Deafness - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References

DEAFNESS A M EDICAL D ICTIONARY , B IBLIOGRAPHY , AND A NNOTATED R ESEARCH G UIDE TO I NTERNET R E FERENCES J AMES N. P...

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DEAFNESS A M EDICAL D ICTIONARY , B IBLIOGRAPHY , AND A NNOTATED R ESEARCH G UIDE TO I NTERNET R E FERENCES

J AMES N. P ARKER , M.D. AND P HILIP M. P ARKER , P H .D., E DITORS

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ICON Health Publications ICON Group International, Inc. 4370 La Jolla Village Drive, 4th Floor San Diego, CA 92122 USA Copyright 2003 by ICON Group International, Inc. Copyright 2003 by ICON Group International, Inc. All rights reserved. This book is protected by copyright. No part of it may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without written permission from the publisher. Printed in the United States of America. Last digit indicates print number: 10 9 8 7 6 4 5 3 2 1

Publisher, Health Care: Philip Parker, Ph.D. Editor(s): James Parker, M.D., Philip Parker, Ph.D. Publisher's note: The ideas, procedures, and suggestions contained in this book are not intended for the diagnosis or treatment of a health problem. As new medical or scientific information becomes available from academic and clinical research, recommended treatments and drug therapies may undergo changes. The authors, editors, and publisher have attempted to make the information in this book up to date and accurate in accord with accepted standards at the time of publication. The authors, editors, and publisher are not responsible for errors or omissions or for consequences from application of the book, and make no warranty, expressed or implied, in regard to the contents of this book. Any practice described in this book should be applied by the reader in accordance with professional standards of care used in regard to the unique circumstances that may apply in each situation. The reader is advised to always check product information (package inserts) for changes and new information regarding dosage and contraindications before prescribing any drug or pharmacological product. Caution is especially urged when using new or infrequently ordered drugs, herbal remedies, vitamins and supplements, alternative therapies, complementary therapies and medicines, and integrative medical treatments. Cataloging-in-Publication Data Parker, James N., 1961Parker, Philip M., 1960Deafness: A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References / James N. Parker and Philip M. Parker, editors p. cm. Includes bibliographical references, glossary, and index. ISBN: 0-597-83875-5 1. Deafness-Popular works. I. Title.

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Disclaimer This publication is not intended to be used for the diagnosis or treatment of a health problem. It is sold with the understanding that the publisher, editors, and authors are not engaging in the rendering of medical, psychological, financial, legal, or other professional services. References to any entity, product, service, or source of information that may be contained in this publication should not be considered an endorsement, either direct or implied, by the publisher, editors, or authors. ICON Group International, Inc., the editors, and the authors are not responsible for the content of any Web pages or publications referenced in this publication.

Copyright Notice If a physician wishes to copy limited passages from this book for patient use, this right is automatically granted without written permission from ICON Group International, Inc. (ICON Group). However, all of ICON Group publications have copyrights. With exception to the above, copying our publications in whole or in part, for whatever reason, is a violation of copyright laws and can lead to penalties and fines. Should you want to copy tables, graphs, or other materials, please contact us to request permission (E-mail: [email protected]). ICON Group often grants permission for very limited reproduction of our publications for internal use, press releases, and academic research. Such reproduction requires confirmed permission from ICON Group International Inc. The disclaimer above must accompany all reproductions, in whole or in part, of this book.

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Acknowledgements The collective knowledge generated from academic and applied research summarized in various references has been critical in the creation of this book which is best viewed as a comprehensive compilation and collection of information prepared by various official agencies which produce publications on deafness. Books in this series draw from various agencies and institutions associated with the United States Department of Health and Human Services, and in particular, the Office of the Secretary of Health and Human Services (OS), the Administration for Children and Families (ACF), the Administration on Aging (AOA), the Agency for Healthcare Research and Quality (AHRQ), the Agency for Toxic Substances and Disease Registry (ATSDR), the Centers for Disease Control and Prevention (CDC), the Food and Drug Administration (FDA), the Healthcare Financing Administration (HCFA), the Health Resources and Services Administration (HRSA), the Indian Health Service (IHS), the institutions of the National Institutes of Health (NIH), the Program Support Center (PSC), and the Substance Abuse and Mental Health Services Administration (SAMHSA). In addition to these sources, information gathered from the National Library of Medicine, the United States Patent Office, the European Union, and their related organizations has been invaluable in the creation of this book. Some of the work represented was financially supported by the Research and Development Committee at INSEAD. This support is gratefully acknowledged. Finally, special thanks are owed to Tiffany Freeman for her excellent editorial support.

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About the Editors James N. Parker, M.D. Dr. James N. Parker received his Bachelor of Science degree in Psychobiology from the University of California, Riverside and his M.D. from the University of California, San Diego. In addition to authoring numerous research publications, he has lectured at various academic institutions. Dr. Parker is the medical editor for health books by ICON Health Publications. Philip M. Parker, Ph.D. Philip M. Parker is the Eli Lilly Chair Professor of Innovation, Business and Society at INSEAD (Fontainebleau, France and Singapore). Dr. Parker has also been Professor at the University of California, San Diego and has taught courses at Harvard University, the Hong Kong University of Science and Technology, the Massachusetts Institute of Technology, Stanford University, and UCLA. Dr. Parker is the associate editor for ICON Health Publications.

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About ICON Health Publications To discover more about ICON Health Publications, simply check with your preferred online booksellers, including Barnes & Noble.com and Amazon.com which currently carry all of our titles. Or, feel free to contact us directly for bulk purchases or institutional discounts: ICON Group International, Inc. 4370 La Jolla Village Drive, Fourth Floor San Diego, CA 92122 USA Fax: 858-546-4341 Web site: www.icongrouponline.com/health

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Table of Contents FORWARD .......................................................................................................................................... 1 CHAPTER 1. STUDIES ON DEAFNESS ................................................................................................. 3 Overview........................................................................................................................................ 3 The Combined Health Information Database................................................................................. 3 Federally Funded Research on Deafness ...................................................................................... 28 E-Journals: PubMed Central ....................................................................................................... 84 The National Library of Medicine: PubMed ................................................................................ 86 CHAPTER 2. NUTRITION AND DEAFNESS ..................................................................................... 111 Overview.................................................................................................................................... 111 Finding Nutrition Studies on Deafness..................................................................................... 111 Federal Resources on Nutrition ................................................................................................. 116 Additional Web Resources ......................................................................................................... 116 CHAPTER 3. ALTERNATIVE MEDICINE AND DEAFNESS ............................................................... 119 Overview.................................................................................................................................... 119 National Center for Complementary and Alternative Medicine................................................ 119 Additional Web Resources ......................................................................................................... 126 General References ..................................................................................................................... 127 CHAPTER 4. DISSERTATIONS ON DEAFNESS ................................................................................. 129 Overview.................................................................................................................................... 129 Dissertations on Deafness.......................................................................................................... 129 Keeping Current ........................................................................................................................ 142 CHAPTER 5. CLINICAL TRIALS AND DEAFNESS ............................................................................ 143 Overview.................................................................................................................................... 143 Recent Trials on Deafness.......................................................................................................... 143 Keeping Current on Clinical Trials ........................................................................................... 144 CHAPTER 6. PATENTS ON DEAFNESS ............................................................................................ 147 Overview.................................................................................................................................... 147 Patents on Deafness ................................................................................................................... 147 Patent Applications on Deafness ............................................................................................... 171 Keeping Current ........................................................................................................................ 179 CHAPTER 7. BOOKS ON DEAFNESS................................................................................................ 181 Overview.................................................................................................................................... 181 Book Summaries: Federal Agencies............................................................................................ 181 Book Summaries: Online Booksellers......................................................................................... 197 The National Library of Medicine Book Index ........................................................................... 203 Chapters on Deafness................................................................................................................. 205 Directories.................................................................................................................................. 219 CHAPTER 8. MULTIMEDIA ON DEAFNESS ..................................................................................... 221 Overview.................................................................................................................................... 221 Video Recordings ....................................................................................................................... 221 Bibliography: Multimedia on Deafness...................................................................................... 224 CHAPTER 9. PERIODICALS AND NEWS ON DEAFNESS .................................................................. 227 Overview.................................................................................................................................... 227 News Services and Press Releases.............................................................................................. 227 Newsletters on Deafness ............................................................................................................ 231 Newsletter Articles .................................................................................................................... 232 Academic Periodicals covering Deafness ................................................................................... 234 CHAPTER 10. RESEARCHING MEDICATIONS................................................................................. 235 Overview.................................................................................................................................... 235 U.S. Pharmacopeia..................................................................................................................... 235 Commercial Databases ............................................................................................................... 236

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APPENDIX A. PHYSICIAN RESOURCES .......................................................................................... 239 Overview.................................................................................................................................... 239 NIH Guidelines.......................................................................................................................... 239 NIH Databases........................................................................................................................... 241 Other Commercial Databases..................................................................................................... 246 The Genome Project and Deafness ............................................................................................. 246 APPENDIX B. PATIENT RESOURCES ............................................................................................... 259 Overview.................................................................................................................................... 259 Patient Guideline Sources.......................................................................................................... 259 Associations and Deafness ......................................................................................................... 282 Finding Associations.................................................................................................................. 286 APPENDIX C. FINDING MEDICAL LIBRARIES ................................................................................ 289 Overview.................................................................................................................................... 289 Preparation................................................................................................................................. 289 Finding a Local Medical Library................................................................................................ 289 Medical Libraries in the U.S. and Canada ................................................................................. 289 ONLINE GLOSSARIES................................................................................................................ 295 Online Dictionary Directories ................................................................................................... 295 DEAFNESS DICTIONARY.......................................................................................................... 297 INDEX .............................................................................................................................................. 369

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FORWARD In March 2001, the National Institutes of Health issued the following warning: "The number of Web sites offering health-related resources grows every day. Many sites provide valuable information, while others may have information that is unreliable or misleading."1 Furthermore, because of the rapid increase in Internet-based information, many hours can be wasted searching, selecting, and printing. Since only the smallest fraction of information dealing with deafness is indexed in search engines, such as www.google.com or others, a non-systematic approach to Internet research can be not only time consuming, but also incomplete. This book was created for medical professionals, students, and members of the general public who want to know as much as possible about deafness, using the most advanced research tools available and spending the least amount of time doing so. In addition to offering a structured and comprehensive bibliography, the pages that follow will tell you where and how to find reliable information covering virtually all topics related to deafness, from the essentials to the most advanced areas of research. Public, academic, government, and peer-reviewed research studies are emphasized. Various abstracts are reproduced to give you some of the latest official information available to date on deafness. Abundant guidance is given on how to obtain free-of-charge primary research results via the Internet. While this book focuses on the field of medicine, when some sources provide access to non-medical information relating to deafness, these are noted in the text. E-book and electronic versions of this book are fully interactive with each of the Internet sites mentioned (clicking on a hyperlink automatically opens your browser to the site indicated). If you are using the hard copy version of this book, you can access a cited Web site by typing the provided Web address directly into your Internet browser. You may find it useful to refer to synonyms or related terms when accessing these Internet databases. NOTE: At the time of publication, the Web addresses were functional. However, some links may fail due to URL address changes, which is a common occurrence on the Internet. For readers unfamiliar with the Internet, detailed instructions are offered on how to access electronic resources. For readers unfamiliar with medical terminology, a comprehensive glossary is provided. For readers without access to Internet resources, a directory of medical libraries, that have or can locate references cited here, is given. We hope these resources will prove useful to the widest possible audience seeking information on deafness. The Editors

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From the NIH, National Cancer Institute (NCI): http://www.cancer.gov/cancerinfo/ten-things-to-know.

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CHAPTER 1. STUDIES ON DEAFNESS Overview In this chapter, we will show you how to locate peer-reviewed references and studies on deafness.

The Combined Health Information Database The Combined Health Information Database summarizes studies across numerous federal agencies. To limit your investigation to research studies and deafness, you will need to use the advanced search options. First, go to http://chid.nih.gov/index.html. From there, select the “Detailed Search” option (or go directly to that page with the following hyperlink: http://chid.nih.gov/detail/detail.html). The trick in extracting studies is found in the drop boxes at the bottom of the search page where “You may refine your search by.” Select the dates and language you prefer, and the format option “Journal Article.” At the top of the search form, select the number of records you would like to see (we recommend 100) and check the box to display “whole records.” We recommend that you type “deafness” (or synonyms) into the “For these words:” box. Consider using the option “anywhere in record” to make your search as broad as possible. If you want to limit the search to only a particular field, such as the title of the journal, then select this option in the “Search in these fields” drop box. The following is what you can expect from this type of search: •

Death of Deafness? The Choice to Hear: Setting the Baseline Source: Hearing Health. 16(3): 54-57. May-June 2000. Contact: Available from Voice International Publications, Inc. P.O. Drawer V, Ingleside, TX 78362-0500. Voice/TTY (361) 776-7240. Fax (361) 776-3278. Website: www.hearinghealthmag.com. Summary: Anecdotal evidence from the hearing health care field indicates that the vast majority of new parents with deaf babies favor technological intervention which enables their babies to hear. This article, the first in a series on the death of deafness, reviews the statistics that support this apparent trend. The author outlines the problem of defining and quantifying the number of Americans who are deaf, then discusses the sources of his data, the use of probability analysis, the impact of new technology on the Deaf

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culture, input from other sources, the demographics of deafness, and public policy issues. The author concludes by describing recent programs designed to maximize early identification of hearing loss. The author stresses that the most urgent task is to find all deaf babies at birth and provide them with the hearing aids, cochlear implants, or schooling in sign language they need; identifying infants and children who lose their hearing after birth is also crucial. Further research on the economic effects of deafness is called for. The contact information for two resource organizations (The National Campaign for Hearing Health and Project Hope Center for Health Affairs) is provided. 3 figures. •

Mediated Communication in the Postsecondary Education of Deaf Students Source: JADARA. Journal of the American Deafness and Rehabilitation Association. 30(1): 1-8. 1996. Summary: As increasing numbers of students with impaired hearing seek postsecondary education, the naivete of many postsecondary institutions about these students' needs assumes greater importance. Students report and staff confirm that representative facilities often fail to distinguish between students who are deaf and students who hard of hearing. Such confusion leads to inappropriate accommodations, when any are made. This article considers the use of mediated communication in the postsecondary education of students who are deaf. The authors suggest antidotes for the neglect and confusion, and offer ideas for alterations in educational administration, for preparing and deploying interpreters, and for research. 1 table. 36 references. (AA-M).

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Renal Failure and Deafness: Branchio-Oto-Renal Syndrome Source: American Journal of Kidney Diseases. 32(2): 334-337. August 1998. Contact: Available from W.B. Saunders Company. P.O. Box 628239, Orlando, FL 328628239. (800) 654-2452. Fax (800) 225-6030. E-mail: [email protected]. Website: www.ajkd.org. Summary: Branchio oto renal (BOR) syndrome is a rare autosomal dominant condition that may present with hearing loss, branchial cysts, and renal failure. The characteristic phenotypic expression of the full syndrome may be partial or complete, and a whole range of renal (kidney) abnormalities may be present. The similarity of BOR to Alport's syndrome may lead to misdiagnosis. This article presents a case report of adult onset renal failure in a 44 year old white man with deafness, previously believed to have Alport's syndrome. The patient's hearing loss was believed to be congenital, and he was prescribed hearing aids. By the age of 19 years, deafness had worsened significantly, and a diagnosis of bilateral moderately severe mixed hearing loss was made. The authors review the relevant literature. The authors note that it is not clear why there is an association between renal malformation and abnormalities of the ear. 2 figures. 18 references.

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Nature of Deaf Culture: Implications for Speech and Hearing Professionals Source: Journal of the Academy of Rehabilitative Audiology (J.A.R.A.). Volume 29: 7184. 1996. Contact: Available from Academy of Rehabilitative Audiology. Circulation Manager, P.O. Box 26532, Minneapolis, MN 55426. (612) 885-0095. Summary: Deaf culture is a lifestyle for many individuals who are born deaf or with hearing impairments and for other people with acquired hearing loss who have been

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enculturated into the Deaf world. Deaf culture has its own history, values, traditions, folklore, and communication behaviors. Its language is American Sign Language (ASL), which gives the culturally Deaf group its identity. This article provides an overview of Deaf culture; its unique behavioral characteristics, values, and traditions; and suggestions for ways to improve interaction between Deaf people and communication professionals. The author stresses that people who are culturally Deaf do not consider deafness a pathology or abnormality. They view themselves as a cultural minority. Even though speech is not compatible with ASL, culturally Deaf people may be interested in spoken English because they realize they must interact with hearing people. The author emphasizes that audiology and speech-language pathology professionals should acknowledge and respect Deaf culture in order for training in English communication to be successful. Deaf people are responsive to professionals who are interested in helping them gain access to the same rights and privileges that hearing people enjoy. 40 references. (AA-M). •

Empowering the Deaf. Let the Deaf Be Deaf Source: Journal of Epidemiology and Community Health. 54(1): 40-44. January 2000. Contact: Available from BMJ Publishing Group. P.O. Box 590A, Kennebunkport, ME 04046. (800) 236-6265. Summary: Deafness is often regarded as a singular phenomenon of homogeneous quality. The medical viewpoint usually classifies deafness by pathology attributable to an inner ear disorder are segregated from pathologies attributable to an outer or middle ear disorder. In this article, the authors argue that hearing loss is a very complex phenomenon which has many and serious consequences for the person with hearing loss. Medical intervention has tended to be concerned more with the origin, degree, type of loss, onset, and structural pathology of deafness than with communicative disability and the implications there may be for the patient. These implications include dependency, denial of abnormal hearing behavior, low self esteem, rejection of the prosthetic help, and the breakdown of social relationships. The authors stress that empowering strategies, aimed at promoting not only a more traditional psychological empowerment but also a community one, should primarily focus on the removal of communication barriers. The authors offer specific strategies on enhancing legislation regarding communication barriers, providing the necessary information, improving the health care setting, and improving patient-physician communication. 42 references.

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Special Literacy Issue: A Reading and Writing Program for Deaf and Hard of Hearing Students Source: Perspectives in Education and Deafness. 17(5): 1-56. May-June 1999. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. Summary: In recognition of the challenge and importance of developing literacy skills in students who are deaf or hard of hearing, this special journal issue (a combination of two publications on education and deafness) offers a detailed description of the comprehensive literacy program offered at the Kendall Demonstration Elementary School (KDES) and the Model Secondary School for the Deaf (MSSD), on the campus of Gallaudet University. The publication includes a separate article on each of the nine components of this literacy program: Reading to Children; Dialogue Journals;

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Independent Reading; Guided Reading and Writing; Shared Reading and Writing; Journals and Logs; Research Reading and Writing; Language Experience; and Writer's Workshops. The publication also includes articles on Gallaudet's pre-college literacy workshops; on the interplay of literacy, auditory training, and speech in a bilingual program; and on storytelling. The publication includes a list of related references and websites, a calendar of events, and a section of practical suggestions for classroom and library activities. The publication is illustrated with black and white photographs of children reading and experiencing other activities related to literacy. •

Sexuality and Deafness. A Call for Education Source: Hearing Health. 14(4): 30-32. July-August 1998. Contact: Available from Voice International Publications, Inc. P.O. Drawer V, Ingleside, TX 78362-0500. Voice/TTY (361) 776-7240. Fax (361) 776-3278. Website: www.hearinghealthmag.com. Summary: Limited access to sexuality information puts children who are deaf or hard of hearing at risk for sexual abuse. This article discusses this issue and proposes a greater emphasis on sex education for these children. The author notes that, because a majority of children who are deaf are born to hearing parents who do not develop effective communication tools, they do not receive sexual education within the home. Barriers to communication in the outside world and poor reading levels further complicate the situation. Unlike their hearing peers, these children often miss information that is obtained vicariously. They do not have the same access to conversations, radio, television, or written sources. The author contends that schools can help bridge the gap by providing accessible information. Sexuality education can potentially reduce the risk of sexual abuse by providing information about behaviors that are appropriate or inappropriate; assistance in establishing boundaries regarding the body; vocabulary enabling children to recount their experiences; and skills for developing positive, appropriate relationships. Once children understand these points, they may be less likely to experience sexual abuse. (AA-M).

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Maternally Inherited Diabetes and Deafness: A Multicenter Study Source: Annals of Internal Medicine. 134(9 Part 1): 721-728. May 1, 2001. Contact: Available from American College of Physicians-American Society of Internal Medicine. 190 North Independence Mall West, Philadelphia, PA 19106-1572. Summary: Maternally inherited diabetes and deafness (MIDD), which is seen in 0.5 percent to 2.8 percent of patients with type 2 diabetes mellitus, is related to a point mutation at position 3243 of mitochondrial (mt) DNA. The clinical description of MIDD is incomplete. This article reports on a study of the clinical presentation and complications of diabetes in patients with MIDD, undertaken to identify clinical characteristics that may help select patients with diabetes for mtDNA mutation screening. The study included 54 patients with type 2 diabetes mellitus and the mtDNA 3243 mutation, from 16 French departments of internal medicine, diabetes, and metabolic diseases. On average, patients with MIDD were young at diabetes onset and presented with a normal or low body mass index. None were obese; 73 percent of subjects had a maternal family history of diabetes. Diabetes was non insulin dependent at onset in 87 percent of patients; however, 46 percent of patients had non insulin dependent disease at onset but progressed to insulin therapy after a mean duration of approximately 10 years. Neurosensory hearing loss was present in almost all patients. Of the patients who received an ophthalmologic examination, 86 percent had macular

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pattern dystrophy (a specific retinal lesion). Myopathy (muscle weakness and wasting) was found in 43 percent of patients, 15 percent had cardiomyopathy (wasting and weakness of the heart muscles), and 18 percent (9 of 51 patients) had neuropsychiatric symptoms. The prevalence of diabetic retinopathy (eye disease) was 8 percent among patients who received an ophthalmologic examination, lower than expected after a mean 12 year duration of diabetes; prevalence of kidney disease was 28 percent. This suggests that a specific renal involvement was the result of mitochondrial disease. The authors conclude that MIDD has a specific clinical profile that may help identify patients with diabetes who should undergo mtDNA testing. 2 figures. 1 table. 46 references. •

Elderly Deaf Patients' Health Care Experiences Source: Journal of the American Board of Family Practice. 13(1): 17-22. January-February 2000. Contact: Available from Drohan Management Group. 11250 Roger Bacon Drive, Suite 8, Reston, VA 20190-5202. (703) 437-4377. Fax (703) 435-4390. E-mail: [email protected]. Summary: Most medical school curricula and major textbooks characterize deafness as a pathologic condition only, which is at odds with the movement to understand the Deaf population as a minority group with a unique language and cultural tradition. Physicians might therefore be unprepared to meet the needs of deaf patients effectively and sensitively. This article reports on a study that investigated the health care experiences of elderly Deaf adults in Richmond, Virginia. The authors conducted focus groups of elderly Deaf persons. Real time voice interpretation of the sign language communication allowed for tape recording and full transcription. Results showed that participants experienced many practical barriers to effective health care, including problems with scheduling appointments and communicating with providers. They believed that providers are ill prepared to care for them and worried that prejudice might be a more subtle obstacle. Participants seemed resigned to these circumstances. The authors suggest a possible explanation for this perspective, and make specific recommendations for three levels of competency in caring for deaf patients. The authors conclude that when the provider and the office staff provide methods to communicate with deaf patients using telephone assisted communication, qualified interpreters, and some basic knowledge of lipreading or sign language, the care of deaf patients is greatly enhanced by the physician patient relationship. 29 references.

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Sudden Total Bilateral Deafness Due to Asymptomatic Mumps Infection Source: International Journal of Pediatric Otorhinolaryngology. 45(2): 167-169. October 1998. Contact: Available from Elsevier Science. P.O. Box 945, New York, NY 10159-0945. (888) 437-4636. Fax (212) 633-3680. E-mail: [email protected]. Summary: Mumps is the most common cause of unilateral (one side) acquired sensorineural hearing loss in children. Although it usually affects the salivary glands, the inner ear may be involved. Deafness is usually unilateral, sudden in onset, profound, and permanent. Bilateral total sensorineural hearing loss has rarely been reported in the English literature. This article presents a case of total deafness due to asymptomatic mumps infection. The 4 year old girl presented with a 10 day history of not answering to the parents sound stimuli. The parents had not noticed parotid swelling or tenderness, vertigo, nausea or meningeal signs, but marked a mild fever and fatigue before the event. The authors report the diagnostic tests used with the child, and

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notes that she was given the standard sudden sensorineural hearing loss regimen of immunosuppressive agents. Hearing loss did not resolve on this regimen after a week, and the drugs were discontinued. The authors conclude by hypothesizing that sudden hearing loss develops with an immune mediated reaction after a viral induction (except some clear traumatic etiologies, for example). The hearing aid was ineffective in this patient, and a rehabilitation therapy was introduced. There was no improvement 3 months later and she was accepted as a candidate for cochlear implantation. 2 tables. 12 references. •

Carrier Rates in the Midwestern United States for the GJB2 Mutations Causing Inherited Deafness Source: JAMA. Journal of the American Medical Association. 281(23): 2211-2216. June 16, 1999. Summary: Mutations in the GJB2 gene are the most common known cause of inherited congenital severe-to-profound deafness. The carrier frequency of these mutations is not known. This article reports on a study undertaken to determine the carrier rate of deafness causing mutations in GJB2 in the midwestern U.S. and the prevalence of these mutations in persons with congenital sensorineural hearing loss ranging in severity from moderate to profound, and to derive revised data for counseling purposes. The study included 52 subjects younger than 19 years, sequentially referred to a midwestern tertiary referral center for hearing loss or cochlear implantation, with moderate to profound congenital hearing loss of unknown cause, parent nonconsanguinity, and nonsyndromic deafness with hearing loss limited to a single generation; 560 control neonates were screened for the 35delG mutation. Of 52 sequential probands referred for congenital sensorineural hearing loss, 22 (42 percent) were found to have GJB2 mutations. The 35delG mutation was identified in 29 of the 41 mutant alleles. Of probands sib, all homozygotes and compound heterozygotes had deafness. Fourteen of 560 controls were 35delG heterozygotes, for a carrier rate expressed as a mean of 2.5 percent. The carrier rate for all recessive deafness causing GJB2 mutations was determined to be 3.01 percent. The authors conclude that this data suggests that mutations in GJB2 are the leading cause of moderate to profound congenital inherited deafness in the midwestern U.S. Screening of the GJB2 mutation can be offered to individuals with congenital deafness with high sensitivity and specificity by screening only for the 35delG mutation. A positive finding should establish an etiologic diagnosis and affect genetic counseling.

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Organization Resources for Families of Children with Deafness or Hearing Loss Source: Pediatric Clinics of North America. 46(1): 153-162. February 1999. Contact: Available from W.B. Saunders Company. 6277 Sea Harbor Drive, Orlando, FL 32887-4800. (800) 654-2452. Summary: Pediatricians, as part of an interdisciplinary team, can provide families of children with hearing loss or deafness with information about important resources to expedite their child's habilitative process. This article, from a monograph on hearing loss in children, describes the emotional stages that parents and caregivers may experience upon initial diagnosis of hearing loss and the support that pediatricians can offer during this critical time of discovery when families seek guidance. The authors discuss the variety of communication strategies and options available to children and families, based on the child's specific needs, diagnostic findings, educational opportunities, family culture, and preferences. A listing of programs, agencies, and organizations

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pertinent to persons with deafness or hearing loss is provided to facilitate linkages from diagnosis to intervention. 1 appendix. 15 references. •

Self-Report of Cochlear Implant Use and Satisfaction by Prelingually Deafened Adults Source: Ear and Hearing. 17(3): 198-210. June 1996. Summary: Prelingually deafened adults tend to demonstrate smaller improvements in speech recognition after cochlear implantation than do post-lingually deafened adults. This article reports on a study undertaken to evaluate cochlear implant use and satisfaction by prelingually deafened adults. A questionnaire was administered to 12 prelingually deafened adult cochlear implant patients to evaluate cochlear implant efficacy and satisfaction Questionnaire results were contrasted with performance on speech recognition tasks. Results showed that although these patients demonstrated little or no improvements in speech recognition 12 months postoperatively, most patients reported that they used their device regularly, that they were satisfied with their device, and that using the cochlear implant improved both their expressive and receptive communication skills. The authors conclude that procedures other than traditional speech recognition measures should be used to evaluate cochlear implant benefit, particularly with prelingually deafened adults. An appendix reprints the questionnaire used in this study. 1 figures. 10 tables. 8 references. (AA-M).

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Role of Educators of the Deaf in the Early Identification of Hearing Loss Source: American Annals of the Deaf. 144(1): 19-23. March 1999. Contact: Available from American Annals of the Deaf. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Summary: Professionals in deaf education, as well as deaf and hard of hearing individuals, play an important role in the development of comprehensive systems of care for deaf and hard of hearing infants and young children. Universal newborn hearing screening programs typically are linked to health systems rather than educational systems. This article explores the need for new links between health and education services in order for educators of deaf and hard of hearing children to be involved in early identification and intervention programs. Identification of hearing loss in infancy, followed by appropriate intervention by age 6 months, can result in normal language development, regardless of degree of hearing loss. As the average age of identification of hearing loss moves downward toward 2 months, children with hearing loss will enter the educational system earlier and with language skills commensurate with those of their hearing peers. Early interventionists will need to have specialized training in deafness and hearing loss, and to have expertise in providing services to very young children and to children with hearing loss in the broad range from mild to profound. One section of the article discusses the early identification and intervention strategies in place at the Marion Downs National Center for Infant Hearing, a center for the promotion of programs for screening, diagnosis, and intervention for newborns and infants who are deaf or hard of hearing. 1 table. 20 references.

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Reading Optimally Builds on Spoken Language: Implications for Deaf Readers Source: Journal of Deaf Studies and Deaf Education. 5(1): 32-50. Winter 2000.

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Contact: Available from Oxford University Press, Journals Customer Service, 2001 Evans Road, Cary, NC 27513. (800) 852-7323 or (919) 677-0977. Fax (919) 677-1714. E-mail: [email protected]. Summary: Reading builds fundamentally on primary language processes. For hearing readers, this means that spoken language processes, including phonological processes, are critical to high achievement in reading. In this article, the authors examine the implications of this fact for deaf readers by considering the relationship between language and reading and by reviewing the research on the use of phonology by deaf readers. The research, although mixed in its results, suggests that the use of phonology is associated with higher levels of reading skills among deaf readers. The authors examine related questions, including the additional semantic and visual strategies available to deaf readers, how some deaf readers gain access to the spoken structure of language, and implications for how to improve reading achievement. 65 references. •

Attitudes of Deaf Adults Toward Genetic Testing for Hereditary Deafness Source: American Journal of Human Genetics. 63(4): 1175-1180. October 1998. Contact: Available from University of Chicago Press. Journals Division, 5720 South Woodlawn, Chicago, IL 60637. Fax (773) 753-0811. E-mail: [email protected]. Summary: Recent advances within molecular genetics to identify the genes for deafness mean that it is now possible for genetic counseling services to offer genetic testing for deafness to certain families. This article reports on a study undertaken to document the attitudes of deaf adults toward genetic testing for deafness. A structured, selfcompletion questionnaire was given to delegates at an international conference on the Deaf Nation held at the University of Central Lancashire (United Kingdom), in 1997. Eighty-seven deaf delegates from the UK returned completed questionnaires. The questionnaire was designed to quantitatively assess attitudes toward genetics, interest in prenatal diagnosis (PND) for deafness, and preference for having deaf or hearing children. The results from this study demonstrate evidence of a predominantly negative attitude toward genetics and its impact on people who are deaf, in a population for whom genetic counseling services are relevant. Fifty-five percent of the sample thought that genetic testing would do more harm than good, 46 percent thought that its potential use devalued deaf people, and 49 percent were concerned about new discoveries in genetics. When asked about testing in pregnancy, 16 percent of participants said that they would consider having PND, and, of these, 29 percent said that they would prefer to have deaf children. The authors note that geneticists need to appreciate that some deaf persons may prefer to have deaf children and may consider the use of genetic technology to achieve this. Any genetic counseling service set up for families with deafness can only be effective and appropriate if clinicians and counselors take into consideration the beliefs and values of the deaf community at large. 5 figures. 18 references. (AA).

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Development of Communication and Language in Deaf and Severely Hard of Hearing Children: Implications for the Future Source: International Journal of Pediatric Otorhinolaryngology. 49(Supplement 1): S39S43. October 1999. Contact: Available from Elsevier Science. P.O. Box 945, New York, NY 10159-0945. (888) 437-4636. Fax (212) 633-3680. E-mail: [email protected].

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Summary: Severe hearing impairment is seldom detected in children before the age of 6 to 12 months, because parent infant interaction at that stage resembles hearing parent child interaction. This is probably due to an innate capacity of infants to take information in one sensory modality and translate it into another, called amodal perception. This article traces the development of communication and language in children who are deaf or severely hard of hearing. The author traces the roots of language to early protoconversations, as well as to early pretend play. Relationships are viewed as the context in which socialization takes place, basic competencies emerge, regulations of emotions develop, and communication skills are acquired. If habilitation after diagnosis of a severe hearing impairment primarily is focused on an oral aural approach, natural patterns of communication between parent and child will gradually disappear, which will have negative implications on the development of these children. If, instead, they are allowed to develop those means of communication that are easy for them to produce and to perceive, positive consequences have been registered on the development of communication and language, as well as on their socioemotional and cognitive development. When these children have been given opportunities to become bilingual with a signed, written, or spoken language, it has improved their overall quality of life. 17 references. •

Principles and Practices of Literacy Development for Deaf Learners: A Historical Overview Source: Journal of Deaf Studies and Deaf Education. 5(1): 3-8. Winter 2000. Contact: Available from Oxford University Press, Journals Customer Service, 2001 Evans Road, Cary, NC 27513. (800) 852-7323 or (919) 677-0977. Fax (919) 677-1714. E-mail: [email protected]. Summary: Since the very beginning of formal approaches to deaf education, the development of literacy has been a priority issue. The history of educational initiatives in this area is entwined with the history of prevailing attitudes and practices toward the impact of deafness on the development of deaf children more generally. In particular, arguments about whether a visual input (reading) can take the place of a diminished auditory input and whether educators should accommodate or seek to ameliorate the effects of the special linguistic characteristics of deaf learner readers have resulted in a wide variety of practices and perspectives. These varied practices and perspectives continue to have impacts on current educational debate and practice. This article provides a brief historical overview of these educational endeavors and the authors note the enduring questions and issues that remain for the field to address. 32 references.

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Evaluation of Anti-hsp70 Antibody Screening in Sudden Deafness Source: Ear and Hearing. June 2003;24;233-5. Contact: Available from Ear and Hearing. Web site: www.ear-hearing.com. Summary: The authors of this article reports on a study conducted to assess the diagnostic utility of anti-hso70 antibody screening in sudden deafness. In the study sera from 27 patients with deafness and 100 healthy blood donors were analyzed. The authors concluded that the anti-hsp70 WB test lacks clinical utility for diagnostic screening in patients with sudden deafness.

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Individual Differences in Effectiveness of Cochlear Implants in Children Who Are Prelingually Deaf: New Process Measures of Performance Source: Volta Review. 101(3): 111-164. Summer 1999. Contact: Available from Alexander Graham Bell Association for the Deaf and Hard of Hearing. Subscription Department, 3417 Volta Place, NW, Washington, DC 20007-2778. Voice/TTY (202) 337-5220. Website: www.agbell.org. Summary: The efficacy of cochlear implants in children who are deaf has been firmly established in the literature. However, the effectiveness of cochlear implants varies widely and is influenced by demographic and experiential factors. This article reports on new process measures of performance that are being used to measure these individual differences in effectiveness of cochlear implants in children. Several key findings suggest new directions for research on central auditory factors that underlie the effectiveness of cochlear implants. First, individual differences have been observed in both adults and children on a wide range of audiological outcome measures. Some patients show large increases in speech perception scores after implantation, whereas others display only modest gains on standardized tests. Second, age of implantation and length of deafness affect all outcome measures. Children implanted at younger ages do better than children implanted at older ages, and children who have been deaf for shorter periods do better than children who have been deaf for longer periods. Third, communication mode affects outcome measures (i.e., children from oral only environments do better than children who use Total Communication). Fourth, there are no preimplant predictors of outcome performance in young children. Finally, there are no significant differences in audiological outcome measures among current implant devices or processing strategies. Taken together, this overall pattern of results suggests that higher level central processes such as perception, attention, learning, and memory may play important roles in explaining the large individual differences observed among users of cochlear implants. 10 figures. 6 tables. 69 references.

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Subclinical Audiological Findings in Carriers of Recessive Genes for Deafness Source: Journal of Basic and Clinical Physiology and Pharmacology. 10(3): 201-208. 1999. Contact: Available from Freund Publishing House, LTD, Scientific Publications Division. P.O.B. 35010, Tel Aviv, Israel 61350. E-mail: [email protected]. Website: www.angelfire.com/il/freund. Summary: The existence of subclinical signs in the hearing of carriers of recessive mutations for deafness has aroused much controversy in the literature. This article reports on a study that comprised 30 carriers of recessive mutations for deafness, and a control group of 30 healthy volunteers, matched for gender and age. All participants underwent a series of hearing tests, including pure tone audiometry, speech tests, Bekesy audiometry and notch noise tests. The main results were: hearing loss in high frequencies (3000 and 4000 Hz), an elevation of the acoustic reflex threshold, as well as an elevation of the identification of 2000 Hz pure tone in the presence of white noise and notch noise. A notch in the Bekesy audiogram was also identified in several carriers. An interaction was found at 4000 Hz, and in the ipsilateral and contralateral acoustic reflex at 500 Hz and 1000Hz. Gender differences were found in pure tone as well as the acoustic reflex tests. This study suggests that the carrier state of autosomal recessive deafness might be identifiable by simple audiological tests. However, routine clinical use of these methods should be deferred until further studies confirm the results and technical improvements provide better differentiation between groups. The authors

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conclude that these subclinical signs may be complementary to DNA research in the investigation of genetic deafness of unknown origin. 1 table. 16 references. •

Hearing Improvement After Therapy for Hyperlipidemia in Patients with ChronicPhase Sudden Deafness Source: Annals of Otology, Rhinology and Laryngology. 110(2): 105-108. February 2001. Contact: Available from Annals Publishing Company. 4507 Laclede Avenue, St. Louis, MO 63108. Summary: The hearing of patients with chronic phase sudden deafness and associated hyperlipidemia (high levels of fats in the blood) tends to improve with therapy for the hyperlipidemia. This article reports on a study of 12 patients with unilateral sudden deafness and hyperlipidemia in whom more than 1 month had elapsed since the onset of the hearing disturbance. The disturbance was considered to be irreversible without therapy. The 4 men and 8 women ranged in age from 32 to 73 years, with a mean age of 54.3 years. Hyperlipidemia was diagnosed when the total blood cholesterol level was 230 mg per dL or greater. The therapy for hyperlipidemia consisted of diet therapy and the administration of antilipemic drugs. The hearing level was measured both before therapy and when the total blood cholesterol level had decreased to less than 230 mg per dL. After therapy, the mean hearing level had improved significantly at each of 125, 250, 500, and 2,000 Hertz (Hz), but the changes in the level were not significant at 1,000, 4,000, or 8,000 Hz. The authors conclude that with therapy for hyperlipidemia, hearing tends to improve in patients with chronic phase sudden deafness and associated hyperlipidemia, even when more than 1 month has elapsed since the onset of the presumably otherwise irreversible hearing loss. 3 figures. 1 table. 8 references.

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Smoking and Deaf Adults: Associations with Age at Onset of Deafness Source: American Annals of the Deaf. 144(1): 44-50. March 1999. Contact: Available from American Annals of the Deaf. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Summary: The prevalence of smoking in different populations is thought to be influenced by sociocultural and linguistic factors. Although smoking and hearing loss are positively correlated, little is known about the smoking habits of deaf populations. This article reports on a study that used national survey data to determine the smoking prevalence in two socioculturally distinct deaf populations, based on age at onset of deafness. The smoking prevalence in each deaf population was compared to the smoking prevalence in the hearing population in multivariate analyses that adjusted for sociodemographics and health status. The smoking prevalence among postlingually deafened adults was not significantly different from that among hearing adults. Prelingually deafened adults were found to be less likely to smoke than hearing adults, even though they have less education and lower income, factors both associated with higher smoking prevalence in other populations. The authors hypothesize that the lower smoking prevalence among prelingually deafened adults may be due to cultural differences or to limited exposure to English language tobacco advertising. 1 figure. 4 tables. 32 references.

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Cochlear Implantation in Deaf Children and Adolescents: Effects on Family Schooling and Personal Well-Being Source: International Journal of Pediatric Otorhinolaryngology. 49(Supplement 1): S183S187. October 1999. Contact: Available from Elsevier Science. P.O. Box 945, New York, NY 10159-0945. (888) 437-4636. Fax (212) 633-3680. E-mail: [email protected]. Summary: This article attempts to answer the main questions raised by that part of the deaf community which still considers cochlear implants (CI) an attack against the psychophysical integrity of the prelingually deaf. In this study, the psychological well being of six adolescents and six children was assessed pre and post implantation using various tools, i.e., projective tests, assessment scales, and structured interviews with parents and teachers. The analysis of post implant findings shows a reduction of stereotype elements, more dynamic modes of figurative expression, quite good relationships within their own social environment, and gradual, positive integration both at home and at school. The authors conclude that cochlear implantation seems to cause no psychological disruption. The sample group showed an improvement in their modes of expression (more consistent with the mental and effective age) and a greater awareness of personal limits, together with the ability to judge the appropriateness of their own behavior. 3 tables. 10 references.

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Hypokalemic Salt-Losing Tubulopathy with Chronic Renal Failure and Sensorineural Deafness Source: Pediatrics. 108(1): [9 p.]. July 2001. Contact: Available from American Academy of Pediatrics. 141 Northwest Point Boulevard, Elk Grove Village, IL 60007-1098. (888) 227-1773. Fax (847) 434-8000. E-mail: [email protected]. Website: www.pediatrics.org. Full text of this article is available at www.pediatrics.org/cgi/content/full/108/1/e5. Summary: This article describes a rare inherited hypokalemic (low levels of potassium) salt losing tubulopathy (kidney disease) with linkage to chromosome 1p31. The authors conducted a retrospective analysis of the clinical data for 7 patients in whom cosegregation of the disease with chromosome 1p31 had been demonstrated. In addition, in 1 kindred, prenatal diagnosis in the second child was established, allowing a prospective clinical evaluation. Clinical presentation (symptoms) of the patients was similar and included premature birth attributable to polyhydramnios, severe renal (kidney) salt loss, normotensive hyperreninemia, hypokalemic alkalosis, and excessive hyperprostaglandin E urea, which suggested the diagnosis of hyperprostaglandin E syndrome and antenatal Bartter syndrome. However, the response to indomethacin was only poor, accounting for a more severe variant of the disease. The patients invariably developed chronic renal failure. The majority had extreme growth retardation, and motor development was markedly delayed. In addition, all patients turned out to be deaf. The authors conclude that this hypokalemic salt losing tubulopathy with chronic kidney failure and sensorineural deafness represents not only genetically but also clinically a disease entity distinct from hyperprostaglandin E syndrome and antenatal Bartter syndrome. A pleiotropic (causing multiple, seemingly unrelated symptoms) effect of a single gene defect is most likely causative for syndromic hearing loss. 6 figures. 1 table. 41 references.

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Approaches to Studying in Deaf and Hearing Students in Higher Education Source: Journal of Deaf Studies and Deaf Education. 5(2): 156-173. Spring 2000. Contact: Available from Oxford University Press, Journals Customer Service, 2001 Evans Road, Cary, NC 27513. (800) 852-7323 or (919) 677-0977. Fax (919) 677-1714. E-mail: [email protected]. Summary: This article describes a survey undertaken to compare the responses of 149 deaf students and 121 hearing students taking the same courses to a shortened and adapted version of the Approaches to Studying Inventory. In general, the impact of deafness on approaches to studying was relatively slight, and deaf students appeared to be at least as capable as hearing students of engaging with the underlying meaning of the materials to be learned. The authors used factor analysis to identify eight scales. Differences between the two groups were statistically significant on four of the scales. Discriminant analysis indicated that deaf students found it more difficult to relate ideas on different topics, and that this was more marked in those who preferred to communicate using sign. However, deaf students were more likely than hearing students to adopt a critical approach and to analyze the internal structure of the topics studied. 4 tables. 97 references.

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Deaf Mentor Experimental Project for Young Children Who Are Deaf and Their Families Source: American Annals of the Deaf. 143(1): 29-39. March 1998. Summary: This article describes the Deaf Mentor Experimental Project, a project that investigated the efficacy of deaf mentor services to young deaf children and their families. These services focused on deaf adults (mentors) who made regular home visits to the children and their families; shared their language (American Sign Language), culture, and personal knowledge of deafness with the families; and served as role models for the children. The children also received regular home visits from a hearing parent advisor who helped the family promote the child's early listening, English, and literacy skills. The result was a bilingual, bicultural home environment for these children. The children who received deaf mentor services were compared to matched children who did not receive these services but who received parent advisor services. Children receiving the early bilingual, bicultural programming made greater language gains during treatment time, had considerably larger vocabularies, and scored higher on measures of communication, language, and English syntax than the matched children. 1 table. 38 references. (AA).

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Understanding the Needs of Our Deaf Community Source: Psychology & AIDS Exchange; Issue 13. Contact: American Psychological Association, Public Interest Directorate, Office AIDS, 750 1st St NE, Washington, DC, 20002-4242, (202) 336-5500, http://www.apa.org. Summary: This article discusses issues of deafness and hearing loss related to HIV. The issues are examined from the following perspectives: 1) an HIV-positive person who experiences deafness as a symptom of the infection; 2) a culturally deaf person who develops HIV; and 3) a deaf professional who has HIV. Persons becoming deaf as a result of HIV are at risk of increased isolation, hopelessness, and vulnerability. They are forced to learn new methods of communication while under extreme stress, and may fail to gain treatment information. The author emphasizes the need to restore a sense of control and improve the quality of life for those who experience a hearing loss. The

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culturally deaf are separated by language and community culture from communitybased support and services for HIV, and suffer from the deaf community's denial of the risks of HIV. A discussion of the use of interpreters highlights the confidentiality problem affecting the deaf, when they must either rely on a third party for communication or seek assistance from services staffed by deaf or signing professionals probably known to them. The author concludes with the following recommendations: develop programs linguistically and culturally sensitive to the deaf; increase access for the deaf in mainstream HIV/AIDS programs; and train HIV/AIDS counselors and therapists to communicate with deaf persons. •

Beyond Parent Education: The Impact of Extended Family Dynamics in Deaf Education Source: American Annals of the Deaf. 145(4): 359-364. October 2000. Contact: Available from American Annals of the Deaf. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Summary: This article discusses the impact of childhood deafness on family dynamics, with particular attention given to the influence and role of grandparents. The author contends that models for the successful involvement of parents in the education of deaf children are already in place and can be applied to extended family, especially grandparents, with promising results. The author reviews an informal questionnaire study that focused on grandparents' support, involving 10 hearing parents of deaf infants and preschool children. How the child is treated within the extended family, how accommodations are made so that the deaf child becomes fully integrated into the extended family and its activities, and how extended family members relate and react to outsiders (medical or educational professionals) who may be involved in the child's care are important considerations for programs involved with young deaf children. Professionals must be aware of the needs of extended family members. Because they recognize that grandparents are mourning the loss of the idealized grandchild and their own idealized role as grandparents, schools and programs for deaf children and adults can provide them with information, networking opportunities, and support groups. 7 references.

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Exploring Assessment Alternatives for Deaf Students Source: Perspectives. 14(4): 5-7, 24. March-April 1996. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. Summary: This article discusses the use of alternative assessment methods for deaf students. For many students who are deaf, as well as for hearing children who learn English as a Second Language (ESL), traditional, standardized English-based tests often do not adequately assess either strengths or weaknesses. The author describes current efforts in locating or designing realistic assessment strategies and discusses the use of student self-assessments, critical thinking analyses, analyzing communication, and the student-generated portfolio. The author encourages teachers to keep data on the assessment techniques they find useful in their own classrooms. 3 references.

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New Genetics of Deafness Source: Advance for Audiologists. 2(5): 63-64. November-December 2000. Contact: Available from Merion Publications, Inc. 2900 Horizon Drive, P.O. Box 61556, King of Prussia, PA 19406-0956. Summary: This article explores how new discoveries in genetics will potentially impact the field of hearing and hearing disorders. For example, future gene therapies could alter the protein output that causes degenerative processes like hair cell deterioration in such conditions as presbycusis (age related hearing loss). The author notes that research is expected to focus on finding a way to prevent the deterioration of hearing in individuals who have a genetic predisposition to hearing loss. Genetic research can also play a part in determining who will be a good candidate for cochlear implantation. The author describes the etiology of hearing loss, how hearing loss is classified, the use of genetics to determine what is occurring in side the previously inaccessible cochlea, ethical concerns, and societal safeguards (notably privacy concerns). 1 figure.

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Infants with Congenital Deafness: On the Importance of Early Sign Language Acquisition Source: American Annals of the Deaf. 145(1): 6-14. March 2000. Contact: American Annals of the Deaf. Gallaudet University Press, 800 Florida Avenue, NE, Washington, DC 20002. V/TTY: (202) 651-5488. Fax: (202) 651-5489. Articles-only reprints are available from Infotrieve: (800) 422-4633; Fax: 310-208-8971; E-mail: [email protected] or Web site: www.infotrieve.com/. PRICE: $12.00 service fee plus copyright royalty and delivery. Summary: This article provides case studies of two boys with profound bilateral hearing impairment. At the time of the study, both boys attended a specialized sign preschool, where hearing-impaired children are integrated with hearing children. The first boy's hearing impairment was detected in the maternity ward with otoacoustic emissions (OAE), but the second boy's hearing impairment was not detected until the age of two. Through interviews, direct observation, and video observation at home and in school, the author describes the characteristic differences between the boys' social and linguistic development as it relates to the time of detection of the hearing impairment.

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Cognitive Diversity in Deaf People: Implications for Communication and Education Source: Scandinavian Audiology. 27(Supplement 49): 109-115. 1998. Contact: Available from Scandinavian University Press North America. 875 Massachusetts Avenue, Suite 84, Cambridge, MA 02139. (800) 498-2877 or (617) 4976515. Fax (617) 354-6875. E-mail: [email protected]. Website: www.scup.no. Summary: This article reports on a research review that considers research conducted to investigate the visual perceptual, visual attention, and memory skills in deaf people. The author reports on how the visual perceptual skills and cognitive processes of deaf people may be different from those in hearing people. The author suggests that deafness and the use of a sign language may selectively contribute to the development of such differences. However, there appears to be no overall enhancement of vision or visual perceptual skills in deaf people. The author discusses use of a global visual perceptual strategy, strategy differences in copying the Rey Osterrieth Complex Figure, diversity in the control of visual attentional skills, visual attention and the Stroop effect, visual attention and attention deficit disorders (ADD), diversity in processing written English,

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and the use of phonological information in deaf signers. The author also discusses implications of the research and its limitations for enhancing the communication and educational experiences of deaf people. 36 references. •

Performance After Cochlear Implantation: A Comparison of Children Deafened by Meningitis and Congenitally Deaf Children Source: Journal of Laryngology and Otology. 114(1): 33-37. January 2000. Contact: Available from Royal Society of Medicine Press Limited. Publications Subscription Department, P.O. Box 9002, London W1A 0ZA, United Kingdom. E-mail: [email protected]. Summary: This article reports on a study in which the speech perception and speech production performance following cochlear implantation of congenitally deaf children and children deafened by meningitis were analyzed. Three groups consisting of 70 congenitally deaf children, 22 children deafened by meningitis before two years of age, and 14 children deafened by meningitis after two years of age were compared. The group deafened by meningitis after two years of age demonstrated significantly better speech perception than the other two groups. Their speech production appeared better, but did not achieve statistical significance compared with the other two groups. There were no significant differences in speech perception or production between the congenitally deaf group and the group deafened by meningitis before two years of age. Further research is required to determine whether this is a consequence of meningitis, whether they have failed to derive benefit from their previous auditory experience, or whether any benefit they may have derived has been lost during their period of deafness. 2 figures. 9 tables. 15 references.

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Changes in Otoacoustic Emissions in Patients with Idiopathic Sudden Deafness Source: Audiology. 36(3): 121-135. May-June 1997. Summary: This article reports on a study of changes in otoacoustic emissions in patients with idiopathic sudden deafness (ISD). Transiently click-evoked, distortion product, and spontaneous otoacoustic emissions were recorded. Changes in these tests were studied during the recovery process in 15 cases of ISD. In all these cases, the amplitudes of clickevoked and distortion product otoacoustic emissions increased concurrently with the recovery of the hearing threshold. Ears with ISD were not different in their otoacoustic emissions characteristics from ears with other forms of sensorineural hearing loss (SNHL). In 4 of the 15 cases, spontaneous otoacoustic emissions could be detected when hearing had recovered. These results suggest that the function of outer hair cells (OHCs) had deteriorated when the hearing threshold was elevated and that OHC activity recovered as hearing improved to nearly normal levels in ISD cases with good outcome. 7 figures. 1 table. 23 references. (AA).

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Conversations Between Deaf Children and Their Hearing Mothers: Pragmatic and Dialogic Characteristics Source: Journal of Deaf Studies and Deaf Education. 5(4): 304-322. Fall 2000. Contact: Available from Oxford University Press, Journals Customer Service, 2001 Evans Road, Cary, NC 27513. (800) 852-7323 or (919) 677-0977. Fax (919) 677-1714. E-mail: [email protected]. Summary: This article reports on a study that examined communication between hearing mothers and their deaf or hearing children longitudinally at child ages 22

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months and at 3 years. The authors analyzed both the effects of child deafness and developmental change on pragmatic and dialogic characteristics of communication. From 22 months to 3 years, deaf and hearing children's communicative skills improved similarly along some dimensions. For example, as they grew older, both deaf and hearing children increased the amount they communicated, became increasingly responsive to their mothers' attentional focus, and were responsible for initiating a higher proportion of the dyads' conversations. On the other hand, deaf children were less skilled at maintaining topics, and the pragmatic function of their communication was more likely to be unclear compared to hearing children. Deaf children were also more likely to direct their mothers and less likely to ask questions than hearing children. Communication by hearing mothers was primarily examined to determine the degree to which they controlled the interactions. Overall, mothers of deaf children were only more controlling along one dimension. Mothers of deaf children used more response controls than mothers of hearing children. However, the majority of measures suggested that they did not exert more topic or turn taking controls than did mothers of hearing children. In addition, mothers of deaf and hearing children seemed equally sensitive to their children's communication abilities. Communication by mothers of both deaf and hearing children changed in similar ways as their children developed. Most of the differences in communication by mothers of deaf and hearing children seemed attributable to the deaf children's linguistic delays. The results suggest that intervention efforts should be focused on fostering linguistic development and not general communication skills or changing maternal conversational control. 8 tables. 42 references. •

Deafness and Mortality: Analyses of Linked Data from the National Health Interview Survey and National Death Index Source: Public Health Reports. 114(4): 330-336. July-August 1999. Contact: Available from Oxford University Press. Journals Costumer Service, 2001 Evans Road, Cary, NC 27513-2009. (800) 852-7323 or (919) 677-0977. Fax (919) 677-1714. E-mail: [email protected]. Summary: This article reports on a study undertaken to examine the association between age at onset of deafness and mortality. The authors analyzed National Health Interview Survey data from 1990 and 1991 (the years the Hearing Supplement was administered) linked with National Death Index data for 1990 to 1995. Adjusting for sociodemographic variables and health status, the authors compared the mortality of three groups of adults older than 19 years: those with prelingual onset of deafness (onset at less than 3 years of age), those with postlingual onset of deafness, and a representative sample of the general population. Multivariate analyses adjusted for sociodemographics and strategies by age found that adults with postlingual onset of deafness were more likely to die in the given time frames than non deaf adults. However, when analyses were also adjusted for health status, there was no difference between adults with postlingual onset of deafness and a control group of non deaf adults. No differences in mortality were found between adults with prelingual onset of deafness and non deaf adults. The authors conclude that adults with postlingual onset of deafness appear to have higher mortality than non deaf adults, which may be attributable to their lower self reported health status. The authors note that their findings also raise questions that suggest more direct inquiry should be made about the modes of communication used by deaf people, in any future studies of this type. 3 tables. 25 references.

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Support Services for Parents and Their Children Who Are Deaf or Hard of Hearing: A National Survey Source: American Annals of the Deaf. 142(4): 278-288. October 1997. Contact: Available from American Annals of the Deaf. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Summary: This article reports on a survey completed in the spring and summer of 1996, by 404 parents of children who are deaf or hard of hearing and were born in 1989 or 1990. The questionnaires asked about early services received and the current status of the children's development. The children were enrolled in 137 different programs in 39 states; about one quarter of the programs participating in Gallaudet's Annual Survey of Deaf and Hard of Hearing Children and Youth. Children who are deaf comprised 46 percent of the group for whom responses were provided; those who are hard of hearing comprised 54 percent. Hearing loss was confirmed at the mean age of 14.5 months for the deaf group, and at the mean age of 28.6 months for the hard of hearing group. Additional conditions place 32 percent at risk for educational or developmental difficulties. One or both parents are deaf in 13 percent of responding families. Almost 40 percent of mothers have some training beyond high school; one third of the children came from minority backgrounds. Communication approaches used in children's initial programs included: speech alone (24 percent), sign and speech (66 percent), sign alone (5 percent), cues (3 percent) and sign and cues (3 percent). Parents gave highly favorable evaluations to intervention programs, and placed teachers at the top of a 'sources of help' list. Parents from minority groups and those with no college training reported that their children showed more behavior problems and less language progress, and gave more negative responses to questions regarding the impact of deafness on their families. This suggests that program personnel may need to increase their intervention efforts for these subgroups of special education consumers. 13 tables. 39 references.

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Residential Schools Offer Students Deaf Culture Source: Perspectives in Education and Deafness. 16(2): 4-5, 24. November-December 1997. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. Summary: This article reports on a survey that investigated residential schools and mainstreaming as educational options for children who are deaf or hard of hearing. Results were analyzed from 115 high school students, from 14 to 21 years old; 55 were from seven mainstream programs and 60 were from 4 residential schools. Almost all the mainstream students had hearing parents and a less than profound hearing loss. One fifth of them also had a compounding disability, ranging from learning disabilities to cerebral palsy. In contrast, one third of the residential students had at least one deaf parent and one half had a deaf or hard of hearing relative. The majority had profound hearing loss of 95 decibels or more. The author concludes that residential schools appeared to offer a stronger representation of deaf culture. These schools averaged 43 percent in hiring deaf teachers. American Sign Language is used to teach in these classrooms. A large majority of the students described themselves as 'Deaf' or 'deaf' and two thirds reported that they 'were happy to be deaf.' In contrast, mainstream programs reported 20 percent of their teachers were deaf and less than one third of the programs included American Sign Language in their instruction. One half of these students called

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themselves 'Deaf' or 'deaf' and one half called themselves 'hard of hearing'; half reported that they were 'happy to be deaf.' The author emphasizes that, if access to deaf culture and student satisfaction are considered important, then it seems that placement in residential schools is ideal for teenagers who are deaf. •

Genes of Silence: Scientists Track Down a Slew of Mutated Genes That Cause Deafness Source: Science News. 153(3): 42-44. January 17, 1998. Summary: This article reports on recent findings in the field of genetics that may provide information about the auditory system and deafness. The deafness gene resembles a fruit fly gene called diaphanous, which has offered some clues to the human gene's possible role in hearing. The scientists are also investigating whether some cases of hearing loss stem from mutations in a second human gene that also resembles diaphanous. These two 'sister' genes are not the first nonsyndromic deafness genes isolated; another gene encoding a protein called connexin 26 was identified some months earlier. The author reports on family studies and on animal studies (in mice). As the genetic data accumulate, physicians may begin to screen for mutations that might cause late-onset hearing loss or counsel people about potential outcomes of future pregnancies. Mice will also serve as the initial testing ground for any treatments that might emerge from the discovery of deafness genes. 2 figures. (AA-M).

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Educational Programs for Deaf Students Source: American Annals of the Deaf. 147 (2): 83-176. Summary: This article reviews controversial issues in the prevention, treatment, and accommodation of deafness in America's educational institutions. The narrative examines topics that have ethical implications for professionals, such as genetic counseling, gene therapy, a parent's right to have a deaf child, cochlear implants, and the increasing federal emphasis on educational outcomes that are not relevant to deaf students. Trends in research and public awareness about deafness are highlighted. A directory provides contact information for special schools and local programs in the United States and Canada. Specific services and communication methods used by the schools are identified in a matrix format. A list of postsecondary programs is also included.

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Genes Involved in Deafness Source: Current Opinion in Genetics and Development. 9(3): 309-314. June 1999. Contact: Available from Elsevier Science. P.O. Box 945, New York, NY 10159-0945. (888) 437-4636 or (212) 633-3730. Fax (212) 633-3680. E-mail: [email protected]. Summary: This article reviews some of the strides that have been made in the field of hereditary deafness. The authors note that, to date, mutations in at least 35 genes are known to cause hearing loss. The authors explore the possible biological roles of human deafness genes, focusing on those that have been identified in the past two years and that are known, or are suspected, to affect inner ear function when mutated. Topics include mutations affecting early inner ear development, mutations affecting sensory hair cells, mutations affecting melanocyte development, and mutations affecting endolymph homeostasis. The authors conclude that increased understanding of the function of these genes has not only provided insight into the molecular basis of deafness, but also of normal auditory function. 1 figure. 53 references (6 annotated).

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Sudden Deafness: An Auditory Mystery Source: Hearing Health. 16(2): 16-18. March-April 2000. Contact: Available from Voice International Publications, Inc. P.O. Drawer V, Ingleside, TX 78362-0500. Voice/TTY (361) 776-7240. Fax (361) 776-3278. Website: www.hearinghealthmag.com. Summary: This article reviews the diagnosis and etiologies of sudden deafness, characterized as an 'auditory mystery.' The authors begin with a brief case report of a woman who realized that she was suddenly deaf in her left ear. The authors encourage readers to obtain a diagnosis, even for mild cases of hearing loss, because not only does the loss of hearing disrupt quality of life, but its occurrence could also signify a serious condition. Topics include diagnostic tests and the use of a detailed patient history in determining why sudden deafness has occurred; the audiological evaluation; sudden deafness in children; possible causes of sudden deafness; the role of trauma (which can interrupt blood flow to the inner ear); recommended actions when sudden deafness strikes; treatment options, including drug therapy and surgery; and spontaneous recovery from sudden deafness. 1 table.

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Genetics and Molecular Biology of Deafness Source: Otolaryngologic Clinics of North America. 32(6): 1067-1088. December 1999. Contact: Available from W.B. Saunders Company. 6277 Sea Harbor Drive, Orlando, FL 32887-4800. Summary: This article reviews the genetics and molecular biology of deafness, stressing that otolaryngologists will increasingly play a role in the evaluation of people with hearing impairments. The authors outline three reasons that knowledge of genetics and molecular biology is becoming more important: during the past two decades, there has been a chance in the most common causes of severe and profound sensorineural hearing loss (i.e., less rubella, fewer trauma induced problems, and reduced use of ototoxic drugs); in the last decade, approximately 49 genes that cause hereditary hearing impairment have been identified; and, as genes that cause hereditary hearing impairment are located and closed, scientists gain information about the biologic mechanisms that likely will lead to new methods for treating and preventing sensorineural hearing loss. Hereditary hearing impairment can present as an isolated occurrence, or in association with a number of anomalies to be part of a syndrome. The screening of newborns for hearing impairment using the techniques of molecular biologists and geneticists will result in early identification and appropriate intervention for those at risk for hereditary hearing impairment. 1 figure. 6 tables. 41 references.

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How Do Children Who Can't Hear Learn to Read an Alphabetic Script?: A Review of the Literature on Reading and Deafness Source: Journal of Deaf Studies and Deaf Education. 5(1): 9-31. Winter 2000. Contact: Available from Oxford University Press, Journals Customer Service, 2001 Evans Road, Cary, NC 27513. (800) 852-7323 or (919) 677-0977. Fax (919) 677-1714. E-mail: [email protected]. Summary: This article reviews the literature on reading and deafness, focusing on the role of three broad factors in acquisition and skilled reading: the method of encoding print; language specific knowledge (i.e., English); and general language knowledge. The author explores the contribution of three communication systems to reading: spoken

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language, English based sign, and American Sign Language (ASL). Their potential contribution to literacy is mediated by four parameters on which they differ: codability, structural isomorphism, accessibility, and processibility. Finally, the author discusses the implications for additional research as well as for education. The author concludes that understanding and facilitating the acquisition of literacy by deaf children requires attention to a multitude of factors. As complex as this developmental task is in hearing children, it is rendered even more complex by the biological constraints attendant upon deafness and complex sociocultural milieu within which deaf children live and grow. 1 figure. 113 references. •

Deafness Is a Scourge (And You Can Say That Again) Source: Geriatrics. 53(8): 65-66, 69, 73. August 1998. Contact: Available from Advanstar Communications. 131 West First Street, Duluth, MN 55802-2065. Summary: This article reviews the problems associated with noise-induced hearing loss (NIHL), particularly in older people. NIHL is caused by three related factors: the intensity of the noise, noise duration, and a genetically based sensitivity to noise induced deafness. The author reviews the occupations and activities that increase one's risk factors for NIHL, discusses eight myths regarding hearing loss and hearing, the contributions of age to hearing loss (including the role of presbycusis, or age-related hearing loss), the role of the general practitioner or gerontologist in identifying and managing hearing loss in their patients, how to help patients adjust to life with a hearing aid, and the kinds of assistive devices and equipment that can be helpful to patients with hearing loss. Topics include the negative impact of hearing loss on quality of life, tinnitus, the indications for hearing aids, the different types of hearing aids, the role of the audiologist, amplification, and listening environments.

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Deaf Children and English: Parents Can Help Source: Perspectives in Education and Deafness. 15(3): 2-3. January-February 1997. Summary: This article reviews the strategies parents can use to help their children who are deaf or have hearing impairments learn English. The author notes that deaf people are so bombarded with the importance of learning English that the result is they some times become scared to use it. This fear can be debilitating, especially for children who are deaf. The article lists strategies for communication and building language skills. These tips include: model good reading behavior every day, watch sign language videotapes with the children, model using a dictionary, model writing at least once a week, communicate directly to the child in writing, buy a few special books and writing supplies for the children, play a word-based game at least once a week, learn special techniques for reading and writing to and with children who are deaf, clarify communication that is misunderstood, and ask others for help in creating a languagerich home environment.

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World Health Organisation and the Prevention of Deafness and Hearing Impairment Caused by Noise Source: Noise and Health. Volume 1: 6-12. September-December 1998. Contact: Available from Noise and Health. Institute of Laryngology and Otology, University College London, 330 Gray's Inn Road, London WC1X 8EE, United Kingdom. +44 171 915 1575. Fax +44 171 278 8041.

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Summary: This article summarizes the activities of the World Health Organization (WHO) in its efforts to prevent deafness and hearing impairment caused by noise. The WHO Programme for Prevention of Deafness and Hearing Impairment (PDH) is especially targeted at developing countries where there is a serious lack of accurate population based data on the prevalence and causes of deafness and hearing impairment, including noise induced hearing loss (NIHL). However, opportunities exist for prevention of NIHL by primary, secondary, and tertiary means and it is necessary for countries to measure the size of the problem and adopt strategies for its prevention. The author describes the two WHO resolutions passed in relation to PDH (1985 and 1995); these resolutions affirmed that much deafness and hearing impairment is avoidable or remediable and that the greatest needs for the problem are in developing countries. WHOPDH addresses problems in this field of public health which are amenable to intervention, giving priority to the poorest developing countries. These problems include ototoxicity, chronic otitis media (ear infection) noise damage to hearing, inherited and congenital causes of deafness, and the provision of appropriate affordable hearing aid services. The PDH program has developed a standardized Ear Disease Assessment Protocol to enable countries to conduct national surveys rapidly. The article also reports briefly on a recent PDH meeting at WHO (Geneva, October 1997); the participants emphasized that NIHL is the most prevalent irreversible industrial disease and the biggest compensable occupational hazard. The meeting recommended that all countries implement the National Programmes for the Prevention of Noise Induced Hearing Loss, integrated with primary health care, including elements on health promotion and measures to reduce noise sources, and introduce legislation and effective hearing conservation. 2 tables. 7 references. (AA-M). •

Cochlear Implants and Education of the Deaf Child Source: The Hearing Review. May 2003. 10(5). p. 18-22+. Contact: Available from CurAnt Communications Inc., Publisher. 6701 Center Drive West, Suite 450, Los Angeles, CA 90045-1535. (310) 642-4400. PRICE: Available free online at www.hearingreview.com. Summary: This article summarizes the results of a five-year study conducted by the Central Institute for the Deaf and funded by the National Institute on Deafness and Other Communication Disorders. The study concludes that deaf children who receive cochlear implants early in life and after appropriate rehabilitation fare better in developing speech skills than those who use hearing aids. The original study appeared in the Feb. 2003 monograph supplement of Ear and Hearing.

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Deaf Children and English Source: Perspectives in Education and Deafness. 15(4): 4-5. March-April 1997. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. Summary: This article, the second in a two-part series, provides suggestions for parents who want to foster English language development with children who are deaf. The author stresses that there are many ways to support formal and informal interaction with children while encouraging them to develop English language skills. Watching sign language videos, modeling the use of a dictionary, and playing weekly word games are a beginning. This article explores some more complex activities that may contribute

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to the process. The activities include communicating regularly through writing, handling public encounters the 'deaf' way (use writing to handle situations with the public), sharing family journal writing, interacting with Deaf adults, reading books by and about Deaf people, keeping up with sign language training, learning new signs regularly, joining the state association of the Deaf and the local Deaf club, organizing an informal signing study group with other interested parents; and making sure the TV has a decoder. Each idea is briefly explained. •

More Deafness Genes Source: Science. 280(5368): 1403. May 29, 1998. Contact: Available from American Association for the Advancement of Science. 1200 New York Avenue NW, Washington, DC 20005. Website: www.sciencemag.org. Summary: This brief article reports on recent findings of new genes underlying genetic deafness. In Nature Genetics, moderate but dominantly inherited hearing impairment in two large families is shown to be a result of a mutation in the TECTA gene, on chromosome 11. Also, a report in this same issue of Science (May 29, 1998) finds that the unconventional myosin gene MY015 is mutated in the shaker-2 mutant mouse and in three human families with recessive, nonsyndromic deafness linked to chromosome 17, DFNB3. In addition, the discovery of alpha-tectorin mutations in people with hearing impairment suggests that the properties of the tectorial membrane are critical for delivering an appropriate stimulus to the hair cells. The authors briefly discuss these findings. The authors conclude by commenting that the difficult work is yet to come: establishing exactly what these molecules do in hair cells, and why each one has such a different effect on the ultrastructure of the developing cell. 10 references.

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Expressive Vocabulary Development of Infants and Toddlers Who Are Deaf or Hard of Hearing Source: Volta Review. 100(5): 1-28. 2000. Contact: Available from Alexander Graham Bell Association for the Deaf and Hard of Hearing. Subscription Department, 3417 Volta Place, NW, Washington, DC 20007-2778. Voice/TTY (202) 337-5220. Website: www.agbell.org. Summary: This chapter is from a text that provides information on the language, speech, and social emotional development of very young children who are deaf or hard of hearing. In this chapter the authors report on a study of the relationships between several demographic variables and expressive vocabulary development. Basing their research on the MacArthur Communicative Development Inventory (CDI), the authors studied a group of 113 children who were deaf or hard of hearing (aged 24 to 37 months). The significant predictors of expressive vocabulary included the child's age, the age of identification of the child's hearing loss (before or after 6 months), the child's cognitive quotient, and the presence or absence of one or more disabilities in addition to hearing loss. The authors present normative data for expressive vocabulary development for children who are deaf or hard of hearing, for ages 8 through 37 months. The authors conclude that despite the apparent advantage in vocabulary ability evident in the early identified normal cognitive group of children, relative to previous reports on children who are deaf or hard of hearing, these children still appear to be delayed relative to data reported on hearing children. 5 figures. 6 tables. 53 references.

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Health Care of the Deaf-Toward a New Understanding (editorial) Source: Journal of the American Board of Family Practice. 13(1): 81-83. January-February 2000. Contact: Available from Drohan Management Group. 11250 Roger Bacon Drive, Suite 8, Reston, VA 20190-5202. (703) 437-4377. Fax (703) 435-4390. E-mail: [email protected]. Summary: This editorial comments on an article in the same issue about the health care experience of elderly deaf patients (Witte and Kuzel). The editorial explores the availability and problems of medical care for the elderly Deaf in Richmond, Virginia (where the research study took place) and comments on health care delivery to the Deaf population at large. The author uses the word deaf to represent people with severe hearing impairment and Deaf (capitalized) to represent the subset that feels linked culturally. The editorial takes a realistic approach, reminding readers that many of the complaints or problems that Deaf people face in accessing health care are the same ones that hearing people face (hard to reach the doctor on the telephone, hard to get laboratory results, doctor uses big words that are hard to understand, nurses and secretaries speak too quickly). The author encourages physicians in communities with large Deaf populations (such as Washington, D.C.) to learn about the Deaf culture, but notes that most physicians in other communities will have neither the time nor the energy to do so. The author recommends some simple strategies that can improve communication with patients, regardless of the presence of a hearing loss. The author concludes by reminding readers that most Deaf do not consider deafness a handicap; rather, they consider it an inconvenience. The physician's caring and attention will serve to make their deafness less of an inconvenience. 1 reference.

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Trip Through the Ear in Search of Deafness (editorial) Source: Pediatric Annals. 28(6): 342-344. June 1999. Contact: Available from SLACK Incorporated. 6900 Grove Road, Thorofare, NJ 080869447. (856) 848-1000. Fax (856) 853-5991. Summary: This editorial reminds primary care physicians of the importance of regular screening for and awareness of the symptoms of hearing loss in infants and children. The author describes the statistics of typical diagnosis of hearing loss in children, briefly considers the impact of universal neonatal screening,and helps readers understand the pathogenesis of hearing loss, with the goal of increasing the likelihood that abnormalities will be recognized. Topics include the qualities of sound: intensity or loudness (measured in decibels, dB) and frequency or pitch (measured in cycles per second, Hz); hearing evaluation tests; the physiology and anatomy of the inner ear; the different types of hearing loss and deafness; and the impact of noise in damaging the hearing. The author reminds readers that other anomalies arising from the same embryology as hearing anomalies are indications that hearing screening should be performed. In addition, it is recommended that a child be referred for a hearing screen if the family history is positive for deafness or if a parent has concerns about the child's hearing. 7 references.

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Maximizing Auditory and Speech Potential for Deaf and Hard-of-Hearing Children: Proceedings of a Clinical Roundtable Source: Pediatric News Journal Reprint 2002. International Medical News Group at Rockville, MD.

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Contact: Available from Oberkotter Foundation, Oral Deaf Education. Voice (877) ORALDEAF (672-5332). TTY/Fax (877) 672-5889. E- mail: [email protected]. Web site Order Form: http://www.oraldeafed.org/materials/orderform.html. PRICE: Available at no cost. Summary: This 'Pediatric News' supplement contains the proceedings from a clinical roundtable at which a panel of six experts discussed some critical issues related to maximizing auditory and speech potential in children who are born with hearing loss or who develop early-onset hearing problems. The topic areas included early diagnosis and intervention, diagnostic protocol, timing of referrals, audiologic evaluation and amplification, cochlear implants, the team approach, review of educational options, and oral deaf education. The supplement concludes with a hearing and language milestones guide for parents and a list of resources for parents with hearing loss. 1 table. •

Programs and Services for the Deaf Source: American Annals of the Deaf. 145(2): 61-230. April 2000. Contact: Available from American Annals of the Deaf. Fowler Hall 409, 800 Florida Avenue, NE, Washington, DC 20002-3695. Summary: This special edition of the American Annals of the Deaf offers a reference to programs and services for the Deaf. The journal begins with an article summarizing the educational and communication needs of deaf and hard of hearing children, with a focus on the principles of fundamental educational choice. The issue then lists schools and programs in the United States, schools and programs in Canada, postsecondary programs, university and college programs for personnel in Deafness, programs for Deaf-blind children and adults, supportive and rehabilitative programs, and research on deafness (a list of related doctoral dissertations). Specific categories include programs for training teachers, professional specialists, and interpreters; Helen Keller Centers for Deaf-blind youth and adults; programs for training teachers of Deaf-blind students; Federal programs; state vocational rehabilitation offices; regional and local programs; national professional organizations and centers; the National Association of the Deaf; professional members of the Conference of Educational Administrators of Schools and Programs for the Deaf; and professional committee members of the Convention of American Instructors of the Deaf. The contact information is provided for all programs noted; a chart summarizes the services available and the communication methods used at the schools and programs in the Directory.

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Health Care Utilization and Adults Who Are Deaf: Relationship with Age at Onset of Deafness Source: HSR: Health Services Research. 37(1): 105-120. February 2002. Summary: This study analyzed data from the Hearing Supplement of the 1990-1991 National Health Interview Surveys to compare healthcare utilization for adults who became deaf before and after the age of three years old. Indicators of healthcare utilization included physician visits, private insurance, and preventive care such as blood pressure monitoring, pap smears, and mammograms. Overall, deaf adults reported a lower health status than hearing adults in the general population. Adults who became deaf prelingually (before the age of three) visited a physician less often than adults in the general population and adults who became deaf postlingually. Healthcare utilization for prelingually deafened adults was comparable to adults from language-based minority groups and may be attributable to language barriers. Postlingually deafened adults visited a physician more often than hearing adults in the

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general population. However, mammograms were less common for postlingually deafened women, revealing a need to address specific prevention services. Future research should focus on the differences between subgroups of the deaf population and methods for improving communication between deaf persons and their healthcare providers. •

Emergency Preparedness Tips for Children and Adults Who Are Deaf or Hard of Hearing Source: Volta Voices. 9(5): 14-15. September/October 2002. Summary: Warning sounds made by fire engines or emergency sirens can't be heard by people who are deaf or hard-of-hearing. This article informs people with hearing impairments how to be aware of their environment and to use their other senses to detect potential danger. Readers also learn how to be prepared for emergencies, and what actions to take if they are involved in an earthquake, fire, or other disaster.

Federally Funded Research on Deafness The U.S. Government supports a variety of research studies relating to deafness. These studies are tracked by the Office of Extramural Research at the National Institutes of Health.2 CRISP (Computerized Retrieval of Information on Scientific Projects) is a searchable database of federally funded biomedical research projects conducted at universities, hospitals, and other institutions. Search the CRISP Web site at http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen. You will have the option to perform targeted searches by various criteria, including geography, date, and topics related to deafness. For most of the studies, the agencies reporting into CRISP provide summaries or abstracts. As opposed to clinical trial research using patients, many federally funded studies use animals or simulated models to explore deafness. The following is typical of the type of information found when searching the CRISP database for deafness: •

Project Title: ASSESMENT

4-D

NANOPROBE

TECHNOLOGY--DEVELOPMENT

AND

Principal Investigator & Institution: Bledsoe, Sanford C.; Director; Otolaryngology; University of Michigan at Ann Arbor 3003 South State, Room 1040 Ann Arbor, Mi 481091274 Timing: Fiscal Year 2001; Project Start 01-AUG-1999; Project End 31-JUL-2003 Summary: (Adapted from Applicant's Abstract): The goal of this project is to develop new micromachined silicon-probe technology with thin-film recording sites to acquire images of brain activity over three spatial dimensions plus the 4th dimension of time. Specifically, the applicants proposed to develop and test two new probe assemblies for chronic experimentation in the central auditory system. First, they would develop a chronic multishank recording probe with intrinsic drug-delivery microchannels and 2

Healthcare projects are funded by the National Institutes of Health (NIH), Substance Abuse and Mental Health Services (SAMHSA), Health Resources and Services Administration (HRSA), Food and Drug Administration (FDA), Centers for Disease Control and Prevention (CDCP), Agency for Healthcare Research and Quality (AHRQ), and Office of Assistant Secretary of Health (OASH).

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pumps to both assess and influence a 3-D tissue environment over time. Drug-delivery probes have been fabricated and used in acute experiments in the central nucleus of the inferior colliculus (CIC). The aim here is to adapt these probes to long-term chronic implantation and drug delivery to this deep auditory structure. Second, they would develop and test a new type of 3-D microelectrode array. It is based on the fabrication of two planar arrays that are connected by a forked silicon ribbon cable and that have recording sites which face inward and are mirror images of one another. This "Brain-ina-Box" (B-I-B) probe will be used in semi-chronic studies in the auditory cortex of awake cats. Major issues to be addressed in this proposal concern 1) chronic recording and drug delivery, 2) flexible but stable interconnect cables and 3) high-channel-count percutaneous connectors. To help guide new directions and developments in fabrication and assembly, the applicants proposed to conduct two animal experiments as test beds for the maturation of the technology. The objectives of the first project are to use 32-site, drug-delivery probes to examine the extent and patterns ("images") of central neural activation in the CIC of guinea pigs to various spatial and temporal configurations of electrical stimulation of the auditory nerve, to correlate these neural patterns with behavioral measures in the same animal and to examine how the patterns and behavior are influenced by time after deafness, intervention and manipulation. The goal of the second project is to use B-I-B probes to characterize the spatio-temporal patterns of cortical activation associated with the coding of soundsource location in the auditory cortex of awake cats. A semi-chronic paradigm will be developed that will permit study of a population of units for some days or weeks, followed by explanation and replacement of the probe at a different cortical site. The probes would sample neurons simultaneously at up to 32 sites, with a spatial scale of some hundreds of micrometers and a temporal scale of milliseconds to hundreds of milliseconds. The practical and logistical information gained from both studies will help guide modifications and refinements of the technology. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: A DEVICE ENABLING DEAF AND HEARING PEOPLE TO COMMUNICATE Principal Investigator & Institution: Curry, Emma; Scomm, Inc. Box 1509, 130 S Limit Sedalia, Mo 65301 Timing: Fiscal Year 2003; Project Start 01-JAN-2003; Project End 31-DEC-2003 Summary: (provided by applicant): The goal of this research program is to explore the feasibility of developing a device to facilitate face-to-face communication between deaf and hearing people. sComm Inc. has had limited, but extremely positive, experience with a prototype device that allows two people to interact directly by typing messages to one another in a format that permits simultaneous display of both messages. Preliminary results suggest this straightforward approach is remarkably better than using an interpreter or handwritten messages. This work will be extended by investigating the willingness of hearing people to use this form of communication with deaf people in various settings, the willingness of deaf individuals to use the device and their experiences, the impact of the device on the employment of deaf people, the general impact of the device on the communication process, and the potential benefits of the device for deaf users beyond communication. In parallel, several design issues will be addressed to establish the feasibility of producing the device affordably. The importance of improved communication between deaf and hearing individuals cannot be overemphasized. Experience with the prototype suggests this device potentially can

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remove many barriers, giving deaf individuals freedom through communication with hearing individuals on a one-on-one basis. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: AAOHNS--CLINICAL TRIALS COOPERATIVE GROUP Principal Investigator & Institution: Brookhouser, Patrick E.; Director; American Academy of Otolaryngology-Head Head and Neck Surgery, Inc. Alexandria, Va 22314 Timing: Fiscal Year 2001; Project Start 15-APR-1997; Project End 31-MAR-2003 Summary: This application seeks support for a Clinical Trials Cooperative Group dedicated to the planning, conduct, analysis, and dissemination of results of clinical studies on the treatment or management of diseases and disorders of hearing, balance, smell, taste, speech, and voice or any combination. The Group structure includes: A Central Office (CO); a Central Trials Coordination Unit (TCU) experienced in the data management and statistical aspects of clinical trials; and a network of participating Investigator Groups (IG). This application proposes the American Academy of Otolaryngology-Head and Neck Surgery Foundation, Inc. (AAO-HNSF) as the CO; a group of senior investigators at the Bowman Gray School of Medicine Dept. of Public Health Sciences as the central TCU, and a group of highly experienced, researchoriented otolaryngologists representing over 20 major public and private university medical centers in the United States as the IG. The Otolaryngology Clinical Trials Cooperative Group (OCTCG) will have overarching responsibilities related to the development of clinical trials, selection of participating units, setting standards for conduct, quality control, and publications for individual clinical trials and for enhancing the disseminatIon of results from OCTCG clinical trials. The first trial proposed will investigate treatment of autoimmune inner ear disease, a potentially devastating condition that, left untreated, can lead to total deafness and disequilibrium in a very short time period. Available treatments for this condition (high dose prednisone methotrexate, cyclophosphamide) are currently utilized without proof of efficacy or assessment of risks. Using a multi institutional, prospective, double blind, placebo controlled design, the study will address the important clinical question of whether it is an acceptable risk to treat AIED with potent and potentially toxic immunosuppressive drugs when corticosteroids fail to control the disease. The dissemination of these results through the OCTCG should allow the timely transfer of information directly to clinical practice. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: AMINO ACID TRANSMITTERS IN THE AUDITORY BRAINSTEM Principal Investigator & Institution: Altschuler, Richard A.; Professor; Otolaryngology; University of Michigan at Ann Arbor 3003 South State, Room 1040 Ann Arbor, Mi 481091274 Timing: Fiscal Year 2002; Project Start 01-DEC-1986; Project End 31-MAR-2007 Summary: Results now show a diversity in amino acid transmitters and receptor subunits that provide for the specialized shaping of neuronal responses and that their changes can underlie auditory brain stem plasticity. Our first Specific Aim is based on our finding decreased GABA release and correlated decreased inhibition in the inferior colliculus (IC) 3 weeks following deafness. It uses immunocytochemistry and tract tracing to test the hypotheses that decreased GABA release is a consequence of decreased GABA in terminals, with a specific GABA sub-circuit to the IC effected, rather than a "global" decrease in all inputs. Caspary's group found GABA-A receptor subunit

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changes that correlated with age-related hearing loss, but couldn't differentiate "pure aging" versus "deafness". Our second specific aim uses quantitative in situ hybridization to test if there will be comparable subunit changes in our more "pure deafness" model, as well as changes in glycine receptor subunits in the DCN where a glycine mediated decrease in inhibition is found. We further hypothesize correlation between changes in transmitter and receptor. We predict GABA but not glycine receptor subunit changes in the CIC and glycine but not GABA receptor subunit changes in fusiform cells. Studies will also uses receptor autoradiography to test a functional correlate, ligand binding. Our final specific aim uses gene micro arrays to screen for differential expression following deafness and test the hypothesis that deafness induces decreased expression of presynaptic neurotransmitter- related genes and compensatory expression of postsynaptic genes. It then uses quantitative in situ hybridization to test the hypothesis that changes will be specific to distinct neuron types. These studies will provide new information on the role of transmitters and receptors in central auditory plasticity. This will, in turn, increase our understanding of the molecular basis of central auditory system dysfunction and provide clues for interventions that might help improve the reintroduction of hearing following deafness, currently with cochlear prostheses and in the future after hair cell regeneration. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: AUDITORY EXPERIENCE AND ADULT NEURON TURNOVER IN BIRDS Principal Investigator & Institution: Kirn, John R.; Professor; Biology; Wesleyan University Middletown, Ct 06459 Timing: Fiscal Year 2001; Project Start 26-JAN-2001; Project End 31-DEC-2003 Summary: (adapted from applicant's abstract) Neurogenesis persists into adulthood in many vertebrates including humans. An understanding of the factors that control neuron addition and survival may ultimately lead to insights on the functions of adult neurogenesis and suggest mechanisms for brain repair. In adult warm-blooded vertebrates, the most widespread production of neurons occurs in the avian telencephalon. In songbirds, many new neurons are inserted into the High Vocal Center (HVC), a region necessary for the perception and production of learned vocalizations. New HVC neurons replace older neurons that have died. Recent work shows that auditory input is necessary for normal rates of neuronal replacement. Available data suggest that deafening decreases the numbers of HVC neurons incorporated and prolongs their subsequent survival. Proposed work will directly test whether the life span of neurons that have been successfully incorporated into HVC prior to deafening is augmented. Additional studies will determine whether deafening influences the production or early survival of adult-formed neurons. A lesion study will test whether the lateral magnocellular nucleus of the anterior neostriatum (lMAN), an area necessary for song learning, participates in the auditory control of HVC neuron turnover. Playbacks of conspecific song produce increased rates of HVC neuron activity and maximal rates are obtained with playbacks of the bird's own song. Thus, the effects of deafening on HVC neuronal turnover may be due to depriving birds of song-related auditory stimulation. Proposed experiments will systematically deprive hearing-intact birds of access to self-generated auditory feedback and/or conspecific song to test this hypothesis. We will contrast these effects with those following manipulations that alter, without completely blocking, self-generated auditory feedback from singing. Collectively, these studies will explore the sensory requirements for the control of adult

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neuron addition and loss within a discrete, well-characterized neural system controlling a learned vocal behavior. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CELL REGENERATION

FORM

AND

GENE

EXPRESSION

IN

HAIR

CELL

Principal Investigator & Institution: Cotanche, Douglas A.; Director of Research, Associate Professo; Children's Hospital (Boston) Boston, Ma 021155737 Timing: Fiscal Year 2001; Project Start 01-JUL-1992; Project End 31-AUG-2006 Summary: (provided by applicant): The long-term objectives of this research are to understand the mechanisms that regulate hair cell regeneration in the avian inner ear and to apply this knowledge to induce regeneration in the mammnalian cochlea. Ultimately, the goal of this research is to utilize hair cell regeneration to ameliorate deafness in humans. Studies from the previous two funding periods of this grant have shown that the auditory epithelium in the postembryonic avian cochlea is mitotically quiescent. However, in response to sound exposure or aminoglycoside administration, the nonsensory supporting cells emerge from quiescence and divide to produce new hair cells that contribute to the structural and functional recovery of the cochlea. In this proposal, we will examine how the proliferative state of supporting cells in the avian cochlea is regulated by activating signals from dying hair cells competing with inhibitory influences generated by connexin expression in gap junctions of differentiated supporting cells. Our hypothesis is that signals released during apoptosis in hair cells induce the supporting cells to exit quiescence and begin proliferating and that connexin43 expression suppresses proliferation in supporting cells of the normal cochlea. The specific aims of this proposal will examine: 1) the role of apoptosis in regulating hair cell death and supporting cell proliferation in the regenerating cochlea; and 2) the role of connexin gene expression in suppressing proliferation of supporting cells in the normal and 'regenerating cochlea. In the first aim we will identify key proteins in the apoptotic pathway, block apoptosis by surgically implanting miniosmotic pumps in birds that will deliver inhibitors of apoptosis directly to the inner ear, and determine how apoptosis regulates hair cell survival and supporting cell proliferation. In the second aim we will define the expression of connexin isoforms in the regenerating cochlea and we will disrupt the regulatory function of connexin43 in normal and regenerating cochlleae by surgically implanting miniosmotic pumps that will deliver inhibitors of gap junction connectivity to the inner ear. These specific aims will be addressed utilizing in situ hybridization and northern blot analysis for mRNA localization and quantification, immunoblots of cochlear protein expression, miniosmotic pump delivery of chemicals directly to the inner ear, and confocal microscopy for immunocytochemical localization of proteins in sectioned and wholemount cochleae. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CELL SURVIVAL IN A NEURAL CIRCUIT FOR LEARNING Principal Investigator & Institution: Johnson, James F.; Anthropology; Florida State University 118 North Woodward Avenue Tallahassee, Fl 323064166 Timing: Fiscal Year 2002; Project Start 01-JAN-1995; Project End 31-JUL-2006 Summary: (provided by applicant): Like humans, songbirds learn the sounds used by their species to communicate during a sensitive period of early development. In many songbird species only males learn to sing and a striking consequence of this behavioral

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sex difference is that the brain regions for song control are prominent in adult males and diminished in size (or absent) in adult females. In the zebra finch, some vocal control regions initially develop in both sexes. However, during the juvenile phase of song learning these regions show substantial neuron death in females, and growth with little neuron death in males. Our working hypothesis is that the quality of song learning and the morphological development of song regions are influenced by how often juvenile birds practice their vocal pattern. A role for use-dependent mechanisms in motor learning and neural growth and plasticity has ample precedent in other animals, including humans. However, the contribution of use-dependent mechanisms in vocal learning (human or songbird) has never been evaluated, partly because of the difficulty of measuring a behavior that is voluntary and that occurs at high levels over the course of development. However, with the use of an event-triggered recording system (which allows continuous monitoring of the vocal output of individual birds), we have shown that daily song production is significantly greater in juvenile birds that are learning to sing than in adults. Individual differences in song production are also greatest in juveniles, and birds that sing more as juveniles have a higher quality song as adults. Thus, songbird vocal learning appears to involve a 'practice-driven' component, which may interact with maturational factors to influence neural development and determine the quality of the adult vocal pattern. Several gene products will be examined in this context to establish links between singing and 1.) storage of the effects of practice, 2.) mechanisms that drive the growth of song regions, and 3.) motivational systems that influence rehearsal of song by juveniles. All of the gene systems that we will study in zebra finches show a high degree of homology to their human counterparts, so what we learn about their role in songbirds may have functional implications for human brain development and vocal learning. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CLINICAL & MOLECULAR ANALYSIS OF NEUROMUSCULAR DISORDERS Principal Investigator & Institution: Kimonis, Virginia E.; Pediatrics; Southern Illinois University Sch of Med Box 19616, 801 Rutledge St Springfield, Il 62794 Timing: Fiscal Year 2001; Project Start 25-JUN-2001; Project End 31-MAR-2002 Summary: (provided by applicant): Since the mid1980s, science has made tremendous progress in understanding genetics, including the roles that genes play in certain diseases. In particular, significant strides have been made towards identifying the molecular basis of neuromuscular disorders. These recent findings have initiated exciting studies of genebased therapies. The focus of our laboratory has been on the clinical and molecular pathogenesis of unique neuromuscular diseases of families from Central Illinois. The original K02 grant was for clinical and molecular delineation of a unique combination of features in a large family with CharcotMarieTooth and deafness. This ongoing study has led to the identification of a unique mutation in the PMP22 gene that cosegregates with the disease phenotype. We have recruited additional families to improve our understanding of this interesting phenotype. Molecular studies propose to examine the mechanism of hearing loss by determining patterns of PMP22 expression in the cochlea of mice throughout development and identifying new PMP22 mutations associated with deafness. As a result of our interest in neuromuscular disorders we have expanded our research interests to include another unique disorder: autosomal dominant limbgirdle muscular dystrophy in combination with Paget disease of bone and Alzheimer disease in some individuals. We have mapped this disorder to a unique focus on chromosome 9. The focus of our research is to identify the gene that disrupts

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basic cell function and causes a myriad of phenotypes in this family. The aim of the current grant is to develop molecular and analytical techniques that will advance our understanding of CharcotMarieTooth disease, limbgirdle muscular dystrophy and other neuromuscular disorders. The suggested training will provide the PI with skills necessary to investigate the clinical and basic molecular pathogenesis of these disorders and aid in the development of novel treatment protocols. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CLONING OF NON-SYNDROMIC DEAFNESS GENES DFNA29 & DFNA32 Principal Investigator & Institution: Li, Xiaoyan C.; House Ear Institute 2100 W 3Rd St Los Angeles, Ca 90057 Timing: Fiscal Year 2001; Project Start 09-FEB-2001; Project End 31-JAN-2004 Summary: (from applicant's abstract): The long-term goal of this study is to understand the function and dysfunction of hearing at the molecular level. Hearing impairment is the most common human sensory disorder. Approximately 70 million people worldwide suffer from hearing loss over 55 dB. Genetic factors are one of the most important causes for hearing impairment. Approximately one in every 2,000 children is born with severe to profound hearing loss due to a genetic cause. Non-syndromic hearing loss occurs in isolation, and it accounts for approximately 80% of hereditary deafness. Studies on the molecular basis of non-syndromic hearing loss, especially the late onset forms, are not only important for improving our understanding of the molecular mechanisms of auditory development and function, but also for developing more precise genetic counseling and therapeutics for both genetic and environmentrelated hearing impairment. We have mapped two new deafness loci, DFNA29 and DFNA32, using linkage analysis on two large US families with non-syndromic autosomal dominant progressive hearing loss. The PI proposes to identify the responsible genes, and to explore their function in the hearing process. The following specific aims are proposed: (1) Refine the critical region of DFNA29 and DFNA32 using recombinational mapping and linkage disequilibrium strategies. (2) Establish permanent cell lines from both affected and unaffected individuals by Epstein-Barr virus transformation. (3) Identify the molecular basis of these loci using a positional candidate gene approach. (4) Establish expression patterns of the genes responsible for DFNA29 and DFNA32 in the developing auditory and vestibular systems using PCR, RT-PCR, and RNA in situ hybridization. This specific aim represents a long-term direction of the study. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: CLONING TWO NEW DEAFNESS MUTATIONS Principal Investigator & Institution: Johnson, Kenneth R.; Research Scientist; Jackson Laboratory 600 Main St Bar Harbor, Me 04609 Timing: Fiscal Year 2001; Project Start 01-JAN-2000; Project End 31-DEC-2004 Summary: Impairment of hearing is the most common sensory deficit in human populations and affects about one of every 1,000 children. The mouse is an excellent model for studying human hearing disorders because of the anatomical and functional similarities between the mouse and human inner ears. In mice, mutations affecting the vestibular system of the inner ear often result in a characteristic circling or head bobbing phenotype; many of these mutations also affect the cochlea and cause deafness. Two independent spontaneous mutations responsible for such abnormal behavior were

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discovered at The Jackson Laboratory and mapped to Chromosomes (Chr) 9 and 17, at positions where no other mouse mutations or deafness genes have been located. Both mutations when homozygous cause deafness, as assessed by the absence of auditory brainstem responses to stimuli greater than 99 dB SPL. Preliminary light microscopic analysis of cross-sections from cochleas indicate that both mutations cause neuroepithelial defects. Mouse mutations with similar defects have been shown to be models for human nonsyndromic hearing loss. On the basis of known human-mouse genetic map relationships, the new mouse mutations may be homologous to the human nonsyndromic deafness genes DFNB16 and DFNA13. The object of this proposal is to clone both of the mouse mutations by the positional-candidate gene approach. Preliminary mapping results have localized each mutation to within a 5 cM interval. These intervals will be further refined to less than 0.2 cM by recombinational analysis of extended linkage crosses. Physical maps will then be constructed and candidate genes within these regions will be screened for mutations. The human homologues of the genes shown to be mutated in mice will be identified for evaluation as candidates for human deafness. Mouse mutations enable studies of inner ear anatomy and development that are not possible in humans; such studies help elucidate pathways critical for the normal development and physiology of the ear. Another objective of this proposal is to establish a time course of pathology and gene expression in inner ears from mutant and control mice. The molecular identification and pathological characterization of these two new mouse mutations causing deafness will provide valuable models for understanding causes of hearing impairment in humans and for developing possible treatments and therapies. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: COMPLEX STIMULUS PERCEPTION WITH COCHLEAR IMPLANTS Principal Investigator & Institution: Chatterjee, Monita; Assistant Scientist; House Ear Institute 2100 W 3Rd St Los Angeles, Ca 90057 Timing: Fiscal Year 2002; Project Start 01-APR-2002; Project End 31-MAR-2007 Summary: (provided by applicant):Cochlear implant listeners today enjoy reasonably good speech understanding in quiet but generally experience severe deterioration in performance in the presence of background sounds. To provide usable speech information through the cochlear implant in the presence of competing sources (speech or nonspeech), basic principles underlying the perception of complex, multichannel stimuli by the electrically stimulated auditory system need to be investigated. This proposal aims to investigate mechanisms underlying complex pattern perception by cochlear implant listeners. The long-term goal of this research is to discover fundamental principles of complex stimulus perception by both electrically stimulated and normal hearing auditory systems. Two hypotheses will be investigated: a) that principles of perceptual organization can be used by cochlear implant listeners for separating out sounds from multiple sources and b) noise is important in signal processing by biological systems in general and by the auditory system in particular, and the right kind of noise introduced into cochlear implants will be beneficial. The proposed experiments are designed to measure the perceptual interactions between pulse-train carriers with similar / dissimilar temporal envelopes exciting adjacent / distant tonotopic locations. Methods will include detection and discrimination of complex stimuli, perceptual difference measures, and auditory streaming measures. Effects of noise on the detection of envelope cues in single- and multi-channel complex stimuli will be measured. It is expected that this research will yield fundamental insight into mechanisms of auditory perception with cochlear implants. Because of their

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fundamental nature, the findings are likely to also further our understanding of the normally functioning auditory system. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CONTROL OF INNER EAR DEVELOPMENT IN THE ZEBRAFISH Principal Investigator & Institution: Weinberg, Eric S.; Professor; Biology; University of Pennsylvania 3451 Walnut Street Philadelphia, Pa 19104 Timing: Fiscal Year 2002; Project Start 01-AUG-1996; Project End 31-MAY-2007 Summary: (provided by applicant): Studies on the genes involved in inner ear development should increase our understanding of human inner ear defects resulting in deafness and/or impairment of vestibular function. Although teleosts have a much reduced portion of the inner ear devoted to auditory reception, the vestibular portion of the inner ear is remarkably similar to that of mammals. Furthermore, the fundamental structure of the sensory epithelium is conserved in fish and mammals. The zebrafish embryo offers a number of advantages in the identification of genes that may be involved in inner ear defects, and in the experimental testing of the function of genes known to be expressed during the development of the inner ear. This proposal continues work that has been carried out in our laboratory on zebrafish inner ear development. We propose four Specific Aims. The first Aim directly continues our work on fate mapping of the specialized structures of the inner ear. In addition to completion of the fate map of the 24 hpf otic vesicle, we will carry out fate mapping of statoacoustic and anterior lateral line ganglia precursors. We will also trace cell movements and determine relative proliferation rates for various otic vesicle territories. These studies with wildtype embrv are essential to understand the cellular defects of mutants with defects in inner ear development. The se ond Aim continues our work on dog-eared, a gene we have demonstrated by positional candidate gene cloning to encode the Eyes Absent 1 (Eyal) protein. This finding demonstrates that the zebrafish is an excellent model for the study of human deafness disorders since mutations of the human EYA1 result in branchio-oto-renal syndrome. It is thus likely that other zebrafish inner ear mutants will be ot great relevance to the study of human deafness and vestibular disorders. In the third Aim of the proposal, we propose to molecularly identify quadro, a gene required for proper formation of the otic placode. quadro mutant embryos develop defects in otic placodal precursors between 12 and 13 hpf, resulting in a failure to properly express early markers of the placode and the dispersal and fragmentation of placodal tissue. We will apply the methods successfully utilized for dog-eared to the mapping and characterization of the quadro gene. The fourth aim of the proposal is a continuation of our mutant screen for defects in otic vesicle formation and patterning. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: CORE SUPPORT FOR HEARING RESEARCH Principal Investigator & Institution: Liberman, M. Charles.; Director; Massachusetts Eye and Ear Infirmary 243 Charles St Boston, Ma 02114 Timing: Fiscal Year 2002; Project Start 01-JUN-2002; Project End 31-MAY-2007 Summary: (provided by applicant): Three Research Cores are proposed to facilitate interdisciplinary research into hearing and deafness at the Massachusetts Eye and Ear Infirmary (MEEI). The Research Center comprises 19 NIDCD-funded principal investigators, all affiliated with the Eaton-Peabody Laboratory (EPL). They include clinicians and basic scientists, with academic ties to graduate programs and departments at Harvard Medical School and MIT. The Research Base covers a wide

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range of basic and applied research projects from peripheral mechanics to cortical processing, from in vitro systems to human patients, from animal models to neural nets. The EPL research group has a long history of fruitful collaboration based on sharing of equipment, resources and scientific expertise via a system of research cores supported by a sunsetting program project grant. The current proposal builds on the present core structure and personnel to maintain existing, and facilitate further, interdisciplinary research efforts into hearing and deafness. Each of the Cores will support highly experienced personnel to 1) staff, stock, maintain and upgrade existing shared research facilities; 2) train users and/or render expert technical services in these facilities; and 3) provide the necessary expertise to enhance research productivity and facilitate the fusion of different research approaches across the many disciplines represented in the Research Center. The three Cores and the major aims of each include 1) an Engineering Core to design, build and maintain data-acquisition systems, custom acoustical devices, stimulus generation systems, a distributed multi-platform system for computational infrastructure and the local area network to link it all together 2) a Surgery/Histology Core to maintain existing shared facilities and assist research teams in animal surgery and histological preparation for both light and electron microscopy; and 3) an Imaging Core to support the growing needs of Center investigators for digital image acquisition and analysis, including confocal microscopy, computer-aided anatomical reconstruction, automation of morphometry, 3-D reconstruction/rendering, and analysis of functional imaging data. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CORE--ENGINEERING Principal Investigator & Institution: Delgutte, Bertrand; Associate Massachusetts Eye and Ear Infirmary 243 Charles St Boston, Ma 02114

Professor;

Timing: Fiscal Year 2002; Project Start 01-JAN-2002; Project End 31-DEC-2002 Summary: (provided by applicant): Scientific advances at our Research Center crucially depend on specialized computer-controlled experimental facilities for auditory research that are powerful and flexible enough to allow the efficient implementation of new paradigms. A major role of the Engineering Core is to design and maintain such specialized data-acquisition systems (Aim 1). This complex task involves selecting commercial instruments that are compatible with each other, installing these instruments, writing special software allowing them to be flexibly controlled by users, maintaining them, and upgrading them. Core Engineers also design and build electronic, acoustic and mechanical devices that are not commercially available (e.g. acoustic systems) to meet the constantly evolving requirements of experiments (Aim 2). Our diverse scientific needs require using several computer platforms (Macintosh, Windows and UNIX) inter-connected by a "Core Network 7" which insures highly reliable intra-laboratory communication. Continuing engineering effort is needed (Aim 3) to maintain the high reliability of this multi-platform network, reliability that is necessary for our networked data acquisition systems, and to meet new computational and data-storage challenges arising from the increasing use of gene chips, imaging and multi-electrode neural recordings. The function of the Engineering Core is to facilitate both current research projects and new initiatives by taking advantage of emerging and mature technologies. The Engineering Core makes it possible for individual investigators to use sophisticated (or simply complicated) techniques that they would otherwise not be able to develop or use. By providing shared experimental facilities, the Engineering Core also encourages collaborative projects among the investigators using these facilities. Finally, the Engineering Core makes the scientific research of the Center

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more efficient by relieving investigators from many routine housekeeping computer tasks, and by allowing the same custom designs to be re-utilized in multiple setups. In short, the Engineering Core increases the quantity and quality of the Center's scientific output, allowing us to better attack new issues in hearing and deafness. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CORE--SURGERY/HISTOLOGY Principal Investigator & Institution: Adams, Joe C.; Professor; Massachusetts Eye and Ear Infirmary 243 Charles St Boston, Ma 02114 Timing: Fiscal Year 2002; Project Start 01-JAN-2002; Project End 31-DEC-2002 Summary: (provided by applicant): The Surgery/Histology Core will provide the intellectual and physical environment in which Center Investigators, their trainees and support staff can learn and perform a variety of surgical and histological procedures. The Core will 1) maintain existing shared facilities for animal surgery, histological processing and electron microscopy and 2) provide technical expertise and training in surgical techniques (both acute and chronic) and histological techniques for tissue processing of both peripheral and central auditory structures at the light- and electronmicroscopic levels. Some complex surgical techniques, such as preparation of the anesthetized cat for neurophysiological study, will be routinely offered as a technical service. Some histological processing will be performed as a technical service, but only when task complexity task and limited project scope makes it inefficient to train the relevant group. Facility maintenance will include 1) ordering of supplies, 2) preparation of stock solutions, 3) equipment repair, and 4) compliance with local and federal regulations. All research groups in the Center will benefit by the time saved in not duplicating these basic services. All investigators will also benefit in several ways from the repository of experience and expert advice the Core represents. First, significant time will be saved by offloading to Core personnel the task of training new students and new investigator-specific support staff. Second, Core expertise will enhance the research endeavors of all participants by 1) facilitating the incorporation of new techniques and 2) by allowing them to use a wide range of techniques, each on a phasic basis, without having to maintain all the requisite skills within their own group at any one point in time. Core personnel include three experienced individuals (only partial support is requested for each) with demonstrated expertise in all relevant areas, including animal surgery, immunohistochemistry and a wide range of embedding and staining techniques for light- and electron-microscopic evaluation of peripheral and central auditory structures. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: CORTICAL AND BEHAVIORAL RESPONSES TO COCHLEAR IMPLANTS Principal Investigator & Institution: Middlebrooks, John C.; Professor; Otolaryngology; University of Michigan at Ann Arbor 3003 South State, Room 1040 Ann Arbor, Mi 481091274 Timing: Fiscal Year 2001; Project Start 01-JAN-2000; Project End 31-DEC-2004 Summary: Clinical experience and psychophysical research indicate that the configuration of electrical currents on each channel of a cochlear prosthesis can have great impact on recognition of speech and other signals. The goal of this research program is to elucidate mechanisms relevant to normal and prosthetic hearing that can guide development of clinical stimulation strategies. The proposed experiments will

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compare the quality of stimulus representation in the auditory cortex determined by various cochlear current configurations. Monopolar and other configurations that are presumed to produce diffuse neural activation will be compared with bipolar and other configurations thought to produce more focal activation. Experiments will be conducted in guinea pigs. Specific Aim 1 is to characterize the distribution of unit activity across cortical location and post-stimulus time in response to stimulation of a single cochlear channel. Multi-channel cortical recording probes will permit simultaneous unit recording at 16 cortical sites. Artificial neural-network techniques will test the accuracy with which cochlear stimulation channels can be identified on the basis of patterns of cortical activity. Stimulus current levels will be expressed relative to psychophysical thresholds assessed in individual subjects. Specific Aim 2 will test interactions between cochlear channels, using single pulses and amplitude-modulated pulse trains. Cortical phase locking to stimulus modulation will be quantified, then the influence of a simultaneous or interleaved masking pulse train presented on a second cochlear channel will be assessed. Animal psychophysical experiments in Specific Aim 3 will measure thresholds for modulation detection in the presence and absence of a masker on a second cochlear channel. Stimulus conditions will be compared that, in the cortical experiments, either minimize or maximize interaction between cochlear channels. Specific Aim 4 is to identify current configurations that are particularly suited to various histories of deafness and stimulation. The results will be used to examine the central mechanisms that underlie effects of duration of deafness and stimulation. These results will be used to examine the central mechanisms that underlie effects of duration of deafness on prosthesis function. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CPEA DATA COORDINATING CENTER Principal Investigator & Institution: Dukes, Kimberly A.; Dm-Stat, Inc. 407 Rear Mystic Ave, Unit 11A Medford, Ma 02155 Timing: Fiscal Year 2003; Project Start 26-SEP-2003; Project End 31-MAY-2008 Summary: (provided by applicant): Since 1997, the National Institute of Child Health and Human Development (NICHD) and the National Institute on Deafness and Other Communication Disorders (NIDCD) has provided funding for a Network on the Neurobiology and Genetics of Autism: Collaborative Programs of Excellence in Autism (CPEA). The CPEA network of 10 multidisciplinary projects is being extended for an additional five years to continue studies of genes, brain structure, brain function and behavior and the clinical course of autism. Three separate entities (DMSTAT, Inc., the Boston University Statistics and Consulting Unit at the College of Arts and Sciences and the Department of Biostatistics at the Boston University School of Public Health) have formed a group, DMSTAT/BU, proposing to collaborate with the CPEA network and to serve as its centralized data coordinating center (DCC). Our multi-disciplinary team of biostatisticians, epidemiologists, statistical geneticists, project managers, programmers and data managers can effectively and efficiently serve the ongoing and changing needs of the CPEA network. The primary goal of DMSTAT/BU is to provide integrated support to all CPEA project staff on two fronts: 1) consistent, efficient, cost-effective and high quality data management, and 2) biostatistical expertise in study design, randomization schemes, matching procedures, statistical genetics and multivariable analysis. DMSTAT/BU will assist project staff in the following areas: study design and protocol development, innovative processes to streamline data collection and increase data quality, administrative management, staff training, study monitoring, regulatory compliance, statistical design, analysis and reporting, and study documentation, in

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addition to applying our proven data management and statistical analysis procedures, DMSTAT/BU will develop a website for the network which will serve as the backbone of the study infrastructure. The website will have administrative (e.g., e-mail links, meeting information, personnel contact information, project documentation, expense tracking) and data collection (distributed tracking and data entry systems) components. A reporting module in the website will make all of the administrative and data management information accessible to authorized members of the CPEA network. The team we have assembled has the requisite experience to effectively serve the needs of the CPEA network. The qualities that set us apart from our competitors are: innovation in streamlining and organizing processes, our abilities to educate and communicate with study staff at all levels, the breadth and depth of our analytic and statistical knowledge, our commitment to integrity in all aspects of data management, and our outstanding track record on studies of similar size and scope. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CYTOGENETIC APPROACHES TO FINDING AUDITORY GENES Principal Investigator & Institution: Williamson, Robin E.; Brigham and Women's Hospital 75 Francis Street Boston, Ma 02115 Timing: Fiscal Year 2002; Project Start 01-JUL-2002; Project End 31-MAR-2005 Summary: (provided by applicant): The goal of the research proposed is to use a cytogenetic approach to discover new deafness genes. Many genes of the auditory system have been identified through linkage studies of families that carry deafness as a heritable trait. This process is limiting in that a large number of affected individuals need to be analyzed to gain significant data. By ascertaining deaf individuals that carry balanced translocations, it becomes possible to discover disrupted genes with the genetic material of a single person. In this proposal, I plan to study two cases where the individual is deaf and carries a balanced translocation. Using the vast number of resources available now due to the progress made by the Human Genome Project, the sequence disrupted by these translocations will be identified. This will be done by progressively narrowing the breakpoint with FISH experiments using BACs and PCR products known to hybridize in the region. Candidate genes will then be identified by comparing the disrupted sequence to those already deposited in GenBank, or by analyzing these sequences with predication programs and methods. Ultimately, a mouse mutant may be created to confirm the pathogenesis of the disrupted gene. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: DEAFNESS AND OSSIFICATION IN LABYRINTHITIS OSSIFICANS Principal Investigator & Institution: Brodie, Hilary A.; Otolaryngology; University of California Davis Sponsored Programs, 118 Everson Hall Davis, Ca 95616 Timing: Fiscal Year 2001; Project Start 01-JAN-2000; Project End 31-DEC-2004 Summary: (Adapted from the Investigator's Abstract) Labyrinthitis ossificans (LO) is the growth of pathologic new bone within the lumen of the cochlea. It is multifactorial in origin and may result in deafness. Profound hearing loss and LO in children are most commonly associated with meningogenic labyrinthitis. The relationship of LO to meningogenic cochlear pathology and its mechanism of induction have not been clearly defined. Cochlear implants are a significant treatment option for improving hearing and quality of life in these patients. However, LO can reduce the efficacy of cochlear implantation. The long-term objective of this research program is to understand the mechanisms which lead to the development, progression, and destructive aspects of LO.

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Such an understanding may lead to new strategies to prevent the devastating effects of hearing loss associated with this disease. The specific aims of this application are: (1) to correlate hearing loss with the temporal and spatial progression of bacterial meningitis from the subarachnoid space to the cochlea; (2) to correlate hearing loss with the temporal and spatial sequence for both labyrinthine fibrosis and ossification and the histopathology of cochlear tissues: spiral ganglion, organ of Corti, Reissner's membrane, stria vascularis, and spiral ligament; (3) to determine the relationship of bone lining cells to osteoblast formation and recruitment during labyrinthine neo-ossification; and (4) to determine the effects of decomplementation, non-steroidal anti-inflammatory compounds, and bacteriostatic vs. bactericidal antibiotics on neo-ossification and hearing loss. There are four hypotheses/specific aims outlined and they are as follows: Bacterial invasion of the cochlear labyrinth from the subarachnoid space correlates with hearing loss and occurs principally via the cochlear aqueduct and not the internal auditory canal; The destruction of cochlear tissue occurs subsequent to the arrival of inflammatory cells and not with the appearance of bacteria within the cochlea. Hearing loss may occur prior to observable pathology and reflect central auditory damage related to meningitis; Bone lining cells of the endosteum are activated and become mature osteoblasts and are the principal source of neo-ossification in labyrinthitis ossificans; and The inflammatory response to suppurative labyrinthitis includes fibrosis and neo-ossification formation and causes cochlear tissue destruction. Inhibition of this process will result in a reduction in both hearing loss and bone deposition. Methods used: The investigators propose to use an experimental gerbilline model of LO, histomorphometry, fluorescent bone histomorphometry, transmission and scanning electron microscopy, autoradiography, and auditory brainstem evoked response. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: DEAFNESS IN WS1: SEARCH FOR GENETIC MODIFIERS Principal Investigator & Institution: Pandya, Arti; Human Genetics; Virginia Commonwealth University Richmond, Va 232980568 Timing: Fiscal Year 2001; Project Start 01-AUG-1998; Project End 31-JUL-2003 Summary: As a pediatric geneticist, the P.I. has a keen interest in understanding the molecular pathology of inherited disorders, especially in children. The long term goal is to establish a career in academic medicine, which would allow the PI to study the importance of modifier genes for phenotypic variation in clinically relevant genetic disorders. Current Departmental projects are highly relevant to this proposal and will provide the necessary building blocks for the PI's career development. Hearing loss affects at least 5 percent of the population with the incidence of profound deafness at birth or during early childhood being estimated as about 0.8 per 1000. It is etiologically heterogenous, with genetic factors accounting for half of all cases of profound deafness, 10-20 percent of which are due to a specific hereditary syndrome. Waardenburg syndrome (WS) is a symptom complex that includes deafness, dystopia canthorum, white forelock and heterochromia. We participated in mapping the gene to 2q35 region, the discovery of genetic heterogeneity in WS, and in the demonstration of mutational heterogeneity at the PAX3 locus. The extensive phenotypic variation observed within WS1 families combined with the striking concordance for phenotype in MZ twins strongly suggests that modifier gene(s) contribute to this variability. The present proposes to identify and characterize the gene(s) causing deafness in individuals who have inherited a gene for WS type 1. We have identified 22 WS1 families with a minimum of 2 or more WS1 siblings who are both affected with deafness. This is ideal for mapping modifier gene(s) by an "affecteds" only approach. A two tiered mapping

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strategy involving a candidate gene search followed by a genome wide scan is proposed. Nonparametric methods of analysis will be used in addition to the traditional parametric approach to extract maximum information from families being studies. We will also further characterize the spectrum of PAX3 mutations in 30 available families with WS1 and study their potential interaction with the modifier gene(s). The successful identification of modifier genes which cause deafness in WS could lead to improved predictive testing. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: DEVELOPMENT OF CEREBRAL SPECIALIZATIONS Principal Investigator & Institution: Neville, Helen J.; Professor; Psychology; University of Oregon Eugene, or 97403 Timing: Fiscal Year 2001; Project Start 01-APR-1978; Project End 31-MAR-2002 Summary: (Adapted From The Investigator's Abstract): The broad goals are to characterize experience-dependent changes in the developing human brain and to test the hypothesis that they are determined by multiple, specific critical periods. The investigators will characterize the effects of auditory deprivation on the functional organization of the visual system, and the effects of acquisition of a visual-manual language (American Sign Language, ASL) and the effects of delayed exposure to language on the functional organization of different language systems of the brain. The investigators will test different conceptions of the identity and organization of subsystems within vision and language in studies of normal hearing adults. They will determine the nature of the effects and the time periods when altered experience affects the normal development of these systems in studies of congenitally and later deafened individuals, and in native and late leamers of ASL and English. The investigators will record event-related brain potentials (ERPS) and changes in blood oxygenation levels employing functional magnetic resonance imaging (FMRI) to characterize the timing and the location of neural activation as these groups of subjects perform tasks designed to activate specific aspects of sensory and language functions. They will assess the hypothesis, raised by our previous behavioral, ERP and FMRI studies, that there is considerable functional specificity in the alterations in the visual system and the language systems that can occur following auditory deprivation and that: 1. congenitally deaf subjects are more accurate and display faster and more extensive neural activation than normal hearing Ss when detecting, localizing and attending to visual events in the far periphery of the visual fields; 2. the neural systems that mediate this processing are more extensive in deaf Ss and include areas that process auditory information in normal hearing Ss; 3. there are several distinct subsystems within language and these differ in the degree to which they are dependent on and modified by language experience; 4. there is overlap in the neural systems within the left hemisphere that process ASL and English, but there is also extensive activation of the temporal and parietal regions of the right hemisphere for processing ASL only. Since these studies will determine the multiple, different time periods in human development when specific inputs from the environment have the greatest impact, the results will carry implications for the time periods when specific educational programs would optimize development in hearing and deaf children. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: DEVELOPMENT OF INHIBITATION IN THE AUDITORY SYSTEM Principal Investigator & Institution: Sanes, Dan H.; Professor; Center for Neural Science; New York University 15 Washington Place New York, Ny 10003

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Timing: Fiscal Year 2001; Project Start 01-AUG-1989; Project End 31-MAR-2005 Summary: The long-term objective of this proposal is to identify the cellular mechanisms that regulate inhibitory synapse function during development and following hearing loss. The research plan is divided into three areas: (1) The regulation of inhibitory synapse strength will be studied in the developing gerbil lateral superior olivary nucleus (LSO), using a brain slice preparation. The activity-dependent depression of inhibitory synapses from the medial nucleus of the trapezoid body (MNTB) to the LSO will be examined with whole-cell voltage-clamp recordings. The relationship between depression and inhibitory synapse refinement will be tested with paired recordings from dye-filled MNTB and LSO neurons. The cellular basis for depression will be examined by direct activation or blockade of GABA and glycine receptors, and the use of transgenic receptor deletions in mice. The postsynaptic signalling pathway that induces depression will be tested by intracellular application of kinase and phosphatase antagonists. (2) The affect of deafness on inhibitory synapse function will be examined in the gerbil inferior colliculus. Using a brain slice preparation, gramicidin perforated-patch recordings will be obtained from IC neurons, and the evoked synaptic currents will be monitored in response to lemnisal or commissural stimulation. To determine why inhibitor synapse reversal potential depolarizes in deafened animals, postsynaptic chloride homeostasis will be examined in normal and deafened animals using chloride pump and voltage-gated chloride channel antagonists. The ability of synaptic activity to regulate chloride homeostasis will be assessed by monitoring inhibitory reversal potential before and after a prolonged period of excitatory or inhibitory activity. (3) The in vivo occurrence of inhibitory synaptic plasticity will be examined wit extracellular recordings from juvenile gerbils during sound stimulation. The strength of sound evoked inhibition in the LSO will be assessed before and after coactivation of excitatory and inhibitory pathways, using stimulus patterns that induce inhibitory depression in the LSO brain slice. To determine whether inhibitory synaptic strength declines in LSO following deafferentation, the inhibitory pathway will be stimulated electrically following cochlear ablation. The proposed experiments will demonstrate how inhibitory synapse physiology can be modified in the central auditory system, and suggest how inhibitory dysfunction could affect acoustic processing following profound deafness. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: DFNA1 IN INHERITED HEARING LOSS Principal Investigator & Institution: King, Mary-Claire; American Cancer Society Professor; Medicine; University of Washington Seattle, Wa 98195 Timing: Fiscal Year 2001; Project Start 01-JUN-1991; Project End 31-JUL-2003 Summary: The investigators have studied a large Costa Rican family with autosomal dominant progressive non-syndromic hearing loss (DFNA1), and have recently through positional cloning identified a protein truncation mutation in the human homologue of the Drosophila diaphanous gene. This mutation was found in all deaf family members, and not in normal hearing family members or over 300 control individuals. The goals of this application are (1) to provide functional support for the pathogenicity of this mutation through a) generating mutant mice through homologous recombination and showing that these mouse mutants have a deafness phenotype (specific aims 1 and 3) and b) demonstrating that the protein abnormality leads to abnormal stability or intracellular localization (specific aims 2 and 4), (2) to start the study of proteins interacting with diaphanous which will elucidate the pathogenic pathway and provide candidate genes for other deafness genes (specific aim 5), (3) to study three other

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deafness families toward the identification of new deafness genes (specific aim 6), and (4) to collect 200 deaf individuals with some family members from the Kaiser Permanente system as a general resource, and screen them for mutations in the diaphanous system (specific aim 7). Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: DROSOPHILA DEAFNESS GENES: ANALYSIS OF CHORDOTONAL ORGAN Principal Investigator & Institution: Eberl, Daniel F.; Biological Sciences; University of Iowa Iowa City, Ia 52242 Timing: Fiscal Year 2001; Project Start 01-APR-2001; Project End 31-MAR-2006 Summary: The long-term objectives of this proposal are to understand the molecular and cellular mechanisms of auditory mechanosensation. These goals will be achieved by using a new genetic model system for hearing in Drosophila. The public availability of almost the entire genome sequence, together with the genetic, developmental and molecular tools for manipulating Drosophila make this a very powerful model. Hearing in insects is mediated by chordotonal organs, which are related to vertebrate auditory and vestibular hair cells because they are developmentally specified by homologs of the same gene, atonal. The first approach will be to identify mutations that specifically disrupt chordotonal organ function, to clone the corresponding genes and to elucidate the cellular location and molecular function of their gene products. Mutations in three genes, beethoven, smetana and touch-insensitive-larva-B will be subjected to this analysis. The second approach will make use of enhancer trap strains, whose engineered transposon inserts express a reporter gene specifically in chordotonal organs. Four enhancer trap strains identified by this criterion will be used as starting points to clone the flanking sequences to identify candidate chordotonal-specific genes. If the transposon does not disrupt the gene, imprecise excision derivatives will be generated as a way to introduce mutations in the gene. The transposons therefore act not only as reporters, but also as molecular tags and as a mutagen. The third approach will use known human genes associated with deafness as a starting point to identify Drosophila homologs and then to use reverse genetics to identify mutations in these genes to test for function. Methods for this reverse genetic approach will include characterization of nearby transposon insertions or their imprecise excision derivatives as well as recently described gene replacement strategies. Identifying auditory genes by any of these approaches and elucidating the molecular roles of their products will provide very important insight into the fundamental but poorly understood process of mechanosensation. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: DRUG MANIPULATION OF NOISE-INDUCED HEARING LOSS Principal Investigator & Institution: Bobbin, Richard P.; Professor; Otorhinolaryngology; Louisiana State Univ Hsc New Orleans New Orleans, La 70112 Timing: Fiscal Year 2001; Project Start 01-AUG-2001; Project End 31-JUL-2003 Summary: (provided by applicant): The long-term objective of this research is to develop drugs that will decrease the effects of intense sound on the cochlea. Recent results from our laboratory have shown that PPADS, an ATP antagonist, decreases the effect of intense sound on the cochlea as monitored with distortion product otoacoustic emissions (DPOAEs). Our working hypothesis is that PPADS is blocking the effects of endogenous ATP acting on receptors on the perilymph surface of Deiters' cells.

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Exogenously applied ATP depolarizes Deiters' cells and increases their intracellular free calcium levels. Deiters' cells are innervated by what appears to be branches of type II afferents to the outer hair cells (OHCs). This suggests that the source of the ATP acting on ATP receptors on the Deiters' cells may be the terminals of these nerve fibers. Anatomically, Deiters' cells are attached to the base and apex of OHCs, to the basilar membrane, and reticular lamina. Thus Deiters' cells may play a significant role in the transduction process carried out by the OHCs and their stereocilia. Deiters' cells may do this by altering their own stiffness. The hypothesis to be tested is that ATP, proposed as a neurotransmitter in the cochlea, reacts with receptors on the Deiters' cells to enhance the deleterious effects of intense sound on the cochlea. This hypothesis will be tested by experiments that: (1) determine if endogenous ATP enhances the deleterious effects of intense sound by testing ATP agonists and antagonists during intense sound exposure; (2) determine if the mechanism of action of ATP involves a movement of Deiters' cell or a stiffness change; and (3) determine if the receptor mechanism on Deiters' cells involves the metabotropic P2Y type of receptor. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: EXPRESSION OF ION CHANNELS IN THE AUDITORY SYSTEM Principal Investigator & Institution: Kaczmarek, Leonard K.; Professor; Pharmacology; Yale University 47 College Street, Suite 203 New Haven, Ct 065208047 Timing: Fiscal Year 2001; Project Start 01-APR-1993; Project End 31-MAR-2006 Summary: The firing patterns of neurons in central auditory pathways encode specific features of sound stimuli, such as frequency, intensity and localization in space. The generation of the appropriate pattern depends, to a major extent, on the properties of the voltage-dependent potassium channels in these neurons. The Shaw-family Kv3.1 and Kv3.3 channels and the two-pore family rTWIK channel are expressed at high levels in neurons that are capable to firing at very rapid rates and that lock their action potentials to specific phases of auditory stimuli. We plan to determine the mechanisms that regulate these channels in the presynaptic terminals of cochlear nucleus neurons and their postsynaptic targets, neurons of the medial nucleus of the trapezoid body. To test the roles of these channels in timing and regulation of transmitter release, we shall record the responses of neuronal terminals and somata in which the genes for these channels have been eliminated by homologous recombination. We shall test the hypothesis that phosphorylation of the channel proteins alters the transmission of information through this synaptic pathway. Using transgenic animals in which the promoters for the channels are coupled to a fluorescent reporter gene, we shall test whether the naturally occurring differences in the level of expression of the Kv3.l channel along tonotopic axes can be induced by changes in the pattern of activity to which the neurons are exposed. An understanding of how ion channels are regulated in central auditory neurons is likely to lead to therapies for certain forms of deafness, as well as for tinnitus, disorders in the interpretation of auditory stimuli, and states of hyperexcitability such as audiogenic seizures. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: EXTRACELLULAR MATRIX IN SYNAPSE FORMATION IN THE CNS Principal Investigator & Institution: Brunken, William J.; Anatomy and Cellular Biology; Tufts University Boston Boston, Ma 02111 Timing: Fiscal Year 2001; Project Start 01-AUG-2000; Project End 31-JUL-2003

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Summary: (Verbatim from applicant's abstract): Laminins are biologically-active molecules which function as cell adhesion molecules, regulate various aspects of development, and serve to stabilize complex anatomical structures. They are large extracellular matrix molecules that are composed of three subunit chains, designated alpha, beta and gamma. Five alpha, three beta and three gamma chains have been identified. Several disorders of the nervous system are linked to laminin genes: some congenital muscular dystrophies involve the alpha2 chain (merosin); the beta2 chain is reduced in Walker-Warburg syndrome, and a complex group of CNS developmental disorders (muscle-brain-eye disease; retinitis pigmentosa with deafness (RP21 with deafness); Walker-Warburg syndrome) map to the site of the gamma3 gene. Laminins are widely expressed in the CNS; we have shown that in the human, rat, bovine and mouse retina, four laminin chains (alpha3, alpha4, beta2 and gamma3) are found in the interphotoreceptor matrix and in the matrix of the outer plexiform layer (OPL). These chains are likely to form two heterotrimers, laminin-13 and laminin-14. The retinal laminin chains appear to play important roles in the morphogenesis of photoreceptors; first, these chains are expressed prior to the onset of rod genesis and persist into adulthood; second, ablation of the gene encoding one of the chains, beta2, results in the production of dysmorphic photoreceptors with aberrant function. Specifically, photoreceptor outer segments are reduced in length; the photoreceptor terminal in the OPL are disrupted: finally, in ERGs, the amplitude of the b-wave is drastically diminished, suggesting that transmission between photoreceptors and bipolar cells is disrupted by loss of laminin beta2 chain function. We hypothesize that laminins-13 and 14 are critical mediators of synapse formation and stabilization between photoreceptors and second order cells in the OPL. Furthermore, we hypothesize that laminins-13 and 14 form unique substrates with which photoreceptor axons interact and to which they adhere in order to elaborate synapses, and, finally, that the molecular structure of the synapse is dependent on the interactions between these laminins and their receptors. We propose to test several aspects of this hypothesis. We will ask several specific questions: (1) what the spatial and temporal expression of laminin-binding molecules is in the OPL; (2) whether these molecules mediate the binding of cells to OPL laminins; and (3) what anatomical and physiological alterations in the photoreceptor synapse result from laminin gene disruptions. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: FMRI OF PEDIATRIC COCHLEAR IMPLANT CANDIDATES Principal Investigator & Institution: Holland, Scott K.; Professor; Children's Hospital Med Ctr (Cincinnati) 3333 Burnet Ave Cincinnati, Oh 45229 Timing: Fiscal Year 2001; Project Start 01-AUG-2001; Project End 31-JUL-2003 Summary: (Provided by Applicant): This R21 proposal describes a feasibility study targeted for funding under the Neurosciences Technology Development program (PA98-050) through NICHD. The proposal outlines three aims necessary to develop functional magnetic resonance imaging (fMRI) technology as a tool for evaluation of pediatric candidates for cochlear implantation. FMRI is a relatively new and highly sophisticated technology, which can provide a window into the functional human brain. Cochlear implantation is also a cutting edge technology in approximately the same development phase as fMRI. The integration of these technologies presents a challenge. Successful integration of these technologies in very young children is a formidable proposition but offers the opportunity for new understanding of human deafness, cortical plasticity, brain development and connectivity. The proposal has three simple aims, which constitute the necessary preliminary steps toward development of fMRI

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technology for use in pediatric cochlear implant candidates. We aim to: 1) Determine whether auditory cortical activity can be observed with fMRI with sound stimulation in sedated toddlers and young children with normal hearing; 2) Demonstrate that auditory cortical activity can be observed with fMRI using sound stimulation in compliant older children with severe to profound, sensorineutral hearling loss; and 3) Perform fMRI of auditory stimulation in severely to profoundly hearing impaired toddlers and young children under sedation. These aims define a logical course toward establishing that fMRI can be used successfully in pediatric cochlear implant candidates, many of whom are very young. If this feasibility study is successful, it will provide essential data needed to develop the neuroscience technology required to apply functional neuroimaging in severely to profoundly hearing impared toddlers and young children. This project will also more clearly define the potential role of fMRI in the selection and management of cochlear implant patients. The most optimistic outcome of this project would be that fMRI could be used to predict future benefit from a cochlear implant in a child. Short of achieving this high goal, this project will certainly accomplish the stated purpose of the Neurosciences Technology Development program (PA-98-050) of supporting "research that will develop significant enhancements to existing technologies important to neuroscience and research that will translate a scientific concept into the basis for a future technology that may advance understanding of important neuroscience research problems." Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: FUNCTION AND STRUCTURE OF PENDRIN IN THYROID CELLS Principal Investigator & Institution: Kopp, Peter A.; Medicine; Northwestern University Office of Sponsored Programs Chicago, Il 60611 Timing: Fiscal Year 2003; Project Start 01-JAN-2003; Project End 31-DEC-2007 Summary: (provided by applicant): Pendred's syndrome is an autosomal recessive disorder defined by congenital deafness, goiter and an impaired thyroidal iodide organification. It is caused by mutations in the PDS (Pendred's syndrome) gene. Mutations in this gene may be among the most frequent genetic causes of congenital deafness since they are not only associated with Pendred's syndrome, but they also form the molecular basis of two forms of non-syndromic deafness. The PDS gene encodes pendrin, an anion transporter belonging to the Solute Carrier Family 26A (SCL26A4). Pendrin is predominantly expressed in the thyroid, the kidney and the inner ear. Functional studies in Xenopus oocytes revealed that pendrin is able to transport chloride and iodide. In thyroid follicular cells, pendrin is expressed at the apical membrane suggesting that it could be involved in the transport of iodide into the follicular lumen. In the kidney, pendrin is found in beta-intercalated cells of the cortical collecting duct and is thought to function as a chloride/base exchanger. The exact role of pendrin in the inner ear remains unknown. Our preliminary data support the concept that pendrin is an apical iodide transporter. A detailed characterization of the anion transport properties of pendrin is essential for the understanding of its role in iodide transport in thyrocytes and the synthesis of thyroid hormones. At this point, there are no data on the kinetic properties of pendrin-mediated iodide transport, and its regulation. The membrane topology and secondary modifications of pendrin are unknown, and the determinants for PDS gene expression have not been characterized. The goals of this proposal are focused on studies addressing the function and structure of pendrin. The studies in Specific Aim 1 aim at further characterizing the iodide transport properties of pendrin. The experiments outlined in Specific Aim 2 seek to characterize the membrane topology and secondary modifications of pendrin and will

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thus contribute to the elucidation of structure-function relationships. The experiments in Specific Aim 3 will determine the cell specificity of the pendrin promoter and study its regulation. These studies will provide fundamental insights into the (patho) physiology of this novel anion transporter that has important functions in the thyroid, the kidney and the inner ear. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: FUNCTIONAL AND MOLECULAR CHARACTERIZATION OF PENDRIN Principal Investigator & Institution: Karniski, Lawrence P.; Associate Professor; Internal Medicine; University of Iowa Iowa City, Ia 52242 Timing: Fiscal Year 2001; Project Start 01-FEB-2001; Project End 31-JAN-2005 Summary: (Adapted from applicant's abstract): Pendred syndrome, manifested by sensorineural hearing loss and goiter is the result of mutations in the PDS gene. PDS encodes a protein labeled pendrin that functions as a chloride, formate and iodide transporter and is expressed in the thyroid, inner ear and kidney. Pendrin's function is similar to a previously described chloride/formate exchanger that plays an important role in NaCl transport across epithelial cells, suggesting that pendrin might perform a similar role in the inner ear. Recent evidence suggests that some individuals with mutations in the PDS gene do not develop thyroid abnormalities but instead have nonsyndromic deafness with dilated vestibular aqueducts (DFNB4). The aims of this proposal are to characterize pendrin in terms of its function, location and regulation and determine how different PDS mutations affect pendrin. The following approach will be taken to achieve these aims: Polyclonal anti-pendrin antibodies (already generated by the Principal Investigator) will be used to identify the cell types in which pendrin is expressed. Pendrin function will be analyzed by determining substrate specificity, inhibitor profile, kinetics of transport and regulation, and chloride/bicarbonate exchange. The effect of different mutations in PDS on protein production, processing, regulation and transport properties will be examined. A knock out mouse model will be used to study the mechanisms of ion transport in cells where pendrin is normally expressed but rendered inactive. This work is a first step towards understanding the physiologic role of pendrin and determining how defects in pendrin lead to the clinical manifestations of Pendred syndrome. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: FUNCTIONAL STIMULATION

EFFECTS

OF

DEAFNESS

AND

NEURAL

Principal Investigator & Institution: Pfingst, Bryan E.; Professor; Otolaryngology; University of Michigan at Ann Arbor 3003 South State, Room 1040 Ann Arbor, Mi 481091274 Timing: Fiscal Year 2001; Project Start 30-SEP-1997; Project End 31-JUL-2006 Summary: adapted from applicant's abstract): Deafness resulting from destruction of the inner ear is accompanied by partial degeneration of the auditory nerve as well as anatomical, physiological and molecular changes throughout the central auditory nervous system. These changes vary considerably among deaf individuals. By hypothesis, these individual differences are associated with the large differences across subjects in psychophysical and speech recognition performance with cochlear implants. In the set of experiments proposed in this application, we will use neurophysiological measures to characterize the large psychophysical changes that occur following

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deafening and implantation of the cochlea. We will then examine the functional effects of post-deafening treatments, such as chronic electrical stimulation of the cochlea, that are intended to retard the long-term deleterious effects of deafness on the auditory system. The functional responses to electrical stimulation through cochlear implants will be assessed at the psychophysical level in humans and guinea pigs by measuring detection threshold functions and discrimination functions, and at the neurophysiological level in guinea pigs by measuring spatial and temporal responses of neurons in the inferior colliculus to electrical stimulation and by measuring gross potentials that reflect summed neural activity at peripheral (ECAP and EABR) and central (EMLR) sites along the auditory pathway. Three aims will be addressed. In Aim 1 we will compare psychophysical responses obtained from humans and guinea pigs to determine the relevance of guinea pig data to humans. In Aim 2 we will characterize the pronounced short-term effects of deafening and implantation procedures on the psychophysical and neurophysiological responses to electrical stimulation using the guinea-pig animal model. In Aim 3, we will test hypotheses about the functional benefits of post-deafening treatment of the ear. The results of these experiments will lead to better use of animal (guinea pig) models for auditory prosthesis experiments, they will provide a better understanding of the functional (diagnostic) implications of commonly used psychophysical measures such as detection threshold level, and they will have direct application to treatment protocols for deaf and severely hearingimpaired patients. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: GAP JUNCTION CHANNEL FORMATION AND GATING Principal Investigator & Institution: Bukauskas, Feliksas; Neuroscience; Yeshiva University 500 W 185Th St New York, Ny 10033 Timing: Fiscal Year 2001; Project Start 01-FEB-1999; Project End 31-JAN-2003 Summary: Direct cell-to-cell diffusion of ions and cytosolic molecules is mediated by gap junction (GJ) channels. Each channel is a multimer of connexin (Cx) subunits that forms an intercellular aqueous pore b docking two pre-assembled hemichannels, one from each of two apposed cells. The first aim of this proposal is to study GJ channel formation as it relates to the events that occur after the establishment of cell-cell contact We utilize our ability to manipulate cells into contact while recording and uniquely combine electrophysiology of GJ formation with fluorescence imaging of Cx distribution in vivo real time. Cxs will be fused with enhanced GFP to allow their visualization. We will examine how the dynamics of Cx redistribution, such as plaque formation is associated with the establishment of electrical coupling. We will examine whether Cxs arrive at the junctional membrane from cytoplasmic stores of adjacent membranes and how membrane fluidity and extracellular Ca2+ affect the association between Cx distribution and GJ formation. We will also study the biophysical properties of de novo GJ channel openings, a process that we propose is a form of gating elicited by hemichannel docking. The second aim of the proposal is to test the hypothesis that the de novo opening of a GJ channel involves Cx domain(s) that are part of a ~common~ gate that is acted upon by different agents. Based on our studies of voltage and chemical gating of GJ channels formed of different Cxs, activators of this common gate include alkanols, H+ and membrane voltage. These agents close GJ channels completely, and the ~common gate~ may represent the principal means by which coupling is dynamically regulated in cell populations where voltages remain uniform. We will also examine gating by transjunctional voltage (Vi). We demonstrate that the Vi gate only partially closes GJ channels to a residual state whose conductance is Cx specific. We will examine

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the ionic selectivity of the residual state and will assess the pore size of the residual and fully open states by measuring intercellular diffusion of uncharged dyes. The third aim of this proposal is to explore how Vi gating combined with the rectifying properties of open and residual states can play a role in regulating electronic transmission between excitable cells. Using heterotypic junctions, with combinations of Cxs that are present in the nervous system, we demonstrate conditions under which some heterotypic junctions exhibit nearly unidirectional transmission. These studies focus on defining the mechanisms that control GJ channel formation and gating which are ultimately necessary for determining the role of intercellular communication in normal and diseased states. Human genetic disorders that have been linked to Cx dysfunction include C-linked CMT disease, visceroatrial heterotaxia and sensorineural deafness. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: GENETICS AND PEDIATRIC NONSYNDROMIC HEARING LOSS Principal Investigator & Institution: Kenna, Margaret A.; Children's Hospital (Boston) Boston, Ma 021155737 Timing: Fiscal Year 2002; Project Start 16-AUG-2002; Project End 31-JUL-2006 Summary: (provided by applicant): Sensorineural hearing loss (SNHL) occurs in 4/1000 newborns. Until recently the work-up used a "shotgun" approach; many tests were ordered but the etiology remained unknown in 50% of cases. However, the recent use of genetic testing and high-resolution imaging has increased the yield of the SNHL evaluation. Mutations in the connexin 26 gene (Cx26) are responsible for approximately 50% of all recessive nonsyndromic SNHL allowing Cx26 gene testing to greatly expand the ability to provide an etiology for SNHL. This information can provide diagnostic as well as prognostic information to the patients, their families, and their physicians. The current proposal seeks to address several areas that have not been addressed by our studies or those of others. First, the audiologic phenotype of infants and children with SNHL and Cx26 mutations, both at the time of presentation and longitudinally, needs further definition. Second, it is not clear whether the audiologic phenotype varies with specific mutations or combination of mutations. Third, it is not clear whether CT scans of the temporal bones are ever routinely indicated in a patient with biallelic Cx26 mutations. Finally, in families of children with SNHL, it is not clear how well patients and families are being counseled to understand the benefits, drawbacks, and limitations of genetic testing or the results and implications of their genetic tests. It is also unclear why parents choose or decline genetic testing, and whether proper consent is obtained for such testing. We propose a clinical study to evaluate infants and children with Cx26related nonsyndromic SNHL. To accomplish these aims we will use 2 large patient populations from Children's Hospital Boston (CHB) including 1.Newborns referred after failing a newborn hearing screen; 2.Non-newborns with newly identified SNHL. There are four areas of concentration: 1.) Identification of Cx26 positive infants and children using genetic testing as part of the initial evaluation for newly-identified SNHL. 2.) The phenotype of the Cx26 related hearing loss will be studied, both at the time of diagnosis and prospectively over time. The rate and percentage of progression of Cx26 related hearing loss will also be evaluated. 3.) The diagnostic evaluation of the hearing loss will include temporal bone imaging studies, as the presence of anatomic temporal bone abnormalities is frequently associated with progression of the hearing loss and thus would affect the prognosis if present. Although temporal bone abnormalities have not been sought in large numbers of Cx26 positive patients, they are thought to be uncommon, at least for the two most frequently identified mutations, 35deIG and 67delT. However, recent studies suggest that temporal bone abnormalities may be

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present in some patients with Cx26 mutations. If this were so, the prognosis of the hearing loss would be affected. 4.) For families of children with SNHL, we will utilize a questionnaire to determine why parents choose to have/not have genetic testing and to ascertain parents understanding of genetic testing. This information will be used to evaluate the effectiveness of genetic counseling associated with gene testing for deafness. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: GENETICS OF A NEW MOUSE MODEL FOR DEAFNESS Principal Investigator & Institution: Zheng, Qing Y.; Jackson Laboratory 600 Main St Bar Harbor, Me 04609 Timing: Fiscal Year 2001; Project Start 01-AUG-2000; Project End 31-JUL-2003 Summary: (adapted from applicant's abstract): Genetic impairment of hearing affects about one in every 2,000 children. Identification of genes leading to hearing impairment is essential for understanding factors necessary for normal auditory function and for development of therapeutic strategies. The genetic analysis of mouse deafness mutations has proven instrumental in the identification of several human deafness genes. A new mutation causing hearing and balance defects spontaneously arose in a colony of BALB/cByJ inbred mice at The Jackson Laboratory. The recessive mutation was named 'hypoplasia of the membranous labyrinth' (symbol hml) because it mainly affects inner ear morphology and development. The human homolog of the gene responsible for the hml phenotype in mice may play a role in the etiology and pathogenesis of some cases of human inherited deafness. Currently the pathogenetic mechanism and the identity of hml are not known. A small panel of intercross mice was used to map the hml locus to the middle region of mouse chromosome (Chr) 10. The genetic map position of the mouse hml gene suggests that the homologous human gene may be located on Chr 9pl3, where a particular form of genetic deafness in humans (DFNB15) has been previously mapped. In order to characterize the genetic and developmental dysfunction responsible for this morphogenetic inner ear mutation in mice, and ultimately extend that new found knowledge to improve our understanding of DFNB15 or other deafness traits in humans, the identification and characterization of the hml gene needs to be carried out. Aim 1. Generate a high-resolution genetic map of the region surrounding hml and screen candidate genes for the mutation. Aim 2. Construct a physical contig of YACS and BACS clones across the minimal genetic region. Aim 3. Fully characterize the development and pathology of inner ears from hml/hml mice compared with hml/+ and +/+ controls. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: GENETICS OF NOISE RESISTANCE IN THE AGING EAR Principal Investigator & Institution: Kujawa, Sharon G.; Massachusetts Eye and Ear Infirmary 243 Charles St Boston, Ma 02114 Timing: Fiscal Year 2001; Project Start 01-AUG-2001; Project End 31-JUL-2003 Summary: (provided by applicant): Hearing losses that accumulate with chronic exposure to high-level sound (noise-induced hearing loss; NIHL) and with age (agerelated hearing loss; AHL or presbycusis) are major health problems. They are common, their consequences are permanent, and their impact on human communication and quality of life is significant. Important advances have been made in characterizing the structural changes in the ear that underlie both NIHL and AHL, however, the mechanisms underlying these changes are still poorly understood. In human

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populations, hearing losses secondary to noise exposure and those attributed to aging are highly variable between individuals: some have 'tough' ears, while others have 'tender' ears. In contrast, work with laboratory mice has shown significantly less variability in NIHL and/or AHL within one inbred strain, while there are striking differences between strains. Our long-term goal is to exploit these strain differences in mouse models to study the genetic factors influencing susceptibility to NIHL and AHL, and interactions between them. Our immediate goals focus on the remarkable resistance to NIHL in the inbred mouse strain l29S6/SvEvTac (129S6), recently reported by the Liberman laboratory. Whereas NIHL typically grows rapidly once a critical exposure level is reached, NIHL in 129S6 grows 10 times more slowly over a wide range of exposure energy. Preliminary results from the Kujawa laboratory suggest that 129S6 also shows a type of AHL, demonstrating that AHL vulnerability need not be linked to NIHL vulnerability, as they appear to be in C57BL/6 mice. Further preliminary results from a Kujawa/Tempel collaboration suggest that both the AHL and the NIHL resistance in 129S6 are heritable as recessive traits. With this foundation, we propose to 1) better characterize the AHL and the influence of AHL on NIHL resistance in 129S6 using a number of physiological and histopathological measures, 2) provide a chromosomal localization for the major gene(s) influencing noise resistance in 129S6 using well-established techniques for genetic mapping, and 3) using the same genetic techniques, establish whether the AHL in 129S6 is mediated by the same gene locus associated with AHL in the C57BL/6 strain. The ultimate characterization of the genes influencing susceptibility to AHL and NIHL will provide fundamental insight into the cellular and molecular processes underlying cochlear degeneration, which, in turn, will be key to devising effective strategies to preserve hearing in human populations. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: HAIR CELL DEVELOPMENT IN THE INNER EAR Principal Investigator & Institution: Jiang, Rulang; Assistant Professor; Eastman Dentistry; University of Rochester Orpa - Rc Box 270140 Rochester, Ny 14627 Timing: Fiscal Year 2001; Project Start 01-JAN-2000; Project End 31-DEC-2002 Summary: The long-term goal of this proposal is to understand the molecular mechanisms underlying hair cell development and patterning in the mammalian inner ear. Hair cells are specialized mechno-receptors that are responsible for sensing auditory and vestibular stimuli. Congenital or pathological loss of hair cells in human cochlea causes hearing loss, with a severity that correlates with the extent of missing hair cells. Recent analysis of mice carrying a targeted mutation in the Jagged2 gene, which encodes a cell surface ligand for the Notch family receptors indicates that Notch signaling mediates hair cell differentiation in the mammalian cochlea. Of the four known mouse Notch genes, only Notchl is expressed in the inner ear epithelium that gives rise to both hair cells and supporting cells, suggesting that Notchl may play an essential role in the hair cell development. interestingly, an autosomal dominantly inherited deafness syndrome has been mapped to the same chromosomal region as the human Notchl gene. However, targeted null mutations in the mouse Notchl gene cause early embryonic lethality. To characterize Notchl function in hair cell development, this pilot study examines potential defects in hair cell patterning in the Notchl heterozygous mice. In addition, new gain-of-function and hypomorphic alleles of mouse Notchl will be generated to further characterize the roles of Notch1 in hair cell development. These studies will lead to a better understanding of Notch function in hair cell development and will provide insights to studies of congenital deafness. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: HISTOLOGY CORE Principal Investigator & Institution: Doucet, John P.; Johns Hopkins University 3400 N Charles St Baltimore, Md 21218 Timing: Fiscal Year 2002; Project Start 01-JAN-2003; Project End 31-DEC-2007 Summary: (provided by applicant): The objective of the Histology Research Core is to increase efficiency and productivity of on-going research and to facilitate interactions among members to promote the application of new anatomical tools and/or the collaboration between laboratories with different expertise. Advice and instruction will be given to those needing to incorporate microscopic anatomy into their research. Subject matter will include details of fixation, sectioning, staining, and microscopic image analysis. When appropriate, core staff will perform the technical work for the investigator, but in the majority of cases, the individual researchers will be responsible for the hands-on work. Experimental procedures will consist of standard histology, immunocytochemistry, in situ hybridization and pathway tracing techniques. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: ID OF THE MOUSE DEAFNESS (DN) GENE ON CHROMOSOME 19 Principal Investigator & Institution: Keats, Bronya J.; Professor; Genetics; Louisiana State Univ Hsc New Orleans New Orleans, La 70112 Timing: Fiscal Year 2001; Project Start 01-AUG-1999; Project End 31-JUL-2003 Summary: (Adapted from investigator's abstract) The overall goal is to identify the gene (dn) that is defective in the deafness (dn/dn) mouse. The applicants have mapped the dn gene to mouse chromosome 19 and it is likely to be the ortholog of the human DFNB7/11 gene on chromosome 9q13-q21. The deafness mouse is an autosomal recessive mutant of the curly-tail (CT) stock showing degeneration of the organ of Corti, stria vascularis, and the saccular macula. Ultrastructural abnormalities of the inner hair cells have been shown to be present at birth, and by 15-20 days after birth there are abnormalities of the extracellular spaces of the organ of Corti as well as loss of inner and outer hair cells. By 45 days after birth the inner and outer hair cells have degenerated completely, and the organ of Corti has no distinguishable cell types from base to apex. Interestingly, regeneration of cells other than hair cells occurs in the apical turn between 45 and 90 days after birth. Unlike other mouse models for deafness, the dn/dn mouse does not exhibit circling behavior, indicative of vestibular dysfunction, and therefore may provide insight into the auditory system as distinct from the vestibular system. Preliminary linkage and physical mapping data suggest that the deafness phenotype is associated with a chromosomal rearrangement, probably an inversion. Based on these results, the applicants propose to identify both breakpoints of the inversion, identify and characterize candidate genes, and create a hearing dn/dn mouse using transgenic technology. The specific aims are to: (1) Isolate and determine the sequence at both inversion breakpoints; (2) Identify candidate genes in the vicinity of the chromosomal rearrangement; (3) Isolate cDNAs and analyze for abnormal expression and gene structure in the dn/dn mouse relative to the +/+ mouse; and (4) Rescue the deafness phenotype via the transfer of BAC DNA directly into dn/dn mouse embryos. The deafness mouse is a model for nonsyndromic profound hearing impairment, and identifying the defective gene will be a valuable contribution to our understanding of the genes needed for normal cochlear function. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: IN UTERO NEURODEVELOPMENT

INFECTION:

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Principal Investigator & Institution: Andrews, William W.; Professor; Obstetrics and Gynecology; University of Alabama at Birmingham Uab Station Birmingham, Al 35294 Timing: Fiscal Year 2003; Project Start 17-APR-2003; Project End 31-MAR-2006 Summary: (provided by applicant): Preterm birth is the highest risk factor for cerebral palsy, other adverse neurological outcomes (such as blindness deafness, and mental retardation), and long-term adverse child health outcomes accounting for up to 50% of these adverse sequelae. Most of this morbidity is concentrated among the subset of preterm births that deliver at less than 32 weeks' gestation. Because of the increasing rate of preterm birth in the United States and the increased survival of infants delivered at the earlier gestational ages, the incidence of adverse long-term sequelae is rising. It is now well established that clinically silent upper genital tract intrauterine bacterial infection is strongly associated with a majority of preterm births that occur prior to 32 weeks' gestation. Additionally, emerging data implicate fetal in utero exposure to bacterial infection/inflammation as an independent risk factor for development of cerebral palsy and other adverse health outcomes, it is speculated that proinflammatory cytokine exposure may represent the link to these adverse outcomes. Most of the studies linking infection to adverse neurological outcomes have been limited to short-term neonatal outcomes including markers for subsequent development of cerebral palsy such as periventricular leukomalacia. Long-term follow-up studies in this area are sparse, are largely limited to cerebral palsy as the primary outcome, and provide limited information regarding the maternal/neonatal clinical course. The current literature lacks a longitudinal study with extensive maternal and neonatal clinical, laboratory, microbiological, and histological data on a cohort of maternal-infant pairs on whom long-term outcome evaluation is available including not only cerebral palsy but also other important neurodevelopmental and health outcomes. We hypothesize that in utero exposure to infection/inflammation is associated with increased risk of adverse neurodevelopmental and child health outcomes at age 4-7 years. We propose to test this hypothesis in a cohort of 424 maternal-infant pairs that delivered at our institution between 24 weeks 0 days and 31 weeks 6 days gestation. This cohort has already been studied resulting in an extensive database that includes almost every detail of antepartum, intrapartum, and neonatal clinical data. Also already available from this cohort are histological, microbiological, and biochemical data including umbilical cord blood and neonatal cultures, umbilical cord blood cytokine data, placental histology, and placental cultures. We propose a longitudinal follow-up study of this preterm cohort in order to systematically evaluate the relationship between maternal/fetal infection, fetal exposure to inflammation, neonatal short-term outcomes and ultimate neurodevelopmental outcomes at age 4 to 7 years (Aims 1 and 2) including the potential influence of effect modifiers such as environmental factors and caregiver characteristics (Aim 3). We will also determine if umbilical cord blood markers of neuronal damage are associated with short-term or long-term outcomes (Aim 4) and the strength of association between in utero infection/inflammation, bronchopulmonary dysplasia and long-term indicators of pulmonary dysfunction (Aim 5). Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: INFLUENCES OF HYPOXIA ON NOISE INDUCED HEARING LOSS Principal Investigator & Institution: Chen, Guang-Di; Pharmaceutical Sciences; University of Oklahoma Hlth Sciences Ctr Health Sciences Center Oklahoma City, Ok 73126

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Timing: Fiscal Year 2001; Project Start 15-AUG-2001; Project End 31-JUL-2004 Summary: (provided by applicant): Noise-induced hearing loss (NIHL) is the most common occupational disease in the United States, yet it's mechanisms are not fully understood. The goal of this application is to determine whether and how hypoxia can interrupt auditory function transiently. People may suffer from insufficient blood oxygen supply due to pulmonary or cardiovascular disease, altitude, and environmental pollution by chemical asphyxiates. The risk of environmental noise may be tremendously increased when the noise is under hypoxic conditions. As indirect evidence, carbon monoxide (CO) exposure, which among other effects reduces oxygen supply to tissues, potentiates permanent NIHL at a level that alone does not cause a permanent threshold loss. The investigation of effect of hypoxic inhalation on NIHL will provide a direct test of the susceptibility of the cochlea to reduced oxygen tension. In this application, noise intensity will be varied from lower than the current permissible exposure level (PEL) to 120 dB SPL. Noise intensity higher than 120 dB SPL may cause some mechanical damage to the cochlea. Oxygen level in the exposure chamber will be varied from the normal level (21%) to 6%. Hypoxic inhalation alone with oxygen level lower than 6% may cause a temporary auditory function loss. The designed experiments will measure: (1) noise-induced and noise+hypoxia-induced hearing loss and hair cell loss 4 weeks after the exposure; (2) free radical generation, succinate dehydrogenase (SDH) activity reduction and apoptotic cell death in the cochlea immediately after the exposure; and (3) time course of these biochemical alterations and the protective effect of a free radical scavenger against SDH-activity reduction and apoptosis. We hypothesize that the noise under hypoxic conditions will generate more free radicals than noise alone, which then impair mitochondria, causing a reduction in SDH activity and also release of cytochrome c that may cause apoptosis. We further hypothesize that free radical scavenging will protect against SDH activity reduction and apoptosis. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: INNER EAR CONSORTIUM (CORE CENTER) Principal Investigator & Institution: Baird, Richard A.; Senior Scientist & Center Head; Central Institute for the Deaf 4560 Clayton Ave St. Louis, Mo 63110 Timing: Fiscal Year 2001; Project Start 26-SEP-2001; Project End 31-AUG-2006 Summary: (provided by applicant): We are requesting funds to support a recently constituted Inner Ear Core Consortium designed to enhance exiting research programs and to promote cooperative interactions among 18 scientists at Central institute for the Deaf (CID), Washington University Medical School (WUMS), and St. Louis University (SLU). This consortium consists of an administrative Core Center, three researchoriented core facilities (Digital Imaging Core Electron Microscopy Core, and Gene Expression Core), and one service-oriented core facility (Electronic Services Core). These core facilities are physically located in a new CID research building, recently completed and occupied, immediately adjacent to the south end of the WUMS campus. The scientists in the Inner Ear Consortium have research backgrounds in anatomy, biochemistry, cell biology, developmental biology, molecular biology, molecular genetics, and physiology. These scientists have primary appointments in the Fay and Carl Simons Center for Biology of Hearing and Deafness, one of two centers of excellence at CID, the Departments of Otolaryngology, Anatomy and Neurobiology, Anesthesiology, Molecular Biology and Pharmacology at WUMS, and the Department of Anatomy and Neurobiology at SLU. The research programs of these scientists address crucial issues in the development, function, and regeneration of sensory receptors and their neuronal innervation in the vertebrate inner ear. The Inner Ear

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Consortium maintains expensive common equipment, supports the common needs of inner ear scientists for digital imaging, ultrastructural analysis, and studies of gene expression. It encourages research collaborations that cross existing research programs by promoting interactions among (1) researchers who study the morphology and physiology of the normal, developing, and regenerating inner ear, (2) researchers who study the inner ear on systemic, cellular, and subcellular levels, and (3) researchers who study the auditory and vestibular systems. It promotes the use of mutant and transgenic animal models and trains inner ear scientists in new research techniques, fostering the transfer of these techniques among inner ear laboratories. The Inner Ear Consortium also provides limited assistance for pilot projects, especially those that advance existing scientific programs, involve multiple investigators, or promise to provide new techniques of general interest to inner ear scientists. Finally, it enhances the research training that consortium members provide to residents and medical students in the Department of Otolaryngology and graduate students in the Departments of Speech and Hearing Sciences at CID and the Division of Biology and Biological Sciences at WUMS. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: INNER EAR GAP JUNCTIONS FOR HEARING Principal Investigator & Institution: Zhao, Hong-Bo; Otolaryngology; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 01-AUG-2000; Project End 31-JUL-2002 Summary: The long-term goal of this project is to investigate how gap junctional coupling in the organ of Corti contributes to normal hearing. Gap junctional coupling is extensive between the cochlear non-sensory cells. There is no gap junctional coupling between sensory cells (inner and outer hair cells) or between sensory cells and supporting cells. Disturbances of inner ear gap junctions are known to be associated with a high incidence of non-syndromic deafness. Hypothetical functions of gap junctions in the cochlea may involve nutrition, passage of ions and small molecules, and maintaining intracochlear electrochemical gradients. A gap junction channel is composed of connexin proteins; each cell side has 6 connexin subunits. More than 5 connexin genes have been identified in the cochlea. Recording of macroscopic current (containing many channels) has demonstrated that gap junctional coupling in supporting cells has variables transjunctional voltage (Vj) dependence and membrane potential (Vm) dependence. This indicates complex channel constitutions. The specific objective in this proposal is to identify types of gap junctional channels in the cochlear supporting cells by single channel recording. A double voltage clamp technique will be used to record the single channel activity. Transjunctional current will be measured on weakly coupled cell pairs or using uncoupling agents, such as CO2 and octanol, to diminish the cell coupling. Single channel conductance, kinetics and voltage dependence on either cell side will be examined to identify the channel configuration. Acetylcholine (Ach) and cyclic nucleotide will be applied to cells to test the possible gating regulation. The results of these studies may provide clues as to functional gap junctional pathways in the inner ear, and improve our understanding of their contributions to normal mammalian hearing. This information may be clinically relevant for physicians treating patients with deafness induced by connexin mutations. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: INTERACTION OF AGE RELATED & NOISE INDUCED HEARING LOSS Principal Investigator & Institution: Boettcher, Flint A.; Assistant Professor; Otolaryngology-Head & Neck Surgery; Medical University of South Carolina 171 Ashley Ave Charleston, Sc 29425 Timing: Fiscal Year 2001; Project Start 01-JUN-1999; Project End 31-MAY-2004 Summary: (Adapted from Abstract): Dr. Boettcher will conduct a series of experiments to investigate the additivity of age-related and noise-induced hearing loss in the gerbil. Animals will be raised in a sound-isolating environment, and hearing testing will be performed on each ear before and after exposure of a single ear to noise at a level intended to produce a mild hearing loss. Dr. Boettcher will investigate whether susceptibility to noise-induced hearing loss varies as a function of age, and whether a non- traumatic conditioning sound can reduce the magnitude of age- related hearing loss. Dr. Boettcher will measure auditory threshold using auditory brainstem responses, and will also, in a subset of animals, obtain measures of histopathology of the stria vacularis, hair cells and eighth nerve fibers. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: LANGUAGE, MODALITY AND THE BRAIN Principal Investigator & Institution: Emmorey, Karen D.; Senior Staff Scientist; Salk Institute for Biological Studies 10010 N Torrey Pines Rd San Diego, Ca 92037 Timing: Fiscal Year 2001; Project Start 01-JUL-1979; Project End 31-MAR-2004 Summary: Sign languages provide a powerful tool for investigating the neurobiology and cognitive architecture of human language. For perception, these languages depend upon high-level vision and motion processing systems; for production, they require the integration of motor systems involving the hands and face. What impact does this different biological base have on language processing? How does it affect non-linguistic cognitive functions? Are the same neural systems involved? We investigate these issues by studying neurologically-intact deaf signers, focusing on a coherent set of cognitive functions implicated in the processing of sign language. We proceed along the primary lines of inquiry: I. The effects of spatialization on language. We investigate a unique aspect of sign language: the linguistic use of physical space. We will examine on-line comprehension of spatialized co- reference, the nature of linguistic use of physical space. We will examine on-line comprehension of spatialized co-reference, the nature of linguistic versus cognitive spatial categories, and the relation between spatial gestures and spatial signs. For the first time, we will investigate the representation and processing of cross-linguistic study of how different sign languages express topological spatial relations. Together, these studies will indicate how the visual-spatial modality impacts grammatical form and language processing. II. The interplay between language and visual-spatial cognition. Our proposed studies are motivated by exciting findings under the current grant which show that processing the complex spatial system of American Sign Language (ASL) exerts selective enhancement of non-linguistic visualspatial processing. We investigate the impact of sign language use on the following cognitive functions; motion perception and analysis, the recognition and categorization of human faces and facial expressions, and visual-spatial imagery. A major goal is to determine how the language one uses can affect cognitive processes in non-linguistic domains. III. The neural bases for language and spatial cognition in deaf signers. The unusual sensory and linguistic experience of deaf ASL signers provides a natural opportunity to investigate neural plasticity and the determinants of brain organization.

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We will investigate cerebral specialization for sign language and its cognitive underpinnings, using standard hemifield techniques. We also propose two studies using fMRI which investigate face processing and visual imagery; fMRI will provide an exquisitely detailed picture of the brain bases for these linguistic and cognitive functions. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MAPPING AND IDENTIFICATION OF DEAFNESS LOCI TO LP36.3 Principal Investigator & Institution: Heilstedt, Heidi; Molecular and Human Genetics; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 01-JAN-2000; Project End 31-DEC-2003 Summary: This abstract is not available. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MAPPING DEAFNESS GENES USING GENOMIC MISMATCH SCANNING Principal Investigator & Institution: Cheung, Vivian G.; Assistant Professor; Children's Hospital of Philadelphia 34Th St and Civic Ctr Blvd Philadelphia, Pa 19104 Timing: Fiscal Year 2001; Project Start 01-FEB-1998; Project End 31-JAN-2003 Summary: Hearing loss affects over 40 million people in the United States. It is the most common form of sensory defect in humans. The mechanism of hearing is still largely unknown despite recent advances in molecular medicine. The goal of this proposal is to apply two molecular biology techniques, genomic mismatch scanning and DNA microarray, to gain a better understanding of the genetic basis of hearing loss. This will eventually affect the prevention, treatment and care of hearing disorders. Genomic mismatch scanning (GMS) is a high-throughput genetic linkage technique that allows physical isolation of the identical-by-descent DNA fragments shared between two related individuals. In this proposal, we plan to apply GMS to confirm the localization for DFNBI, the gene for non-syndromic autosomal recessive deafness that has been mapped by linkage to chromosome 13q. Then, we will narrow the DFNBI candidate region using linkage disequilibrium mapping. This has been difficult with traditional mapping strategies due to the need for a very dense set of polymorphic markers. However, in GMS, a dense set of completely informative markers is scanned simultaneously on a whole genome level. In addition, during the grant period, we will develop a DNA microarray that will allow mapping of the identical-by-descent DNA fragments isolated by GMS on a whole genome level in one hybridization step. This DNA microarray will allow mapping of other deafness loci with unknown genomic locations. Finally, we will develop a linkage analysis model for the identity-by-descent maps generated by GMS. Upon achieving the goals delineated in this proposal, we hope to have contributed to the understanding of the genetic control of hearing and have provided a robust method that is a promising tool for the next generation of gene mapping. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MARKING HAIR CELL PROGENITORS WITH BAC TRANSGENICS Principal Investigator & Institution: Segil, Neil; Scientist Ii; House Ear Institute 2100 W 3Rd St Los Angeles, Ca 90057 Timing: Fiscal Year 2003; Project Start 01-APR-2003; Project End 31-MAR-2005

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Summary: (provided by applicant): Sensory hair cell loss is the leading cause of deafness in humans. The mammalian cochlea cannot regenerate its complement of sensory hair cells and thus at present, the only treatment for deafness due to sensory hair cell loss is the use of prosthetics such as hearing aids and cochlear implants. Strategies for hair cell repair that involve either stimulation of quiescent cells in the mature organ of Corti, or which involve transplantation of progenitor cells able to differentiate into hair cells will require markers to identify such cells, as well as a detailed understanding of hair cell precursor biology. However, progenitor cells that give rise to hair cells of the mammalian inner ear are currently uncharacterized, largely because no definitive makers are available for their identification. We propose a pilot project to develop molecular genetic markers that identify unique subpopulations of otic epithelial progenitors. To accomplish this, we will use an important new resource (The Gensat Project) to identify markers of sub-populations of cells in the developing inner ear. This is a collection of transgenic animals that harbor bacterial artificial chromosomes (BACS) as transgenes and which express Green Fluorescent Protein (GFP) under the control of a single gene contained in the BAC. In each transgenic animal, GFP is expressed in a unique sub-population of cells dictated by the expression pattern of the gene in question. We have identified current BAC Transgenic Collection, with a list of genes reported in the literature to be expressed in the inner ear has identified twentythree genes expressed in the inner ear. To explore the usefulness of the BAC Transgenic collection, we propose to analyze four BAC transgenic animals chosen from among this group, based on their potential to add to our knowledge of sensory hair cell progenitors. In Specific Aim 1, each transgenic line will be characterized developmentally for expression of GFP in otic sub-populations each sub-populations will be purified by Fluorescence Activated Cell Sorting. In Specific Aim 2, GFP-expressing sub-populations will be assayed for their ability to differentiate into hair cells using a newly developed dissociated culture system for sensory epithelium. In the Specific Aim 3, the same purified otic epithelial sub-populations will be profiled using micro-arrays to provide initial information about gene expression networks in the developing inner ear. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MEASURING HIV/AIDS KNOWLEDGE AMONG THE DEAF Principal Investigator & Institution: Goldstein, Marjorie F.; Social Sciences Innovations Corporation 71 W 23Rd St, 8Th Fl New York, Ny 10010 Timing: Fiscal Year 2002; Project Start 27-SEP-2000; Project End 31-MAY-2004 Summary: (provided by applicant): This Phase II SBIR application proposes to use Interactive Video Questionnaire technology to interview individuals who are deaf about their HIV/AIDS knowledge, attitudes, and behaviors using a sign language questionnaire to be self-administered on laptop computer. In order to meet the linguistic and cultural needs of the deaf population who use American Sign Language (ASL) as their primary communication modality, survey options will include: standard ASL, and highly contextualized ASL (each with optional captioning), and a captions-only version. Building on translation work that began in Phase I (using a bilingual translation team) and former related research, we will translate 200 HIV/AIDS related items, videotape these and digitize them for use on 12 laptop computers. The survey will then be administered to 1000 individuals who are deaf. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MEMBRANE TRAFFICKING AND CHANNEL ABUNDANCE Principal Investigator & Institution: Jan, Lily Y.; Professor and Hhmi Investigator; University of California San Francisco 500 Parnassus Ave San Francisco, Ca 94122 Timing: Fiscal Year 2001; Project Start 15-AUG-2001; Project End 31-JUL-2006 Summary: (Provided by Applicant): For signaling to proceed normally in the nervous system, there has to be the right number of the right type of ion channels and transmitter receptors on the neuronal membrane. What kind of quality control machinery can ensure the proper assembly of these membrane protein complexes? How does a cell control the number of channels and receptors on its cell membrane? We have found a novel quality control mechanism that curtails the trafficking of inadequately assembled membrane protein complexes from the endoplasmic reticulum (ER) to the cell membrane. This ensures surface expression of fully assembled ATP-sensitive potassium (K-ATP channels with four Kir6.2 and four SUR subunits, and of properly assembled, heterodimeric GABA-B receptors that can functionally couple to the G protein-activated inwardly rectifying potassium (GIRK or Kir3) channels. The ER retention/retrieval signals in the K-ATP channels also limit the number of these channels on the cell surface. How general might be the use of ER retention/retrieval in the quality control of membrane protein complexes? Does the cell regulate other steps of membrane trafficking to control the number and type of ion channels and transmitter receptors? We have developed new methods to test the hypothesis that the numbers of different potassium channels are subjected to different membrane trafficking regulations. The examples to be used in our study are potassium channels that mediate slow synaptic potentials, control neuronal excitability, and potentially protect central neurons under stress. Mutations of potassium channel proteins are known to cause ataxia, epilepsy, deafness, arrhythmia, hypertension, and unchecked insulin release leading to hypoglycemia. Indeed, human epilepsy could result from mutations that reduce the M-type potassium channel activity by only 25 percent. And some of the disease-causing mutations alter the amount of functional potassium channels on the cell membrane. Our goal is to achieve better understanding as to how membrane trafficking regulates channel number and type. This may help us appreciate in the long run how regulation of membrane trafficking might contribute to synaptic plasticity, and whether malfunctions of this process contribute to mental and neurological diseases. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MINK-RELATED PEPTIDES(MIRPS): STRUCTURE AND FUNCTION Principal Investigator & Institution: Goldstein, Steve a N.; Professor and Chief; Pediatrics; Yale University 47 College Street, Suite 203 New Haven, Ct 065208047 Timing: Fiscal Year 2001; Project Start 01-AUG-1996; Project End 31-JUL-2005 Summary: MinK is a small ion channel subunit with a single transmembrane span. It is active only after assembly with a pore-forming subunit. Nonetheless, MinK is required for normal channel function in some tissues. Last period we learned why: MinK determines the gating kinetics, unitary conductance, ion selectivity, regulation and pharmacology of these mixed channel complexes. In the heart and ear, Mink assembles with KCNQ1 to form Iks channels. Inherited Mink mutations are associated with altered IKs function, cardiac arrhythmia and deafness. Mink was thought to be unique until last year when we cloned 3 genes encoding Mink-related peptides (MiRPs). In the last period, we studied the function, dysfunction and structure/function of MinK. We learned how disease-associated mutations altered Mink function and identified residues critical for activity. This allowed isolation of the genes for MiRP1, MiRP2 and MIRP3.

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We then found that MiRP1 assembled with the pore-forming subunit HERG to reconstitute attributes of cardiac IKr channels. This led to our identification of MIRP1 mutations associated with sporadic long QT syndrome (LQTS), a rare disorder that predisposes to sudden death. More significantly, we later discovered that a MiRP1 polymorphism present in approximately 1.6 percent of healthy individuals places this large group at risk for a common, life-threatening disorder: drug-induced LOTS. Our most recent studies reveal that MiRPs operate not only with KCNQ1 and HERG but also with classical voltage-gated potassium channel subunits throughout the body. The central goal of this application arises directly from our studies of Mink over the last five years: we seek to learn how M1RPs (including Mink) operate in normal and disease states. The four specific aims are designed to evaluate (1) newly identified native MiRPpartner complexes from skeletal muscle, heart and brain; (2) newly identified diseaseassociated mutants; (3) MiRP domains and residues that mediate channel function; and, (4) sites of contact in MiRP-partner complexes. We argue these small subunits merit intense scrutiny. First, they are important to normal physiology and disease pathogenesis. Second, they have potential to reveal mechanisms of ion channel function from a unique vantage point: a peptide intimate with (but not of) the pore-forming subunit. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR BASIS OF MATERNALLY TRANSMITTED DEAFNESS Principal Investigator & Institution: Fischel-Ghodsian, Nathan; Professor of Pediatrics; Cedars-Sinai Medical Center Box 48750, 8700 Beverly Blvd Los Angeles, Ca 90048 Timing: Fiscal Year 2002; Project Start 01-MAR-1992; Project End 31-MAR-2006 Summary: (provided by applicant): The broad long term objectives of this research proposal are to elucidate the molecular basis of maternally inherited deafness, and globally to shed light on the mechanism(s) of phenotypic expression of pathogenic mitochondrial DNA mutations. The specific aims of this proposal mainly revolve around the identification and functional characterization of a nuclear gene that is responsible for modifying the clinical expression of the A1555G mitochondrial DNA mutation associated with hearing impairment in humans. The health-relatedness of these aims is to provide the basis for the rational design of therapeutic interventions to prevent, correct, or circumvent the clinical expression of maternally transmitted hearing impairment specifically and mitochondrial DNA diseases more generally. The experimental approach will focus on the identification of a modifier gene on chromosome 8 using genetic mapping and the analysis of candidate genes in the region. In parallel, genetic linkage and candidate gene analysis will be used to identify additional loci and genes that may be modifiers of the clinical phenotype. Once the chromosome 8 modifier gene has been identified, the gene and its functional pathway will be functionally characterized using expression studies, cellular localization, identification of proteins binding to the modifier, and binding assays of both the mutated and non-mutated forms of the modifier protein. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MOLECULAR CONTROL OF PATTERNING IN EARLY EAR DEVELOPMENT Principal Investigator & Institution: Barald, Kate F.; Associate Professor; Cell and Developmental Biology; University of Michigan at Ann Arbor 3003 South State, Room 1040 Ann Arbor, Mi 481091274

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Timing: Fiscal Year 2001; Project Start 01-APR-2000; Project End 31-MAR-2003 Summary: (Adapted from the Investigator's Abstract) Inner ear development has been described as one of the most remarkable displays of precision microengineering in the vertebrate body" (Swanson et al, 1990). The long term goal of this research is to examine the function of genes that shape very early events in inner ear development in order to understand the mechanisms that produce many severe forms of congenital deafness. The experiments in this proposal examine the functional role of two genes expressed early in the development of the auditory system: bone morphogenetic protein 4 (BMP4) and noggin. The first and second specific aims test the hypothesis that the morphogen BMP4 expressed in localized cell foci in the otic epithelium and the BMP4 antagonist, noggin, expressed in the surrounding periotic mesenchyme, are responsible for modeling the semicircular canals (SCC) and sensory tissue (hair cells) within them. The chick is used as the model system in these studies because the levels of these important molecules can be manipulated in the chick system and the effects of altering the environment on auditory system development can he assessed. These experiments cannot he done in the mouse or zebra fish because BMP "knockout" mice and zebra fish mutations die before the inner ear forms, and therefore, any effects of altering the levels of expression of these genes can not be assessed. These experiments depend on misexpression of BMP4 and noggin, by implanting agarose beads bearing cells that express noggin or BMP4 into ectopic locations in the early developing inner ear. Noggin bead implants eliminate SCC selectively; BMP4 implants "rescue" the lost canals. The third specific aim tests the hypothesis that BMP/I expression is critical for sensory cell (hair cell and supporting cell) development in the inner ear. This laboratory has produced BMP4 expressing immortalized otocyst cells from the 9 day Immortomouse. These cells were used to "rescue" the loss of SCC in the preliminary experiments on which these studies are based. The cells in culture not only express hair cell markers in the order expected for hair cells in the embryo, but also serve as a model system to study the role of the relevant genes in the production of hair cell/supporting cells in the inner ear. Furthermore, a BMP-4-expressing IMO cell line also produces the statoacoustic ganglion (SAG) neurite outgrowth/survival factor that directs the emerging neurites of the SAG back to BMFP-expressing sensory cell targets in the developing otocyst. IMO cell lines can therefore be used in molecular studies to identify the molecular cascades that produce sensory cell lineages as well as the SAG neurite outgrowth factor. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR DEVELOPMENT OF THE ENDOLYMPHATIC DUCT AND SAC Principal Investigator & Institution: Choo, Daniel I.; Assistant Professor; Children's Hospital Med Ctr (Cincinnati) 3333 Burnet Ave Cincinnati, Oh 45229 Timing: Fiscal Year 2001; Project Start 02-APR-2001; Project End 31-MAR-2006 Summary: (from applicant's abstract): Homeostasis of inner ear endolymph is critical to sensory transduction in the inner ear. Failure to maintain endolymph homeostasis is thought to result in deafness, vestibular dysfunction and tinnitus in pathologies such as Meniere's disease or certain forms of hereditary hearing impairment. The endolymphatic duct and sac (ELDS) are key structures in maintaining this fluid homeostasis. Therefore, data on the molecular development of the ELDS are very relevant. By focusing on a mouse mutant (kreisler) with an ELDS phenotype, this application seeks to define the molecular pathways involved in induction and differentiation of the ELDS. To determine early targets of kr signaling, this application will test the hypothesis that expression of early molecular markers of the ELDS anlage is

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down-regulated in homozygote kr embryos at embryonic day 10-11 compared to controls. The effects of kr mutation on cellular differentiation within the developing ELDS will be studied by testing the hypothesis that expression of a battery of genes specific for cells in the embryonic day 12 to 18 ELDS is down-regulated in kreisler homozygotes compared to controls. To facilitate direct experimental manipulation of the developing ELDS, this application will develop an in vitro model of the developing kreisler otocyst and ELDS. Experiments will first test the hypothesis that cultured kreisler otocysts developmentally mimic the in vivo system morphologically and functionally. This model will then be used to test the hypothesis that virally mediated expression of kr can rescue the ELDS phenotype in vitro. To test the hypothesis that hindbrain sources of kr induce ELDS differentiation, we will culture kreisler otocysts with wild-type hindbrain explants. Such data will provide insights into the molecular pathways involved in kr signaling and in development of the ELDS. The PI's obvious commitment to medicine and science has been demonstrated by his extensive pursuit of training in the clinical and basic science facets of inner ear biology. The success of these efforts are reflected in his publications which also demonstrate his ability to accomplish quality basic science investigation. In combination with the outstanding academic environment at Children's Hospital Research Foundation, the RCA will allow the PI to continue a rigorous scientific training and successfully address the specific aims outlined in the application. The proposed program of study and the science generated will undoubtedly advance the PI toward his goals of successfully competing for a future R01, and in the long term, becoming a successful independent Clinician scientist. Significantly, this proposal includes challenging but achievable goals that will provide important knowledge to the field of inner ear development. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR DISSECTION OF MYOSIN VIIA Principal Investigator & Institution: Hasson, Tama W.; Biology; University of California San Diego 9500 Gilman Dr, Dept. 0934 La Jolla, Ca 92093 Timing: Fiscal Year 2001; Project Start 01-FEB-2001; Project End 31-JAN-2005 Summary: (applicant's description): The unconventional myosin, myosin-VIIa is critical for the development and function of the inner ear and for the maintenance and function of the retina. Mutations in myosin-VIIa result in Usher disease, characterized by profound deafness and retinal degeneration. Myosin-VIIa mutations also cause recessive and dominant deafness in humans and deafness in the mouse. Classically, myosins are described as motors that hydrolyze ATP and transport protein or organelle cargo along actin filaments. Various genetic and localization studies hypothesize that myosin-VIIa may act in protein transport, organelle movement, phagocytosis, endocytosis, or assembly of the inner ear actin cytoskeleton. Although this list is impressive, we don't actually know if myosin-VIIa is a motor and, significantly, no protein or organelle cargo have been identified. Our long term goal is to elucidate the function of myosin-VIIa in sensory cells and to identify the molecular cargo that this motor is transporting. Towards this aim, we will purify myosin-VIIa and investigate its mechanochemical properties using in vitro actin-binding, ATPase, and motility assays. We will use mutagenesis studies to characterize the effects of Usher disease mutations on myosin-VIIa enzyme function in vitro. To determine the molecular cargo of myosinVIIa, we will continue our analysis of three Myosin interacting Proteins, MYP6, MYP13 and MYP-kelch, which we identified as proteins that bind to the tail of myosin-VIIa. We will determine how these MYPs assemble with myosin-VIIa in vivo and also assess their roles in myosin-VIIa function. Ultimately we will ascertain whether binding of

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molecular cargo directly affects myosin motility. To investigate the functions of myosinVIIa, we have identified two retina pigmented epithelium cell lines that recreate the expression of myosin-VIIa seen in vivo. Using transfection methods we will experimentally assess the hypotheses that myosin-VIIa functions in cytoskeletal assembly, phagocytosis, and membrane transport. Finally, we will extend upon our observation that myosin-VIIa is phosphorylated in vivo, by investigating the importance of this phosphorylation on myosin-VIIa function in the cultured retina lines. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR GENETICS OF DEAFNESS IN THE AMES WALTZER MODEL Principal Investigator & Institution: Alagramam, Kumar N.; Pediatrics; Case Western Reserve University 10900 Euclid Ave Cleveland, Oh 44106 Timing: Fiscal Year 2002; Project Start 01-APR-2002; Project End 31-MAR-2006 Summary: (provided by applicant): We are interested in identifying and studying mammalian genes that are involved in auditory function and development of the neuroepithelia of the inner ear. The molecular analysis of hearing loss mutations in the mouse is an ideal way of gaining access to such genes. This proposal focuses on one particular mouse mutation called Ames Waltzer (av). The av is a recessive mutation, which causes deafness and vestibular dysfunction associated with degeneration of the inner ear neuroepithelia. We identified the gene that harbors the av mutation, Pcdh15 (Protocadherin 15). Sequence analysis showed that Pcdh15 is a novel member of the cadherin super family. We recently showed that mutation in the human homologue of mouse Pcdh15 causes Usher syndrome type 1F and thus, establishing av mouse as a model for deafness in USH1F. Protocadherins represent a large family of non-classic cadherins that are structurally and functionally divergent from the classic cadherins. In higher vertebrates, based on expression data, protocadherins are thought to be involved in a variety of functions, including neural development, neural circuit formation, and formation of the synapse. The role of protocadherins in the peripheral sensory apparatus, such as the inner ear, is unknown. Ames waltzer mice provide genetic evidence that a protocadherins is required for inner ear development and function. In the proposed study, we plan to determine the function of Pcdh15 in the normal development of the inner ear neuroepithelia. Specifically, we will study temporal and spatial expression patterns of Pcdh15 to understand the role of this gene in the normal structure and function of the inner ear. By using in situ hybridization and immunohistochemical methods, we plan to determine the primary site of action of the gene product during the course of inner ear development. We also plan to conduct detailed morphological analysis (using electron microscopy) to correlate ultrastructure and the nature of the mutation in the different alleles of av. Additionally, we also plan to identify proteins that interact with the protein encoded by Pcdh15. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MOLECULAR GENETICS OF NON-SYNDROMIC DEAFNESS Principal Investigator & Institution: Liu, Xue Z.; Otolaryngology; University of Miami Box 248293 Coral Gables, Fl 33124 Timing: Fiscal Year 2001; Project Start 15-SEP-2001; Project End 31-AUG-2006 Summary: (Investigator's abstract): The goal of this proposal is to identify molecular basis of non-syndromic deafness by using a multidisciplinary Approach in several unique resources of patients/families from Far Eastern populations. We have a large

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and expanding collection of genomic DNA from patients/families with non-syndromic deafness from China and Japan First, because of genetic heterogeneity, large multigenerational families and consanguineous families have been selected for study that are independently capable of yielding evidence of for linkage. We will then seek to identify and ultimately clone the relevant genes by the positional candidate gene approach. Second, a novel sequential screening strategy will be used to identify new genes for deafness in probands from multiplex families. Candidate genes to be screened will include the human orthologs of murine genes for deafness and other members of gene families in which some are known to be the cause of deafness. The research will yield important information o ethnic differences in the frequency and distribution of mutations at currently recognized genes for non-syndromic deafness. Using available information on outcome variables such as age of onset, audiologic findings, and the mating structure of the deaf population, these data will permit a search for clinically relevant genotype-phenotype correlations and a clearer understanding of the cause for secular changes in the frequency of specific forms o genetic deafness. Finally, we will use yeast 2-hybrid systems and the immunoaffinity chromatography to search for proteins that interact with the products of the MYO7A and USH1C genes. The identification of such interactions could lead to identifying potential modifiers and exciting new therapeutic approaches to attenuate the specific effects of mutations on the cochlear. The proposed work will identify the new gene (s) for non-syndromic deafness and enable us to understand better cellular and molecular basis of genetic deafness. Our studies will develop a more comprehensive picture of the involvement of genes in nonsyndromic deafness to gain further insight into the function of these genes in the inner ear. This knowledge is an essential prerequisite to the development and provision of molecular diagnostic services for families with non-syndromic deafness. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR MECHANISMS IN HEREDITARY NEPHRITIS Principal Investigator & Institution: Hudson, Billy G.; Chairman; University of Kansas Medical Center Msn 1039 Kansas City, Ks 66160 Timing: Fiscal Year 2001; Project Start 01-JUN-2001; Project End 31-MAY-2002 Summary: Alport Syndrome is a hereditary disorder characterized by progressive nephropathy which is frequently associated with sensorineural deafness and ocular abnormalities. The nephropathy has been linked to a structural abnormality in the glomerular basement membrane (GBM) which is caused by mutations in the gene encoding the alpha3, alpha4 and alpha 5 chains of type IV collagen. The most common form is X-linked, in which over 200 mutations have been found in the COL4A5 gene encoding the alpha5 chain. These mutations interfere with the formation of the alpha3(IV)/alpha4(IV)/alpha5(IV) supramolecular network of type IV collagen. Most male patients have near normal kidney function at birth, which deteriorates over time leading to end-stage renal disease by mechanisms that are not understood. The central thrust of this proposal is to test the hypothesis that the progression to end-stage renal disease in X -linked hereditary nephritis evolves from a congenital malformation of the glomerular basement membrane (GBM) which involves COL4A5 mutations that arrest a developmental switch from the immature alpha1(IV)/alpha2(IV) network to the nature alpha3(IV)/alpha4(IV)/alpha5(IV) network, and the persistence of this immature network predisposes the GBM to proteolytic degradation. The cornerstone of the research plan is a use of the canine X-linked model of the human disease in which the COL4A5 gene mutation is a premature stop codon. The specific aims are: Aim 1: Determine the nature and timing of the switch from immature to mature GBM in

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normal dog kidney in comparison to affected male dogs. Aim 2: Determine which glomerular cells synthesize the alpha3(IV), alpha4(IV) chains. Aim 3: Examine the relationship between the expression of the alpha3(IV), alpha4(IV) and alpha5(IV) chains. Aim 4: Determine the temporal relationship at both the message nd protein levels of the expression of the alpha1(IV)-alpha6(IV) chains in normal dogs compared to affected male dogs during progression of their disease. Aim 5: Determine the susceptibility of type IV collage of GBM to proteolytic degradation in normal and affected male dogs. The achievement of these aims requires application of the techniques of molecular biology, biochemistry, immunochemistry and cell biology. It is anticipated that the achievement of the aims will yield new insights into the mechanisms underlying the pathogenesis of Alport Syndrome. An understanding of these mechanisms is fundamental to the development of therapeutic measures to correct the disorder by gene therapy or to delay progression to end stage renal disease. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR MECHANISMS OF INNER EAR FUNCTION Principal Investigator & Institution: Heller, Stefan; Massachusetts Eye and Ear Infirmary 243 Charles St Boston, Ma 02114 Timing: Fiscal Year 2001; Project Start 01-SEP-2001; Project End 31-JUL-2006 Summary: Normal hearing and balance critically depend on the function of mechanosensitive receptors. In the inner ear, these receptors are involved in at least two fundamental processes, transduction by the hair cell stereocilia and regulation of the osmotic pressure of endolymphatic fluid in the scala media. We have recently identified an ion channel that is activated by exposure to osmotic stress and direct mechanical stimulation. This novel receptor, vanilloid receptor-related osmotically activated channel (VR-OAC), is expressed by inner ear hair cells and marginal cells of the stria vascularis. The Vroac gene is a positional candidate for the human deafness disorder DFNA25 and the deaf murine mutant Bronx Waltzer. We hypothesize that it is involved in mechano-reception by hair cells, measurement of the osmotic pressure of endolymphatic fluid, or both. Our long-term goal is to understand the physiological role of VR- OAC and its associated proteins in inner ear function. We will address this question through the parallel accomplishment of four specific aims. We have preliminary evidence that a second mechanoreceptive ion channel, related to VR-OAC, is expressed in the inner ear. We plan to search for additional VR-OAC-like channels (Aim 1). In Aim 2, we will determine the cellular and subcellular localization of VROAC and any other related channel that we identify in the inner ear via established methods of in situ hybridization and immunocytochemistry. Because our previous results implicate an essential interaction of VR-OAC with other intracellular proteins, we plan to identify these proteins utilizing the yeast two-hybrid screening system (Aim 3), an approach we have used previously to identify interaction partners of inner ear proteins. In Aim 4, we address the physiological relevance of heteromeric channels formed between VR-OAC and related channels, including the one we already have found. VR- OAC is currently the only mechanoreceptive ion channel that can be assessed by expression in a heterologous system, and we plan to physiologically characterize heteromultimeric channels using calcium imaging and whole cell recordings. We will also test VR- OAC's in vivo role by creating a mouse line carrying a null mutation. Phenotypic assessment of the resulting animals will provide fundamental clues as to the in vivo relevance of VR-OAC for inner ear mechanosensation, or osmoregulation, or both. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MOUSE DEAFNESS AND STUDY OF A MOUSE DEAFNESS GENE Principal Investigator & Institution: Kohrman, David C.; Assistant Professor; Otolaryngology; University of Michigan at Ann Arbor 3003 South State, Room 1040 Ann Arbor, Mi 481091274 Timing: Fiscal Year 2001; Project Start 01-APR-2001; Project End 31-MAR-2006 Summary: (Adapted from applicant's abstract): The primary objective of this proposal is the identification and characterization of the gene responsible for inherited deafness in the mouse mutant known as 'spinner'. Mutation of this gene results in sensorineural hearing loss similar to that found in many human nonsyndromic deafness disorders. The approaches described will attempt to correlate effects at the single gene level with those occurring at the cellular level and the level of the intact cochlea. A positional cloning strategy will be used to identify the affected gene. Gene localization information will be derived from an existing high resolution genetic cross and a set of genomic DNA clones that span the candidate region. Positional candidate genes mapped to this region in mouse, and to the region of conserved linkage in humans, will be evaluated for mutation in spinner mice. To further narrow the candidate region, genomic DNA clones from the candidate region will be microinjected into sr/sr zygotes. DNA clones that contain a functional version of the normal gene are expected to produce phenotypic correction in the resulting transgenic progeny. This DNA will be directly screened for the presence of the affected gene. The biological role of the spinner gene in the cochlea will be examined using several approaches. High resolution phenotypic analysis of affected mice will be performed to identify early defects in the cochlea that result from mutation of the spinner gene. Sensory cell function in the cochlea will be assessed by measurement of evoked responses in spinner mice. Ultrastructural and immunocytochemical analyses will be performed to detail the degenerative process in the mutant cochlea. Database analysis of the gene's primary sequence will be used to identify related genes and protein motifs that may provide insight into gene function. The expression pattern of the spinner gene, and the subcellular localization of its encoded protein, will be determined. Based upon comparative genetic data, the human version of the spinner gene is a positional candidate for the gene affected in a nonsyndromic deafness disorder, DFNB6. The human gene will be directly evaluated for mutations in individuals with inherited defects at the DFNB6 locus. This project will result in the identification of a critical gene in the mammalian inner ear, provide the basis for a model of this gene's role in the cochlea, and investigate the involvement of the gene in human nonsyndromic hearing loss. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MURINE MODELS OF HYPOTHYROIDISM AND CONGENITAL DEAFNESS Principal Investigator & Institution: Walsh, Edward J.; Professor; Director; Boys Town National Research Hospital 555 N 30Th St Omaha, Ne 68131 Timing: Fiscal Year 2001; Project Start 24-SEP-2001; Project End 31-AUG-2006 Summary: (provided by applicant): The overall, long-term goal of the research proposed in this application is to determine the anatomical and physiological basis of peripheral auditory anomalies associated with congenital hypothyroidism and to initiate an investigation into the molecular nature of associated deficits. In doing so, we hope to expand our understanding of thyroid hormone's role in the normal development of the inner ear and extend our understanding of the auditory consequences of hypothyroidism by considering the relative contribution that discrete inner ear tissues

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and structures make in the pathogenesis of the disease. The Tshrhyt mutant mouse is ideally suited to meet the needs of the proposed research and will serve as the primary model of hypothyroidism in the proposed study. We also propose to study normal BALB/c mice rendered hypothyroid through the actions of the anti-thyroid drugs, propylthiouracil (PTU) and methimazole (MMI), in an effort to establish a model of hypothyroidism-induced otopathology that will allow us to clarify differences reported in the literature regarding the effects of thyroid hormone deficiency on the auditory periphery, as well as the capacity of thyroid hormone to restore function in diseased animals. The chief hypothesis being tested in this proposal is that abnormal cochlear amplification represents an enduring defect of hypothyroidism-induced otopathology and that normal passive transduction is acquired developmentally over a protracted time course. In that context, in addition to experiments designed to address the primary question directly using in vivo recordings (ABR, CM and DPOAE), as well as in vitro recordings from outer hair cells, we plan to study the morphological and the functional properties of the tectorial membrane, along with its chemical composition, to determine its role in the pathogenesis of the disease. The role of other passive aspects of transduction (e.g., endocochlear potential) will be assessed directly. We also plan to take the first step in a study aimed at determining the molecular and genetic basis of hypothyroidism, using RT-PCR and in situ hybridization to study the expression of transcripts that may affect the expression of cochlear amplification. Finally, we propose to explore the intriguing possibility that the efferent OC system may influence the development of peripheral auditory function. If the main hypotheses proposed here are affirmed, the existing model of auditory system pathology induced by hypothyroidism will be fundamentally revised. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MYOSIN 15 GENETICS, PATHOLOGY AND THERAPEUTIC POTENTIAL Principal Investigator & Institution: Camper, Sally A.; Professor; Human Genetics; University of Michigan at Ann Arbor 3003 South State, Room 1040 Ann Arbor, Mi 481091274 Timing: Fiscal Year 2001; Project Start 01-AUG-2001; Project End 31-JUL-2006 Summary: Hereditary inner ear disease is prevalent and has significant implications for quality of life. There is currently no available clinical cure for hereditary inner ear disease. The mouse serves as an ideal mammalian model for understanding genetic inner ear disease and for developing therapeutic measures. Mouse models have facilitated the discovery of genes that underlie hereditary disease in humans, have made it possible to study the role of these genes in inner ear development and function, and hold great promise as models for developing treatments for hereditary inner ear disease. This grant application builds on our discovery that mutations in the unconventional myosin gene, Myo15, are responsible for profound congenital deafness in humans with DFNB3 and in two spontaneous mouse mutants with profound recessive deafness, shaker 2 and shaker 2J. We propose to use these two mouse models of DFNB3 to establish the structure and functional properties of this large myosin, the requirement for expression in utero and early postnatal life, and the developmental basis for pathology in affected individuals. As each of these goals are accomplished we will move closer toward the ultimate objective of gene therapy for congenital deafness in children and expand our molecular understanding of the basic hearing process. In the first aim, we will rigorously test the long-term functional and structural outcome of the phenotypic correction of deafness in shaker 2 mice that we accomplished by

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complementation with a BAC transgene. The second aim tests the hypothesis that MYOSIN XV-deficient hair cells fail to detach from the basement membrane during development, leading to cellular pathology. The third aim examines whether loss of Myo15 function is partially compensated by the function of other myosins. The fourth aim tests whether the unusual N-terminal third of the MYOXV protein is important for function using transgenic mice. Finally the feasibility of gene therapy for DFNB3 in newborns or young children will be assessed in the last aim using inducible expression of Myo15 transgenes in young, postnatal shaker 2 mice. Our investigative team has a track record for accomplishments resulting from cross disciplinary collaboration, which has brought expertise in otolaryngology and morphology together with expertise in molecular and developmental genetics. Our interdisciplinary approach is essential to fully exploit the animal models that will be the basis for developing and testing therapy for hereditary inner ear disease. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MYOSIN-X: A NOVEL MYOSIN WITH PH DOMAINS Principal Investigator & Institution: Cheney, Richard E.; Assistant Professor; Cellular/Molecular Physiology; University of North Carolina Chapel Hill Office of Sponsored Research Chapel Hill, Nc 27599 Timing: Fiscal Year 2002; Project Start 01-JUN-1997; Project End 31-MAY-2007 Summary: Myosin-X is an unconventional myosin of the MyTH-FERM super class that is broadly expressed in vertebrate tissues. Although little is known about the functions of the MyTH-FERM myosins, mutations in one member of this group are the leading cause of hereditary deaf-blindness in children. We recently discovered that myosin-X undergoes a novel form of motility within filopodia, and we hypothesize that myosin-X is a component of a widespread but previously uncharacterized system for intracellular transport on actin-rich structures such as filopodia. Myosin-X also exhibits a remarkable localization to the tips of filopodia and overexpressing it leads to increased number and length of filopodia, suggesting that myosin-X functions in the largely unknown pathways regulating filopodial dynamics. Myosin-X also binds to integrins and one of its light chains is known to be dramatically down regulated in many tumors. Together these data strongly suggest that myosin-X and the novel form of motility associated with it play fundamental but largely unexplored roles in the basic cell biology underlying human health and disease. We propose to: I. Determine the fundamental properties, mechanisms, and regulation of this novel form of motility. II. Identify the structures and molecular cargo(s) transported by intrafilopodial motility. III. Use myosin-X as a marker to identify the components of a putative filopodial tip complex and determine if myosin-X is a component of signaling pathways that regulate filopodial dynamics. IV. Determine the functions of myosin-X in key cell biological processes such as phagocytosis, filopodial dynamics, and cell crawling. This research will determine the fundamental properties of a novel and previously uncharacterized form of motility that has critical implications for a host of important cell biological processes including integrin function, cell adhesion, filopodial dynamics, macrophage function, and nerve regrowth. The proposed research will thus answer critical questions about the basic cell biology underlying many human diseases including cancer, hereditary deafness, retinitis pigmentosa, and nerve injury. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: NICOTINIC RECEPTORS IN COCHLEAR HAIR CELL PHYSIOLOGY Principal Investigator & Institution: Fuchs, Paul A.; Professor; Otolaryn & Head & Neck Surgery; Johns Hopkins University 3400 N Charles St Baltimore, Md 21218 Timing: Fiscal Year 2003; Project Start 01-APR-2003; Project End 31-MAR-2006 Summary: (provided by applicant) Recent years have seen important developments in the study of hearing, especially the emergence of molecular genetics to probe the underlying mechanisms of hearing loss. It is estimated that 1 in every 1,000 newborns is profoundly deaf, while nearly 1 in 20 has a significant hearing impairment. In more than half of these cases, the cause is genetic. As of February 2002, twenty-nine specific genes have been associated with different forms of nonsyndromic human deafness. In addition, many other gene products involved in normal cochlear function have been identified. The foreign collaborator (Elgoyhen) cloned and characterized two novel nicotinic receptor genes (alpha 9 and alpha 10) that are expressed in cochlear hair cells. Alpha 9 and 10 encode receptor proteins that mediate the effect of acetylcholine (ACh) released by efferent neurons onto cochlear hair cells. The Principal Investigator (Fuchs) showed that calcium entry through the ACh receptor leads to hair cell hyperpolarization, reducing transmitter release to cause a loss of tuning and sensitivity in the auditory nerve fibers. This FIRCA proposal brings together the expertise of the Fuchs and Elgoyhen laboratories to conduct a series of electrophysiological and molecular genetic experiments to elucidate the properties and function of these cholinergic receptors in hair cell physiology. The long-term goal of this proposal is to define the physiological role of the hair cell's cholinergic receptor. In addition, it is expected that the results obtained will contribute to our understanding of the role of efferent cholinergic input in the genesis and potential treatment of hearing impairment produced by loud sound or ototoxic drugs. The immediate aims of this application are three-fold: first, the pharmacological and physiological comparison of recombinant alpha9/alpha10 receptors expressed in Xenopus laevis oocytes with native cholinergic receptors of inner and outer hair cells in acute cochlear explants; second, the characterization of the ontogeny of cholinergic responses in inner hair cells and third, the physiological analysis of synaptic contacts onto inner and outer hair cells in mice with genetic modifications in the Acra9 gene, namely the alpha9 null mutant mouse and a knock-in mouse bearing a gain of function mutation. This research will be done primarily in Argentina at INGEBI (National Research Council) in collaboration with Ana Belen Elgoyhen as an extension of NIH grant #R01 DC01508. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: PHONOLOGICAL SYSTEMS OF PEDIATRIC COCHLEAR IMPLANT USERS Principal Investigator & Institution: Chin, Steven B.; Assistant Scientist; OtolaryngologyHead and Neck Surgery; Indiana Univ-Purdue Univ at Indianapolis 620 Union Drive, Room 618 Indianapolis, in 462025167 Timing: Fiscal Year 2003; Project Start 01-SEP-2003; Project End 31-AUG-2008 Summary: (provided by applicant): The long-term goal of this project is to increase our knowledge and understanding of the phonological systems acquired by profoundly deaf children who have used cochlear implants for at least five years. We propose to apply methods from theoretical and clinical linguistics to produce detailed analyses of the phonological systems that underlie speech production by deaf children after cochlear implantation. These detailed linguistic analyses of sound segment inventories, constraints on sequencing, variation in phonetic realizations of morphemes, and

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implementation of phonological rules will then be compared to both all phonological systems in general and the phonological systems of deaf children who use conventional hearing aids, children who have normal hearing, and fully developed English. Longitudinal analyses of the phonological systems of children who use cochlear implants will identify possible directions and extent of changes in those systems and provide information regarding constraints on changes over time. Subtypes of phonological systems, based on structural similarities, will be identified and related to speech perception abilities and demographic factors such as age at implantation and communication mode to identify specific factors that may affect how phonological systems are structured. This research project will thus address the critical question of how cochlear implants shape the ultimate linguistic systems of children who use them. Findings will provide valuable new empirical and theoretical information about phonological development, which should have important implications for device design, the development and implementation of new intervention programs, and choices in educational placement. Finally, the proposed project on phonological systems of deaf children following cochlear implantation will provide new insights into the ability of the human brain to adapt to electrical hearing as a catalyst for the development of structured linguistic systems underlying spoken language development. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: PORCINE SUBMAXILLARY MUCIN AND HUMAN NORRIN Principal Investigator & Institution: Hill, Robert L.; Professor and Chairman; Biochemistry; Duke University Durham, Nc 27706 Timing: Fiscal Year 2001; Project Start 01-SEP-1978; Project End 31-DEC-2002 Summary: The structure/function relationships of two related proteins will be examined. Porcine submaxillary mucin is a glycoprotein containing about 67 percent by weight carbohydrate. Mucins lubricate the respiratory, gastrointestinal and urogenital tracts and protect epithelial cells from dehydration and injury. Much of porcine submaxillary mucin structure has been elucidated, but not all, including how the monomeric mucin molecules assemble to form dimeric and multimeric mucins that are the mature, functional molecules. The half-cystines that form the inter- and intrachain disulfide bonds in dimers and multimers will be determined. Particular attention will be given to the structural motif CGLCG and its role in assembly of multimeric mucin. These studies will utilize site-directed mutagenesis and expression of mutant constructs in animal cells in culture. Similar studies will be performed with norrin, a 133 residue human protein that is defective in Norrie disease, an X-linked, recessive human neurological disorder characterized by blindness, deafness and mental retardation. This protein is very similar structurally to the domain in some mucins that forms disulfidelinked multimers. The half-cystines in norrin that form inter- and intrachain disulfide bonds will be determined by the same methods used with submaxillary mucin. Hopefully, these studies will reveal how the disulfide-rich domains in proteins in which they occur lead to oligomer formation. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: PREVENTING NIHL BY ENHANCING GLUTATHIONE PATHWAYS Principal Investigator & Institution: Kopke, Richard D.; Co-Director; U.S. Naval Medical Center (San Diego) 34800 Bob Wilson Dr (Cid) San Diego, Ca 92134 Timing: Fiscal Year 2001; Project Start 30-SEP-2001; Project End 31-JUL-2003

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Summary: (provided by applicant): The primary objective of these studies is to test and develop pharmacologic agents to protect against noise-induced hearing loss (NIHL). The long-term objective is to develop agents for oral administration in clinical populations. The primary hypothesis is that pharmacologically optimizing the glutathione pathway will ameliorate or prevent NIHL and that this protection can be obtained by either injection or by oral administration of carefully selected glutathione enhancing agents. The secondary hypothesis is that noise exposure will alter the glutathione pathway as measured by the ratio of reduced to oxidized glutathione and the affiliated enzymes glutathione reductase (GR) and glutathione peroxidase (GSH-Px) during the first 6 hours of noise exposure and that these changes will correlate with the level of lipid peroxidation as measured by malondialdehyde (MDA) levels. The first specific aim is to determine the time course of any changes in the cochlear levels of reduced glutathione, and the ratio of reduced (GSH) vs. oxidized (GSSG) glutathione prior to noise exposure, and.5, 2, 4, and 6 hours after onset of noise exposure. This aim will be accomplished by measuring cochlear glutathione, both the reduced GSH and oxidized GSSG, in animals sacrificed at those times. The critical measures will be the reduced GSH, and the GSH/GSSG ratio, a measure of oxidative stress. The second specific aim is to determine the time course of any changes in cochlear GR and GSH-Px, critical enzymes of the glutathione anti-oxidant pathway, at those same times. The third specific aim is to determine the time course of changes in cochlear lipid peroxidation at those same times. The second and third aims will be accomplished by measuring GR, GSH-Px, and MDA, a marker for cell membrane oxidative damage, in animals sacrificed at those times. The fourth specific aim is to determine the efficacy of 3 putative otoprotective agents in reducing noise induced cochlear damage with auditory brainstem response thresholds and outer hair cell counts as primary measures. Protection efficacy will also be measured by GSH/GSSG, GR, GSH-Px, and MDA measures. The fifth specific aim is to compare the efficacy of these same agents orally administered to the IP administration, utilizing the same outcome measures. These studies should lay the basis for clinical trials of oral agents to prevent NIHL. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: PROTEIN IMPORT INTO YEAST MITOCHONDRIA Principal Investigator & Institution: Jensen, Robert E.; Associate Professor; Cell Biology and Anatomy; Johns Hopkins University 3400 N Charles St Baltimore, Md 21218 Timing: Fiscal Year 2001; Project Start 01-JAN-1993; Project End 31-DEC-2004 Summary: (applicant's description): The long range goal of this research proposal is to understand how proteins of the mitochondrial inner membrane and intermembrane space mediate two events: translocation of proteins into the mitochondrial matrix, and protein sorting to the inner membrane. Mitochondria are essential organelles, whose function requires the import of hundreds of different proteins that are encoded in the nucleus, synthesized in the cytosol and imported into the organelle. Protein import is a multistep pathway which includes the binding of precursor proteins to surface receptors, translocation across one or both mitochondrial membranes, and folding and assembly of the imported protein inside the mitochondrion. To understand how these processes occur, we have isolated and are analyzing mutants in the yeast, Saccharomyces cerevisiae, that are defective in mitochondrial protein import. Many studies indicate that mitochondria in yeast import proteins into their mitochondria by a process virtually identical to the way mammalian mitochondria import proteins. Hence our studies with yeast will yield valuable information about mitochondria biogenesis in all organisms. Supporting this view, a human disease called Mohr-Tranebjaerg

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syndrome, a neurodegenerative disease characterized by deafness, dystonia , mental retardation and blindness, has recently been shown to result from a defect in mitochondrial protein import. The human mutation is in a gene encoding a protein called DDP, homologous to the yeast Tim8 protein. We anticipate that our future studies will continue to provide insights into the normal functions of mitochondria and their relationships to human disease. We have recently shown that the mitochondrial inner membrane contains two translocation complexes: the TIM23 complex, which is required to translocate precursors across the inner membrane into the matrix and the TIM22 complex, which is required for the insertion of polytopic proteins into the inner membrane. Tim54p, Tim22p and Tim18p comprise the membrane subunits of the TIM22 complex. In this proposal, we ask several questions aimed at understanding the mechanism by which proteins are inserted into the inner membrane. (1) What other proteins comprise the TIM22 complex? (2) Does Tim18p play a direct role in import? (3) Does Tim54p play a docking role in the insertion process? (4) What is the precise pathway that proteins traverse during their import and insertion into the inner membrane? (5) Can inner membrane protein insertion be reconstituted using purified components? Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: REGULATING DORSOVENTRAL POLARITY WITHIN THE INNER EAR Principal Investigator & Institution: Epstein, Douglas J.; Genetics; University of Pennsylvania 3451 Walnut Street Philadelphia, Pa 19104 Timing: Fiscal Year 2003; Project Start 01-AUG-2003; Project End 31-JUL-2008 Summary: (provided by applicant): The auditory and vestibular structures of the inner ear mediate our senses of hearing and balance, respectively. Recent progress has been made in identifying some of the causes of hereditary forms of deafness and vestibular disease in humans; however, a detailed understanding of the genetic pathways coordinating inner ear development remains limited. By identifying the genetic networks regulating inner ear morphogenesis, our understanding of the association between otic development and disease should improve. The principal components for hearing (the cochlea) and balance (the semicircular canals, utricle and saccule) are formed from ventral and dorsal outgrowths, respectively, of a common bilateral structure, the otocyst. Organization of the inner ear into auditory and vestibular components is dependent on localized patterns of gene expression within the otic vesicle. Surrounding tissues are known to influence compartmentalization of the otic vesicle, yet the participating signals remain unclear. The notochord and floor plate are sources of the secreted protein Sonic hedgehog (Shh) that functions in both short and long range signaling events to promote growth and differentiation of progenitor cells in the ventral neural tube and paraxial structures. In the absence of Shh, ventral otic derivatives including the cochlear duct and cochleovestibular ganglia fail to develop. The origin of the inner ear defects in Shh -/- embryos can be attributed to alterations in the expression of a number of genes previously implicated in the specification of cochlear, neuronal and chondrogenic lineages. Although the effects of Shh signaling are detected in the otic epithelium, adjacent tissues including the periotic mesenchyme and neural tube are also targets of Shh action. This brings into question the relative contribution of Shh signaling in each of the tissues impacting on inner ear development. Experiments outlined in this proposal are aimed at elucidating the mechanism by which Shh specifies auditory cell fates in the ventral otocyst. The temporal and spatial requirements of Hedgehog (Hh) signaling in inner ear development will be addressed

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by the conditional inactivation of Smoothened, an essential transducer of all Hh signals, in each of the tissues impacting on the otic vesicle. Experiments to identity the downstream effectors of Shh signaling in otic development are also proposed. Finally, despite preliminary insights into how auditory cell fates are specified, little is known of the extrinsic cues that establish vestibular (dorsal) structures in the otic vesicle. Introduction of specific pathway inhibitors into the dorsal otocyst using transgenic approaches as well as the assessment of mouse mutants in candidate dorsalizing factors should contribute towards our overall understanding of how polarity is established along the dorsoventral axis of the inner ear. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: REGULATION OF MICROTUBULES BY RHO GTPASES Principal Investigator & Institution: Gundersen, Gregg G.; Associate Professor; Anatomy and Cell Biology; Columbia University Health Sciences New York, Ny 10032 Timing: Fiscal Year 2001; Project Start 01-APR-2001; Project End 31-MAR-2005 Summary: (provided by applicant) Microtubules (MTs) are ubiquitous elements of the cytoskeleton that are essential for the generation of cellular asymmetry during development and differentiation. In undifferentiated cells, most MTs are highly dynamic, whereas in polarizing or differentiated cells, certain MTs become stabilized and organized into specific arrays that are necessary for supporting polarized cell function. In many cases, alterations in proteins that regulate the formation of these MT arrays result in disease. Thus, lissencephaly is caused by alterations in the Lisl protein, which affects dynein functions in cell division and polarity. The nonsyndromic deafness syndrome, DFNA1, is cause by mutations in human Dia, a protein we have found regulates microtubule stabilization in fibroblasts. Our overall goal in this project is to determine the molecular mechanisms controlling MT stabilization and polarization and to explore their significance for cell function using the simple model system of in vitro wound healing. We have found that there are two distinct molecular pathways that regulate MTs during cell polarization and migration into wounds. These pathways resemble those described in yeast, where they function to position the nucleus and spindle. This suggests that MT-based polarization pathways have been conserved from yeast to mammals. One pathway involves the stabilization of MT oriented toward the wound and is regulated by the small GTPase Rho and its downstream effector mDia. We will explore how mDia regulates MT stabilization during in vitro wound healing by identifying the domain(s) of mDia important for MT stabilization and by identifying downstream effectors of mDia. Human Dia is mutated in DFNA1, and we will test the possibility that the mutation in DFNA1 alters MT stabilization. The second -pathway involves the reorientation of the MT organizing center (MTOC) toward the leading edge of cells adjacent to the wound margin. We have found that MTOC reorientation is regulated by CDC42 and is controlled independently of MT stabilization. We will study dynein and other potential downstream effectors to understand how CDC42 regulates MTOC reorientation. Lisl protein interferes with dynein function when overexpressed and we will test whether Lis1 overexpression effects MTOC orientation. We will study whether inhibition of MT stabilization or MTOC reorientation interferes with the polarization of cells, the migration of the cells into the wound and other wound responses. These studies should establish the molecular pathways by which MTs are regulated in cells and will contribute to our understanding of how cell polarity is established by MTs in all eukaryotic cells. These studies will also contribute to our understand of the basic molecular pathways involved in wound responses and will help

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explore the basic cell biological consequences of mutations in human Dia that causes deafness. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: REORGANIZATION OF VISUAL FUNCTIONS AFTER EARLY DEAFNESS Principal Investigator & Institution: Bavelier, Daphne I.; Associate Professor; Brain and Cognitive Sciences; University of Rochester Orpa - Rc Box 270140 Rochester, Ny 14627 Timing: Fiscal Year 2001; Project Start 01-FEB-2001; Project End 31-JAN-2006 Summary: (Adapted from Applicant's abstract): The goal of this research is to assess and separate the effect of deafness and of acquisition of a sign language on the organization of the visual system. Visual abilities in hearing individuals will be compared with those of deaf signers. To separately document the role of deafness from that of signing, deaf with minimal exposure to sign and hearing individuals who are native signers will also be included. Indeed, the existing literature indicates that deafness and acquisition of American Sign Language have different and separate effects on the reorganization of visual functions. The aspects of the visual system that have been modified will be first determined by comparing behavioral indices of visual processes in these four populations. Modifications in brain organization that accompany these behavioral differences will be then characterized with functional magnetic resonance imaging (fMRI). The existing literature indicates that congenital auditory deprivation is associated with specific enhancement of behavioral performance and neural activity in response to moving stimuli and visual stimuli in the periphery. Since this type of information is predominantly represented along the dorsal visual pathway, it has been proposed that early deafness affects predominantly the dorsal visual pathway. This work will test the hypothesis that dorsal visual functions are altered after early deafness, by studying and comparing motion processing and spatial localization in deaf and hearing. The contribution of deafness and signing will be separately assessed by looking at deaf with minimal signing experience and hearing who are native signers. The second goal of this work is to test whether plastic changes are indeed specific to the dorsal pathway as has been hypothesized in the literature or if plastic changes can be induced by early dearness or signing in the other main visual pathway, i.e. the ventral pathway. For that purpose, face processing and handshape processing will be compared in deaf and hearing as a function of their signing ability. These experiments will provide new information about which sub-systems within vision are most altered by early auditory deprivation and/or early acquisition of signs, and will help to characterize how these changes are implemented at the cortical level. Since these studies will determine the aspects of visual perception that are most likely to reorganize when the environment is altered early on, these results will also carry implications for the kind of training that would optimize visual and attentional skills in children. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: SPEECH AND LANGUAGE IN CHILDREN WITH COCHLEAR IMPLANTS Principal Investigator & Institution: Miyamoto, Richard T.; Professor & Chairman; Otolaryngology-Head and Neck Surgery; Indiana Univ-Purdue Univ at Indianapolis 620 Union Drive, Room 618 Indianapolis, in 462025167 Timing: Fiscal Year 2001; Project Start 01-JUL-1987; Project End 31-MAR-2003

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Summary: This proposal requests continued support for a program of research on perlingually deaf children with cochlear implants. The long-term goal of the project is to assess the effect of cochlear implants (CIs) on the development of speech production and language. In particular, we will compare the speech and language abilities of CI users to those of hearing aid (HA) users to determine the potential benefits of implantation compared to HA use. We will also compare the performance of children implanted early (below age 3-4) to those implanted later in life, to investigate whether early implantation may limit the negative consequences of auditory deprivation in the development of language and speech production. We will also study the influence of communication mode on speech and language development by comparing the performance of users of oral communication with users of total communication. We also plan to assess whether the latest clinically available processing strategies will allow children to reach superior levels of speech and language development, compared to older stimulation strategies. Finally, the proposed studies will provide information that will improve our basic understanding about the relation between the development of speech perception and speech production and about the development of language in children receiving impoverished auditory input. These goals will be achieved by a longitudinal assessment and description of the speech production and language abilities of a large group of deaf children, and by more intensive studies that focus on a smaller number of subjects. The proposed research extends our earlier work on speech production in a number of new directions. Project I will study the acoustic and physiological characteristics of the speech of implanted children and normal-hearing control subjects. Project II will examine the acquisition of English phonology by implanted children. Project III will assess the longitudinal changes in speech intelligibility by children with CIs and control groups of profoundly deaf children with hearing aids. Finally, Project IV will investigate the language development of implanted children and compare their performance to unimplanted deaf controls and normal-hearing children. The four projects of the proposed research address the efficacy of CIs in facilitating the development of specific abilities that all children must acquire to develop spoken language: the ability to control the physiological mechanisms used in speech production, to organize speech sounds into a coherent phonological system, to produce intelligible speech, and to control the creative, meaningful aspects of language. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: SPONTANEOUS SIGN SYSTEMS IN FOUR CULTURES Principal Investigator & Institution: Goldin-Meadow, Susan J.; Irving B. Harris Professor of Psychology; Psychology; University of Chicago 5801 S Ellis Ave Chicago, Il 60637 Timing: Fiscal Year 2001; Project Start 01-SEP-1988; Project End 31-JUL-2006 Summary: (provided by applicant): What might it mean to say that language is innate or, alternatively, that language must be learned? This proposal attempts to articulate these questions in terms that lend themselves to experimental investigation, asking which aspects of language development are so over-determined that they will appear even under learning conditions that vary widely from the norm. The participants are deaf children whose hearing parents have not yet exposed them to sign language, and whose hearing losses are so profound as to preclude acquisition of spoken language. Despite their lack of an accessible conventional language model, these children do communicate, developing gesture systems that are structured in language-like ways. Moreover, Chinese deaf children of hearing parents develop the same gesture systems as their American counterparts, suggesting that the deaf children's gesture systems are resilient, not only to the absence of a conventional language model, but also to cultural

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variation. Where do these deaf children's gesture systems come from? One candidate is the gestures hearing adults produce as they talk. Indeed, the gestures of Mandarin speakers are similar in type to those of English speakers. However, the gestures adults use when speaking languages typologically distinct from Mandarin and English - verbframed languages such as Spanish or Turkish - differ strikingly from the gestures used by speakers of satellite-framed languages such as English or Mandarin. These four cultures thus offer an opportunity to examine effects of hearing speakers' gestures on the gesture systems developed by deaf children. If deaf children in all four cultures develop gesture systems with the same structure despite differences in the gestures they see, the children themselves must be bringing strong biases to the communication situation. The project has four aims: (1) To describe how adult speakers of Spanish or Turkish gesture differently from adult speakers of Mandarin or English. (2) To determine whether gesture systems created by Spanish and Turkish deaf children of hearing parents are structured as are gesture systems created by Chinese and American deaf children despite differences in the gestural models they see. (3) To describe how Spanish, Turkish, Chinese and American hearing children use gestures differently from deaf children in these same cultures even though they see hearing adults using the same gestures. (4) To determine whether Spanish, Turkish, Chinese and American hearing adults, when asked to use gesture as their sole means of communication, produce gestures structured like the deaf child's. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: STIMULI PROMOTING SURVIVAL OF SPIRAL GANGLION NEURONS Principal Investigator & Institution: Green, Steven H.; Associate Professor; Biology; University of Iowa Iowa City, Ia 52242 Timing: Fiscal Year 2002; Project Start 01-MAY-1996; Project End 31-MAY-2007 Summary: Spiral ganglion neurons (SGNs) die gradually following the loss of hair cells by the process of apoptosis. Electrical stimulation promotes the survival of such deafferented SGNs in vivo, raising the possibility of using electrical stimulation to maintain survival of SGNs in deaf individuals - in effect allowing cochlear implants to replace the trophic as well as the sensory function of hair cells. Our first goal is to provide a detailed timecourse of the key molecular events characteristic of apoptosis during the death of SGNs following deafening and describe how these events are affected by electrical stimulation. Rats will be deafened using ototoxin to destroy the hair cells and, during the approximately 100 day period over which SGNs die, the levels, the phosphorylation state and the subcellular localization of key representative apoptosis regulators and mediators will be determined immunohistochemically and biochemically. This will reveal at what stage(s) in the apoptotic process the SGNs exist following loss of hair cells, thereby identifying the time during which potential therapies for preventing SGN death would be most successfully applied. The second goal is to test the hypothesis implied by our previous studies: depolarization recruits three distinct kinase systems that independently and additively promote SGN survival, acting in distinct cellular compartments and on distinct substrates. Cyclic AMPdependent protein kinase and Ca2+/calmodulin-dependent protein kinase (CaMK) II, appear to function in the cytoplasm and phosphorylate mitochondrial apoptotic regulators, with CaMKII doing so by an indirect route involving tyrosine kinases. CaMKIV functions in the nucleus, phosphorylating the transcription factor CREB. To test this hypothesis, a lentiviral vector will be used to introduce genes encoding activated protein kinase mutants or kinase inhibitor proteins into SGNs, with these

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proteins restricted to specific subcellular locations, e.g., mitochondria or nucleus, by means of physiological targeting sequences. For these studies, we use the system we have developed, in which SGNs are electrically stimulated or depolarized in vitro to maintain their survival. In parallel, we will infuse pharmacological inhibitors or activators of these kinase systems into the cochleae of hearing or deafened rats, or deafened rats with implanted stimulating electrodes. These experiments will determine the extent to which these intracellular signals promote survival in vivo and verify that they mediate the survival-promoting effect of electrical stimulation in vivo as they do in vitro. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: STRUCTURE AND DYNAMICS OF CONNEXIN26 GAP JUNCTIONS Principal Investigator & Institution: Sosinsky, Gina E.; Professor; Neurosciences; University of California San Diego 9500 Gilman Dr, Dept. 0934 La Jolla, Ca 92093 Timing: Fiscal Year 2003; Project Start 01-AUG-2003; Project End 31-JUL-2007 Summary: (provided by applicant): Gap junctions serve an essential role in the passage of molecules from the cytoplasm of one cell to its neighbor in both functional and homeostatic capabilities. They are defined as clusters of closely packed intercellular membrane channels embedded in the plasma membranes of two adjoining cells. The channels are composed of two hexamers of a protein (connexon) from a family of integral membrane proteins known as connexins. Here, we focus on the structure and function of connexin26 (Cx26), the smallest of the family. Mutations in the DNA sequence can result in hereditary sensorineural deafness and account for between one third to one half of the cases of prelingual inherited deafness in Caucasian populations. We have isolated preparations of Cx26 gap junctions in pure and sufficient amounts for biochemical and structural studies. These 2D crystals are amenable to electron microscopy (EM) structure determination and conformational dynamics as revealed with atomic force microscopy (AFM) done under hydrated conditions. In SPECIFIC AIM 1, we will determine the structure of the Cx26 hemichannel beyond 10 Angstroms using state of the art cryo-EM and improvements on image processing procedures. This involves improving specimen preparation, imaging at either liquid nitrogen or liquid helium temperature and implementation of a combined single particle/ 2D crystallographic approach to circumvent imperfect crystal lattices. In SPECIFIC AIM 2, we will construct Cx26 wild type and mutant cell lines with a tetracysteine domain genetic tag to improve isolation with FlAsH ligand affinity bead purification, stably express these in HeLa cells or in baculovirus-infected Sf9 insect cells and isolate the gap junctions or connexons for structural analysis using the methods developed in Specific Aim 1. We will construct two Cx26 mutants (P97L and T135A), each containing a single point mutation in one of the transmembrane helices that changes the effective pore properties. These mutations should be reflected in conformational changes in the 3D structure. In SPECIFIC AIM 3, we will expand coordinated AFM/EM experiments for visualizing conformational changes due to treatments known to close or alter gap junction mediated communication. Preliminary AFM images have visualized conformational changes at submolecular resolution. We have chosen five treatments that known to induce closure of Cx26 channels or hemichannels and are physiologically relevant. Conformational changes identified by AFM imaging will be further imaged using EM. Each of these goals is intended to complement the others and lead to structural and physiological models of Cx26 germane to the entire connexin family. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: STRUCTURE OF DECODING SITE AND TELOMERASE RNA DOMAINS Principal Investigator & Institution: Rechkoblit, Olga A.; Sloan-Kettering Institute for Cancer Res New York, Ny 10021 Timing: Fiscal Year 2003; Project Start 01-AUG-2003; Project End 31-JUL-2005 Summary: (provided by applicant): RNA and RNA-protein interactions play a key role in many biological processes and diseases. Although, the majority of drugs target cellular, bacterial or viral proteins known to be associated with a particular human disease, some already existing highly efficient medications interact with RNA or RNAprotein complexes. A better understanding of RNA structure would provide access to design and optimization of new drugs targeting RNA. The emphasis of my research proposal is on the X-ray crystallographic determination of key RNA structures and their complexes. The proposal is subdivided into two major projects: antibiotic-human mitochondrial ribosomal decoding site recognition, and structure of the key domains of human telomerase RNA. The goal of the first project is to evaluate the structures of human mitochondrial ribosomal decoding site and aminoglycoside antibiotics induced deafness-associated mutant, both alone and in a complex with aminoglycosides. The available structural literature is focused on the prokaryotic decoding site and its complexes. The goal of the second project is to solve the structure of four crucial domains of human telomerase RNA and congenital dyskeratosis syndrome-associated mutants that, if successful, could provide deep insights into telomerase structure and function. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: STRUCTURE/FUNCTION ANALYSIS OF LOW PL CONNEXIN ISOFORMS Principal Investigator & Institution: Hertzberg, Elliot L.; Professor; Neuroscience; Yeshiva University 500 W 185Th St New York, Ny 10033 Timing: Fiscal Year 2002; Project Start 01-APR-2002; Project End 31-MAR-2004 Summary: (provided by applicant): Gap junctions are assemblages of cell-cell channels occurring in regions of cell contact. These channels permit direct intercellular communication by passive diffusion of low molecular weight hydrophilic molecules, including calcium, cyclic nucleotides, inositol phosphates and other signaling molecules. Several gap junction proteins, termed connexins, serve prominent roles in the nervous system. Among these, Connexin43 (Cx43) is expressed at high levels in astrocytes where they provide a mechanism of spatial buffering of ions and exert a modulatory influence on neuronal activity. Mutations of Cx32, found in myelin, lead to a progressive peripheral neuropathy termed X-linked Charcot-Marie-Tooth disease. Mutations in Cx26 underlie the most common forms of non-syndromic deafness. Interestingly, connexins are the only family of plasma membrane proteins that do not appear to be glycosylated. Preliminary studies of Cxs 43, 32 and 26 indicate that they are significantly more negatively charged than anticipated based upon their sequence and known covalent modifications. That these pI variants are indistinguishable by SDS-PAGE indicates that the molecular basis for acidic pI variants is of low molecular weight. The specific aims of this proposal are to (1) identify this modification and the altered amino acid residues and (2) determine its role in the assembly and functioning of gap junction channels. Our approach to determining the chemical basis of connexin will be biochemical, relying especially on mass spectrometry, and genetic engineering. The role of this modification will be assessed using pharmacology and site-direct mutagenesis.

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Identification of a low Mr anion covalently attached to connexins might provide the first target for pharmacological intervention in gap junction function. Knowledge of where charged residues exist will profoundly influence modeling studies of connexins and, likely, other membrane proteins, in which alterations of electrostatic interactions play a role in channel selectivity and gating. Many of the experiments will push experimental procedures for use in the study of membrane proteins. Establishing the basis of connexin charge alteration will introduce a new player in chemical modification of proteins and their properties. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: STRUCTURE/FUNCTION STUDIES OF GAP JUNCTIONS Principal Investigator & Institution: Bargiello, Thaddeus A.; Neuroscience; Yeshiva University 500 W 185Th St New York, Ny 10033 Timing: Fiscal Year 2001; Project Start 01-FEB-1992; Project End 31-JUL-2004 Summary: Gap junctions formed by Cx26 and Cx32 differ in molecular permeability and in sensitivity to transjunctional voltage. These differences will be exploited to define the molecular mechanisms that underlie voltage dependence and ionic fluxes of intercellular and membrane channels formed by proteins of the connexin gene family. In the long term, integration of data from biophysical, molecular genetic, NMR and computer modeling studies will lead to understanding of how conformational changes in this membrane protein and others dynamically relate to their function and how fixed charges modulate ion flux. In the proposed studies the pore-lining sequence of the Cx32 channel will be further defined and the atomic structure of the amino terminus of wild type and mutated Cx32 will be solved by high resolution NMR. The position of the voltage sensor and its relation to the transjunctional electric field will be explored. Studies are proposed to test the hypothesis that the conformational flexibility of a proline kink motif underlies the conformational changes required for voltage dependent gating. Studies are also proposed to distinguish between concerted and individual subunit gating models. Data from molecular and electrophysiological studies of ionic flux will be examined using the electrodiffusive model of Chen and Eisenberg to further define the roles of charged amino acids in determining permeation and selectivity of gap junction channels. X-linked Charcot-Marie-Tooth disease (CMTX) is caused by mutations of human Cx32, and nonsyndromic deafness by mutations of Cx26. Biophysical and molecular studies have shown that changes in permeability of Cx32 are likely to underlie the etiology of CMTX. The proposed studies will advance the basic scientific knowledge required to define the molecular basis of this and other connexinrelated diseases, and the biological role of gap junctions. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: STUDY OF A GENETIC NETWORK INVOLVED IN EAR DEVELOPMENT Principal Investigator & Institution: Friedman, Rick A.; Chief, Section on Hereditary Disorders o; House Ear Institute 2100 W 3Rd St Los Angeles, Ca 90057 Timing: Fiscal Year 2001; Project Start 28-SEP-2001; Project End 31-AUG-2006 Summary: (provided by applicant): Tremendous progress has been made during the last five years in the mapping and cloning of genes responsible for syndromic and nonsyndromic hereditary hearing loss. The mouse is an excellent animal model for the study of these human conditions because the anatomy, function and hereditary abnormalities of the ear have been shown to be similar in both humans and mice. We

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have recently described one such model resulting from a spontaneous mutation in a C3HIHeJ colony of mice at the Jackson Laboratory. The insertion of a retrotransposon (intracisternal A particle) into an intron of Eyal was associated with reduced expression of the normal Eya] message and inner ear and kidney abnormalities. We have designated this mutation Eya1 bor The human homologue of this gene, EYAJ, has been shown to underlie Branchio-Oto-Renal (B OR) syndrome, an autosomal dominant disorder characterized by hearing loss with associated branchial and renal anomalies. The function of this new class of nuclear protein is poorly understood. Our preliminary data, and that of our collaborators, suggests a critical role for this gene, and other members of this gene family (Eyal-4), in inner ear morphogenesis and postnatal function. In this proposal we intend to explore the hypothesis that Eyal participates in a regulatory network, as Jescribed in the Drosophila eye (eya), that is conserved and critical, in a dose dependent manner, to the early inductive events in mammalian ear development and maintenance of the mature auditory phenotype. We will examine the dose dependent effects on the ear and related structures, in viva, anatomically, functionally and molecularly. This will be accomplished by studying the Fl mice resulting from a cross between C3H/HeJ Eyalb0r/+ and BALB/cJ Eya1+/- and Eyal over-expression transgenic mutants. Additionally, we will identify the regions of the Eya homologous region (EyaHR) that are critical to the protein-protein interactions with other members of this conserved transcriptional regulatory complex. These and future experiments represent the natural progression from my Mentored Clinical Scientist Development Award (K08). Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: THE ZEBRAFISH EAR Principal Investigator & Institution: Malicki, Jarema; Assistant Professor; Massachusetts Eye and Ear Infirmary 243 Charles St Boston, Ma 02114 Timing: Fiscal Year 2001; Project Start 01-JUL-2001; Project End 31-MAY-2005 Summary: (Investigator's abstract) The long-term goals are to identify in the sensory epithelia of the zebrafish. The identification of genetic mechanisms involved in the generation and regeneration of hair cells is potentially of great practical importance because the components of these mechanisms may be used to trigger hair cell regeneration in the human auditory system. Understanding the genetics of hair cell maintenance might lead to new treatment regimens for progressive hearing loss. The small quantities of tissue available from the vertebrate inner ear constrain the understanding of the molecular biology of the inner ear to genes expressed in high quantity or to those affected in naturally occurring mutations in (primarily) man and mouse. Zebrafish forward genetic analysis, through random mutagenesis approaches, provides an opportunity to find genes that may be critical, but expressed in very small quantities. Although the mammalian inner ear is more complex than that of the fish, the fundamental elements of inner ear function appear to be the same in both. To identify such genes, we propose to screen for recessive deafness in chemically mutagenized fish using F2 parthenogenetic diploids. The specific aims are: (1) to use chemical mutagenesis and early pressure to generate parthenogenetic diploid embryos from the progeny of mutangenized fish; (2) to search for defects in hair cell generation, regeneration, function, and maintenance by screening for morphological defects in inner ear structures in early larvae; for balance problems in larvae; for defects in hair cell generation in the lateral line organ with a hair cell selective fluorescent dye (daspei); for defects in hair cell regeneration after neomycin induced destruction of hair cells, and for functional defects in hearing at day 7 and at 3 months with an automated behavioral

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screen that uses image analysis of the startle response to acoustic stimulation.; (3) to genetically characterize the mutants that are isolated (4) to determine the developmental and cellular bases of the isolated mutant phenotypes. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: THYROID HORMONE RECEPTORS AND GENETIC CONTROL OF HEARING Principal Investigator & Institution: Forrest, Douglas; Assistant Professor; Human Genetics; Mount Sinai School of Medicine of Nyu of New York University New York, Ny 10029 Timing: Fiscal Year 2002; Project Start 01-AUG-1997; Project End 31-JUL-2007 Summary: (provided by applicant): Thyroid hormone is essential for the development of hearing and inadequate hormone levels at early stages of development lead to profound deafness. Although the need for thyroid hormone is well known, less is understood of how it acts and what it controls in the development of the auditory system.Thyroid hormone receptors in target tissues transmit the hormonal signal into cellular responses. The study of these receptors is therefore expected to reveal critical steps in the chain of events that control the development of the auditory system. A primary site of action of these receptors in the auditory system is in the cochlea.Thyroid hormone receptors act as hormone-activated transcription factors and are encoded by two related genes, Thra and Thrb. Targeted mutagenesis in mice indicates that Thrb has the primary role and Thra a lesser role in the control of cochlear development. The mutant phenotypes indicate that thyroid hormone receptors regulate relatively late stages of maturation of the cochlea prior to the onset of auditory function.This renewal application aims to advance our understanding of the role of these receptors in cochlear -development by investigation of: 1) The role of thyroid hormone-metabolizing enzymes (deiodinases) as regulators of thyroid hormone receptor function in the cochlea; 2) The developmental signals that induce Thrb gene expression in the cochlea; 3) A novel gene that was identified using Thrb-null mice to screen for genes involved in the maturational stages of cochlear development. This gene expresses a novel extracellular matrix protein in the cochlear basilar membrane. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: TISSUE ENGINEERING OF THE INNER EAR Principal Investigator & Institution: Miller, Josef M.; Professor & Director of Research; Otolaryngology; University of Michigan at Ann Arbor 3003 South State, Room 1040 Ann Arbor, Mi 481091274 Timing: Fiscal Year 2001; Project Start 01-APR-2000; Project End 31-MAR-2003 Summary: Three critical findings have been made over the last several years with important implications to the treatment of the deafened auditory system. First, there are significant changes to the peripheral auditory system as a consequence of deafness in the adult animal. Second, these changes impact on the reintroduction of input after deafness. Third, it is now possible to intervene and influence deafness-related changes to permit optimal processing of re-introduced input. Such interventions can be termed "Tissue Engineering" and their basis development, and application drive our proposed studies. A great deal of our current knowledge regarding interventions that may effect the survival and function of the sensory nerves derives from in vitro studies of cells in culture and organotypic preparations. The goal of this investigation is to verify the validity of these interventions in vivo, and to test the efficacy and safety of potential

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interventions to "engineer" the tissues of the inner ear in vivo. Our first specific aim is to test the utility and synergy of chemical and activity factors involved in the survival of the auditory nerve for enhanced spiral ganglion survival after deafness. We hypothesize that there are naturally multiple factors involved in the maintenance of spiral ganglion cells, which not only provides redundancy (for increased protection) but also synergy in effect, and we can achieve maximal enhancement with multiple factors. Our second set of studies how neurotrophic factors and stimulation induced activity can not only enhance spiral ganglion cell survival but also induce regrowth of auditory nerve peripheral process, which regress after inner hair cell loss. Our ultimate goal is to develop the knowledge base and the tools to intervene in and influence the deafened auditory system to provide the best possible environment for re-introduction of auditory information. Hopefully, these in vivo studies will provide a critical step in the transfer of this technology to human application. The interventions that are developed will provide the substrate essential for reconnecting regenerated hair cells in the future. The should directly enhance the benefit of cochlear prostheses at present. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: TRANSDUCTION MECHANISM OF HAIR CELLS Principal Investigator & Institution: Hudspeth, Albert J.; Professor; Lab of Sensory Neuroscience; Rockefeller University New York, Ny 100216399 Timing: Fiscal Year 2002; Project Start 01-JUL-1983; Project End 31-MAR-2007 Summary: Hair cells are the sensory receptors of the internal ear, where they underlie the responsiveness of both the auditory and the vestibular systems. Each hair cell is a mechanoreceptor that responds when a sound or an acceleration applies a force to its hair bundle, a cluster of a few dozen to a few hundred mechanically sensitive filaments protruding from the cell's top surface. Because most hearing loss and many forms of dysequilibrium result from damage to hair cells, the proposed experiments are meant to reveal more about how these cells perform their essential functions. First, a combination of electron-microscopic and biophysical experiments will be used to determine whether a hair cell adapts to a prolonged stimulus by physically resetting the molecular apparatus in its hair bundle. The proposed experiments should show whether the myosin molecules thought to mediate adaptation occur at the appropriate sites and whether these molecules actually move as the hair cell adapts. A second set of experiments is meant to determine whether the hair bundle helps amplify the ear's mechanical inputs, thus augmenting the sensitivity of hearing and sharpening its frequency discrimination. In addition to providing information about the nature of the ear's amplificatory process, this investigation may indicate why hearing is vulnerable to overstimulation and whether the ear's amplifier can be protected from or regenerated after injury. In a final set of studies, genetic and molecular- biological techniques will be used to identify proteins essential to hearing and balance. More than a hundred genes are involved in heritable forms of deafness and vertigo, which affect one child in a thousand at birth and a similar proportion later in life. The zebrafish, a tractable model organism suitable for large-scale genetic analysis, will be used to identify novel genes and to determine the functions of recently discovered genes in the inner ear. After the corresponding human genes have been found, their roles in the ear and their possible involvement in human hearing loss and dysequilibrium will be examined. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: VACCINATION FOR LASSA FEVER Principal Investigator & Institution: Buchmeier, Michael J.; Professor; Scripps Research Institute 10550 N Torrey Pines Rd La Jolla, Ca 920371000 Timing: Fiscal Year 2002; Project Start 01-JAN-2002; Project End 31-DEC-2005 Summary: (Provided by Applicant) Lassa virus, endemic in West Africa, infects 100,000300,000 people annually, killing 5,000-10,000, and leaving about 30,000 with residual sequelae such as nerve deafness. The overall goal of this grant proposal is to understand the protective immune response to Lassa virus, thus positioning us to develop a vaccine against Lassa fever. Lassa virus is a Biosafety level 4 (BSL4) agent, and the constraints imposed by this fact have significantly impeded our ability to study the agent. Nevertheless, vaccine studies were carried out almost a decade ago, in guinea pigs and primates, and showed clearly that protective immunity could be conferred against this disease. However, the protective component(s) of vaccine-induced immunity were not identified. We are now in a much stronger position to evaluate the protective components, and shall do so in the experiments we propose. Our group has extensive experience with emerging viruses in general, and Lassa virus in particular. In addition we have many years of experience in designing vaccines against Old World arenaviruses. The constructs we will use will exploit recent advances that greatly enhance the immune responses induced by DNA vaccines, optimizing the induction of T cells (CD8+ and CD4+), and antibodies. There are only two reliable challenge models for Lassa fever, guinea pigs and primates. The Scripps collaborators will first evaluate the immunogenicity of the vaccines in guinea pigs with immunized animals also being sent to USAMRIID for live virus challenge trials. In the second year the study will then move to the primate models in Houston, where we will use rhesus macaques (Indian origin) characterized in the MAMU I allele in order to evaluate the immune response in primates in a model that closely parallels the human responses to the same viral antigens. Finally the vaccinated monkeys will be moved to USAMRIID where challenge studies will be performed under the supervision of Dr. Peter B. Jahrling, and where all the collaborators will combine efforts on site to ensure an exhaustive evaluation of the responses of the individual primates. These studies are expected to lead to the identification of a candidate Lassa vaccine that can be tested in humans. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

E-Journals: PubMed Central3 PubMed Central (PMC) is a digital archive of life sciences journal literature developed and managed by the National Center for Biotechnology Information (NCBI) at the U.S. National Library of Medicine (NLM).4 Access to this growing archive of e-journals is free and unrestricted.5 To search, go to http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Pmc, and type “deafness” (or synonyms) into the search box. This search gives you access to fulltext articles. The following is a sample of items found for deafness in the PubMed Central database: 3 4

Adapted from the National Library of Medicine: http://www.pubmedcentral.nih.gov/about/intro.html.

With PubMed Central, NCBI is taking the lead in preservation and maintenance of open access to electronic literature, just as NLM has done for decades with printed biomedical literature. PubMed Central aims to become a world-class library of the digital age. 5 The value of PubMed Central, in addition to its role as an archive, lies in the availability of data from diverse sources stored in a common format in a single repository. Many journals already have online publishing operations, and there is a growing tendency to publish material online only, to the exclusion of print.

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A Human Mitochondrial GTP Binding Protein Related to tRNA Modification May Modulate Phenotypic Expression of the Deafness-Associated Mitochondrial 12S rRNA Mutation. by Li X, Guan MX.; 2002 Nov; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=135671

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Cerebral organization for language in deaf and hearing subjects: Biological constraints and effects of experience. by Neville HJ, Bavelier D, Corina D, Rauschecker J, Karni A, Lalwani A, Braun A, Clark V, Jezzard P, Turner R.; 1998 Feb 3; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=33817

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Designing a curriculum on Internet health resources for deaf high school students. by Gregg AL, Wozar JA, Wessel CB, Epstein BA.; 2002 Oct; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=128959

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Enteroviruses and sudden deafness. by Schattner A, Halperin D, Wolf D, Zimhony O.; 2003 May 27; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=155958

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Human deafness dystonia syndrome is a mitochondrial disease. by Koehler CM, Leuenberger D, Merchant S, Renold A, Junne T, Schatz G.; 1999 Mar 2; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=26750

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Identifying the genes of hearing, deafness, and dysequilibrium. by Corwin JT.; 1998 Oct 13; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=33905

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In vitro 3[prime prime or minute]-end endonucleolytic processing defect in a human mitochondrial tRNASer(UCN) precursor with the U7445C substitution, which causes non-syndromic deafness. by Levinger L, Jacobs O, James M.; 2001 Nov 1; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=60182

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KCNQ4, a K + channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway. by Kharkovets T, Hardelin JP, Safieddine S, Schweizer M, El-Amraoui A, Petit C, Jentsch TJ.; 2000 Apr 11; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=18242

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Language-related cortex in deaf individuals: Functional specialization for language or perceptual plasticity? by Caplan D.; 2000 Dec 5; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=34084

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Light eye colour linked to deafness after meningitis. by Cullington HE.; 2001 Mar 10; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=26552

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Mutant ion channel in cochlear hair cells causes deafness. by Trussell L.; 2000 Apr 11; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=33974

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Neurotrophic factor intervention restores auditory function in deafened animals. by Shinohara T, Bredberg G, Ulfendahl M, Pyykko I, Olivius NP, Kaksonen R, Lindstrom B, Altschuler R, Miller JM.; 2002 Feb 5; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=122246

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NMR structure of the LCCL domain and implications for DFNA9 deafness disorder. by Liepinsh E, Trexler M, Kaikkonen A, Weigelt J, Banyai L, Patthy L, Otting G.; 2001 Oct 1; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=125649

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Otoancorin, an inner ear protein restricted to the interface between the apical surface of sensory epithelia and their overlying acellular gels, is defective in autosomal recessive deafness DFNB22. by Zwaenepoel I, Mustapha M, Leibovici M, Verpy E,

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Goodyear R, Liu XZ, Nouaille S, Nance WE, Kanaan M, Avraham KB, Tekaia F, Loiselet J, Lathrop M, Richardson G, Petit C.; 2002 Apr 30; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=122933 •

Patients affected with Fabry disease have an increased incidence of progressive hearing loss and sudden deafness: an investigation of twenty-two hemizygous male patients. by Germain DP, Avan P, Chassaing A, Bonfils P.; 2002; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=134464

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Speech-like cerebral activity in profoundly deaf people processing signed languages: Implications for the neural basis of human language. by Petitto LA, Zatorre RJ, Gauna K, Nikelski EJ, Dostie D, Evans AC.; 2000 Dec 5; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=17683

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The Deafness-Associated Mitochondrial DNA Mutation at Position 7445, Which Affects tRNASer(UCN) Precursor Processing, Has Long-Range Effects on NADH Dehydrogenase Subunit ND6 Gene Expression. by Guan MX, Enriquez JA, FischelGhodsian N, Puranam RS, Lin CP, Maw MA, Attardi G.; 1998 Oct; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=109173

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The Gene for an Inherited Form of Deafness Maps to Chromosome 5q31. by Leon PE, Raventos H, Lynch E, Morrow J, King M.; 1992 Jun 1; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&rendertype=abstr act&artid=49253

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Transient "deafness" accompanies auditory development during metamorphosis from tadpole to frog. by Boatright-Horowitz SS, Simmons AM.; 1997 Dec 23; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=25131

The National Library of Medicine: PubMed One of the quickest and most comprehensive ways to find academic studies in both English and other languages is to use PubMed, maintained by the National Library of Medicine.6 The advantage of PubMed over previously mentioned sources is that it covers a greater number of domestic and foreign references. It is also free to use. If the publisher has a Web site that offers full text of its journals, PubMed will provide links to that site, as well as to sites offering other related data. User registration, a subscription fee, or some other type of fee may be required to access the full text of articles in some journals. To generate your own bibliography of studies dealing with deafness, simply go to the PubMed Web site at http://www.ncbi.nlm.nih.gov/pubmed. Type “deafness” (or synonyms) into the search box, and click “Go.” The following is the type of output you can expect from PubMed for deafness (hyperlinks lead to article summaries):

6

PubMed was developed by the National Center for Biotechnology Information (NCBI) at the National Library of Medicine (NLM) at the National Institutes of Health (NIH). The PubMed database was developed in conjunction with publishers of biomedical literature as a search tool for accessing literature citations and linking to full-text journal articles at Web sites of participating publishers. Publishers that participate in PubMed supply NLM with their citations electronically prior to or at the time of publication.

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A case of a de novo A3243G mutation in mitochondrial DNA in a patient with diabetes and deafness. Author(s): Maassen JA, Biberoglu S, 't Hart LM, Bakker E, de Knijff P. Source: Archives of Physiology and Biochemistry. 2002 July; 110(3): 186-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12221518&dopt=Abstract

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A case of diabetes, deafness, cardiomyopathy, and central sleep apnea: novel mitochondrial DNA polymorphisms. Author(s): Sakaue S, Ohmuro J, Mishina T, Miyazaki H, Yamaguchi E, Nishimura M, Fujita M, Nagashima K, Tagami S, Kawakami Y. Source: The Tohoku Journal of Experimental Medicine. 2002 March; 196(3): 203-11. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12002277&dopt=Abstract

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A case study of pure word deafness: modularity in auditory processing? Author(s): Pinard M, Chertkow H, Black S, Peretz I. Source: Neurocase : Case Studies in Neuropsychology, Neuropsychiatry, and Behavioural Neurology. 2002; 8(1-2): 40-55. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11997484&dopt=Abstract

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A clinical evaluation of ophthalmic assessment in children with sensori-neural deafness. Author(s): Guy R, Nicholson J, Pannu SS, Holden R. Source: Child: Care, Health and Development. 2003 September; 29(5): 377-84. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12904246&dopt=Abstract

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A gene locus for steroid-resistant nephrotic syndrome with deafness maps to chromosome 14q24.2. Author(s): Ruf RG, Wolf MT, Hennies HC, Lucke B, Zinn C, Varnholt V, Lichtenberger A, Pasch A, Imm A, Briese S, Lennert T, Fuchshuber A, Nurnberg P, Hildebrandt F. Source: Journal of the American Society of Nephrology : Jasn. 2003 June; 14(6): 1519-22. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12761252&dopt=Abstract

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A human mitochondrial GTP binding protein related to tRNA modification may modulate phenotypic expression of the deafness-associated mitochondrial 12S rRNA mutation. Author(s): Li X, Guan MX. Source: Molecular and Cellular Biology. 2002 November; 22(21): 7701-11. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12370316&dopt=Abstract

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A mitochondrial tRNA(His) gene mutation causing pigmentary retinopathy and neurosensorial deafness. Author(s): Crimi M, Galbiati S, Perini MP, Bordoni A, Malferrari G, Sciacco M, Biunno I, Strazzer S, Moggio M, Bresolin N, Comi GP. Source: Neurology. 2003 April 8; 60(7): 1200-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12682337&dopt=Abstract

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A new method of partial deafness treatment. Author(s): Skarzynski H, Lorens A, Piotrowska A. Source: Medical Science Monitor : International Medical Journal of Experimental and Clinical Research. 2003 April; 9(4): Cs20-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12709676&dopt=Abstract

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A novel autosomal dominant non-syndromic deafness locus (DFNA48) maps to 12q13-q14 in a large Italian family. Author(s): D'Adamo P, Pinna M, Capobianco S, Cesarani A, D'Eustacchio A, Fogu P, Carella M, Seri M, Gasparini P. Source: Human Genetics. 2003 March; 112(3): 319-20. Epub 2002 December 13. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12596055&dopt=Abstract

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A novel autosomal recessive non-syndromic deafness locus (DFNB35) maps to 14q24.1-14q24.3 in large consanguineous kindred from Pakistan. Author(s): Ansar M, Din MA, Arshad M, Sohail M, Faiyaz-Ul-Haque M, Haque S, Ahmad W, Leal SM. Source: European Journal of Human Genetics : Ejhg. 2003 January; 11(1): 77-80. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12529709&dopt=Abstract

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A novel connexin 26 compound heterozygous mutation results in deafness. Author(s): Harris KC, Erbe CB, Firszt JB, Flanary VA, Wackym PA. Source: The Laryngoscope. 2002 July; 112(7 Pt 1): 1159-62. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12169891&dopt=Abstract

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A novel locus for autosomal dominant non-syndromic deafness (DFNA41) maps to chromosome 12q24-qter. Author(s): Blanton SH, Liang CY, Cai MW, Pandya A, Du LL, Landa B, Mummalanni S, Li KS, Chen ZY, Qin XN, Liu YF, Balkany T, Nance WE, Liu XZ. Source: Journal of Medical Genetics. 2002 August; 39(8): 567-70. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12161595&dopt=Abstract

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A patient database application for Hereditary Deafness Epidemiology and Clinical Research (H.E.A.R.): an effort for standardization in multiple languages. Author(s): Pfister M, Akyildiz S, Gunhan O, Maassen M, Rodriguez JJ, Zenner HP, Apaydin F. Source: Eur Arch Otorhinolaryngol. 2003 February;260(2):81-5. Epub 2002 September 27. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12582784&dopt=Abstract

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A variant of Fine-Lubinsky syndrome: a Japanese boy with profound deafness, cataracts, mental retardation, and brachycephaly without craniosynostosis. Author(s): Nakane T, Mizobe N, Hayashibe H, Nakazawa S. Source: Clinical Dysmorphology. 2002 July; 11(3): 195-8. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12072800&dopt=Abstract

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Abnormal cochlea linked to deafness in transgenic mice expressing human cytokeratin K8. Author(s): Bartolome MV, Casanova ML, Carricondo F, del Castillo E, Jorcano JL, GilLoyzaga P. Source: Histology and Histopathology. 2002; 17(3): 827-36. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12168793&dopt=Abstract

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Aspects of quality of life in persons with pre-lingual deafness using sign language: subjective wellbeing, ill-health symptoms, depression and insomnia. Author(s): Werngren-Elgstrom M, Dehlin O, Iwarsson S. Source: Archives of Gerontology and Geriatrics. 2003 July-August; 37(1): 13-24. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12849069&dopt=Abstract

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Association of mutations in the NALP3/CIAS1/PYPAF1 gene with a broad phenotype including recurrent fever, cold sensitivity, sensorineural deafness, and AA amyloidosis. Author(s): Aganna E, Martinon F, Hawkins PN, Ross JB, Swan DC, Booth DR, Lachmann HJ, Bybee A, Gaudet R, Woo P, Feighery C, Cotter FE, Thome M, Hitman GA, Tschopp J, McDermott MF. Source: Arthritis and Rheumatism. 2002 September; 46(9): 2445-52. Erratum In: Arthritis Rheum 2002 December; 46(12): 3398. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12355493&dopt=Abstract

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ATP6B1 gene mutations associated with distal renal tubular acidosis and deafness in a child. Author(s): Hahn H, Kang HG, Ha IS, Cheong HI, Choi Y. Source: American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation. 2003 January; 41(1): 238-43. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12500243&dopt=Abstract

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Attitudes of the broader hearing, deaf, and hard-of-hearing community toward genetic testing for deafness. Author(s): Martinez A, Linden J, Schimmenti LA, Palmer CG. Source: Genetics in Medicine : Official Journal of the American College of Medical Genetics. 2003 March-April; 5(2): 106-12. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12644780&dopt=Abstract

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Atypical Bartter syndrome with sensorineural deafness with G47R mutation of the beta-subunit for ClC-Ka and ClC-Kb chloride channels, barttin. Author(s): Miyamura N, Matsumoto K, Taguchi T, Tokunaga H, Nishikawa T, Nishida K, Toyonaga T, Sakakida M, Araki E. Source: The Journal of Clinical Endocrinology and Metabolism. 2003 February; 88(2): 781-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12574213&dopt=Abstract

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Autosomal dominant progressive nephropathy with deafness: linkage to a new locus on chromosome 11q24. Author(s): Prakash S, Chung KW, Sinha S, Barmada M, Ellis D, Ferrell RE, Finegold DN, Randhawa PS, Dinda A, Vats A. Source: Journal of the American Society of Nephrology : Jasn. 2003 July; 14(7): 1794-803. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12819239&dopt=Abstract

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Bone-anchored hearing aids in unilateral inner ear deafness. Author(s): Bosman AJ, Hol MK, Snik AF, Mylanus EA, Cremers CW. Source: Acta Oto-Laryngologica. 2003 January; 123(2): 258-60. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12701753&dopt=Abstract

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Branchial cyst, sensorineural deafness, congenital heart defect, and skeletal abnormalities: Branchio-oto-cardio-skeletal (BOCS) syndrome? Author(s): Basel-Vanagaite L, Shohat M, Udler Y, Karmazin B, Levit O, Merlob P. Source: American Journal of Medical Genetics. 2002 November 15; 113(1): 78-81. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12400069&dopt=Abstract

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Broad thumbs and halluces with deafness: a patient with Keipert syndrome. Author(s): Reardon W, Hall CM. Source: American Journal of Medical Genetics. 2003 April 1; 118A(1): 86-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12605449&dopt=Abstract

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Carbogen gas for treatment of sudden deafness. Author(s): Hender KM, Anderson JN, Vallance NA. Source: The Medical Journal of Australia. 2002 April 15; 176(8): 387-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12041636&dopt=Abstract

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CDH23 mutation and phenotype heterogeneity: a profile of 107 diverse families with Usher syndrome and nonsyndromic deafness. Author(s): Astuto LM, Bork JM, Weston MD, Askew JW, Fields RR, Orten DJ, Ohliger SJ, Riazuddin S, Morell RJ, Khan S, Riazuddin S, Kremer H, van Hauwe P, Moller CG, Cremers CW, Ayuso C, Heckenlively JR, Rohrschneider K, Spandau U, Greenberg J, Ramesar R, Reardon W, Bitoun P, Millan J, Legge R, Friedman TB, Kimberling WJ. Source: American Journal of Human Genetics. 2002 August; 71(2): 262-75. Epub 2002 June 19. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12075507&dopt=Abstract

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Change deafness: the inability to detect changes between two voices. Author(s): Vitevitch MS. Source: Journal of Experimental Psychology. Human Perception and Performance. 2003 April; 29(2): 333-42. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12760619&dopt=Abstract

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Changes in the spatial distribution of visual attention after early deafness. Author(s): Proksch J, Bavelier D. Source: Journal of Cognitive Neuroscience. 2002 July 1; 14(5): 687-701. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12167254&dopt=Abstract

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Children's search for targets located within and beyond the field of view: effects of deafness and age. Author(s): Netelenbos JB, Savelsbergh GJ. Source: Perception. 2003; 32(4): 485-97. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12785486&dopt=Abstract

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Choanal stenosis, hypothelia, deafness, recurrent dacryocystitis, neck fistulas, short stature, and microcephaly: report of a case. Author(s): Dumic M, Cvitanovic M, Saric B, Spehar A, Batinica S. Source: American Journal of Medical Genetics. 2002 December 1; 113(3): 295-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12439900&dopt=Abstract

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Clinical and molecular findings in a patient with a novel mutation in the deafnessdystonia peptide (DDP1) gene. Author(s): Binder J, Hofmann S, Kreisel S, Wohrle JC, Bazner H, Krauss JK, Hennerici MG, Bauer MF. Source: Brain; a Journal of Neurology. 2003 August; 126(Pt 8): 1814-20. Epub 2003 June 04. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12805099&dopt=Abstract

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Clinical manifestations of DFNB29 deafness. Author(s): Ahmed ZM, Riazuddin S, Friedman TB, Riazuddin S, Wilcox ER, Griffith AJ. Source: Advances in Oto-Rhino-Laryngology. 2002; 61: 156-60. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12408079&dopt=Abstract

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Cochlear implantation for adolescents and adults with prelinguistic deafness. Author(s): Schramm D, Fitzpatrick E, Seguin C. Source: Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology. 2002 September; 23(5): 698-703. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12218622&dopt=Abstract

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Cochlear implantation in maternal inherited diabetes and deafness syndrome. Author(s): Raut V, Sinnathuray AR, Toner JG. Source: The Journal of Laryngology and Otology. 2002 May; 116(5): 373-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12080997&dopt=Abstract

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Comparison of duration of deafness and tumour invasion to the inner ear from metastatic tumours of the internal auditory canal: human temporal bone pathology. Author(s): Nishimura S, Kaga K, Tsuzuku T, Iino Y. Source: The Journal of Laryngology and Otology. 2002 April; 116(4): 256-60. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11945183&dopt=Abstract

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Comparison of the bone anchored hearing aid implantable hearing device with contralateral routing of offside signal amplification in the rehabilitation of unilateral deafness. Author(s): Niparko JK, Cox KM, Lustig LR. Source: Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology. 2003 January; 24(1): 73-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12544032&dopt=Abstract

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Complete biotinidase deficiency presenting as reversible progressive ataxia and sensorineural deafness. Author(s): Tsao CY, Kien CL. Source: Journal of Child Neurology. 2002 February; 17(2): 146. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11952077&dopt=Abstract

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Congenital myopathy, recurrent secretory diarrhea, bullous eruption of skin, microcephaly, and deafness: a new genetic syndrome? Author(s): Levy J, Chung W, Garzon M, Gallagher MP, Oberfield SE, Lieber E, AnyaneYeboa K. Source: American Journal of Medical Genetics. 2003 January 1; 116A(1): 20-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12476446&dopt=Abstract

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Connexin 26 mutations in cases of sensorineural deafness in eastern Austria. Author(s): Frei K, Szuhai K, Lucas T, Weipoltshammer K, Schofer C, Ramsebner R, Baumgartner WD, Raap AK, Bittner R, Wachtler FJ, Kirschhofer K. Source: European Journal of Human Genetics : Ejhg. 2002 July; 10(7): 427-32. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12107817&dopt=Abstract

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Cortical representation of hearing restoration in patients with sudden deafness. Author(s): Suzuki M, Kouzaki H, Nishida Y, Shiino A, Ito R, Kitano H. Source: Neuroreport. 2002 October 7; 13(14): 1829-32. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12395134&dopt=Abstract

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Craniosynostosis, telecanthus, scalp hair abnormalities, and sensorineural deafness in two sibs. Author(s): Megarbane A, Hersh JH, Chouery E, Fabre M. Source: American Journal of Medical Genetics. 2002 May 15; 109(4): 323-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11992488&dopt=Abstract

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De novo mutation in the gene encoding connexin-26 (GJB2) in a sporadic case of keratitis-ichthyosis-deafness (KID) syndrome. Author(s): Alvarez A, del Castillo I, Pera A, Villamar M, Moreno-Pelayo MA, Moreno F, Moreno R, Tapia MC. Source: American Journal of Medical Genetics. 2003 February 15; 117A(1): 89-91. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12548749&dopt=Abstract

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Deafness and cerebral plasticity. Author(s): Cordes M, Wszolek ZK. Source: Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine. 2003 September; 44(9): 1440-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12960190&dopt=Abstract

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Deafness and CMT disease associated with a novel four amino acid deletion in the PMP22 gene. Author(s): Sambuughin N, de Bantel A, McWilliams S, Sivakumar K. Source: Neurology. 2003 February 11; 60(3): 506-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12578939&dopt=Abstract

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Deafness and renal tubular acidosis in mice lacking the K-Cl co-transporter Kcc4. Author(s): Boettger T, Hubner CA, Maier H, Rust MB, Beck FX, Jentsch TJ. Source: Nature. 2002 April 25; 416(6883): 874-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11976689&dopt=Abstract

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Deafness genes for nonsyndromic hearing loss and current studies in China. Author(s): Xiao Z, Xie D. Source: Chinese Medical Journal. 2002 July; 115(7): 1078-81. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12173596&dopt=Abstract

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Deafness genes. Author(s): Kitamura K, Takahashi K, Tamagawa Y, Noguchi Y, Kuroishikawa Y, Ishikawa K, Hagiwara H. Source: J Med Dent Sci. 2000 March; 47(1): 1-11. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12162522&dopt=Abstract

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Deafness in sub-Saharan Africa. Author(s): Kiyaga NB, Moores DF. Source: Am Ann Deaf. 2003 Spring; 148(1): 18-24. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12765086&dopt=Abstract

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Deafness resulting from mutations in the GJB2 (connexin 26) gene in Brazilian patients. Author(s): Oliveira CA, Maciel-Guerra AT, Sartorato EL. Source: Clinical Genetics. 2002 May; 61(5): 354-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12081719&dopt=Abstract

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Deafness, culture, and choice. Author(s): Levy N. Source: Journal of Medical Ethics. 2002 October; 28(5): 284-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12356951&dopt=Abstract

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Deafness: from bedside to bench and back. Author(s): Smith RJ. Source: Lancet. 2002 August 31; 360(9334): 656-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12241869&dopt=Abstract

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Deafness--a complication of nasopharyngeal airways. Author(s): Jones GL, Redfern RM, Jerwood DC. Source: Anaesthesia and Intensive Care. 2002 December; 30(6): 807-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12500523&dopt=Abstract

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Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31. Author(s): Mburu P, Mustapha M, Varela A, Weil D, El-Amraoui A, Holme RH, Rump A, Hardisty RE, Blanchard S, Coimbra RS, Perfettini I, Parkinson N, Mallon AM, Glenister P, Rogers MJ, Paige AJ, Moir L, Clay J, Rosenthal A, Liu XZ, Blanco G, Steel KP, Petit C, Brown SD. Source: Nature Genetics. 2003 August; 34(4): 421-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12833159&dopt=Abstract

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Dextran-induced pulmonary edema in patients with sudden deafness. Author(s): Kuo ST, Hsu WC, Young YH. Source: Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology. 2002 September; 23(5): 661-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12218616&dopt=Abstract

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Drawing insight from pictures: the development of concepts of false drawing and false belief in children with deafness, normal hearing, and autism. Author(s): Peterson CC. Source: Child Development. 2002 September-October; 73(5): 1442-59. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12361311&dopt=Abstract

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Dystonia with and without deafness is caused by TIMM8A mutation. Author(s): Swerdlow RH, Juel VC, Wooten GF. Source: Adv Neurol. 2004; 94: 147-54. Review. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=14509668&dopt=Abstract

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Effectiveness of sequencing connexin 26 (GJB2) in cases of familial or sporadic childhood deafness referred for molecular diagnostic testing. Author(s): Wu BL, Lindeman N, Lip V, Adams A, Amato RS, Cox G, Irons M, Kenna M, Korf B, Raisen J, Platt O. Source: Genetics in Medicine : Official Journal of the American College of Medical Genetics. 2002 July-August; 4(4): 279-88. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12172394&dopt=Abstract

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Electrophysiological findings in two bilateral cochlear implant cases: does the duration of deafness affect electrically evoked auditory brain stem responses? Author(s): Thai-Van H, Gallego S, Truy E, Veuillet E, Collet L. Source: The Annals of Otology, Rhinology, and Laryngology. 2002 November; 111(11): 1008-14. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12450176&dopt=Abstract

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EMG analysis of the upper and lower fascicles of the orbicularis oris muscle in deaf individuals. Author(s): Regalo SC, Vitti M, Hallak JE, Semprini M, Mattos MG, Tosello DO, Constancio RF, Pegoraro ME, Lopes RA. Source: Electromyogr Clin Neurophysiol. 2003 September; 43(6): 367-72. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=14535049&dopt=Abstract

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Endolymphatic deafness: a particular variety of cochlear disorder. Author(s): Tran BH. Source: Orl; Journal for Oto-Rhino-Laryngology and Its Related Specialties. 2002 MarchApril; 64(2): 120-4. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12021503&dopt=Abstract

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Enteroviruses and sudden deafness. Author(s): Schattner A, Halperin D, Wolf D, Zimhony O. Source: Cmaj : Canadian Medical Association Journal = Journal De L'association Medicale Canadienne. 2003 May 27; 168(11): 1421-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12771071&dopt=Abstract

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Etiology of deafness at the Yeditepe School for the deaf in Istanbul. Author(s): Egeli E, Cicekci G, Silan F, Ozturk O, Harputluoglu U, Onur A, Egeli A, Yildiz A. Source: International Journal of Pediatric Otorhinolaryngology. 2003 May; 67(5): 467-71. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12697348&dopt=Abstract

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Evaluation of anti-hsp70 antibody screening in sudden deafness. Author(s): Samuelsson AK, Hyden D, Roberg M, Skogh T. Source: Ear and Hearing. 2003 June; 24(3): 233-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12799545&dopt=Abstract

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Evaluation of dHPLC for CX26 mutation screening in patients from southern France with sensorineural deafness. Author(s): Pallares-Ruiz N, Blanchet P, Mondain M, Low-Hong S, Demaille J, Claustres M, Roux AF. Source: Genetic Testing. 2001 Winter; 5(4): 339-43. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11960582&dopt=Abstract

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Exploring the clinical and epidemiological complexity of GJB2-linked deafness. Author(s): Gualandi F, Ravani A, Berto A, Sensi A, Trabanelli C, Falciano F, Trevisi P, Mazzoli M, Tibiletti MG, Cristofari E, Burdo S, Ferlini A, Martini A, Calzolari E. Source: American Journal of Medical Genetics. 2002 September 15; 112(1): 38-45. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12239718&dopt=Abstract

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Familial deafness, congenital heart defects, and posterior embryotoxon caused by cysteine substitution in the first epidermal-growth-factor-like domain of jagged 1. Author(s): Le Caignec C, Lefevre M, Schott JJ, Chaventre A, Gayet M, Calais C, Moisan JP. Source: American Journal of Human Genetics. 2002 July; 71(1): 180-6. Epub 2002 May 17. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12022040&dopt=Abstract

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Fatal septicemia in an infant with keratitis, ichthyosis, and deafness (KID) syndrome. Author(s): Gilliam A, Williams ML. Source: Pediatric Dermatology. 2002 May-June; 19(3): 232-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12047643&dopt=Abstract

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Fits and deafness. Author(s): Iwata NK, Hayashi T, Numaga J, Yamamichi N, Sakurai M, Kanazawa I. Source: Lancet. Neurology. 2002 October; 1(6): 387-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12849401&dopt=Abstract

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Frequency of mtDNA A1555G and A7445G mutations among children with prelingual deafness in Turkey. Author(s): Tekin M, Duman T, Bogoclu G, Incesulu A, Comak E, Fitoz S, Yilmaz E, Ilhan I, Akar N. Source: European Journal of Pediatrics. 2003 March; 162(3): 154-8. Epub 2003 January 21. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12655418&dopt=Abstract

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Functional analysis of a dominant mutation of human connexin26 associated with nonsyndromic deafness. Author(s): Bruzzone R, Gomes D, Denoyelle E, Duval N, Perea J, Veronesi V, Weil D, Petit C, Gabellec MM, D'Andrea P, White TW. Source: Cell Communication & Adhesion. 2001; 8(4-6): 425-31. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12064630&dopt=Abstract

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Functional analysis of connexin-26 mutants associated with hereditary recessive deafness. Author(s): Wang HL, Chang WT, Li AH, Yeh TH, Wu CY, Chen MS, Huang PC. Source: Journal of Neurochemistry. 2003 February; 84(4): 735-42. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12562518&dopt=Abstract

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Functional studies of human skin disease- and deafness-associated connexin 30 mutations. Author(s): Common JE, Becker D, Di WL, Leigh IM, O'Toole EA, Kelsell DP. Source: Biochemical and Biophysical Research Communications. 2002 November 15; 298(5): 651-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12419304&dopt=Abstract

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Genetic evaluation and counseling for congenital deafness. Author(s): Green GE, Cunniff C. Source: Advances in Oto-Rhino-Laryngology. 2002; 61: 230-40. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12408089&dopt=Abstract

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Genetic information but not termination: pregnant women's attitudes and willingness to pay for carrier screening for deafness genes. Author(s): Ryan M, Miedzybrodzka Z, Fraser L, Hall M. Source: Journal of Medical Genetics. 2003 June; 40(6): E80. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12807979&dopt=Abstract

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Genetic screening for deafness. Author(s): Smith RJ, Hone S. Source: Pediatric Clinics of North America. 2003 April; 50(2): 315-29. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12809325&dopt=Abstract

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Genetic testing for deafness--GJB2 and SLC26A4 as causes of deafness. Author(s): Smith RJ, Robin NH. Source: Journal of Communication Disorders. 2002 July-August; 35(4): 367-77. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12160355&dopt=Abstract

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Genetics of congenital deafness in the Palestinian population: multiple connexin 26 alleles with shared origins in the Middle East. Author(s): Shahin H, Walsh T, Sobe T, Lynch E, King MC, Avraham KB, Kanaan M. Source: Human Genetics. 2002 March; 110(3): 284-9. Epub 2002 February 08. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11935342&dopt=Abstract

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GJB2 deafness gene shows a specific spectrum of mutations in Japan, including a frequent founder mutation. Author(s): Ohtsuka A, Yuge I, Kimura S, Namba A, Abe S, Van Laer L, Van Camp G, Usami S. Source: Human Genetics. 2003 April; 112(4): 329-33. Epub 2003 January 31. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12560944&dopt=Abstract

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Health care utilization and adults who are deaf: relationship with age at onset of deafness. Author(s): Barnett S, Franks P. Source: Health Services Research. 2002 February; 37(1): 105-20. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11949915&dopt=Abstract

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Hemifacial microsomia, external auditory canal atresia, deafness and Mullerian anomalies associated with acro-osteolysis: a new autosomal recessive syndrome? Author(s): Brady AF, Winter RM, Wilson LC, Tatnall FM, Sheridan RJ, Garrett C. Source: Clinical Dysmorphology. 2002 July; 11(3): 155-61. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12072792&dopt=Abstract

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Hereditary deafness and phenotyping in humans. Author(s): Bitner-Glindzicz M. Source: British Medical Bulletin. 2002; 63: 73-94. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12324385&dopt=Abstract

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High-throughput screening for GJB2 mutations--its clinical application to genetic testing in prelingual deafness screening for GJB2 mutations. Author(s): Sugata A, Fukushima K, Sugata K, Fukuda S, Kimura N, Gunduz M, Kasai N, Usami S, Smith RJ, Nishizaki K. Source: Auris, Nasus, Larynx. 2002 July; 29(3): 231-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12167443&dopt=Abstract

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Human connexin26 (GJB2) deafness mutations affect the function of gap junction channels at different levels of protein expression. Author(s): Thonnissen E, Rabionet R, Arbones ML, Estivill X, Willecke K, Ott T. Source: Human Genetics. 2002 August; 111(2): 190-7. Epub 2002 June 22. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12189493&dopt=Abstract

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Identification of CRYM as a candidate responsible for nonsyndromic deafness, through cDNA microarray analysis of human cochlear and vestibular tissues. Author(s): Abe S, Katagiri T, Saito-Hisaminato A, Usami S, Inoue Y, Tsunoda T, Nakamura Y. Source: American Journal of Human Genetics. 2003 January; 72(1): 73-82. Epub 2002 December 06. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12471561&dopt=Abstract

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Imaging in sensorineural deafness. Author(s): Decat M, Cosnard G. Source: Acta Otorhinolaryngol Belg. 2002; 56(4): 335-6. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12528249&dopt=Abstract

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Incidence of cochlear involvement in hyperbilirubinemic deafness. Author(s): Oysu C, Aslan I, Ulubil A, Baserer N. Source: The Annals of Otology, Rhinology, and Laryngology. 2002 November; 111(11): 1021-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12450178&dopt=Abstract

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Infantile progressive bulbar palsy with deafness. Author(s): Voudris KA, Skardoutsou A, Vagiakou EA. Source: Brain & Development. 2002 October; 24(7): 732-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12427524&dopt=Abstract

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Isolation and characterization of the putative nuclear modifier gene MTO1 involved in the pathogenesis of deafness-associated mitochondrial 12 S rRNA A1555G mutation. Author(s): Li X, Li R, Lin X, Guan MX. Source: The Journal of Biological Chemistry. 2002 July 26; 277(30): 27256-64. Epub 2002 May 14. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12011058&dopt=Abstract

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Keratitis, ichthyosis and deafness syndrome with development of multiple hair follicle tumours. Author(s): Kim KH, Kim JS, Piao YJ, Kim YC, Shur KB, Lee JH, Park JK. Source: The British Journal of Dermatology. 2002 July; 147(1): 139-43. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12100197&dopt=Abstract

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Keratitis, ichthyosis, and deafness (KID) syndrome. Author(s): Miteva L. Source: Pediatric Dermatology. 2002 November-December; 19(6): 513-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12437553&dopt=Abstract

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Keratitis-ichthyosis-deafness syndrome and carotenaemia. Author(s): Ahmadi S, McKenna K. Source: Clinical and Experimental Dermatology. 2003 July; 28(4): 394-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12823302&dopt=Abstract

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Late-onset hearing loss in a mouse model of DFN3 non-syndromic deafness: morphologic and immunohistochemical analyses. Author(s): Xia AP, Kikuchi T, Minowa O, Katori Y, Oshima T, Noda T, Ikeda K. Source: Hearing Research. 2002 April; 166(1-2): 150-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12062767&dopt=Abstract

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Leftward cradling bias, prosodic speech, and deafness: the deaf are not dumb. Author(s): Woll B, Sieratzki JS. Source: The Journal of Genetic Psychology; Child Behavior, Animal Behavior, and Comparative Psychology. 2002 March; 163(1): 126-8. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11952261&dopt=Abstract

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Loss-of-function and residual channel activity of connexin26 mutations associated with non-syndromic deafness. Author(s): Bruzzone R, Veronesi V, Gomes D, Bicego M, Duval N, Marlin S, Petit C, D'Andrea P, White TW. Source: Febs Letters. 2003 January 2; 533(1-3): 79-88. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12505163&dopt=Abstract

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Macrothrombocytopenia and progressive deafness is due to a mutation in MYH9. Author(s): Mhatre AN, Kim Y, Brodie HA, Lalwani AK. Source: Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology. 2003 March; 24(2): 205-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12621333&dopt=Abstract

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Making sense of nonsyndromic deafness. Author(s): Smith RJ, Huygen PL. Source: Archives of Otolaryngology--Head & Neck Surgery. 2003 April; 129(4): 405-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12707186&dopt=Abstract

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Management of unilateral acoustic neuroma in a 72-year-old patient with contralateral congenital deafness. Author(s): Branch MP, Hirsch BE. Source: Otolaryngology and Head and Neck Surgery. 2002 November; 127(5): 483-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12447251&dopt=Abstract

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Midbrain deafness with normal brainstem auditory evoked potentials. Author(s): Johkura K. Source: Neurology. 2002 October 22; 59(8): 1293. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12391380&dopt=Abstract

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Molecular and functional effects of the T14709C point mutation in the mitochondrial DNA of a patient with maternally inherited diabetes and deafness. Author(s): Perucca-Lostanlen D, Taylor RW, Narbonne H, Mousson de Camaret B, Hayes CM, Saunieres A, Paquis-Flucklinger V, Turnbull DM, Vialettes B, Desnuelle C. Source: Biochimica Et Biophysica Acta. 2002 December 12; 1588(3): 210-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12393175&dopt=Abstract

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Molecular characterization of a 12q22-q24 deletion associated with congenital deafness: confirmation and refinement of the DFNA25 locus. Author(s): Petek E, Windpassinger C, Mach M, Rauter L, Scherer SW, Wagner K, Kroisel PM. Source: American Journal of Medical Genetics. 2003 March 1; 117A(2): 122-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12567408&dopt=Abstract

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Molecular diagnosis of deafness: impact of gene identification. Author(s): Usami S, Koda E, Tsukamoto K, Otsuka A, Yuge I, Asamura K, Abe S, Akita J, Namba A. Source: Audiology & Neuro-Otology. 2002 May-June; 7(3): 185-90. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12053143&dopt=Abstract

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Molecular diagnosis of neurosensory deafness: the gap between basic research and diagnostic application is increasing. Author(s): Van Camp G. Source: Acta Otorhinolaryngol Belg. 2002; 56(4): 337-40. Review. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12528250&dopt=Abstract

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Molecular mechanism of a frequent genetic form of deafness. Author(s): Michel V, Hardelin JP, Petit C. Source: The New England Journal of Medicine. 2003 August 14; 349(7): 716-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12917317&dopt=Abstract

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Morphometric changes in the cochlear nucleus in patients who had undergone cochlear implantation for bilateral profound deafness. Author(s): Chao TK, Burgess BJ, Eddington DK, Nadol JB Jr. Source: Hearing Research. 2002 December; 174(1-2): 196-205. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12433410&dopt=Abstract

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Mutation spectrum of the connexin 26 (GJB2) gene in Taiwanese patients with prelingual deafness. Author(s): Hwa HL, Ko TM, Hsu CJ, Huang CH, Chiang YL, Oong JL, Chen CC, Hsu CK. Source: Genetics in Medicine : Official Journal of the American College of Medical Genetics. 2003 May-June; 5(3): 161-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12792423&dopt=Abstract

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Mutations in COCH that result in non-syndromic autosomal dominant deafness (DFNA9) affect matrix deposition of cochlin. Author(s): Grabski R, Szul T, Sasaki T, Timpl R, Mayne R, Hicks B, Sztul E. Source: Human Genetics. 2003 October; 113(5): 406-16. Epub 2003 August 20. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12928864&dopt=Abstract

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Mutations in the alternatively spliced exons of USH1C cause non-syndromic recessive deafness. Author(s): Ouyang XM, Xia XJ, Verpy E, Du LL, Pandya A, Petit C, Balkany T, Nance WE, Liu XZ. Source: Human Genetics. 2002 July; 111(1): 26-30. Epub 2002 June 18. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12136232&dopt=Abstract

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Mutations of Cx26 gene (GJB2) for prelingual deafness in Taiwan. Author(s): Wang YC, Kung CY, Su MC, Su CC, Hsu HM, Tsai CC, Lin CC, Li SY. Source: European Journal of Human Genetics : Ejhg. 2002 August; 10(8): 495-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12111646&dopt=Abstract

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Mutations of MYO6 are associated with recessive deafness, DFNB37. Author(s): Ahmed ZM, Morell RJ, Riazuddin S, Gropman A, Shaukat S, Ahmad MM, Mohiddin SA, Fananapazir L, Caruso RC, Husnain T, Khan SN, Riazuddin S, Griffith AJ, Friedman TB, Wilcox ER. Source: American Journal of Human Genetics. 2003 May; 72(5): 1315-22. Epub 2003 April 08. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12687499&dopt=Abstract

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Myocardial dysfunction in maternally inherited diabetes and deafness. Author(s): Silveiro SP, Canani LH, Maia AL, Butany JW, Gross JL. Source: Diabetes Care. 2003 April; 26(4): 1323-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12663628&dopt=Abstract

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Nevus of Ota associated with ipsilateral deafness. Author(s): Alvarez-Cuesta CC, Raya-Aguado C, Vazquez-Lopez F, Garcia PB, PerezOliva N. Source: Journal of the American Academy of Dermatology. 2002 November; 47(5 Suppl): S257-9. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12399743&dopt=Abstract

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Non-syndromic autosomal-dominant deafness. Author(s): Petersen MB. Source: Clinical Genetics. 2002 July; 62(1): 1-13. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12123480&dopt=Abstract

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Nonsyndromic recessive deafness DFNB18 and Usher syndrome type IC are allelic mutations of USHIC. Author(s): Ahmed ZM, Smith TN, Riazuddin S, Makishima T, Ghosh M, Bokhari S, Menon PS, Deshmukh D, Griffith AJ, Riazuddin S, Friedman TB, Wilcox ER. Source: Human Genetics. 2002 June; 110(6): 527-31. Epub 2002 May 03. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12107438&dopt=Abstract

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Non-syndromic recessive deafness in Jordan: mapping of a new locus to chromosome 9q34.3 and prevalence of DFNB1 mutations. Author(s): Medlej-Hashim M, Mustapha M, Chouery E, Weil D, Parronaud J, Salem N, Delague V, Loiselet J, Lathrop M, Petit C, Megarbane A. Source: European Journal of Human Genetics : Ejhg. 2002 June; 10(6): 391-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12080392&dopt=Abstract

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Nonsyndromic sensorineural deafness associated with the A1555G mutation in the mitochondrial small subunit ribosomal RNA in a Balinese family. Author(s): Malik S, Sudoyo H, Sasmono T, Winata S, Arhya IN, Pramoonjago P, Sudana W, Marzuki S. Source: Journal of Human Genetics. 2003; 48(3): 119-24. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12624722&dopt=Abstract

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Novel mutations in GJB2 encoding connexin-26 in Japanese patients with keratitisichthyosis-deafness syndrome. Author(s): Yotsumoto S, Hashiguchi T, Chen X, Ohtake N, Tomitaka A, Akamatsu H, Matsunaga K, Shiraishi S, Miura H, Adachi J, Kanzaki T. Source: The British Journal of Dermatology. 2003 April; 148(4): 649-53. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12752120&dopt=Abstract

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Of specialty interest: publications of the National Institute on Deafness and Other Communication Disorders. Author(s): Thomas PC. Source: Orl Head Neck Nurs. 2002 Spring; 20(2): 26-30. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12055978&dopt=Abstract

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Origins and frequencies of SLC26A4 (PDS) mutations in east and south Asians: global implications for the epidemiology of deafness. Author(s): Park HJ, Shaukat S, Liu XZ, Hahn SH, Naz S, Ghosh M, Kim HN, Moon SK, Abe S, Tukamoto K, Riazuddin S, Kabra M, Erdenetungalag R, Radnaabazar J, Khan S, Pandya A, Usami SI, Nance WE, Wilcox ER, Riazuddin S, Griffith AJ. Source: Journal of Medical Genetics. 2003 April; 40(4): 242-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12676893&dopt=Abstract

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Otoancorin, an inner ear protein restricted to the interface between the apical surface of sensory epithelia and their overlying acellular gels, is defective in autosomal recessive deafness DFNB22. Author(s): Zwaenepoel I, Mustapha M, Leibovici M, Verpy E, Goodyear R, Liu XZ, Nouaille S, Nance WE, Kanaan M, Avraham KB, Tekaia F, Loiselet J, Lathrop M, Richardson G, Petit C. Source: Proceedings of the National Academy of Sciences of the United States of America. 2002 April 30; 99(9): 6240-5. Epub 2002 Apr 23. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11972037&dopt=Abstract

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Outcomes of hemisphericity questionnaires correlate with unilateral dichotic deafness. Author(s): Morton BE. Source: Brain and Cognition. 2002 June; 49(1): 63-72. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12027393&dopt=Abstract

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Pathogenesis of the deafness-associated A1555G mitochondrial DNA mutation. Author(s): Giordano C, Pallotti F, Walker WF, Checcarelli N, Musumeci O, Santorelli F, d'Amati G, Schon EA, DiMauro S, Hirano M, Davidson MM. Source: Biochemical and Biophysical Research Communications. 2002 April 26; 293(1): 521-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12054632&dopt=Abstract

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Pathogenic mutations but not polymorphisms in congenital and childhood onset autosomal recessive deafness disrupt the proteolytic activity of TMPRSS3. Author(s): Lee YJ, Park D, Kim SY, Park WJ. Source: Journal of Medical Genetics. 2003 August; 40(8): 629-31. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12920079&dopt=Abstract

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Performance of cochlear implant recipients with GJB2-related deafness. Author(s): Green GE, Scott DA, McDonald JM, Teagle HF, Tomblin BJ, Spencer LJ, Woodworth GG, Knutson JF, Gantz BJ, Sheffield VC, Smith RJ. Source: American Journal of Medical Genetics. 2002 May 1; 109(3): 167-70. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11977173&dopt=Abstract

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PET evidence of neuroplasticity in adult auditory cortex of postlingual deafness. Author(s): Lee JS, Lee DS, Oh SH, Kim CS, Kim JW, Hwang CH, Koo J, Kang E, Chung JK, Lee MC. Source: Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine. 2003 September; 44(9): 1435-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12960189&dopt=Abstract

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Postimplantation audition and educational attainment in children with prelingually acquired profound deafness. Author(s): Boothroyd A, Boothroyd-Turner D. Source: Ann Otol Rhinol Laryngol Suppl. 2002 May; 189: 79-84. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12018356&dopt=Abstract

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Preserved use of spatial cues for sound segregation in a case of spatial deafness. Author(s): Thiran AB, Clarke S. Source: Neuropsychologia. 2003; 41(9): 1254-61. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12753964&dopt=Abstract

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Prevalence of GJB2 mutations in prelingual deafness in the Greek population. Author(s): Pampanos A, Economides J, Iliadou V, Neou P, Leotsakos P, Voyiatzis N, Eleftheriades N, Tsakanikos M, Antoniadi T, Hatzaki A, Konstantopoulou I, Yannoukakos D, Gronskov K, Brondum-Nielsen K, Grigoriadou M, Gyftodimou J, Iliades T, Skevas A, Petersen MB. Source: International Journal of Pediatric Otorhinolaryngology. 2002 September 2; 65(2): 101-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12176179&dopt=Abstract

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PROMM and deafness: exclusion of ZNF9 as the disease gene in DFNA18 suggests a polygenic origin of the PROMM/DM2 phenotype. Author(s): Bonsch D, Neumann C, Lang-Roth R, Witte O, Lamprecht-Dinnesen A, Deufel T. Source: Clinical Genetics. 2003 January; 63(1): 73-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12519376&dopt=Abstract

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Selection for deafness? Author(s): Meyer CG, Amedofu GK, Brandner JM, Pohland D, Timmann C, Horstmann RD. Source: Nature Medicine. 2002 December; 8(12): 1332-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12457154&dopt=Abstract

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Subcellular localisation, secretion, and post-translational processing of normal cochlin, and of mutants causing the sensorineural deafness and vestibular disorder, DFNA9. Author(s): Robertson NG, Hamaker SA, Patriub V, Aster JC, Morton CC. Source: Journal of Medical Genetics. 2003 July; 40(7): 479-86. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12843317&dopt=Abstract

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Substitutions in the conserved C2C domain of otoferlin cause DFNB9, a form of nonsyndromic autosomal recessive deafness. Author(s): Mirghomizadeh F, Pfister M, Apaydin F, Petit C, Kupka S, Pusch CM, Zenner HP, Blin N. Source: Neurobiology of Disease. 2002 July; 10(2): 157-64. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12127154&dopt=Abstract

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Sudden bilateral simultaneous deafness with vertigo as a sole manifestation of vertebrobasilar insufficiency. Author(s): Lee H, Yi HA, Baloh RW. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2003 April; 74(4): 539-41. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12640087&dopt=Abstract

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Sudden deafness and anterior inferior cerebellar artery infarction. Author(s): Lee H, Sohn SI, Jung DK, Cho YW, Lim JG, Yi SD, Lee SR, Sohn CH, Baloh RW. Source: Stroke; a Journal of Cerebral Circulation. 2002 December; 33(12): 2807-12. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12468774&dopt=Abstract

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Sudden deafness and Lyme disease. Author(s): Lorenzi MC, Bittar RS, Pedalini ME, Zerati F, Yoshinari NH, Bento RF. Source: The Laryngoscope. 2003 February; 113(2): 312-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12567088&dopt=Abstract

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Sudden deafness as a manifestation of the rupture of a cerebral arteriovenous malformation. Author(s): Fernandez CA, Carceller MA, Garcia JR, Garcia CG, Alegria JB. Source: Otolaryngology and Head and Neck Surgery. 2003 April; 128(4): 592-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12707669&dopt=Abstract

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Sudden deafness: long-term follow-up and recurrence. Author(s): Furuhashi A, Matsuda K, Asahi K, Nakashima T. Source: Clinical Otolaryngology and Allied Sciences. 2002 December; 27(6): 458-63. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12472512&dopt=Abstract

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Sudden onset visual impairment and deafness in a patient with “long standing rheumatoid arthritis”. Author(s): Gaitonde S, Joshi VR. Source: J Assoc Physicians India. 2003 February; 51: 178-82. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12725263&dopt=Abstract

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Sudden unilateral deafness after bilateral knee replacement. Author(s): Phillips JS, Prinsley PR. Source: The Journal of Laryngology and Otology. 2003 April; 117(4): 310-1. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12816223&dopt=Abstract

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Test your knowledge: pathology quiz. Rash and deafness. Author(s): Ryder N. Source: Aust Fam Physician. 2002 November; 31(11): 1017-8. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12471959&dopt=Abstract

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The C66W mutation in the deafness dystonia peptide 1 (DDP1) affects the formation of functional DDP1.TIM13 complexes in the mitochondrial intermembrane space. Author(s): Hofmann S, Rothbauer U, Muhlenbein N, Neupert W, Gerbitz KD, Brunner M, Bauer MF. Source: The Journal of Biological Chemistry. 2002 June 28; 277(26): 23287-93. Epub 2002 April 15. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11956200&dopt=Abstract

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The detection of childhood deafness in Ayrshire. Author(s): Kubba H. Source: Health Bull (Edinb). 1999 November; 57(6): 399-405. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12811872&dopt=Abstract

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The genetics of deafness. Author(s): Nance WE. Source: Mental Retardation and Developmental Disabilities Research Reviews. 2003; 9(2): 109-19. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12784229&dopt=Abstract

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The neonatal variant of Bartter syndrome and deafness: preservation of renal function. Author(s): Shalev H, Ohali M, Kachko L, Landau D. Source: Pediatrics. 2003 September; 112(3 Pt 1): 628-33. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12949294&dopt=Abstract

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The roles of unconventional myosins in hearing and deafness. Author(s): Libby RT, Steel KP. Source: Essays Biochem. 2000; 35: 159-74. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12471897&dopt=Abstract

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The transmembrane serine protease (TMPRSS3) mutated in deafness DFNB8/10 activates the epithelial sodium channel (ENaC) in vitro. Author(s): Guipponi M, Vuagniaux G, Wattenhofer M, Shibuya K, Vazquez M, Dougherty L, Scamuffa N, Guida E, Okui M, Rossier C, Hancock M, Buchet K, Reymond A, Hummler E, Marzella PL, Kudoh J, Shimizu N, Scott HS, Antonarakis SE, Rossier BC. Source: Human Molecular Genetics. 2002 November 1; 11(23): 2829-36. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12393794&dopt=Abstract

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Transcranial contralateral cochlear stimulation in unilateral deafness. Author(s): Wazen JJ, Spitzer JB, Ghossaini SN, Fayad JN, Niparko JK, Cox K, Brackmann DE, Soli SD. Source: Otolaryngology and Head and Neck Surgery. 2003 September; 129(3): 248-54. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12958575&dopt=Abstract

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Two large French pedigrees with non syndromic sensorineural deafness and the mitochondrial DNA T7511C mutation: evidence for a modulatory factor. Author(s): Chapiro E, Feldmann D, Denoyelle F, Sternberg D, Jardel C, Eliot MM, Bouccara D, Weil D, Garabedian EN, Couderc R, Petit C, Marlin S. Source: European Journal of Human Genetics : Ejhg. 2002 December; 10(12): 851-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12461693&dopt=Abstract

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Vascular defects and sensorineural deafness in a mouse model of Norrie disease. Author(s): Rehm HL, Zhang DS, Brown MC, Burgess B, Halpin C, Berger W, Morton CC, Corey DP, Chen ZY. Source: The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. 2002 June 1; 22(11): 4286-92. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12040033&dopt=Abstract

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Vestibular evoked myogenic potentials are intact after sudden deafness. Author(s): Wu CC, Young YH. Source: Ear and Hearing. 2002 June; 23(3): 235-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12072615&dopt=Abstract

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WFS1 mutations in Spanish patients with diabetes mellitus and deafness. Author(s): Domenech E, Gomez-Zaera M, Nunes V. Source: European Journal of Human Genetics : Ejhg. 2002 July; 10(7): 421-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12107816&dopt=Abstract

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X-linked mixed deafness syndrome with congenital fixation of the stapedial footplate and perilymphatic gusher (DFN3). Author(s): Cremers CW, Snik AF, Huygen PL, Joosten FB, Cremers FP. Source: Advances in Oto-Rhino-Laryngology. 2002; 61: 161-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12408080&dopt=Abstract

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CHAPTER 2. NUTRITION AND DEAFNESS Overview In this chapter, we will show you how to find studies dedicated specifically to nutrition and deafness.

Finding Nutrition Studies on Deafness The National Institutes of Health’s Office of Dietary Supplements (ODS) offers a searchable bibliographic database called the IBIDS (International Bibliographic Information on Dietary Supplements; National Institutes of Health, Building 31, Room 1B29, 31 Center Drive, MSC 2086, Bethesda, Maryland 20892-2086, Tel: 301-435-2920, Fax: 301-480-1845, E-mail: [email protected]). The IBIDS contains over 460,000 scientific citations and summaries about dietary supplements and nutrition as well as references to published international, scientific literature on dietary supplements such as vitamins, minerals, and botanicals.7 The IBIDS includes references and citations to both human and animal research studies. As a service of the ODS, access to the IBIDS database is available free of charge at the following Web address: http://ods.od.nih.gov/databases/ibids.html. After entering the search area, you have three choices: (1) IBIDS Consumer Database, (2) Full IBIDS Database, or (3) Peer Reviewed Citations Only. Now that you have selected a database, click on the “Advanced” tab. An advanced search allows you to retrieve up to 100 fully explained references in a comprehensive format. Type “deafness” (or synonyms) into the search box, and click “Go.” To narrow the search, you can also select the “Title” field.

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Adapted from http://ods.od.nih.gov. IBIDS is produced by the Office of Dietary Supplements (ODS) at the National Institutes of Health to assist the public, healthcare providers, educators, and researchers in locating credible, scientific information on dietary supplements. IBIDS was developed and will be maintained through an interagency partnership with the Food and Nutrition Information Center of the National Agricultural Library, U.S. Department of Agriculture.

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The following information is typical of that found when using the “Full IBIDS Database” to search for “deafness” (or a synonym): •

A human mitochondrial GTP binding protein related to tRNA modification may modulate phenotypic expression of the deafness-associated mitochondrial 12S rRNA mutation. Author(s): Division and Program in Human Genetics and Center for Hearing and Deafness Research, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229-3039, USA. Source: Li, X Guan, M X Mol-Cell-Biol. 2002 November; 22(21): 7701-11 0270-7306

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Acupuncture treatment of sudden deafness. Source: Liu, Y L J-Tradit-Chin-Med. 1987 March; 7(1): 27-8 0254-6272

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Atypical muscle pathology and a survey of cis-mutations in deaf patients harboring a 1555 A-to-G point mutation in the mitochondrial ribosomal RNA gene. Author(s): Department of Otolaryngology, University of Tokyo, Tokyo, Japan. Source: Yamasoba, Tatsuya Goto, Yu ichi Oka, Yoshitomo Nishino, Ichizo Tsukuda, Katsunori Nonaka, Ikuya Neuromuscul-Disord. 2002 June; 12(5): 506-12 0960-8966

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Complete biotinidase deficiency presenting as reversible progressive ataxia and sensorineural deafness. Author(s): Department of Pediatrics, The Ohio State University, Columbus, USA. [email protected] Source: Tsao, C Y Kien, C L J-Child-Neurol. 2002 February; 17(2): 146 0883-0738

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Could antioxidant therapy reduce the incidence of deafness following bacterial meningitis? Author(s): Department of Pathology, Houston Medical Center, Warner Robins, GA 31093, USA. Source: Maurizi, C P Med-Hypotheses. 1999 January; 52(1): 85-7 0306-9877

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Deafness induced up-regulation of GluR2/3 and NR1 in the spiral ganglion cells of the rat cochlea. Author(s): Department of Otolaryngology and Sensory Organ Surgery, Osaka University Graduate School of Medicine, Suita, Japan. Source: Hasegawa, T Doi, K Fuse, Y Fujii, K Uno, Y Nishimura, H Kubo, T Neuroreport. 2000 August 3; 11(11): 2515-9 0959-4965

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Dextran-induced pulmonary edema in patients with sudden deafness. Author(s): Department of Otolaryngology, National Taiwan University Hospital, Taipei. Source: Kuo, S T Hsu, W C Young, Y H Otol-Neurotol. 2002 September; 23(5): 661-4 1531-7129

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Early bilateral deafening prevents calretinin up-regulation in the dorsal cortex of the inferior colliculus of aged CBA/CaJ mice. Author(s): Department of Surggery, University of Rochester School of Medicine and Dentistry, NY 14642-8629, USA. [email protected] Source: Zettel, M L O'Neill, W E Trang, T T Frisina, R D Hear-Res. 2001 August; 158(1-2): 131-8 0378-5955

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Electronystagmographic findings and recovery of cochlear and vestibular function in patients suffering from sudden deafness with a special reference to the effect of anticoagulation. Author(s): Department of Otorhinolaryngology, University Hospital of Turku, Finland. Source: Laurikainen, E Aantaa, E Kallinen, J Audiology. 1989; 28(5): 262-7 0020-6091

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Evaluation of hearing recovery in patients with sudden deafness. Author(s): Department of Otolaryngology, School of Medicine, Keio University, Tokyo, Japan. Source: Yamamoto, M Kanzaki, J Ogawa, K Ogawa, S Tsuchihashi, N Acta-OtolaryngolSuppl. 1994; 51437-40 0365-5237

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Evaluation of prostaglandin E1 therapy for sudden deafness. Author(s): Department of Otorhinolaryngology, Nagoya University School of Medicine, Japan. Source: Nakashima, T Kuno, K Yanagita, N Laryngoscope. 1989 May; 99(5): 542-6 0023852X

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Functional analysis of connexin-26 mutants associated with hereditary recessive deafness. Author(s): Department of Physiology, Chang Gung University School of Medicine, Kwei-San, Tao-Yuan, Taiwan, Republic of China. [email protected] Source: Wang, H L Chang, W T Li, A H Yeh, T H Wu, C Y Chen, M S Huang, P C JNeurochem. 2003 February; 84(4): 735-42 0022-3042

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Ginkgo biloba extract EGb 761 or pentoxifylline for the treatment of sudden deafness: a randomized, reference-controlled, double-blind study. Author(s): Department of Otolaryngology, University of Heidelberg, Germany. [email protected] Source: Reisser, C H Weidauer, H Acta-Otolaryngol. 2001 July; 121(5): 579-84 0001-6489

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Hearing recovery in sudden deafness patients using a modified defibrinogenation therapy. Author(s): Department of Otolaryngology, Osaka University Medical School, Japan. Source: Shiraishi, T Kubo, T Okumura, S Naramura, H Nishimura, M Okusa, M Matsunaga, T Acta-Otolaryngol-Suppl. 1993; 50146-50 0365-5237

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Human connexin26 (GJB2) deafness mutations affect the function of gap junction channels at different levels of protein expression. Author(s): Institut fur Genetik, Universitat Bonn, Romerstrasse 164, 53117 Bonn, Germany. Source: Thonnissen, E Rabionet, R Arbones, M L Estivill, X Willecke, K Ott, T HumGenet. 2002 August; 111(2): 190-7 0340-6717

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Hypogonadotrophic hypogonadism associated with prelingual deafness due to a connexin 26 gene mutation. Author(s): Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hjpital d'Enfants Armand Trousseau, Paris, France. [email protected] Source: Houang, M Gourmelen, M Moatti, L Le, Bouc Y Garabedian, E N Denoyelle, F JPediatr-Endocrinol-Metab. 2002 February; 15(2): 219-23

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Hypokalemic salt-losing tubulopathy with chronic renal failure and sensorineural deafness. Author(s): Departments of Pediatrics, Marburg, Germany. Source: Jeck, N Reinalter, S C Henne, T Marg, W Mallmann, R Pasel, K Vollmer, M Klaus, G Leonhardt, A Seyberth, H W Konrad, M Pediatrics. 2001 July; 108(1): E5 10984275

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Idiopathic sudden sensorineural deafness--an approach to the problem. Source: Khanijow, V K Raman, R Singapore-Med-J. 1988 February; 29(1): 76-7 0037-5675

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Influence of neurotrophins on the synaptogenesis of inner hair cells in the deaf Bronx waltzer (bv) mouse organ of Corti in culture. Author(s): Neurology Department, University of Wisconsin, 1300 University Avenue, Room 75 MSC, Madison 53706, USA. [email protected] Source: Sobkowicz, H M August, B K Slapnick, S M Int-J-Dev-Neurosci. 2002 November; 20(7): 537-54 0736-5748

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Initial steroid hormone dose in the treatment of idiopathic sudden deafness. Author(s): Department of Otolaryngology, Head Neck Surgery, Kumamoto University School of Medicine, Japan. Source: Minoda, R Masuyama, K Habu, K Yumoto, E Am-J-Otol. 2000 November; 21(6): 819-25 0192-9763

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Isolation and characterization of the putative nuclear modifier gene MTO1 involved in the pathogenesis of deafness-associated mitochondrial 12 S rRNA A1555G mutation. Author(s): Division of Human Genetics, Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH 45229, USA. Source: Li, Xiaoming Li, Ronghua Lin, Xinhua Guan, Min Xin J-Biol-Chem. 2002 July 26; 277(30): 27256-64 0021-9258

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Japanese and Western diet and risk of idiopathic sudden deafness: a case-control study using pooled controls. Author(s): Department of Hygiene, Hamamatsu University School of Medicine, Japan. [email protected] Source: Nakamura, M Whitlock, G Aoki, N Nakashima, T Hoshino, T Yokoyama, T Morioka, S Kawamura, T Tanaka, H Hashimoto, T Ohno, Y Int-J-Epidemiol. 2001 June; 30(3): 608-15 0300-5771

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Mutations in SLC19A2 cause thiamine-responsive megaloblastic anaemia associated with diabetes mellitus and deafness. Author(s): Department of Genetics, Tamkin Human Molecular Genetics Research Facility, Technion-Israel Institute of Technology, Bruce Rappaport Faculty of Medicine, Haifa. Source: Labay, V Raz, T Baron, D Mandel, H Williams, H Barrett, T Szargel, R McDonald, L Shalata, A Nosaka, K Gregory, S Cohen, N Nat-Genet. 1999 July; 22(3): 300-4 1061-4036

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Preventive attitudes and beliefs of deaf and hard-of-hearing individuals. Author(s): Medical College of Ohio, Toledo, USA. Source: Tamaskar, P Malia, T Stern, C Gorenflo, D Meador, H Zazove, P Arch-Fam-Med. 2000 June; 9(6): 518-25, discussion 526 1063-3987

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Probational treatment of sudden deafness with prostacyclin: a pilot study. Author(s): Klinik and Poliklinik fur Hals-Nasen-Ohren-Heilkunde, Universitat zu Koln, F.R.G. Source: Michel, O Matthias, R Auris-Nasus-Larynx. 1991; 18(2): 115-23 0385-8146

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Protection and regrowth of the auditory nerve after deafness: neurotrophins, antioxidants and depolarization are effective in vivo. Author(s): University of Michigan, Kresge Hearing Research Institute, Ann Arbor, MI 48109-0506, USA. [email protected] Source: Miller, Josef M Miller, Amy L Yamagata, Takahiko Bredberg, Goran Altschuler, Richard A Audiol-Neurootol. 2002 May-June; 7(3): 175-9 1420-3030

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Reversible deafness caused by biotinidase deficiency. Author(s): Department Pediatrics, Schneider Children's Medical Center of Israel, Sackler School of Medicine, Tel Aviv University, Petah Tiqva, Israel. Source: Straussberg, R Saiag, E Harel, L Korman, S H Amir, J Pediatr-Neurol. 2000 September; 23(3): 269-70 0887-8994

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Risk factors for sudden deafness: a case-control study. Author(s): Department of Otorhinolaryngology, Nagoya University School of Medicine, Japan. Source: Nakashima, T Tanabe, T Yanagita, N Wakai, K Ohno, Y Auris-Nasus-Larynx. 1997 July; 24(3): 265-70 0385-8146

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Senile deafness and metabolic bone disease. Author(s): Department of Neurotology, Sapporo City General Hospital, Japan. Source: Yamazaki, T Ogawa, K Imoto, T Hayashi, N Kozaki, H Am-J-Otol. 1988 September; 9(5): 376-82 0192-9763

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Sudden deafness: a retrospective evaluation of dextran therapy. Author(s): Department of Otorhinolaryngology, University Hospital, Uppsala, Sweden. Source: Hultcrantz, E Stenquist, M Lyttkens, L ORL-J-Otorhinolaryngol-Relat-Spec. 1994 May-June; 56(3): 137-42 0301-1569

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The effects of coenzyme Q10 treatment on maternally inherited diabetes mellitus and deafness, and mitochondrial DNA 3243 (A to G) mutation. Author(s): Third Department of Internal Medicine, Tohoku University School of Medicine, Sendai, Japan. Source: Suzuki, S Hinokio, Y Ohtomo, M Hirai, M Hirai, A Chiba, M Kasuga, S Satoh, Y Akai, H Toyota, T Diabetologia. 1998 May; 41(5): 584-8 0012-186X

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The efficacy of Lasix-vitamin therapy (L-V therapy) for sudden deafness and other sensorineural hearing loss. Author(s): Department of Otolaryngology, Osaka City University Medical School, Japan. Source: Konishi, K Nakai, Y Yamane, H Acta-Otolaryngol-Suppl. 1991; 48678-91 03655237

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The gene mutated in thiamine-responsive anaemia with diabetes and deafness (TRMA) encodes a functional thiamine transporter. Author(s): Division of Hematology, Children's Hospital, Dana Farber Cancer Institute, and Harvard Medical School, Boston, Massachusetts 02115, USA. Source: Fleming, J C Tartaglini, E Steinkamp, M P Schorderet, D F Cohen, N Neufeld, E J Nat-Genet. 1999 July; 22(3): 305-8 1061-4036

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The syndrome of renal tubular acidosis and nerve deafness. Discordant manifestations in dizygotic twin brothers. Author(s): Department of Paediatrics, Hospital Universitario NS Covadonga, University of Oviedo, Asturias, Spain. Source: Santos, F Rey, C Malaga, S Rodriguez, L M Orejas, G Pediatr-Nephrol. 1991 March; 5(2): 235-7 0931-041X

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Treatment of 100 cases of nerve deafness with injectio radix salviae miltiorrhizae. Author(s): Dongzhimen Hospital of Beijing College of TCM. Source: Hu, Y Ge, Y Zhang, Y Liu, J Li, X Liu, Q J-Tradit-Chin-Med. 1992 December; 12(4): 256-8 0254-6272

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Federal Resources on Nutrition In addition to the IBIDS, the United States Department of Health and Human Services (HHS) and the United States Department of Agriculture (USDA) provide many sources of information on general nutrition and health. Recommended resources include: •

healthfinder®, HHS’s gateway to health information, including diet and nutrition: http://www.healthfinder.gov/scripts/SearchContext.asp?topic=238&page=0

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The United States Department of Agriculture’s Web site dedicated to nutrition information: www.nutrition.gov

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The Food and Drug Administration’s Web site for federal food safety information: www.foodsafety.gov

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The National Action Plan on Overweight and Obesity sponsored by the United States Surgeon General: http://www.surgeongeneral.gov/topics/obesity/

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The Center for Food Safety and Applied Nutrition has an Internet site sponsored by the Food and Drug Administration and the Department of Health and Human Services: http://vm.cfsan.fda.gov/

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Center for Nutrition Policy and Promotion sponsored by the United States Department of Agriculture: http://www.usda.gov/cnpp/

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Food and Nutrition Information Center, National Agricultural Library sponsored by the United States Department of Agriculture: http://www.nal.usda.gov/fnic/

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Food and Nutrition Service sponsored by the United States Department of Agriculture: http://www.fns.usda.gov/fns/

Additional Web Resources A number of additional Web sites offer encyclopedic information covering food and nutrition. The following is a representative sample: •

AOL: http://search.aol.com/cat.adp?id=174&layer=&from=subcats

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Family Village: http://www.familyvillage.wisc.edu/med_nutrition.html

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Google: http://directory.google.com/Top/Health/Nutrition/

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Healthnotes: http://www.healthnotes.com/

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Open Directory Project: http://dmoz.org/Health/Nutrition/

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Yahoo.com: http://dir.yahoo.com/Health/Nutrition/

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WebMDHealth: http://my.webmd.com/nutrition

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WholeHealthMD.com: http://www.wholehealthmd.com/reflib/0,1529,00.html

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The following is a specific Web list relating to deafness; please note that any particular subject below may indicate either a therapeutic use, or a contraindication (potential danger), and does not reflect an official recommendation: •

Vitamins Vitamin D Source: Healthnotes, Inc.; www.healthnotes.com

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CHAPTER 3. ALTERNATIVE MEDICINE AND DEAFNESS Overview In this chapter, we will begin by introducing you to official information sources on complementary and alternative medicine (CAM) relating to deafness. At the conclusion of this chapter, we will provide additional sources.

National Center for Complementary and Alternative Medicine The National Center for Complementary and Alternative Medicine (NCCAM) of the National Institutes of Health (http://nccam.nih.gov/) has created a link to the National Library of Medicine’s databases to facilitate research for articles that specifically relate to deafness and complementary medicine. To search the database, go to the following Web site: http://www.nlm.nih.gov/nccam/camonpubmed.html. Select “CAM on PubMed.” Enter “deafness” (or synonyms) into the search box. Click “Go.” The following references provide information on particular aspects of complementary and alternative medicine that are related to deafness: •

“Deaf hearing”: unacknowledged detection of auditory stimuli in a patient with cerebral deafness. Author(s): Garde MM, Cowey A. Source: Cortex. 2000 February; 36(1): 71-80. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10728898&dopt=Abstract

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A deaf woman learning to control her excessive vocal loudness through a portable feedback system. Author(s): Lancioni GE, Markus S. Source: Percept Mot Skills. 1999 June; 88(3 Pt 2): 1347-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10485120&dopt=Abstract

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A morphometric analysis of auditory brain regions in congenitally deaf adults. Author(s): Emmorey K, Allen JS, Bruss J, Schenker N, Damasio H.

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Source: Proceedings of the National Academy of Sciences of the United States of America. 2003 August 19; 100(17): 10049-54. Epub 2003 Aug 06. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12904582&dopt=Abstract •

A new method of partial deafness treatment. Author(s): Skarzynski H, Lorens A, Piotrowska A. Source: Medical Science Monitor : International Medical Journal of Experimental and Clinical Research. 2003 April; 9(4): Cs20-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12709676&dopt=Abstract

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Brain activities of prelingually and postlingually deafened children using cochlear implants. Author(s): Fujiki N, Naito Y, Hirano S, Kojima H, Shiomi Y, Nishizawa S, Honjo I. Source: Ann Otol Rhinol Laryngol Suppl. 2000 December; 185: 12-4. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11140983&dopt=Abstract

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Confirmation of deafness in infancy. Author(s): Watkin PM, Baldwin M. Source: Archives of Disease in Childhood. 1999 November; 81(5): 380-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10519708&dopt=Abstract

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Congenital deafness and sinoatrial node dysfunction in mice lacking class D L-type Ca2+ channels. Author(s): Platzer J, Engel J, Schrott-Fischer A, Stephan K, Bova S, Chen H, Zheng H, Striessnig J. Source: Cell. 2000 July 7; 102(1): 89-97. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10929716&dopt=Abstract

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Cortical representation of hearing restoration in patients with sudden deafness. Author(s): Suzuki M, Kouzaki H, Nishida Y, Shiino A, Ito R, Kitano H. Source: Neuroreport. 2002 October 7; 13(14): 1829-32. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12395134&dopt=Abstract

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Deafness and cerebral plasticity. Author(s): Cordes M, Wszolek ZK. Source: Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine. 2003 September; 44(9): 1440-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12960190&dopt=Abstract

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Deafness disrupts chloride transporter function and inhibitory synaptic transmission. Author(s): Vale C, Schoorlemmer J, Sanes DH.

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Source: The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. 2003 August 20; 23(20): 7516-24. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12930790&dopt=Abstract •

Does the addition of hyperbaric oxygen therapy to the conventional treatment modalities influence the outcome of sudden deafness? Author(s): Aslan I, Oysu C, Veyseller B, Baserer N. Source: Otolaryngology and Head and Neck Surgery. 2002 February; 126(2): 121-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11870340&dopt=Abstract

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Downregulation of otospiralin, a novel inner ear protein, causes hair cell degeneration and deafness. Author(s): Delprat B, Boulanger A, Wang J, Beaudoin V, Guitton MJ, Venteo S, Dechesne CJ, Pujol R, Lavigne-Rebillard M, Puel JL, Hamel CP. Source: The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. 2002 March 1; 22(5): 1718-25. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11880501&dopt=Abstract

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Exploring the perceived world of the deaf-blind: on the development of an instrument. Author(s): Ronnberg J, Samuelsson E, Borg E. Source: International Journal of Audiology. 2002 March; 41(2): 136-43. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12212859&dopt=Abstract

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Feeling vibrations: enhanced tactile sensitivity in congenitally deaf humans. Author(s): Levanen S, Hamdorf D. Source: Neuroscience Letters. 2001 March 23; 301(1): 75-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11239720&dopt=Abstract

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Functional differentiation of the auditory association area in prelingually deaf subjects. Author(s): Hirano S, Naito Y, Kojima H, Honjo I, Inoue M, Shoji K, Tateya I, Fujiki N, Nishizawa S, Konishi J. Source: Auris, Nasus, Larynx. 2000 October; 27(4): 303-10. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10996488&dopt=Abstract

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Functional magnetic resonance imaging may avoid misdiagnosis of cochleovestibular nerve aplasia in congenital deafness. Author(s): Thai-Van H, Fraysse B, Berry I, Berges C, Deguine O, Honegger A, Sevely A, Ibarrola D, Van HT.

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Source: The American Journal of Otology. 2000 September; 21(5): 663-70. Erratum In: Am J Otol 2002 May; 23(3): 412. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10993455&dopt=Abstract •

Ginkgo biloba extract EGb 761 or pentoxifylline for the treatment of sudden deafness: a randomized, reference-controlled, double-blind study. Author(s): Reisser CH, Weidauer H. Source: Acta Oto-Laryngologica. 2001 July; 121(5): 579-84. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11583389&dopt=Abstract

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Hearing after congenital deafness: central auditory plasticity and sensory deprivation. Author(s): Kral A, Hartmann R, Tillein J, Heid S, Klinke R. Source: Cerebral Cortex (New York, N.Y. : 1991). 2002 August; 12(8): 797-807. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12122028&dopt=Abstract

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Hyperbaric oxygen therapy for sudden deafness. Author(s): Nakashima T, Fukuta S, Yanagita N. Source: Advances in Oto-Rhino-Laryngology. 1998; 54: 100-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9547880&dopt=Abstract

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Impact of early deafness and early exposure to sign language on the cerebral organization for motion processing. Author(s): Bavelier D, Brozinsky C, Tomann A, Mitchell T, Neville H, Liu G. Source: The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. 2001 November 15; 21(22): 8931-42. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11698604&dopt=Abstract

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Lack of deafness in Crigler-Najjar syndrome type 1: a patient survey. Author(s): Suresh G, Lucey JF. Source: Pediatrics. 1997 November; 100(5): E9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9347003&dopt=Abstract

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Lesion site in sudden deafness: study with electrocochleography and transiently evoked otoacoustic emission. Author(s): Ota Y, Oda M. Source: Acta Oto-Laryngologica. 1999 January; 119(1): 33-41. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10219382&dopt=Abstract

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Neuro-functional imaging and profound deafness. Author(s): Truy E.

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Source: International Journal of Pediatric Otorhinolaryngology. 1999 February 15; 47(2): 131-6. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10206360&dopt=Abstract •

Neurotrophic factor intervention restores auditory function in deafened animals. Author(s): Shinohara T, Bredberg G, Ulfendahl M, Pyykko I, Olivius NP, Kaksonen R, Lindstrom B, Altschuler R, Miller JM. Source: Proceedings of the National Academy of Sciences of the United States of America. 2002 February 5; 99(3): 1657-60. Epub 2002 January 29. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11818566&dopt=Abstract

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Of kittens and kids: altered cortical maturation following profound deafness and cochlear implant use. Author(s): Ponton CW, Eggermont JJ. Source: Audiology & Neuro-Otology. 2001 November-December; 6(6): 363-80. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11847464&dopt=Abstract

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PET evidence of neuroplasticity in adult auditory cortex of postlingual deafness. Author(s): Lee JS, Lee DS, Oh SH, Kim CS, Kim JW, Hwang CH, Koo J, Kang E, Chung JK, Lee MC. Source: Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine. 2003 September; 44(9): 1435-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12960189&dopt=Abstract

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Prolonged deafness limits auditory system developmental plasticity: evidence from an evoked potentials study in children with cochlear implants. Author(s): Ponton CW, Moore JK, Eggermont JJ. Source: Scand Audiol Suppl. 1999; 51: 13-22. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10803910&dopt=Abstract

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Results of total deafness treatment in young pre- and postlingually deafened children. Author(s): Szuchnik J, Skarzynski H, Geremek A, Zawadzki R. Source: Scand Audiol Suppl. 2001; (52): 42-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11318479&dopt=Abstract

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Sign language activated the auditory cortex of a congenitally deaf subject: revealed by positron emission tomography. Author(s): Nishimura H, Doi K, Iwaki T, Hashikawa K, Nishimura T, Kubo T. Source: Advances in Oto-Rhino-Laryngology. 2000; 57: 60-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11892216&dopt=Abstract

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Targeted disruption of otog results in deafness and severe imbalance. Author(s): Simmler MC, Cohen-Salmon M, El-Amraoui A, Guillaud L, Benichou JC, Petit C, Panthier JJ. Source: Nature Genetics. 2000 February; 24(2): 139-43. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10655058&dopt=Abstract

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The effect of bilateral deafness on excitatory and inhibitory synaptic strength in the inferior colliculus. Author(s): Vale C, Sanes DH. Source: The European Journal of Neuroscience. 2002 December; 16(12): 2394-404. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12492434&dopt=Abstract

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The effects of congenital deafness on auditory nerve synapses and globular bushy cells in cats. Author(s): Redd EE, Pongstaporn T, Ryugo DK. Source: Hearing Research. 2000 September; 147(1-2): 160-74. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10962182&dopt=Abstract

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The effects of congenital deafness on auditory nerve synapses: Type I and Type II multipolar cells in the anteroventral cochlear nucleus of cats. Author(s): Redd EE, Cahill HB, Pongstaporn T, Ryugo DK. Source: Journal of the Association for Research in Otolaryngology : Jaro. 2002 December; 3(4): 403-17. Epub 2002 March 26. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12486596&dopt=Abstract

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The neonatal variant of Bartter syndrome and deafness: preservation of renal function. Author(s): Shalev H, Ohali M, Kachko L, Landau D. Source: Pediatrics. 2003 September; 112(3 Pt 1): 628-33. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12949294&dopt=Abstract

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The neural correlates of 'deaf-hearing' in man: conscious sensory awareness enabled by attentional modulation. Author(s): Engelien A, Huber W, Silbersweig D, Stern E, Frith CD, Doring W, Thron A, Frackowiak RS. Source: Brain; a Journal of Neurology. 2000 March; 123 Pt 3: 532-45. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10686176&dopt=Abstract

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The pattern of hearing impairment among schoolboys in an Institute for deaf subjects. Author(s): Abolfotouh MA, Al-Ghamdi SA.

Alternative Medicine 125

Source: Saudi Med J. 2000 September; 21(9): 873-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11376367&dopt=Abstract •

Transcranial contralateral cochlear stimulation in unilateral deafness. Author(s): Wazen JJ, Spitzer JB, Ghossaini SN, Fayad JN, Niparko JK, Cox K, Brackmann DE, Soli SD. Source: Otolaryngology and Head and Neck Surgery. 2003 September; 129(3): 248-54. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12958575&dopt=Abstract

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Transient deafness due to temperature-sensitive auditory neuropathy. Author(s): Starr A, Sininger Y, Winter M, Derebery MJ, Oba S, Michalewski HJ. Source: Ear and Hearing. 1998 June; 19(3): 169-79. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9657592&dopt=Abstract

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Ultrasound activates the auditory cortex of profoundly deaf subjects. Author(s): Imaizumi S, Hosoi H, Sakaguchi T, Watanabe Y, Sadato N, Nakamura S, Waki A, Yonekura Y. Source: Neuroreport. 2001 March 5; 12(3): 583-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11234768&dopt=Abstract

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Use of audiovisual information in speech perception by prelingually deaf children with cochlear implants: a first report. Author(s): Lachs L, Pisoni DB, Kirk KI. Source: Ear and Hearing. 2001 June; 22(3): 236-51. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11409859&dopt=Abstract

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Vascular defects and sensorineural deafness in a mouse model of Norrie disease. Author(s): Rehm HL, Zhang DS, Brown MC, Burgess B, Halpin C, Berger W, Morton CC, Corey DP, Chen ZY. Source: The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. 2002 June 1; 22(11): 4286-92. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12040033&dopt=Abstract

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Visual contrast sensitivity in deaf versus hearing populations: exploring the perceptual consequences of auditory deprivation and experience with a visual language. Author(s): Finney EM, Dobkins KR. Source: Brain Research. Cognitive Brain Research. 2001 March; 11(1): 171-83. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11240120&dopt=Abstract

126 Deafness

Additional Web Resources A number of additional Web sites offer encyclopedic information covering CAM and related topics. The following is a representative sample: •

Alternative Medicine Foundation, Inc.: http://www.herbmed.org/

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AOL: http://search.aol.com/cat.adp?id=169&layer=&from=subcats

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Chinese Medicine: http://www.newcenturynutrition.com/

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drkoop.com: http://www.drkoop.com/InteractiveMedicine/IndexC.html

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Family Village: http://www.familyvillage.wisc.edu/med_altn.htm

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Google: http://directory.google.com/Top/Health/Alternative/

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Healthnotes: http://www.healthnotes.com/

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MedWebPlus: http://medwebplus.com/subject/Alternative_and_Complementary_Medicine

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Open Directory Project: http://dmoz.org/Health/Alternative/

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HealthGate: http://www.tnp.com/

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WebMDHealth: http://my.webmd.com/drugs_and_herbs

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WholeHealthMD.com: http://www.wholehealthmd.com/reflib/0,1529,00.html

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Yahoo.com: http://dir.yahoo.com/Health/Alternative_Medicine/

The following is a specific Web list relating to deafness; please note that any particular subject below may indicate either a therapeutic use, or a contraindication (potential danger), and does not reflect an official recommendation: •

General Overview Mumps Source: Integrative Medicine Communications; www.drkoop.com Rubella Source: Integrative Medicine Communications; www.drkoop.com

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Alternative Therapy Chiropractic Source: Integrative Medicine Communications; www.drkoop.com Chiropractic Source: WholeHealthMD.com, LLC.; www.wholehealthmd.com Hyperlink: http://www.wholehealthmd.com/refshelf/substances_view/0,1525,681,00.html

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•

Chinese Medicine Bushen Yinao Pian Alternative names: Bushen Yinao Tablets Source: Pharmacopoeia Commission of the Ministry of Health, People's Republic of China Hyperlink: http://www.newcenturynutrition.com/cgilocal/patent_herbs_db/db.cgi?db=default&Chinese=Bushen%20Yinao%20Pian&m h=10&sb=---&view_records=View+Records

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Herbs and Supplements Elder Source: The Canadian Internet Directory for Holistic Help, WellNet, Health and Wellness Network; www.wellnet.ca Ginkgo Alternative names: Ginkgo biloba Source: Alternative Medicine Foundation, Inc.; www.amfoundation.org Ginkgo Source: The Canadian Internet Directory for Holistic Help, WellNet, Health and Wellness Network; www.wellnet.ca Ginkgo Biloba Source: Healthnotes, Inc.; www.healthnotes.com

General References A good place to find general background information on CAM is the National Library of Medicine. It has prepared within the MEDLINEplus system an information topic page dedicated to complementary and alternative medicine. To access this page, go to the MEDLINEplus site at http://www.nlm.nih.gov/medlineplus/alternativemedicine.html. This Web site provides a general overview of various topics and can lead to a number of general sources.

129

CHAPTER 4. DISSERTATIONS ON DEAFNESS Overview In this chapter, we will give you a bibliography on recent dissertations relating to deafness. We will also provide you with information on how to use the Internet to stay current on dissertations. IMPORTANT NOTE: When following the search strategy described below, you may discover non-medical dissertations that use the generic term “deafness” (or a synonym) in their titles. To accurately reflect the results that you might find while conducting research on deafness, we have not necessarily excluded non-medical dissertations in this bibliography.

Dissertations on Deafness ProQuest Digital Dissertations, the largest archive of academic dissertations available, is located at the following Web address: http://wwwlib.umi.com/dissertations. From this archive, we have compiled the following list covering dissertations devoted to deafness. You will see that the information provided includes the dissertation’s title, its author, and the institution with which the author is associated. The following covers recent dissertations found when using this search procedure: •

A Comparative Study of Leader Behavior among Deaf and Hearing Supervisors by Sutcliffe, Ronald Eugene, Phd from University of Maryland College Park, 1980, 145 pages http://wwwlib.umi.com/dissertations/fullcit/8117384

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A Comparative Study of the Attitudes toward International Students As a Function of Selected Characteristics of Deaf College Preparatory Students (social Distance) by Lewis, Nancy Jones, Phd from The American University, 1993, 96 pages http://wwwlib.umi.com/dissertations/fullcit/9400718

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A Comparison of the Opinions of Deaf and Hearing Professionals Regarding the Research Needs in the Field of Deafness (deaf Professionals) by Palmer, J. Ursula, Edd from East Texas State University, 1989, 205 pages http://wwwlib.umi.com/dissertations/fullcit/9004600

130 Deafness

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A Cross-cultural Comparative Study: Deafness by Andersson, Yerker Johan Olof, Phd from University of Maryland College Park, 1981, 161 pages http://wwwlib.umi.com/dissertations/fullcit/8205210

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A Deaf Way of Education: Interaction among Children in a Thai Boarding School by Reilly, Charles Banks, Phd from University of Maryland College Park, 1995, 270 pages http://wwwlib.umi.com/dissertations/fullcit/9539716

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A Descriptive Study of Changes in Deafness Etiology from 1985 to 1995: a Comparison of Texas School for the Deaf and Northwest Harris County Cooperative for the Hearing Impaired by Chinn, Kathleen Mary, Edd from Lamar University Beaumont, 1997, 145 pages http://wwwlib.umi.com/dissertations/fullcit/9817000

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A Follow-up Study of the Relationship between the Abilities of Adults with Deafness at the Time of Vocational Evaluation and Status Ten Years Later by Holl, Marylou, Edd from Columbia University Teachers College, 1993, 108 pages http://wwwlib.umi.com/dissertations/fullcit/9432525

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A Multisensory Model for the Instruction of Deaf Children in the Public Schools in Maine by Loughin, Bertha Norris, Edd from Peabody College for Teachers of Vanderbilt University, 1981, 111 pages http://wwwlib.umi.com/dissertations/fullcit/8121566

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A National Study of Deaf Entrepreneurs and Small Business Owners: Implications for Career Counseling by Pressman, Sue Ellen, Phd from Virginia Polytechnic Institute and State University, 1999, 131 pages http://wwwlib.umi.com/dissertations/fullcit/9923376

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A Phenomenological Study of the Prelinguistic Deaf and Family Members of the Deaf by Taylor, Kathleen Heitzeg, Phd from The University of Michigan, 1989, 272 pages http://wwwlib.umi.com/dissertations/fullcit/8920625

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A Pilot Study of the Effect of Training in Interpersonal Process Recall on the Affective Sensitivity and Empathy of Hearing Parents of Deaf Children by Anthony, Robert Alan, Phd from Michigan State University, 1980, 89 pages http://wwwlib.umi.com/dissertations/fullcit/8020685

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A School Contact with a Cognitive Experience on Deafness to Measure the Acceptance of the Severely Hearing-impaired Population by Hearing Junior High School Students (curriculum Attitudes) by Fleischer, Gerald Guy, Phd from New York University, 1984, 147 pages http://wwwlib.umi.com/dissertations/fullcit/8505418

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A Study of Deaf High School Students' Vocational Interests and Attitudes by Farrugia, David L., Edd from Northern Illinois University, 1981, 141 pages http://wwwlib.umi.com/dissertations/fullcit/8122225

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A Study of Parental Deafness As a Factor in the Development of Self-concept in Samples of Deaf and Hearing College Students by Searls, James Matthew, Phd from The American University, 1989, 117 pages http://wwwlib.umi.com/dissertations/fullcit/9014654

Dissertations 131

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A Study of Resource/service Needs of Schools Serving Hearing-impaired Students in Texas As Perceived by Reputational Leaders at the Texas School for the Deaf and Teachers and Positional Leaders from the Schools by Zenor, Jerry Wayne, Phd from The University of Texas at Austin, 1987, 241 pages http://wwwlib.umi.com/dissertations/fullcit/8806444

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A Study of the Coping Strategies Developed by Older Adults Who Have Been Deaf since Adolescence and Possible Application of the Strategies to the Aging Process (nebraska) by Tidball, L. Kaye, Phd from The University of Nebraska - Lincoln, 1986, 200 pages http://wwwlib.umi.com/dissertations/fullcit/8620821

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A Study of the Quality of Posed Voluntary Facial Expression in Blind, Deaf, and Control Students in Residential and Public School Settings (residential Schools) by Langeler, Margaret Diane, Phd from University of Maryland College Park, 1991, 129 pages http://wwwlib.umi.com/dissertations/fullcit/9133110

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A Survey of Parents of Black Hearing Impaired Children and Their Attitudes Regarding the Educational and Personal Effects of Deafness in Their Children by Jones, Ronald Count, Phd from University of Cincinnati, 1981, 116 pages http://wwwlib.umi.com/dissertations/fullcit/8201236

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Acoustic Characteristics of the Speech of the Postlingually Deafened: Implications for the Role of Auditory Feedback during Speech Production by Waldstein, Robin S., Phd from Brown University, 1989, 136 pages http://wwwlib.umi.com/dissertations/fullcit/9002320

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Acquisition and Use of Visual/gestural and Aural/oral Bilingualism: a Phenomenological Study on Bilingualism and Deafness (visual Gestural Bilingualism, Language Acquisition) by Mason, David G., Phd from University of Alberta (canada), 1990, 269 pages http://wwwlib.umi.com/dissertations/fullcit/NN60379

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An Analysis of Deaf Issues and Their Social Setting As Dramatized by Representative Playscripts by Zachary, Samuel J., Phd from Bowling Green State University, 1984, 312 pages http://wwwlib.umi.com/dissertations/fullcit/8508381

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An Analysis of Specialized Cognitive Functions in Deaf and Hearing Signers (laterality) by Mckee, David Edwin, Phd from University of Pittsburgh, 1987, 182 pages http://wwwlib.umi.com/dissertations/fullcit/8809180

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An Ethnographic Examination of Deaf Culture and Sign Language in Japan by Fedorowicz, Steven Charles; Phd from Washington State University, 2002, 195 pages http://wwwlib.umi.com/dissertations/fullcit/3058778

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An Evaluation of the Effectiveness of an Objective-based Criteria-referenced Physical Fitness Module with a Deaf-blind Population by Powell, Theresa A., Edd from Boston University School of Education, 1980, 161 pages http://wwwlib.umi.com/dissertations/fullcit/8024145

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An Examination of How One Deaf Person Constructs Meaning in Music: a Phenomenological Perspective by Cruz, Ana Lucia De Carvalho, Phd from The University of Tennessee, 1997, 140 pages http://wwwlib.umi.com/dissertations/fullcit/9809933

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An Examination of Some Selected Factors That Affected the Education and Socialization of the Deaf of Ontario, 1870 - 1900 by Winzer, Margaret Ann, Edd from University of Toronto (canada), 1981 http://wwwlib.umi.com/dissertations/fullcit/f521478

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An Exploration of Intergenerational Relations Through Dance between Profoundly Deaf Individuals Who Are 'total Communicators' by Sherman, Andrea, Phd from New York University, 1993, 238 pages http://wwwlib.umi.com/dissertations/fullcit/9333939

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An Exploration of the Life Experiences of Deaf Persons after Receiving Cochlear Implants by Mccartin, Eileen Frances; Phd from George Mason University, 2003, 266 pages http://wwwlib.umi.com/dissertations/fullcit/3086709

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An Investigation of Knowledge and Sources of Information about Acquired Immunodeficiency Syndrome among Deaf Gay Males (immune Deficiency) by Taylor, Charles Reid, Phd from New York University, 1993, 95 pages http://wwwlib.umi.com/dissertations/fullcit/9317684

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An Investigative Study Identifying the Factors Which Influence Parents As They Make Educational Placement Decisions for Their Children Who Are Deaf by Wolfe, Vicki L.; Edd from The University of Tennessee, 1999, 181 pages http://wwwlib.umi.com/dissertations/fullcit/9944302

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Antecedent Cognitive Skills Related to Science Inquiry: an Assessment with Deaf Children by Grossman, Edward Boris, Edd from Columbia University Teachers College, 1987, 197 pages http://wwwlib.umi.com/dissertations/fullcit/8721115

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Arrangements for the Display of Deafness: an Ethnographic Account of Communication in a Hispanic Family by Rosa-lugo, Linda Iris, Edd from Columbia University Teachers College, 1989, 270 pages http://wwwlib.umi.com/dissertations/fullcit/9013574

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Art Made Accessible: Redefining Accessibility and Cross-cultural Communication for the Deaf and Hard-of-hearing in the American Theatre Institution (sign Language Theater) by Ruebhausen, David Keith, Phd from University of Minnesota, 1996, 276 pages http://wwwlib.umi.com/dissertations/fullcit/9635886

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Aspects of the Home Care of Young Deaf Children of Deaf Parents by Hartley, Gillian M., Phd from University of Nottingham (united Kingdom), 1988, 359 pages http://wwwlib.umi.com/dissertations/fullcit/D-84729

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Attitudes of Rehabilitation Counselors with the Deaf toward Deafness and Deaf People by Galloway, Victor Henry, Edd from The University of Arizona, 1973, 119 pages http://wwwlib.umi.com/dissertations/fullcit/7301144

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Attitudes toward Deafness, Motivation and Expectations of Students Enrolled in Manual Communication Classes. by Miller, Lucille Vida, Edd from Brigham Young University, 1976, 121 pages http://wwwlib.umi.com/dissertations/fullcit/7618343

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Biding the Time: American Deaf Cultural History, 1900 to World War Ii by Burch, Susan; Phd from Georgetown University, 1999, 349 pages http://wwwlib.umi.com/dissertations/fullcit/9955573

Dissertations 133

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Bionic Ears and Genetic Mistakes: the Cultural Construction of Deafness in Clinic Settings by Terstriep, Amy Lynn, Phd from University of Kansas, 1998, 296 pages http://wwwlib.umi.com/dissertations/fullcit/9903075

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Body and Soul: Deafness and Identity in Ruth Schaumann's Autobiographical Novel 'das Arsenal' (germany) by Mullens, Margaret Emily; Phd from University of Maryland College Park, 2001, 342 pages http://wwwlib.umi.com/dissertations/fullcit/3035806

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Building Bridges: Case Studies in Literacy and Deafness by Bowen, Sandra Kay; Phd from The University of Arizona, 1999, 301 pages http://wwwlib.umi.com/dissertations/fullcit/9957981

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Cognitive Functioning of Deaf Young Adults As Determined by Wechsler Performance Scales by White, Bettie Sue Florey, Phd from Texas A&m University, 1990, 99 pages http://wwwlib.umi.com/dissertations/fullcit/9027289

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Concept Formation and Prevocational Competence in Deaf Students by Herrick, Helen Marie, Edd from Boston University School of Education, 1980, 70 pages http://wwwlib.umi.com/dissertations/fullcit/8024107

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Context: Its Affect on the Interactions between a Hearing Mother and Her Hearingimpaired Child (deafness) by Plapinger, Donald S., Edd from University of Cincinnati, 1985, 173 pages http://wwwlib.umi.com/dissertations/fullcit/8605506

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Conversation at Home: a Case Study of the Communication Experiences of a Young Deaf Child in a Large Hearing Family by Evans, Judith Freed, Phd from New York University, 1994, 347 pages http://wwwlib.umi.com/dissertations/fullcit/9502452

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Correlation of Achievement of Deaf Adolescents with the Engagement Style Measure by Busby, Howard Ray, Phd from The University of Arizona, 1983, 99 pages http://wwwlib.umi.com/dissertations/fullcit/8322637

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Culture, Disability, and the Family: an Exploratory Study and Empowerment Perspective of African American Parents of Deaf and Hard of Hearing Children by Borum, Valerie; Phd from Howard University, 2001, 307 pages http://wwwlib.umi.com/dissertations/fullcit/3040794

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Deaf Adults' Use of Social Networks to Overcome Barriers to Learning: an Exploratory Study by Verlinde, Ruth Ann, Edd from Columbia University Teachers College, 1987, 159 pages http://wwwlib.umi.com/dissertations/fullcit/8804248

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Deaf Children and Their Mothers: the Relationship among Hearing Status of the Parents, Maternal Attitude toward Deafness, Maternal Acceptance of the Child and the Self-image and Academic Achievement of Deaf Children. by Taub, Sandra, Phd from New York University, 1977, 150 pages http://wwwlib.umi.com/dissertations/fullcit/7803035

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Deaf Identities, Sign Languages, and Minority Social Movement Politics in Modern Japan (1868--2000) by Nakamura, Karen; Phd from Yale University, 2001, 409 pages http://wwwlib.umi.com/dissertations/fullcit/3007397

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Deaf Identity Development: Construction and Validation of a Theoretical Model (cultural Identity) by Glickman, Neil Stephen, Phd from University of Massachusetts, 1993, 262 pages http://wwwlib.umi.com/dissertations/fullcit/9329612

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Deaf Sign Language: Identity Change in the Swedish Deaf Community by Fredang, Paivi Aino-leena; Phd from Uppsala Universitet (sweden), 2003, 299 pages http://wwwlib.umi.com/dissertations/fullcit/f247777

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Deaf Students in Mainstreamed College Composition Courses Culture and Pedagogy by Johnson, Tonya Stremlau, Phd from The Louisiana State University and Agricultural and Mechanical Col., 1996, 171 pages http://wwwlib.umi.com/dissertations/fullcit/9706338

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Deaf Students' Perceptions of Their English Language Learning: Rationale for an Experience-based Curriculum Model by Meath-lang, Bonnie Maureen, Edd from The University of Rochester, 1980, 151 pages http://wwwlib.umi.com/dissertations/fullcit/8025048

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Deaf-centered or Hearing-centered: Understanding Deaf Identity by Overstreet, Susan V., Phd from Brigham Young University, 1999, 189 pages http://wwwlib.umi.com/dissertations/fullcit/9931551

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Deafness and Mother-child Interaction by Anderson, Douglas Edward; Phd from University of Alberta (canada), 1982 http://wwwlib.umi.com/dissertations/fullcit/NK56842

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Deafness and Mother-child Interaction: Scaffolded Instruction and the Learning of Problem-solving Skills by Jamieson, Janet Ruth, Phd from Mcgill University (canada), 1989 http://wwwlib.umi.com/dissertations/fullcit/f3128180

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Deafness and Mother-child Interaction: Scaffolded Instruction and the Learning of Problem-solving Skills by Jamieson, Janet Ruth; Phd from Mcgill University (canada), 1989 http://wwwlib.umi.com/dissertations/fullcit/NL52314

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Deafness As an Identity in Relation to Future Educational and Occupational Plans among Deaf High School Students by Emerton, Robert Greg, Phd from Western Michigan University, 1973, 154 pages http://wwwlib.umi.com/dissertations/fullcit/7330301

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Deafness, Communication, and Social Identity: an Anthropological Analysis of Interaction among Parents, Teachers, and Deaf Children in a Preschool by Erting, Carol J., Phd from The American University, 1982, 606 pages http://wwwlib.umi.com/dissertations/fullcit/8306972

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Development Assistance from American Organizations to Deaf Communities in the Developing World: a Qualitative Study in Jamaica by Wilson, Amy Terra; Phd from Gallaudet University, 2001, 269 pages http://wwwlib.umi.com/dissertations/fullcit/3038023

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Differences in Visual Hemifield Processing of Linguistic Materials by Deaf and Hearing Adult Males by Mundel-atherstone, Beverly Jean, Phd from University of Alberta (canada), 1989 http://wwwlib.umi.com/dissertations/fullcit/f3122852

Dissertations 135

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Early Communication and Level of Mental Representation in the Profoundly Deaf (deaf, Communication) by Richards, Sandra Lowe Payne, Phd from Smith College School for Social Work, 1990, 95 pages http://wwwlib.umi.com/dissertations/fullcit/9117388

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Educational Programming for Students Who Are Deaf: Ceasd Standards Supported and Perceived As Practiced in Kansas (conference of Educational Administrators Serving the Deaf, Mainstreaming) by Maile, Robert Arnold, Phd from The University of Oklahoma, 1994, 236 pages http://wwwlib.umi.com/dissertations/fullcit/9422543

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Effects of Ethnically Varied Deaf Client Vignettes, Stage of Interview and Counselor Hearing Status on Counselor Empathy and Counseling Skills by Anderson, Glenn Bernard, Phd from New York University, 1982, 157 pages http://wwwlib.umi.com/dissertations/fullcit/8214863

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Effects of Integrated Training Experiences on the Attitudes of Hearing Adults toward Deaf Adults and Deafness by Blake, Gary Dean, Edd from University of Arkansas, 1971, 161 pages http://wwwlib.umi.com/dissertations/fullcit/7119536

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Effects of Training and Input on the Performance of High and Low Achieving Hearing and Deaf Children by Huberty, Thomas J. Reed, Phd from University of Missouri - Columbia, 1980, 156 pages http://wwwlib.umi.com/dissertations/fullcit/8108806

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Employers' Perceptions of the Americans with Disabilities Act and Its Effects on Vertical Mobility in the Business World (deaf) by Curry, David Allan, Ded from The University of Rochester, 1996, 206 pages http://wwwlib.umi.com/dissertations/fullcit/9630663

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Encoding Processes Used by Deaf Children When Reading Print Sentences by Dodds, Ronald Garrett, Edd from University of Toronto (canada), 1982 http://wwwlib.umi.com/dissertations/fullcit/f3201445

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Experiences of Culturally Diverse Mothers with Early Childhood Deaf Education Programs by Kommatea, Lisa-stravroula A., Phd from New York University, 1998, 163 pages http://wwwlib.umi.com/dissertations/fullcit/9935645

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Fantasies of Deafness, Silence, and Speech (american Sign Language) by Nelson, Jennifer Lee, Phd from University of California, Berkeley, 1995, 208 pages http://wwwlib.umi.com/dissertations/fullcit/9602685

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First Language Learning in Deaf Persons Beyond the Critical Period by Mckinney, Virginia, Phd from The Claremont Graduate University, 1983, 365 pages http://wwwlib.umi.com/dissertations/fullcit/8321056

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Foreigners in Their Own Land: the Cultural Origins of the Campaign against Sign Language in Nineteenth-century America (sign Language, Nineteenth Century, Deaf) by Baynton, Douglas Cameron, Phd from The University of Iowa, 1993, 338 pages http://wwwlib.umi.com/dissertations/fullcit/9421103

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Forensic Issues of Deaf Offenders by Miller, Katrina R.; Edd from Lamar University Beaumont, 2001, 96 pages http://wwwlib.umi.com/dissertations/fullcit/3037920

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From Symbol to Meaning: Processing Skills and Literacy Development in Deaf Students by Weaver-trumble, Barbara, Phd from University of California, Berkeley, 1996, 82 pages http://wwwlib.umi.com/dissertations/fullcit/9723243

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Hearing Parents with a Deaf Child: Factors Influencing Their Acceptance of the Deaf Community (sign Language) by Halpern, Karen P., Edd from University of Toronto (canada), 1994, 176 pages http://wwwlib.umi.com/dissertations/fullcit/NN97303

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Hereditary Deafness and Mast Cell Tumors in Dogs: Sequence Analysis of Candidate Genes from the Melanocyte Development Pathway by Zemke, Daniel; Phd from Michigan State University, 2003, 162 pages http://wwwlib.umi.com/dissertations/fullcit/3092235

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Hereditary Deafness on the Island of Martha's Vineyard: an Ethnohistory of a Genetic Disorder (massachusetts) by Groce, Nora Ellen, Phd from Brown University, 1983, 424 pages http://wwwlib.umi.com/dissertations/fullcit/8325981

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How Communication Technologies Have Historically Isolated Deaf People by Rogers, Thomas, Phd from University of Illinois at Urbana-champaign, 1995, 212 pages http://wwwlib.umi.com/dissertations/fullcit/9522165

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Identifying Factors That Predict Deaf Students' Academic Success in College by Harrison, Richard Hastings, Phd from University of Pennsylvania, 1987, 171 pages http://wwwlib.umi.com/dissertations/fullcit/8714049

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Identifying Learning Disabilities in the Deaf Population by Marlowe, Bruce Alan, Phd from The Catholic University of America, 1991, 87 pages http://wwwlib.umi.com/dissertations/fullcit/9117152

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Ideologies of Deafness: Deaf Education in Hispanic America by Claros Kartchner, Ruth Elizabeth; Phd from The University of Arizona, 2000, 291 pages http://wwwlib.umi.com/dissertations/fullcit/9983913

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Increasing Peer Information about Problems Related to Severe Hearing Impairment (mainstreaming, Deafness) by Youdelman, Karen Schneier, Edd from Columbia University Teachers College, 1984, 199 pages http://wwwlib.umi.com/dissertations/fullcit/8505408

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Information Processing and Deafness: an Exploratory Study by Just, Evelyn, Phd from University of Alberta (canada), 1992, 196 pages http://wwwlib.umi.com/dissertations/fullcit/NN73078

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Investigating Factors in Developing a Culturally Affirmative Discipline Model in Schools for the Deaf by Sorensen, David Alan, Edd from Northern Arizona University, 1996, 117 pages http://wwwlib.umi.com/dissertations/fullcit/9710212

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Investigation of Variables Associated with the Reading Abilities of Eighty-nine Secondary Deaf Students by Morrison, Marcia Mary, Edd from University of Northern Colorado, 1981, 105 pages http://wwwlib.umi.com/dissertations/fullcit/8202715

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Jane's Story: a Description of One Deaf Person's Experiences with Literacy (social Interactions) by Perry, Robert C., Jr., Phd from University of Georgia, 1995, 216 pages http://wwwlib.umi.com/dissertations/fullcit/9604067

Dissertations 137

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Life in a Department: an Educational Criticism of a Mathematics Program for Nontraditional Students (deafness, Handicapped) by Sachs, Marvin Charles, Edd from The University of Rochester, 1985, 292 pages http://wwwlib.umi.com/dissertations/fullcit/8601325

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Misevaluation of the Deaf: a Study of Judgment in the Psychiatric Evaluation of Deaf Persons by Dickert, Jeffery Louis, Phd from Rutgers the State University of New Jersey New Brunswick, 1983, 227 pages http://wwwlib.umi.com/dissertations/fullcit/8406361

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Mother-infant Communication Patterns: Impact of Deafness by Mccarthy, Mary Gage, Phd from The University of Texas at Austin, 1996, 179 pages http://wwwlib.umi.com/dissertations/fullcit/9719431

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Mothers' Mental State Input and Theory of Mind Understanding in Deaf and Hearing Children by Moeller, Mary Pat; Phd from The University of Nebraska - Lincoln, 2002, 131 pages http://wwwlib.umi.com/dissertations/fullcit/3059958

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Neuro-linguistic Programming As an Interviewing Technique with Prelingually Deaf Adults (deafness, Leisure) by Davis, Gerald Loyd, Jr., Edd from Oklahoma State University, 1984, 91 pages http://wwwlib.umi.com/dissertations/fullcit/8515247

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Nonhandicapped Students Attitudes towards Deaf Students by Buckney, Patricia Dewiese, Phd from Loyola University of Chicago, 1990, 109 pages http://wwwlib.umi.com/dissertations/fullcit/9016794

•

Ogden Avenue Baptist Church: a Strategy for Developing Ministry to the Deaf (wisconsin) by Carlson, Leonard A., Dmin from Fuller Theological Seminary, School of Theology, 1986, 163 pages http://wwwlib.umi.com/dissertations/fullcit/8702952

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Opportunity to Learn: Deafness and Literacy during an Age of Standards-based Reform by Cawthon, Stephanie Washbourn; Phd from The University of Wisconsin Madison, 2002, 174 pages http://wwwlib.umi.com/dissertations/fullcit/3049453

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Parental Communication Method and Deaf Children: a Study of Parental Stress, Psychosocial Adjustment, Social Skills, and Educational Achievement by Hagborg, Winston J., Phd from Temple University, 1984, 153 pages http://wwwlib.umi.com/dissertations/fullcit/8419767

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Physicians' Attitudes toward Deaf Persons and the Communication Methods Used with Their Deaf Patients by Mcneil, Ellen Elizabeth, Phd from Oregon State University, 1984, 158 pages http://wwwlib.umi.com/dissertations/fullcit/8418170

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Play Behaviors of Deaf and Hearing Children by Mann, Lynne Firsel, Edd from University of Virginia, 1981, 155 pages http://wwwlib.umi.com/dissertations/fullcit/8205458

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Predicting the Academic Performance of Deaf Students in a Postsecondary Educational Setting by Boyd, Barbara Ella, Phd from University of Southern California, 1983 http://wwwlib.umi.com/dissertations/fullcit/f2745349

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•

Present Roles and Functions of Parent Educators in Schools for the Deaf by Howell, Ruth Frances, Edd from University of Maryland College Park, 1984, 161 pages http://wwwlib.umi.com/dissertations/fullcit/8506537

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Psychological Adjustment of Deaf Children of Hearing Parents: a Study Comparing Groups Using Oral and Total Communication Methods by Gray, Barbara Sue, Phd from Temple University, 1980, 146 pages http://wwwlib.umi.com/dissertations/fullcit/8025177

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Pueblo Individuals Who Are D/deaf: Acceptance in the Home Community, the Dominant Society, and the Deaf Community by Kelley, Walter Paul; Phd from The University of Texas at Austin, 2001, 186 pages http://wwwlib.umi.com/dissertations/fullcit/3035959

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Resource-based Early Intervention with Multicultural Deaf/hard of Hearing Infants, Toddlers and Their Families by Wu, Cheryl Lynn; Psyd from California School of Professional Psychology - Berkeley/alameda, 2002, 200 pages http://wwwlib.umi.com/dissertations/fullcit/3026993

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Responses of Deaf High School Students to an 'attitudes toward Alcohol' Scale by Sabin, Martha C., Phd from Southern Illinois University at Carbondale, 1986, 171 pages http://wwwlib.umi.com/dissertations/fullcit/8728301

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Self-disclosure in Deaf-hearing, Deaf-deaf, and Hearing-hearing Married Couples: a Look at Frequency, Value, and Contexts in Relation to Marital Satisfaction by Mcintosh, Rebecca Anne, Phd from The University of Texas at Austin, 1995, 179 pages http://wwwlib.umi.com/dissertations/fullcit/9534883

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Self-esteem, Family Climate, and Communication Patterns in Relation to Deafness (sign Language) by Desselle, Debra Duke, Phd from University of New Orleans, 1992, 187 pages http://wwwlib.umi.com/dissertations/fullcit/9230595

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Signing for a High: a Study of Alcohol and Drug Use by Deaf and Hard of Hearing Adolescents by Dick, Janet Ellen, Phd from Rutgers the State University of New Jersey New Brunswick, 1996, 344 pages http://wwwlib.umi.com/dissertations/fullcit/9633689

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Silenced Voices, Signed Visions: a Phenomenological Study of the Role of Literacy in the Lives of Five Deaf Individuals by Kimmel, Karen L., Phd from State University of New York at Buffalo, 1996, 188 pages http://wwwlib.umi.com/dissertations/fullcit/9704900

•

Social Agency and Deaf Communities: a Nicaraguan Case Study by Polich, Laura Gail; Phd from The University of Texas at Austin, 1998, 289 pages http://wwwlib.umi.com/dissertations/fullcit/9937121

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Social Cognition and Self-concept of Hearing Adolescents with Deaf Parents by Charlson, Elizabeth Stone, Phd from University of California, Berkeley with San Francisco State Univ., 1989, 124 pages http://wwwlib.umi.com/dissertations/fullcit/9006588

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Sound Knowledge: the Political Meaning of Deafness by Wrigley, Owen Paul, Phd from University of Hawaii, 1992, 388 pages http://wwwlib.umi.com/dissertations/fullcit/9312223

•

Sounds and Signs of Communication: a Comparative Study of Inquiry, Request and Response Behavior in Hearing-impaired and Hearing Preschool Children (deafness)

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by Christensen, Kathee Mangan, Phd from The Claremont Graduate University, 1986, 136 pages http://wwwlib.umi.com/dissertations/fullcit/8607821 •

Sources and Aspects of Code-switching in the Signing of a Deaf Adult and Her Interlocutors by Lee, Dorothy Marian, Phd from The University of Texas at Austin, 1983, 250 pages http://wwwlib.umi.com/dissertations/fullcit/8319628

•

Surditas: the Understandings of the Deaf and Deafness in the Writings of Augustine, Jerome, and Bede (saint Augustine, Saint Jerome, Venerable Bede) by King, Leslie A., Phd from Boston University, 1996, 235 pages http://wwwlib.umi.com/dissertations/fullcit/9625329

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The Acquisition of Occupational Knowledge in Deaf Populations: a Schema Theory Approach (career Guidance) by Clark, David Anthony, Phd from The Florida State University, 1992, 205 pages http://wwwlib.umi.com/dissertations/fullcit/9306029

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The Australian Theatre of the Deaf: Essence, Sensibility, Style by Bradford, Shannon Leigh; Phd from The University of Texas at Austin, 2000 http://wwwlib.umi.com/dissertations/fullcit/3014976

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The Comparison of the Self-concept of Young Deaf Children with Their Academic Achievement and Communication Style (demographic Variables, Hearing-impaired, Deafness, Self-image) by Mcmahon, Margo Elizabeth, Edd from University of Massachusetts, 1985, 534 pages http://wwwlib.umi.com/dissertations/fullcit/8517133

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The Development of an Administrative Model for the Integration of Deaf Children into the Public Schools of Maine by Kelly, Robert Edward, Edd from Peabody College for Teachers of Vanderbilt University, 1981, 110 pages http://wwwlib.umi.com/dissertations/fullcit/8121565

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The Development of God Image in Deaf Children: an Analysis from an Object Relations Theory Perspective by Maxon, William Steven, Phd from Drew University, 1996, 191 pages http://wwwlib.umi.com/dissertations/fullcit/9629118

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The Development of Self-understanding in Deaf Children by Prout, Teresa Feher, Phd from University of Pittsburgh, 1998, 134 pages http://wwwlib.umi.com/dissertations/fullcit/9919324

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The Effect of Three Instructional Modalities on Reading and Vocabulary Comprehension of Deaf Children by Bland, Elwood Leonard, Phd from University of Maryland College Park, 1981, 137 pages http://wwwlib.umi.com/dissertations/fullcit/8202845

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The Effects of Bilingual and Monolingual Reading Videos on Story Recall with Students Who Are Deaf and Hard of Hearing by Meyers-sinett, Kelly D'ann, Edd from Florida International University, 1997, 143 pages http://wwwlib.umi.com/dissertations/fullcit/9727339

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The Effects of Deafness on Play in Four-year-old Boys by Gatty, Janice Catherine, Edd from University of Massachusetts, 1990, 192 pages http://wwwlib.umi.com/dissertations/fullcit/9022685

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•

The Hearing Line: Literary Encounters with Deafness in Nineteenth-century America by Krentz, Christopher Becker; Phd from University of Virginia, 2002, 277 pages http://wwwlib.umi.com/dissertations/fullcit/3035331

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The Humor Response of Deaf Children: an Exploratory Study by Krents, Elisabeth Joan, Phd from Columbia University, 1982, 115 pages http://wwwlib.umi.com/dissertations/fullcit/8222418

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The Identification and Perceived Importance of Special Competencies for Deafness Rehabilitation Specialists by Petty, Debra Martin, Edd from The University of Tennessee, 1987, 193 pages http://wwwlib.umi.com/dissertations/fullcit/8713474

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The Identification of Special Competencies of Deafness Specialists in Postsecondary Education Programs by Kolvitz, Marcia Ellen; Phd from The University of Tennessee, 1999, 203 pages http://wwwlib.umi.com/dissertations/fullcit/9959295

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The Influence of Knowledge and Contact on Hearing Parents' Attitudes towards Deaf People by Addams, Barbara Jean Harling, Dsw from The Catholic University of America, 1988, 228 pages http://wwwlib.umi.com/dissertations/fullcit/8814931

•

The Life Experiences of Deaf High School Graduates in Nigeria by Olubodun, William Olugbemi; Phd from The University of Nebraska - Lincoln, 2003, 352 pages http://wwwlib.umi.com/dissertations/fullcit/3092582

•

The Native American Deaf Experience: Cultural, Linguistic, and Educational Perspectives by Baker, Sharon, Edd from Oklahoma State University, 1996, 144 pages http://wwwlib.umi.com/dissertations/fullcit/9717940

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The Nature and Role of Story Schemata in the Reading of Severely Deaf Children by Banks, James S., Phd from University of Aberdeen (united Kingdom), 1989, 432 pages http://wwwlib.umi.com/dissertations/fullcit/DX91612

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The Perceptions of Deafness and Language Learning of Incoming Asl Students by Peterson, Richard William, Phd from University of California, Riverside, 1999, 259 pages http://wwwlib.umi.com/dissertations/fullcit/9925067

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The Production of Asl Signs (aphasia, Deafness) by Whittemore, Gregory Lynn, Phd from The University of Texas at Austin, 1987, 236 pages http://wwwlib.umi.com/dissertations/fullcit/8717569

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The Relationship between Communication Modes and Achievement of Deaf Students by Nebe, Henry Joseph, Edd from The University of Southern Mississippi, 1980, 132 pages http://wwwlib.umi.com/dissertations/fullcit/8109886

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The Relationship between Perceptions of Stigmatization, Self-concept, and Attributional Style of Deaf Adolescents by Kappy, Barbara, Phd from Wayne State University, 1996, 256 pages http://wwwlib.umi.com/dissertations/fullcit/9715858

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The Relationship between Self-concept of Deaf Children and Selected Other Variables by Warren, Charlotte Jane, Phd from University of Virginia, 1983, 112 pages http://wwwlib.umi.com/dissertations/fullcit/8415431

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The Relationship between Visual Perceptual Ability of Deaf Children and the Ability to Comprehend Sign Language by Ratner, Vivienne, Phd from New York University, 1989, 260 pages http://wwwlib.umi.com/dissertations/fullcit/8916037

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The Role of Language Communication Acquisition Experience in Developing Abstract Thought in Deaf Students by Schine, Rena, Phd from New York University, 1990, 132 pages http://wwwlib.umi.com/dissertations/fullcit/9102645

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The Search for Synchrony: a Hearing Infant with Deaf Parents by Elder, Mary-scovill, Edd from Columbia University Teachers College, 1996, 128 pages http://wwwlib.umi.com/dissertations/fullcit/9713874

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The Silent North: a Case Study on Deafness in a Dene Community by Castleden, Heather Evelyn; Med from University of Alberta (canada), 2002, 194 pages http://wwwlib.umi.com/dissertations/fullcit/MQ69660

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The 'silent South': Growing Up Deaf in the Antebellum Southern States by Joyner, Hannah Ruth; Phd from University of Pennsylvania, 2000, 405 pages http://wwwlib.umi.com/dissertations/fullcit/9989607

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The Social Construction of Reality in a Total Institution: an Ethnography of a State Residential School for the Deaf by Evans, Abbie Donald, Phd from The Louisiana State University and Agricultural and Mechanical Col., 1982, 434 pages http://wwwlib.umi.com/dissertations/fullcit/8312086

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The Social Reference Group As a Correlate to Selected Dimensions of Self-perception in a Sample of Deaf Adolescents by Kessler Poole, Vicki Lynn, Phd from The American University, 1992, 204 pages http://wwwlib.umi.com/dissertations/fullcit/9305778

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Therapy with the Deaf Child (afrikaans Text) by De Wet, Wynand, Phd from University of Pretoria (south Africa), 1993 http://wwwlib.umi.com/dissertations/fullcit/f2395427

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'to Get Back into Society the Way I Was': a Sociological Analysis of Physical Disability and Impairment (dialectics, Deafness, Chronic Pain) by Seidel, John Vail, Phd from University of Colorado at Boulder, 1984, 419 pages http://wwwlib.umi.com/dissertations/fullcit/8422650

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Tone-deafness and Low Musical Abilities an Investigation of Prevalence, Characteristics, and Tractability by Mawhinney, Thomas A; Phd from Queen's University at Kingston (canada), 1987 http://wwwlib.umi.com/dissertations/fullcit/NL38431

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Toward the Development of a Methodology for Adapting a Self-concept Inventory for Use with Deaf Children by Lapham, Robert Francis, Phd from Duke University, 1981, 145 pages http://wwwlib.umi.com/dissertations/fullcit/8129826

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Transculturalism, Affiliation and the Epistemological Verities of 'normative Identity': Deafness and the African Diaspora by Mazard Wallace, G. L.; Phd from University of Massachusetts Amherst, 2001, 232 pages http://wwwlib.umi.com/dissertations/fullcit/3027228

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Trends in the Development of Education of the Deaf in Nigeria between 1950 and 1985: an Interview and Historical Study by Adepoju, Gabriel Ademola, Phd from Gallaudet University, 1991, 344 pages http://wwwlib.umi.com/dissertations/fullcit/9129330

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Use of the Videotaped Ralston Test of Fingerspelled Pseudowords (rtfp) in Exploring Reading among Deaf Adults in College and Postsecondary Training Programs by Ralston, Frances; Phd from Gallaudet University, 2000, 223 pages http://wwwlib.umi.com/dissertations/fullcit/3029919

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Value and Use of American Sign Language and English in Two Pennsylvania Schools for the Deaf by Rinne, Mary Glenn, Edd from University of Pittsburgh, 1995, 216 pages http://wwwlib.umi.com/dissertations/fullcit/9601237

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Words Made Flesh: Nineteenth-century Deaf Education and the Growth of Deaf Culture by Edwards, Rebecca Anne Rourke, Phd from The University of Rochester, 1997, 374 pages http://wwwlib.umi.com/dissertations/fullcit/9729038

Keeping Current Ask the medical librarian at your library if it has full and unlimited access to the ProQuest Digital Dissertations database. From the library, you should be able to do more complete searches via http://wwwlib.umi.com/dissertations.

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CHAPTER 5. CLINICAL TRIALS AND DEAFNESS Overview In this chapter, we will show you how to keep informed of the latest clinical trials concerning deafness.

Recent Trials on Deafness The following is a list of recent trials dedicated to deafness.8 Further information on a trial is available at the Web site indicated. •

Genetic Studies of Tone Deafness Condition(s): Healthy; Tone Deafness Study Status: This study is currently recruiting patients. Sponsor(s): National Institute on Deafness and Other Communication Disorders (NIDCD) Purpose - Excerpt: This study will examine the hereditary basis of tone deafness by identifying regions of the human genome linked to this condition. Both exceptionally good pitch recognition (perfect pitch) and exceptionally poor pitch recognition (tone deafness) run in families. A better understanding of what causes tone deafness may provide new insights into auditory (hearing) function. Individuals with two or more family members 15 years of age or older who are tone deaf or have trouble recognizing different melodies may be eligible for this study. Candidates will be screened with a short listening test for pitch and a short written test. Those identified with poor pitch recognition will fill out a brief questionnaire about their family tree and family members (without identifying names) who have trouble recognizing melodies or tones. Individuals with poor pitch recognition will be asked to help contact family members who may be interested in participating. Members of families with two or more firstdegree relatives (parents, grandparents, siblings) who are tone deaf may enroll in the study. They will provide a blood sample (about 2 tablespoons) for genetic studies and may take a 20-minute hearing test using headphones. Study Type: Observational

8

These are listed at www.ClinicalTrials.gov.

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Contact(s): see Web site below Web Site: http://clinicaltrials.gov/ct/show/NCT00006076

Keeping Current on Clinical Trials The U.S. National Institutes of Health, through the National Library of Medicine, has developed ClinicalTrials.gov to provide current information about clinical research across the broadest number of diseases and conditions. The site was launched in February 2000 and currently contains approximately 5,700 clinical studies in over 59,000 locations worldwide, with most studies being conducted in the United States. ClinicalTrials.gov receives about 2 million hits per month and hosts approximately 5,400 visitors daily. To access this database, simply go to the Web site at http://www.clinicaltrials.gov/ and search by “deafness” (or synonyms). While ClinicalTrials.gov is the most comprehensive listing of NIH-supported clinical trials available, not all trials are in the database. The database is updated regularly, so clinical trials are continually being added. The following is a list of specialty databases affiliated with the National Institutes of Health that offer additional information on trials: •

For clinical studies at the Warren Grant Magnuson Clinical Center located in Bethesda, Maryland, visit their Web site: http://clinicalstudies.info.nih.gov/

•

For clinical studies conducted at the Bayview Campus in Baltimore, Maryland, visit their Web site: http://www.jhbmc.jhu.edu/studies/index.html

•

For cancer trials, visit the National Cancer Institute: http://cancertrials.nci.nih.gov/

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For eye-related trials, visit and search the Web page of the National Eye Institute: http://www.nei.nih.gov/neitrials/index.htm

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For heart, lung and blood trials, visit the Web page of the National Heart, Lung and Blood Institute: http://www.nhlbi.nih.gov/studies/index.htm

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For trials on aging, visit and search the Web site of the National Institute on Aging: http://www.grc.nia.nih.gov/studies/index.htm

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For rare diseases, visit and search the Web site sponsored by the Office of Rare Diseases: http://ord.aspensys.com/asp/resources/rsch_trials.asp

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For alcoholism, visit the National Institute on Alcohol Abuse and Alcoholism: http://www.niaaa.nih.gov/intramural/Web_dicbr_hp/particip.htm

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For trials on infectious, immune, and allergic diseases, visit the site of the National Institute of Allergy and Infectious Diseases: http://www.niaid.nih.gov/clintrials/

•

For trials on arthritis, musculoskeletal and skin diseases, visit newly revised site of the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health: http://www.niams.nih.gov/hi/studies/index.htm

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For hearing-related trials, visit the National Institute on Deafness and Other Communication Disorders: http://www.nidcd.nih.gov/health/clinical/index.htm

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For trials on diseases of the digestive system and kidneys, and diabetes, visit the National Institute of Diabetes and Digestive and Kidney Diseases: http://www.niddk.nih.gov/patient/patient.htm

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•

For drug abuse trials, visit and search the Web site sponsored by the National Institute on Drug Abuse: http://www.nida.nih.gov/CTN/Index.htm

•

For trials on mental disorders, visit and search the Web site of the National Institute of Mental Health: http://www.nimh.nih.gov/studies/index.cfm

•

For trials on neurological disorders and stroke, visit and search the Web site sponsored by the National Institute of Neurological Disorders and Stroke of the NIH: http://www.ninds.nih.gov/funding/funding_opportunities.htm#Clinical_Trials

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CHAPTER 6. PATENTS ON DEAFNESS Overview Patents can be physical innovations (e.g. chemicals, pharmaceuticals, medical equipment) or processes (e.g. treatments or diagnostic procedures). The United States Patent and Trademark Office defines a patent as a grant of a property right to the inventor, issued by the Patent and Trademark Office.9 Patents, therefore, are intellectual property. For the United States, the term of a new patent is 20 years from the date when the patent application was filed. If the inventor wishes to receive economic benefits, it is likely that the invention will become commercially available within 20 years of the initial filing. It is important to understand, therefore, that an inventor’s patent does not indicate that a product or service is or will be commercially available. The patent implies only that the inventor has “the right to exclude others from making, using, offering for sale, or selling” the invention in the United States. While this relates to U.S. patents, similar rules govern foreign patents. In this chapter, we show you how to locate information on patents and their inventors. If you find a patent that is particularly interesting to you, contact the inventor or the assignee for further information. IMPORTANT NOTE: When following the search strategy described below, you may discover non-medical patents that use the generic term “deafness” (or a synonym) in their titles. To accurately reflect the results that you might find while conducting research on deafness, we have not necessarily excluded non-medical patents in this bibliography.

Patents on Deafness By performing a patent search focusing on deafness, you can obtain information such as the title of the invention, the names of the inventor(s), the assignee(s) or the company that owns or controls the patent, a short abstract that summarizes the patent, and a few excerpts from the description of the patent. The abstract of a patent tends to be more technical in nature, while the description is often written for the public. Full patent descriptions contain much more information than is presented here (e.g. claims, references, figures, diagrams, etc.). We

9Adapted

from the United States Patent and Trademark Office: http://www.uspto.gov/web/offices/pac/doc/general/whatis.htm.

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will tell you how to obtain this information later in the chapter. The following is an example of the type of information that you can expect to obtain from a patent search on deafness: •

Apparatus for use in conjunction with lipreading by the profoundly deaf Inventor(s): Fourcin; Adrian J. (London, GB2), Rosen; Stuart M. (London, GB2), Walliker; John R. (Epsom, GB2) Assignee(s): National Research Development Corporation (London, GB2) Patent Number: 4,809,329 Date filed: August 29, 1986 Abstract: The profoundly deaf benefit in lipreading if the larynx frequencies of speech are presented to them by means of acoustic sine waves. In the present invention the sine waves required are generated using a microcomputer and analogue output circuits, with the microcomputer providing digital level samples for the required sine waves from a stored look-up table. The wide frequency range of sine waves required is achieved by omitting some samples in each cycle at high frequencies, and the wide range of output levels required is provided by employing two digital to analogue converters, one acting as an attenuator. Excerpt(s): The present invention relates to apparatus which generates an audio signal from speech and which is of assistance to the profoundly deaf when lipreading. As mentioned in "Speech Pattern Element Stimulation in Electrical Hearing" by Adrian Fourcin, Ellis Douek, Brian Moore, Evelyn Abberton, Stuart Rosen and John Walliker, Archives of Otolaryngology, Vol. 110, March 1984, the profoundly deaf may be assisted in lipreading if larynx frequency information is presented to them acoustically in pure sine wave form. The explanation for this finding is not certain, but may be that a sinusoid is the only waveform which can pass through a dispersive linear filter (transducer, ear canal, tympanic membrane, ossicular chain [if present], cochlea) and still retain its overall shape. A major problem which is encountered in producing the required sine wave output is the generation of an accurate sine wave of varying amplitude over a frequency range from about 30 Hz to about 800 Hz. Web site: http://www.delphion.com/details?pn=US04809329__

•

Apparatus to alert a deaf person Inventor(s): Brugger; Richard D. (5433 Clinton Dr., Erie, PA 16509), Sulkoski; Jerome (7258 Bell Rd., Harborcreek, PA 16511) Assignee(s): none reported Patent Number: 4,380,759 Date filed: November 5, 1980 Abstract: An apparatus to alert a deaf person made up of an alarm device such as a smoke detector adapted to vibrate when actuated by smoke and a transmitter having a vibration sensor connected to the transmitter. The vibration sensor is supported in engagement with the smoke detector and adapted to sense the vibrations of the smoke detector and to transmit a signal to a remotely located receiver. The receiver has a vibrating reed with a tacticle member on its end for engaging a person to alert him when the receiver causes the reed to be vibrated.

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Excerpt(s): Apparatus has been designed and assembled for the purpose of alerting a deaf person to certain specific dangers. One such danger is a fire, where he may be unable to hear the "audible" alarm that is characteristic of Smoke Alarms. This apparatus picks up the sound vibrations from the conventional Smoke Detector and causes a radio signal to be transmitted to a receiver and a "stimulator" located on the deaf person. A novel configuration of piezoelectric reed and special electronic circuitry is a part of this apparatus. The apparatus uses commercially available devices, generally, since the intent was to construct a working model and to create an overall system design. Also, it is less expensive and easier to make changes and modifications at this level than it would be to do those things after miniaturization is performed. The following patents are known to the inventer. U.S. Pat. Nos. 2,582,777; 3,618,070; 3,623,064; 3,786,628; 3,810,170; 3,911,416; 4,028,882 and 4,180,810. Web site: http://www.delphion.com/details?pn=US04380759__ •

Braille deaf-blind communicators Inventor(s): Fewell; William B. (124 Dow Rd., Port Charlotte, FL) Assignee(s): none reported Patent Number: 4,215,490 Date filed: November 16, 1977 Abstract: A hand held and portable device used for communicating between blind-deaf persons and between blind-deaf and nonhandicapped persons who know the Braille system. The transmitter person presses one or a number of the six keys in combinations producing raised projections representing a six unit Braille cell to be felt and read by a receiving person through tactile finger sensation. Excerpt(s): This invention has for an object to provide a portable means for communicating with a deaf-blind person who knows the Braille system. Also the apparatus may be used for communicating between two deaf-blind persons. Another object is to provide a simple means of communicating between handicapped persons having the use of only one hand and three fingers. A further object is to provide an apparatus which is small enough to be carried in the purse of pocket and be hand held when communicating. Web site: http://www.delphion.com/details?pn=US04215490__

•

Cochlear implant auditory prosthesis Inventor(s): Jacobs; Jared J. (14251 Paul Ave., Saratoga, CA 95070) Assignee(s): none reported Patent Number: 5,061,282 Date filed: October 10, 1989 Abstract: A cochlear implant auditory prosthesis corrects sensorineural deafness by generating stimulus signals to neurons connected to the auditory nerve in response to vibrations in the basilar membrane of the cochlea of the inner ear. The prosthesis comprises a plurality of transducer elements disposed along the length of the cochlea adjacent to the basilar membrane, whereby each transducer element responds to vibrations in the basilar membrane at the corresponding location of the respective

150 Deafness

transducer element. Each transducer element comprises a transducer for detecting the respective vibrations of the basilar membrane, and a signal processing element for generating a stimulus signal in response to the vibration. The frequency response and gain of each transducer element of the prosthesis can be tuned by a compact control unit to provide an ideal response for the user. Excerpt(s): The present invention relates to a cochlear implant prosthesis for individuals who have hearing disabilities. More specifically, this invention relates to a cochlear implant auditory prosthesis for generating stimulus signals to neurons connected to the auditory nerve in persons suffering from sensorineural deafness. The cochlea is a fluidfilled organ in the inner ear which aids in the conversion of sound waves to electrochemical stimuli. In a healthy ear, sound normally travels through the external ear canal to the tympanic membrane, also known as the ear drum. The tympanic membrane vibrates in response to pressure changes in the sound waves. The vibrations are transmitted through a series of small bones in the middle ear to the cochlea, or inner ear. The innermost of the small bones, the stapes or more commonly referred to as the stirrup, contacts a membranous opening known as the oval window at the base of the cochlea. The stapes transmits sound vibrations through the oval window to the fluidfilled interior of the cochlea. These sound vibrations are then transmitted through the cochlear fluid which induces vibration in a membrane within the cochlea. This membrane, known as the basilar membrane, follows a spiral path along the length of the cochlea. Longitudinal lines of hair cells on the basilar membrane sway in response to the vibrations. The motion of the cilia of the hair cells causes alternating localized changes in electrical potential that stimulate the auditory nerve fibers. A dysfunction of these hair cells causes sensorineural deafness, whereby the hair cells respond improperly and fail to stimulate the auditory neurons. Each auditory nerve fiber carries a specific modality of sensation to the brain. The type of sensation perceived when a sensory nerve is stimulated is determined by the specific area in the central nervous system to which the nerve fiber leads. Thus, regardless of whether the auditory nerve is stimulated by its sensory end-organ hair cell, or by direct electrical signals, a perception of sound is created. This phenomenon forms the fundamental basis of the implanted cochlear prosthesis. Web site: http://www.delphion.com/details?pn=US05061282__ •

Communication apparatus and method for performing vision testing on deaf and severely hearing-impaired individuals Inventor(s): Hutchinson; Ray Anthony (Olivenhain, CA) Assignee(s): Quo Vadis, Inc. (Encinitas, CA) Patent Number: 5,929,972 Date filed: January 14, 1998 Abstract: An apparatus and method for communicating to a deaf or severely hearing impaired individual while vision testing utilizing indicator lights (52A), (52B), (52C), and (52D) positioned in the same plane as a visual acuity test chart (54) having optotypes (50B). The examiner activates the indicator lights by means such as an infrared remote control receiver (56). This is done in a manner previously disclosed to the examinee permitting the examinee to understand messages from the examiner, while the examiner simultaneously operates a refractor to determine the refractive state of the examinee.

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Excerpt(s): This invention relates to vision testing deaf or severely hearing-impaired individuals, specifically to the means by which the examiner communicates the necessary instructions to the hearing impaired individual during the course of the examination. The usual means of communicating to deaf and hearing-impaired individuals are through the printed word, sign language, finger spelling, lip reading, gestures or by touch. Except for touch, the hearing-impaired individual must be able to observe the one attempting the communication. Other means of non-verbal communication for the handicapped have been developed. U.S. Pat. No. 4,406,998 to Willough, Sep. 27, 1983 discloses a device which allows communication through lighted message blocks. Many devices have been developed which allow communication from a handicapped person to another, such as the apparatus disclosed in U.S. Pat. No. 3,651,512 to Summers, Mar. 21, 1972. A vision examination consists of many parts. Typically, an eye examination will include the patient case history, visual acuity testing, various eye health tests, muscle balance tests, objective refraction and subjective refraction. Visual acuity testing is accomplished using various types of visual acuity test targets and displays. A test distance between the patient and the test targets is typically twenty feet. Visual acuity test targets are known as optotypes. One such optotype is disclosed in U.S. Pat. No. 4,968,131 to Lewis, Nov. 6, 1990. Historically these test targets were presented to the patient as a wall chart having the optotypes imprinted on it. Currently, the most common method of presenting the optotypes to the patient is through the use of a chart projector which projects the optotypes upon a reflective screen. To permit adequate visualization of the projected acuity chart, the room is generally made dark. Alternative methods of displaying visual acuity charts have been developed. One such device is a translucent panel with imprinted optotypes which is rear-illuminated as disclosed in U.S. Pat. No. 5,129,720 to Jovicevic, Jul. 14, 1992. Another method employs a computer to generate optotypes upon a video display unit as disclosed in U.S. Pat. No. 4,239,351 to Williams, et al, Dec. 16, 1980. Testing of visual acuity and the use of optotypes is integral to the vision examination and is measured repeatedly during the subjective testing phase of the examination. Web site: http://www.delphion.com/details?pn=US05929972__ •

Communication device and method for deaf and mute persons Inventor(s): Butnaru; Hanan (1 Somerville Ct., San Antonio, TX 78257), Krueger; Wesley O. (4410 Medical Dr., Suite 340, San Antonio, TX 78229) Assignee(s): none reported Patent Number: 6,240,392 Date filed: August 29, 1997 Abstract: A communication device for deaf, hearing impaired, or mute persons comprises a processor control system which makes use of a microphone and a speech recognizer to receive and process audio data (speech or non-speech) to determine whether or not a dangerous situation exits within the environment surrounding the user. The system comprises a key pad and/or stylus and tablet information input system to accommodate communication from the user to the persons in the surrounding environments and a visual display capability to transmit the information so acquired by way of a projection apparatus in the form of characters and in the language to which the user is accustomed. Indicator signals which correspond to dangerous or cautionary situations relating to abnormally loud noises, or readily recognized sound patterns, such

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as a siren may also be displayed to the user, as may be information related geographic location, distance to a preset destination, or other personally useful information. Excerpt(s): This invention relates generally to a device for communication between persons who are deaf, hearing impaired, or mute. More particularly, the invention is directed toward a communication system for deaf, hearing impaired or mute persons which allows the user to visualize speech and other sounds directed at him through various audio sources. Historically, deaf, hearing impaired, and mute persons have had a difficult time communicating, both among themselves and with others not similarly handicapped. In fact, it is often more difficult to conduct a simple conversation between a non-handicapped person and one that is deaf or mute, because the non-handicapped person usually is not familiar with American Sign Language (ASL). Even when the persons communicating are familiar with ASL, sign language is not a very convenient method of communication when a large group is involved. Expensive television cameras and video screens must be employed to transmit messages to large audiences. That is, unless the receiver of the message is so close to the message transmitter that he or she can distinguish ASL gestures and the expressions on the message transmitter's face, communication is simply not possible. As mentioned previously, if the message receiver has not been trained to understand ASL, then communication is also not possible. Web site: http://www.delphion.com/details?pn=US06240392__ •

Communication system for deaf, deaf-blind, or non-vocal individuals using instrumented glove Inventor(s): George; William R. (Palo Alto, CA), Kramer; James P. (Stanford, CA), Lindener; Peter (E. Palo Alto, CA) Assignee(s): The Board of Trustee of the Leland Stanford Junior University (Stanford, CA) Patent Number: 5,047,952 Date filed: October 14, 1988 Abstract: A communication system for deaf, deaf-blind, or non-vocal individuals includes an instrumented glove for obtaining electrical signals indicative of a hand configuration of a first individual. Strain gage sensors in the glove flex with movement of the hand. Each sensor includes a tension strain gage and a compression strain gage which are serially connected and form two legs in a bridge circuit. Signals from the bridge circuit are amplified and digitized and applied to a computer which includes an adaptive pattern recognition algorithm which is responsive to hand-state vectors for recognizing letter beacons in hand-space. A second individual communicates with the first individual through the computer system using a portable keyboard. The output devices for communicating to the first and second individuals depend on the visual, vocal and hearing capabilities of the individuals and can be selected from a voice synthesizer, LCD monitor, or braille display. Excerpt(s): This invention relates generally to communication devices for deaf, deafblind, or non-vocal individuals, and more particularly the invention relates to a communication system which utilizes an instrumented glove. While many deaf persons communicate effectively using a form of sign language or the fingerspelling alphabet, problems arise when a hearing person, who does not know sign language, attempts to interact with a deaf individual who does not know the "oral" method, i.e., cannot speak

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intelligibly and read lips. For deaf-blind persons, communication is even more difficult since they must be able to touch the hand of the person with whom they wish to interact. These communication difficulties adversely effect interpersonal relationships and vocational activities. Consequently, many deaf, deaf-blind and non-vocal people avoid situations requiring interaction with hearing persons. These disabled individuals often remain unemployed and dependent, and cannot fully participate in community life. The most common ways a nonvocal deaf or deaf-blind person communicates with a hearing person who does not know sign language are via interpreters and notewriting. Although interpreters are effective in translating sign language or fingerspelling into speech and vice versa, they are often expensive and difficult to acquire on a moment's notice. Use of an interpreter also leads to a loss of independence and privacy. Notewriting is used by many nonvocal individuals to communicate with someone who is seated nearby, but it is awkward while walking, standing at a distance, and when more than two persons participate in a conversation. Web site: http://www.delphion.com/details?pn=US05047952__ •

Device for mounting a telecommunications device for the deaf in a public phone booth Inventor(s): King; Bruce A. (3956 Downes Dr., Indianapolis, IN 46236) Assignee(s): none reported Patent Number: 4,903,290 Date filed: July 22, 1988 Abstract: A device for mounting a telecommunictions device for the deaf ("TDD") to a pay telephone booth which provides an enclosure for storing the TDD when not in use, and a mechanism for attaching the TDD to the booth so that it can be moved between a position where it is contained within the enclosure to a position where the handset of the telephone may be received in the handset cradle of the TDD to provide access to public telephone to the deaf and hearing impaired. Excerpt(s): This invention relates to a device for mounting appliances in a public telephone booth and more particularly to a device for mounting a telecommunications device for the deaf in a telephone booth. Deaf and hearing impaired people are unable to use standard telephones for communication unless they and the party with whom they are communicating both have a telecommunications device for the deaf ("TDD"). TDD's typically have a cradle for receiving a telephne handset, a keyboard for typing in outgoing messages, a means for converting typed messages to signals which can be transmitted over telephone lines and a means for converting signals received over telephone lines to a visual display. Thus, TDDs are often fairly large in size and are too cumbersome to make portability practicable. Therefore, TDDs are typically used only in the home or office environment. Because it is not practical for a deaf or hearing impaired person to carry TDDs around with them, the deaf and hearing impaired are essentially denied access to public telephone booths. The inventor is unaware of any prior art which discloses a TDD mounted in a public telephone booth. However the inventor is aware of two U.S. Patents which disclose public telephone booths with devices therein for holding telephone directories. These patents are Chambers (U.S. Pat. No. 2,982,593 issued May 2, 1961) and Hickman et al. (U.S. Pat. No. 4,323,290 issued Apr. 6, 1982). Web site: http://www.delphion.com/details?pn=US04903290__

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Digital hearing aid Inventor(s): Ishige; Ryuuichi (Tokyo, JP), Mitome; Yukio (Tokyo, JP) Assignee(s): Nec Corporation (Tokyo, JP) Patent Number: 5,838,801 Date filed: December 9, 1997 Abstract: A digital hearing aid has input means for converting an input sound into a digital data for generating an input data, analyzing means for analyzing the input data converted by the input means by a digital conversion and calculating an acoustic pressure at each frequency band, control means for inputting a result of calculation by the analyzing means, acoustic sense characteristics storage means for preliminarily storing acoustic sense characteristics of a deafness and a person having healthy acoustic sense from a fitting means, gain calculation data storage means for preliminarily storing an acoustic pressure range the easiest to hear for the deafness from the fitting means, and acoustic sense compensating means for performing acoustic sense compensation process by amplifying the input data with a given gain. The control means calculates the gain of each frequency range on the basis of the acoustic sense characteristics and an acoustic pressure range stored in the acoustic sense characteristics storage means and the gain calculation data storage means. Excerpt(s): The present invention relates a digital hearing aid or hearing aid for sensorineural deafness using a digital signal processing. A hearing impairment, i.e. deafness, is generally classified into two kinds, i.e. conductive deafness and sensorineural deafness. The conductive deafness is a hearing impairment caused for variation of transmission characteristics due to failure of any one or all of external ear, middle ear. This type of hearing impairment can be simply overcome by amplifying input sound. Web site: http://www.delphion.com/details?pn=US05838801__

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Educational apparatus and method for control of deaf individuals in a mixed teaching environment Inventor(s): Sapora; Robert (825 Holiday La., Westminster, MD 21157) Assignee(s): none reported Patent Number: 4,368,459 Date filed: December 16, 1980 Abstract: Radio signaling apparatus is for use, primarily, in classrooms at all educational levels, elementary school through graduate school, in which a teacher and a mixed body of hearing and deaf students participate in a lecture and discussion situation with the support of a trained interpreter in sign language. The radio system comprises a voice actuated pulsed omnidirectional radio transmitter, which is worn by the teacher, and a plurality of portable receiving devices, one for each deaf student. The microphone of the transmitter is selected to discriminate against ambient sounds and background voices. The omnidirectional receivers detect the pulsed radio transmissions and generate by transducer action a non-audible sensory stimulation to alert each individual deaf student, whereupon each such student should renew his or her observation of the interpreter. In a preferred embodiment the radio apparatus provides a flashing visual signal from each deaf student's receiver. The receiver and transducer are to be worn or

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placed within the student's peripheral field of vision when the student is working on or is reading his class notes and texts during classroom sessions. The system is adaptable to more general uses outside the classroom. Excerpt(s): The requirements of the present invention for electronic components, circuits, and radio devices are totally satisfied by well-known and well-developed arts of manufacture in the electronic and radio industries. Furthermore, it is also evident in the existing arts that the loss, by a human, of a sensory capability, such as hearing, can be compensated, at least in part, by augmentation or expanded usage of one or more of the other human senses, i.e., sight, touch, taste and smell. In the case of complete loss of hearing, via the auditory organs, perception and information transfer to the handicapped individual is presently most frequently accomplished through visual or touch sensations. However, in the field of education, specifically of the deaf, reliance upon visual aids including sign language interpretations presently results in deficiencies in information transfer from teacher to student. These deficiencies are of such magnitude as to seriously depreciate the learning process of the deaf. The deaf must, on occasion, look at other legitimate classroom objects and are likely to miss important portions of the instructions. The following references in the existing arts are relevant to compensation for an impairment in hearing ability by augmentation and use of alternative sensory capabilities of the human body. These references do not, however, disclose or teach the present invention. Web site: http://www.delphion.com/details?pn=US04368459__ •

Educational device especially for the blind and the blind-deaf Inventor(s): Sparks; William B. (3413 Kinnamon Rd., Winston-Salem, NC 27104) Assignee(s): none reported Patent Number: 4,378,215 Date filed: June 11, 1981 Abstract: An educational device for teaching or self-teaching the manual alphabet of the deaf to anyone, especially to the blind and/or deaf and for communicating among such persons is disclosed. A plurality of three-dimensional, actual size cast or molded figures of the human hand mounted on a base and each formed in a respective letter of the manual alphabet comprise an instructional kit for teaching the manual alphabet. Each hand figure is provided thereon with embossments of the Braille character and, preferably, the English alphabet letter corresponding to the manual alphabet letter represented by such figure. Another embodiment comprises statues or figures of human beings depicted in various poses communicating with each other by sign language with Braille characters and English alphabet letters affixed on the pedestal of the statues. Excerpt(s): The present invention relates to education and more particularly to educational devices for teaching or self-teaching the manual alphabet of the deaf to those who are blind, deaf, blind-deaf and to those with normal sight and hearing, as well as for teaching and communicating feelings, concepts and ideas to and among such persons. U.S. Pat. No. 1,962,687 to Hodge discloses a communication chart for use by a person to instruct others in the manual alphabet of the deaf. The chart comprises a sheet of flexible material upon which is displayed all the letters of the English alphabet. Flexible tabs are superposed over each letter on the chart and are adapted to be lifted up to expose the underlying letter. The underside of each flexible tab is provided with an illustration of the manual alphabet sign corresponding to the respective underlying

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English letter. U.S. Pat. No. 4,074,444 to Laenger, Sr. et al discloses a method and apparatus for communicating with the blind-deaf by means of a movable anthropomorphic, electromechanical hand which is electronically programmed to form letters of the one-hand manual alphabet. The electromechanical hand is controlled by an electronic buffer which converts intelligence signals from an electric typewriter or teletype machine to a code which causes mechanical motion of the hand to a programmed position corresponding to a character of the one-hand manual alphabet. Web site: http://www.delphion.com/details?pn=US04378215__ •

Extract of pine needle and the use thereof Inventor(s): Ji; Ling (No. 5-400, Dong Da Street, Fengtai District, Beijing, 100071, CN) Assignee(s): none reported Patent Number: 6,329,000 Date filed: October 1, 1999 Abstract: This invention discloses a kind of pine needle extract. The extract can be used to treat the following diseases: hypertension, coronary heart disease, angina pectoris, arrhythmia, diabetes, hyperlipemia, high blood viscosity, high blood aggregation, scleratheroma, cerebral infarction, brain scleratheroma, senile dementia, sudden deafness, etc. Excerpt(s): This invention relates to an extract of pine needle and its uses in medicines, especially its use in treating hypertension and coronary heart disease. This invention also relates to a pharmaceutical composition comprising the extract of pine needle and its pharmaceutical uses. Pine needle is also named pine leaf. Its Latin name is Folium Pini. One important thing is that Pine needle is leaf of plants of Pinaceae and Pinus. It comes from Pinus tabuleaformis carr, Pinus massoniana Lams, Pinus Yuananensis Franch, etc. In China there are 20 species and 10 varieties of Pinacaea plants, 16 species and 2 varieties have been planted. In China, there has been a view that pine needle may be used as a supplement material for some medicines for many years. For example, some of the recipes collected in the Great Dictionary of Chinese Herbs can treat injury, edema, eczema, chronic bronchitis and asomnia, or prevent influzeza, epidemic encephalitis. Pine needle is used as one of the ingredients in these recipes. Web site: http://www.delphion.com/details?pn=US06329000__

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Homozygous mutation in KVLQT1 which causes Jervell and Lange Nielsen syndrome Inventor(s): Keating; Mark T. (Salt Lake City, UT), Splawski; Igor (Salt Lake City, UT) Assignee(s): University of Utah Research Foundation (Salt Lake City, UT) Patent Number: 6,150,104 Date filed: August 17, 1998 Abstract: Jervell and Lange-Nielsen syndrome (JLN) is an autosomal recessive form of long QT syndrome. In addition to QT interval prolongation, this disorder is associated with congenital deafness. JLN is rare, but affected individuals are susceptible to cardiac arrhythmias with a high incidence of sudden death and short life expectancy. A

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homozygous mutation in KVLQT1, the potassium channel gene responsible for chromosome 11-linked long QT syndrome, is shown to be a cause of JLN. Excerpt(s): Jervell and Lange-Nielsen syndrome (JLN) is an autosomal recessive form of long QT syndrome (LQT). In addition to QT interval prolongation, this disorder is associated with congenital deafness. JLN is rare, but affected individuals are susceptible to cardiac arrhythmias with a high incidence of sudden death and short life expectancy. The present invention is directed to a mutation in the KVLQT1 gene which results in Jervell and Lange-Nielsen syndrome and to probes and methods for diagnosing the presence of JLN. JLN is diagnosed in accordance with the present invention by analyzing the DNA sequence of the KVLQT1 gene of an individual to be tested and comparing the respective DNA sequence to the known DNA sequence of a normal KVLQT1 gene. The publications and other materials used herein to illuminate the background of the invention or provide additional details respecting the practice, are incorporated by reference, and for convenience are respectively grouped in the appended l ist of References. Cardiac arrhythmias are a common cause of morbidity and mortality, accounting for approximately 11% of all natural deaths (Kannel, 1987; Willich et al., 1987). In general, presymptomatic diagnosis and treatment of individuals with life-threatening ventricular tachyarrhythmias is poor, and in some cases medical management actually increases the risk of arrhythmia and death (Cardiac Arrhythmia Suppression Trial II Investigators, 1992). These factors make early detection of individuals at risk for cardiac arrhythmias and arrhythmia prevention high priorities. Web site: http://www.delphion.com/details?pn=US06150104__ •

Method and apparatus for communicating information representative of sound waves to the deaf Inventor(s): McConnell; Jeffrey D. (3504 W. Sixth, Spokane, WA 99204) Assignee(s): none reported Patent Number: 4,813,419 Date filed: March 22, 1988 Abstract: A method and apparatus of communicating audio signals to the deaf is described. In accordance with the method described, electrical impulses characteristic of sound waves are applied either directly to the tactile sensory nerves by means of an implant or are applied simultaneously with vibrations representative of the sound waves to the nerve endings located at the skin surface. In accordance with the apparatus, a nerve stimulator applies a voltage representative of the sound waves across a pair of implanted electrodes in contact with the tactile sensory nerve such as the radial nerve branch located in the wrist or applies vibrations representative of the sound waves by means of a transducer to the skin, e.g. the finger tips of the deaf person, and electrical impulses representative of the sound waves across a pair of spaced electrodes carried by the transducer so that the same nerve endings are stimulated with both the vibrations and the electrical impulses. Excerpt(s): The present invention relates to a method and apparatus for communicating information representative of air pressure sound waves to deaf persons, and more particularly to a method and apparatus for translating sound waves into electrical signals and stimulating the tactile sensory nerves of the deaf person in accordance with such electrical signals. The prior art's efforts at providing some measure of hearing for the totally and partially deaf has been directed primarily at stimulating the inner ear or

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cochlea with signals derived from sound waves. The stimulated cochlea would in theory transmit signals to the brain through the auditory nerve, which could be translated into information representing the original sound waves. One example of such prior art efforts is described in an article entitled "Success for the `Bionic Ear,`" appearing in the Mar. 12, 1984 issue of Time Magazine. According to this article, eight wires are implanted in the deaf person's inner ear and connected to an electrical plug extending through the skull. A microphone carried on the ear translates the sound waves into electrical signals which are then processed by a computer and applied to the implanted wires. Such prior art devices require delicate surgery, are expensive, and have not yet met with any great success. Web site: http://www.delphion.com/details?pn=US04813419__ •

Method and apparatus for sound responsive tactile stimulation of deaf individuals Inventor(s): Franklin; David (9 Preston Rd., Somerville, MA 02143), Franklin; Joseph (9 Preston Rd., Somerville, MA 02143), Hughes; Paul (17 Howell Rd., Sudbury, MA 01776) Assignee(s): none reported Patent Number: 5,035,242 Date filed: April 16, 1990 Abstract: A tactile aid for deaf individuals includes a conveniently wearable array of vibration transducers mounted on a flexible carrier strip so as to be positionally biased toward the individuals's skin. Each transducer includes an enclosed magnetically vibratable cantilevered beam, an angled mounting slot to effect the positional bias mounting on the strip, and contact pads rather than conventional pin connectors to both reduce mass and facilitate electrical connection to contact pads on carrier strips in the form of printed circuit boards. Received acoustic signals are processed in at least first and second formant circuits each sub-divided into plural sub-bands corresponding to the number of transducers. In each formant circuit the corresponding formant frequency and amplitude are measured, averaged over a predetermined number of cycles, and a voltage corresponding to the measured amplitude is provided on a corresponding subhand channel to pulse width modulate a pulse train employed to excite the transducers. Spectral resolution can be increased by averaging the amplitude/frequency measurements over fewer cycles. Glottal pulses in the speech signal are monitored to provide a glottal rate signal at a stepped down frequency related to the actual glottal rate. The channel amplitude is modulated by the glottal signal to cause alternation of transducer excitation at the related glottal rate. Additional formants may be detected to derive additional information in the excitation signal. Excerpt(s): The present invention relates to methods and apparatus for converting acoustic signals to signals capable of being perceived by tactile sensing. More particularly, the present invention relates to wearable tactile devices for deaf individuals who have essentially no useful input from their own damaged auditory system. The primary utility of the present invention is to provide a means whereby such deaf individuals have access to the world of sound, both for communication purposes and, in the special case of prelingually deafened children (i.e. those either being born deaf or losing their hearing before attaining significant language capability), for learning speech and language at as early an age as possible and in as nearly natural a way as possible. As such, it is clear that a great emphasis for devices in this class must be on cosmetics and ease of use, as well as processing methods, since continuous wearing is a necessary requirement if the above goals are to be met. Before discussing the prior art in detail, it is

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important to note that, in addition to tactile sensing, there is a somewhat related class of devices called cochlear implants. As the name implies, these are devices having a stimulator implanted directly into the inner ear of a deaf individual for the purpose of obtaining some degree of hearing restoration. Generally speaking, an important distinction between tactile devices and cochlear implant devices is that cochlear implants attempt to restore some degree of the lost sense of hearing while tactile devices attempt to substitute the tactile sensory mode for the lost hearing sense. Hence, the transducers for both types of devices differ markedly, and the processing must also differ in order to accommodate the different transducer functions and different sensory modes. Tactile aid technology dates back to 1927; however, only developments since 1986 are relevant since it is within that time frame that devices with the necessary compactness and convenience to be worn continuously have made their appearance. All such devices presently in use have only one or at most two channels in spite of the fact that it has long been recognized that it is desirable to use multiple channels, each functioning in some way to analyze speech waveforms and other acoustical events according to one of a number of possible paradigms, and to present detailed information about the sound events via a multiplicity of skin exciters. It is generally understood that only in this way can enough detailed information be presented to a user sufficiently clearly and uniquely to enable him to eventually learn to recognize and appreciate the sound events in a manner analogous to that by which the intact hearing system receives, analyzes and appreciates the same sound events. Web site: http://www.delphion.com/details?pn=US05035242__ •

Method and apparatus for visual telecommunications, in particular for use by the deaf Inventor(s): Abramatic; Jean-Francois (Noisy le Roi, FR), Letellier; Philippe (Versailles, FR), Nadler; Morton (La Celle Saint Cloud, FR) Assignee(s): INRIA Institute National de Recherche en Informatique et en Automatique (Le Chesnay, FR) Patent Number: 4,546,383 Date filed: June 17, 1983 Abstract: In a system for visual telecommunications, e.g. for use by deaf people, an electronic camera (20) makes an image of a moving subject for transmission. The TV type image is passed through a contour extractor (25) to be converted to an animated cartoon line-drawing type image. The contour image is then compressed by means including a sampler (28) for image reduction, a difference detector (30) for selecting only points which change from one image to the next, a filter (34) for rejecting isolated points in the contour image, and an encoder (36) for converting sequences of on/off bits into data words for transmission, via a modem (14) over a telephone line (15). At the other end of the line a similar transmitter/receiver system decodes the received words and reconstitutes the successive images for display on a screen (46). The resulting animated cartoon type of display is adequate for communication by sign language or by lip reading, and is capable of being sufficiently compressed to be transmitted over a normal telephone line. Excerpt(s): The present invention relates to telecommunications, and in particular to telecommunications by telephone or telematics lines. The invention aims more particularly at transmitting images over such links, where it is understood that telephone links are not limited to cables or electric lines but also include communication

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links such as microwaves, optical fibers, etc. Telephone lines have traditionally been developed and installed in networks for transmitting voice communications. More recently, they have found a use in transmitting digital type data, by means of modulator-demodulators generally known as modems. Such telephone links have transmission characteristics which are well adapted to transmitting analog signals at frequencies of not more than about 4 kHz. When they are used for transmitting digital signals, they tend to be useable at data rates of up to about 4800 bauds. Such performance limits the volume of information which may be transmitted in given time. Thus, while telephone lines are well adapted to transmitting human speech or to transmitting computer messages in the form of relatively low data rate binary signals, they are not suitable for real time transmission of images which are liable to change quickly in time. Image transmission requires a relatively large amount of information to be transferred in comparison to the volume of information needed for a voice message. The image must be divided into point-like zones or "pixels" which are smaller with increasing definition to be transmitted. A signal representative of the brightness of each pixel must be produced. The set of such signals must then be transmitted, generally in series in the form of a sequence. Each sequence corresponds to an image, and the level of the signal at a given instant in the sequence corresponds to the brightness of a corresponding point in the transmitted image. Web site: http://www.delphion.com/details?pn=US04546383__ •

Method and device for presenting information to deaf persons Inventor(s): Traunmuller; Hartmut (123 Bjornstigen, Solna, SW) Assignee(s): none reported Patent Number: 4,025,732 Date filed: July 22, 1976 Abstract: In a device for presenting information to deaf persons an electroacoustic signal from a microphone is supplied to a high-pass and to a low-pass channel via an amplifier. The relation between the envelope potentials of the high-passed and the lowpassed signals is sensed by means of logarithmic circuits and a differential amplifier, the output potential of which determines the frequency of a voltage controlled oscillator. By means of a dynamic-adapting signal, derived from the electroacoustic signal, an amplitude modulation of the oscillator output is provided. The accordingly obtained amplitude modulated signal is used to drive tactile or auditive transducers. Excerpt(s): The invention relates to a method for presenting information obtained from an electroacoustic signal, especially a speech signal, via tactile or auditive transducers, and to a device for carrying out the method, intended to be used by deaf persons, and presenting such information, which is suitable as a complement to lipreading. Deaf persons without or with only insignificant residual hearing do not get any essential help by using hearing-aids. They are for speech perception constrained to use lip-reading. However, only lipreading does not make a full understanding of speech possible. Different ways of making further information in the speech available at a simultaneous lipreading have been known. A simple method consists in driving a tactile transducer (vibrator) directly with an amplified electroacoustic signal. The vibrator can be sensed by the deaf person for instance with the fingers or via the teeth. The additional information, perceptible in this way, does, however, still not make a full understanding of speech possible.

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Web site: http://www.delphion.com/details?pn=US04025732__ •

Method for detection of susceptibility mutations for ototoxic deafness Inventor(s): Fischel-Ghodsian; Nathan (Los Angeles, CA), Prezant; Toni R. (Reseda, CA) Assignee(s): Cedars-Sinai Medical Center (Los Angeles, CA) Patent Number: 5,506,101 Date filed: June 30, 1993 Abstract: Method for detection of mitochondrial nucleotide associated with predisposition for ototoxic deafness, especially deafness associated with the administration of aminoglycosides, is described. Excerpt(s): This invention relates to the area of genetic diagnosis. More specifically, the invention relates to detection of an alteration of wild-type mitochondrial ribosomal RNA(rRNA) which is associated with ototoxic deafness in humans. Sensorineural deafness, either in conjunction with neuromuscular diseases or with diabetes, has been associated with heteroplasmic mitochondrial DNA (mtDNA) mutations (Shoffner, et al., Adv. Human Genet., 19:267-330, 1990; Ballinger, et al., Nature Genet., 1:11-15, 1992; van den Ouweland, et al., Nature Genet., 1:368-371, 1992; Reardon, et al., Lancet, 34:13761379, 1992). The likelihood of mtDNA mutations has also been suggested in two forms of non-syndromic deafness. A maternal inheritance pattern has been reported in several pedigrees in the Far East with familial aminoglycoside-induced deafness (Higashi, Clin. Genet., 35:433-436, 1989; Hu, et al., J. Med. Genet., 28:79-83, 1991), and a single large Arab-Israeli pedigree with maternally-inherited congenital deafness has been described (Jaber, et al., J. Med. Genet., 29:86-90, 1992). Mitochondrial DNA is transmitted exclusively through mothers, since the sperm apparently contributes no mitochondria to the zygote. This leads to the expectation that a defect in a mitochondrial gene should lead to disease equally in both sexes, but can only be transmitted through the maternal line. There are hundreds of mitochondria in each cell and they serve a variety of metabolic functions, the most important being the synthesis of ATP by oxidative phosphorylation. Each mitochondrion contains several mitochondrial DNA (mtDNA) chromosomes, which in humans are 16,569 basepairs (bp) double-stranded circles. Replication, transcription, and translation of the mtDNA occurs within the mitochondrion. The mitochondrial DNA encodes 13 messenger RNAs, and the large and small ribosomal RNAs and 22 transfer RNAs which are necessary for their translation. The messenger RNAs are translated on mitochondrion-specific ribosomes, using a mitochondrion-specific genetic code, into 13 proteins. These proteins interact with approximately sixty nuclear encoded proteins to form the five enzyme complexes required for oxidative phosphorylation. Web site: http://www.delphion.com/details?pn=US05506101__

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Methods for communicating with a telecommunications device for the deaf Inventor(s): Lynn; Joe J. (Gaithersburg, MD), Morduch; Georg E. (Alexandria, VA) Assignee(s): Microlog Corporation (Germantown, MD) Patent Number: 5,670,957 Date filed: June 7, 1995

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Abstract: An automatic telephone answering system employs a switch activated by any of several techniques to select a language for communication with a caller. A single matrix determines the content of a message to be generated in response to input from the caller. A single matrix can be used because it is arranged in accordance with the content of the various messages and not on the specific language of the messages. All of the messages for each respective language are stored in a separate directory such that a given identifier obtained from the matrix will recall that message in the language determined by identification of the particular directory. A telecommunications device for the deaf (TDD) is treated in the same manner as a language and selection of messages employs the same matrix used for traditional languages. Received TDD signals are converted to ASCII for storage, and messages to be identified by the matrix are stored in ASCII and converted to TDD before transmission. Excerpt(s): This invention relates to the art of automatic telephone systems capable of communicating in several languages. Automated telephone answering systems are known. These systems are capable of delivering prerecorded messages and of receiving inputs from the caller either in the form of signals from the telephone, such as the Touch Tone or rotary dial signals, or voice messages from the caller. These systems communicate with humans either directly by producing the sounds of human speech or indirectly through signals which are interpreted by an electrical or electromechanical device. An automated telephone system generally consists of an input module, a control module, and an output module, even though the line of distinction is sometimes not clear. The control module may be the CPU of a computer while the input and output modules are combined in a single input-output processor (IOP) which may also be located in the computer. Web site: http://www.delphion.com/details?pn=US05670957__ •

Middle ear implant Inventor(s): Dalchow; Carsten (Dusseldorf, DE), Elies; Wolfgang (Bielefeld, DE) Assignee(s): Spiggle & Theis (Dieburg, DE) Patent Number: 6,432,139 Date filed: August 17, 2000 Abstract: Deafness is often caused by damage to the ossicular chain, which at present is partly or fully replaces by surgical implantation of a corresponding titanium prosthesis. Known versions of this type of middle ear implant consist of a flat coupling body (8) designed to rest against the ear drum, an oblong shank (3) connected to the body for bridging the tympanic cavity and a base (4,11) which is joined to the shank (3) and rests against the base of the implant in the tympanic cavity. Because the depth oft he tympanic cavity varies from patient to patient and measurements become known only during surgery, the length of the implant has to be adjusted very quickly. To be able to do this more simply than is possible with the known implants having a fixed length, the middle ear implant provided by the invention is characterized in that the oblong shank (3) has separation zones (6) to allow for individual adjustment of the shank length to the patient-specific depth of the tympanic cavity during the surgical intervention, and in that the base (4, 11) and/or the flat coupling body (1, 8) are embodied as a slide-on connector part which houses the corresponding end of the shank (3). Excerpt(s): The present invention relates to a middle ear implant with a flat coupling body for application against the eardrum, an elongated shaft connected therewith for

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spanning the space in the tympanic cavity, and a shaft-connected base for application against the supporting point for the implant in the tympanic cavity. Impaired hearing ranging up to deafness can have different pathological causes. One oft he causes typically lies in a disease-related change in or degeneration oft he small auditory bones of the ossicular chain in the middle ear, i.e., the three ossicles positioned in the tympanic cavity (Cavum tympani): the hammer, the anvil, and the stirrup. These ossicles carry sound transmission from the eardrum via the vestibule window (vestibular window) to the inner ear, the air in the middle ear making possible almost friction-free vibration of the ossicles combined together in articulated fashion. These articulated ossicles represent a lever mechanism which amplifies the recorded sound waves in the case of humans by about two to three times. If the lever mechanism is disturbed, e.g., through a degenerative change in the bone substance, a person will become hard of-hearing. Web site: http://www.delphion.com/details?pn=US06432139__ •

Mutated polynucleotide corresponding to a mutation responsible for prelingual nonsyndromic deafness in the connexin 26 gene and method of detecting this hereditary defect Inventor(s): Denoyelle-Gryson; Francoise (Arcueil, FR), Guesdon; Jean-Luc (Sevres, FR), Marlin-Duvernois; Sandrine (Colombes, FR), Petit; Christine (Le Plessis-Robinson, FR), Weil; Dominique (Paris, FR) Assignee(s): Institut Pasteur (Paris, FR) Patent Number: 5,998,147 Date filed: August 14, 1998 Abstract: A purified polynucleotide having a chain of nucleotides corresponding to a mutated sequence, which in a wild type form encodes a polypeptide implicated in hereditary sensory defect, wherein said mutated purified polynucleotide presents a mutation responsible for prelingual non-syndromic deafness selected from the group consisting of a specific deletion of at least one nucleotide. Excerpt(s): The present invention concerns a mutation responsible for autosomal prelingual non-syndromic deafness and a method for the detection of this hereditary sensory defect for homozygous and heterozygous individuals. The invention concerns more particularly a specific deletion of at least one nucleotide in the connexin 26 (Cx 26) gene and especially in a guanosine rich region, notably between the nucleotides 27 and 32. The invention is also directed to the use of polynucleotide, or fragments thereof, for example as tools useful for the in vitro detection of a mutation of a gene belonging to the Cx26 gene family. Profound or severe prelingual deafness affects one child in a thousand in developed countries (Morton N E. Genetic epidemiology of hearing impairment. In Genetics of hearing impairment. (The New York Acad Sci, New York 1991; 630:16-31). It is a major handicap as it impedes language acquisition. According to studies performed in a U.S. population of children with non-syndromic (isolated) prelingual deafness and in whom an obvious environmental cause has been excluded, it is estimated that up to two-thirds of the cases have a genetic basis (Marazita M L, Ploughman L M, Rawlings B, Remington E, Arnos K S, Nance W E. Genetic epidemiological studies of early-onset deafness in the U.S. school-age population. Am J Med Genet 1993; 46:486-91). These forms are mainly sensorineural and are almost exclusively monogenic. The major mode of inheritance is autosomal recessive (DFNB), involving 72% to 85% of cases, this fraction increasing to 90% when only profound deafness is taken into account.

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Web site: http://www.delphion.com/details?pn=US05998147__ •

Pharmaceutical composition for treatment of sudden deafness Inventor(s): Fukushima; Hideyuki (Otsu, JP), Kanemaru; Shinichi (Osaka, JP) Assignee(s): Hoffmann-La Roche Inc. (NJ), Sumitomo Pharmaceuticals Company, Ltd. (Osaka, JP), Takeda Chemical Industries, Ltd. (Osaka, JP) Patent Number: 5,698,232 Date filed: November 8, 1996 Abstract: An interferon is administered singly or in combination with other pharmacological agents to patients with idiopathic sudden sensorineural hearing loss, whereby the patients are remarkably improved. Excerpt(s): The present invention relates to a pharmaceutical composition for the treatment of idiopathic sudden sensorineural hearing loss in human patients. Among inner ear deafnesses which have an abrupt onset, deafness with unknown etiology is called idiopathic sudden sensorineural hearing loss (abbreviated ISSHL hereunder). Reference No. 1 (References referred to in the present specification are listed hereinafter), for example, describes: "The Research and Study Group of the Ministry of Health & Welfare: 1973--Idiopathic Sudden Sensorineural Hearing Loss in Special Diseases", as one criteria for diagnosis, calls for "where the time of the initial onset of the symptoms can be clearly determined" and removes from the criteria, "those where etiology can be clearly known". In other words, ISSHL is a syndrome having acute deafness to sound as the main symptom, of which causes are unknown; the onset mechanism and pathema are still under hypotheses. The major proposed hypothetical etiologies are circulation disorder in auris interna, viral infection and labyrinthine window breaks. Those etiologies are merely hypothetical, owing to the impossibility of experimental models of the ISSHL, scarcity of autopsies and those available being obsolete". (see Reference No. 1). Currently, ISSHL patients are treated with a steroids, cardiovascular agents and vitamins in combination therewith for the purpose of improving blood circulation. Because etiology and pathema of the disease are still unknown, such treatment has not resulted in decisive improvements (see Reference Nos. 1, 2 and 3). Particularly, the treatment for the severe type of ISSHL has not reached any satisfactory results, even where the treatment has been undertaken at an early stage of the disease. Thus, the recovery of practical audio abilities could not be currently obtained. Web site: http://www.delphion.com/details?pn=US05698232__

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Receiving caller identification information with a telecommunications device for the deaf Inventor(s): Quagliana; Douglas D. (Edison, NJ) Assignee(s): Intel Corporation (Santa Clara, CA) Patent Number: 6,560,317 Date filed: January 3, 2002 Abstract: A central office (CO) for a telephone connected with a telecommunication device for the deaf (TDD) called by a calling device determines that the called TDD is

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connected with the called CO. The called CO postpones indication that the called TDD has connected with the called CO, until the called CO determines whether the called TDD is willing to connect with the calling device. This causes the calling device to continue to receive an indication that a connection has yet to be established between the calling device and the called TDD. As a result of being connected with the telephone connected with the TDD, the called CO transmits caller identification (CID) information regarding the calling device in a format that can be received by a TDD. Excerpt(s): The present invention relates to receiving caller identification (CID) information with a telecommunications device for the deaf (TDD). Wired telephones are connected to a telephone company's central office (CO) through a two-wire circuit called a local loop. A CO contains switching equipment that connects the telephone lines of a calling telephone terminating at one CO to the telephone lines of a called telephone terminating at another CO. When the switches between a telephone and a CO are open (e.g., because a telephone handset is holding down a switchhook), there is no connection between the telephone and the CO. However, once the switches between a telephone and a CO are closed (e.g., because the telephone handset is removed, causing the switchhook to lift up), the local loop is complete, thus establishing a connection between the telephone and the CO. When a calling telephone is connected with the calling telephone's CO (the "calling CO"), it generates a signal that tells the calling CO that a person wants to make a call. The calling CO returns a dial tone to the calling telephone indicating that the calling CO is ready to accept a telephone number. The person dials a telephone number that is sent to the calling CO. The calling CO transmits the telephone number over a telephone network and connects with a called telephone's CO (the "called CO"). Web site: http://www.delphion.com/details?pn=US06560317__ •

Ringing detector for use by the deaf Inventor(s): Sparber; Richard G. (Wheaton, IL) Assignee(s): Bell Telephone Laboratories, Incorporated (Murray Hill, NJ) Patent Number: 4,379,210 Date filed: May 28, 1981 Abstract: A ringing detector circuit for use by the deaf which may be connected between the tip and ring conductors (11, 12) of a subscriber set to cause an incandescent lamp (40) to flash to half brightness when ringing voltage is applied to the line. Closing a manual switch (22) allows the lamp (40) to burn at half brightness when the phone is not ringing and to flash to full brightness when the phone is ringing. Excerpt(s): This invention relates to telephone ringing detectors for use by the deaf and, more particularly, to such detectors including a visual indicator. One important result of the recent, increased social awareness of the needs and rights of handicapped persons has been the development of telecommunications devices for the deaf (TDD). Such devices, typically comprising a battery-powered, microprocessor-controlled teletypewriter with moving visual display, are acoustically coupled to conventional telephones to permit hearing-impaired persons to communicate readily and conveniently with others. Special provisions must be made to alert hearing-impaired persons of incoming calls. One known ring indicator can be connected to any room lamp to cause the lamp to flash on repeatedly when ringing voltage is present on the telephone line. Further, if the lamp is being used to illuminate the room, the indicator

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will cause the lamp to flash off repeatedly to alert the hearing-impaired person. However, from a human-factors viewpoint, such an indicator is highly undesirable when the lamp used for alerting is also used to illuminate the room because the people in the room will be subjected to darkness during the active part of each ringing cycle. Accordingly, it is an object of the present invention to provide a reliable, inexpensive ringing detector which can be employed in conjunction with a lamp used for room illumination without annoying light interruptions during ringing. Web site: http://www.delphion.com/details?pn=US04379210__ •

Shared bus arbitration apparatus having a deaf node Inventor(s): Abidi; Mohammad V. (Shrewsbury, MA), Cao; Xi-Ren (Acton, MA), ColonOsorio; Fernando (Stow, MA), Lary; Richard F. (Colorado Springs, CO), Quaynor; Nii (Shrewsbury, MA) Assignee(s): Digital Equipment Corporation (Maynard, MA) Patent Number: 5,239,630 Date filed: July 2, 1990 Abstract: An improved arbitration technique for a computer network system in which multiple nodes communicate using a shared bus, and at least one node has no knowledge of the current status of the arbitration taking place between all nodes in the system. Such a node is called a "deaf node". Each node in the system is assigned an initial arbitration count number, for example, N+1, where N is the node number assigned to the node. The arbitration count number is the number of quiet slots a node must count before trying to transmit on the system bus. The length of a quiet slot is determined by a particular system's electrical characteristics. One quiet slot is reserved as the "deaf node quiet slot", during which a deaf node may transmit. In response to a transmission occurring in the deaf node quiet slot, each node in the system reinitializes its arbitration count number to its initial arbitration count number. Additionally, a node that is not a deaf node, but has detected a system error, may deliberately transmit during the deaf node quiet slot to reinitialize the arbitration count numbers or the internal clocks of all nodes in the system. Excerpt(s): This invention relates to computer systems having nodes communicating through a bus, and more particularly to an arbitration apparatus for determining which node transmits next on the bus. A computer system may have a number of nodes communicating with each other through a bus. The nodes may be individual computers, controllers for mass storage devices, or other computer apparatus. A problem arising in such a system architecture is the problem of determining which node transmits next on the bus. For example, a first node may be presently transmitting a message on the bus. Upon the conclusion of that transmission, there may be several nodes seeking to begin transmission. If more than one node begins transmission at the same time, say at the completion of the transmission of the first nodes, then a data collision will occur. The problem of determining which node transmits next on a shared, multiple access, bus is termed the "arbitration problem". The arbitration problem may be solved by having a node test the bus in order to determine if carrier is present on the bus, and then to either begin transmitting in the absence of carrier, or if carrier is detected to initiate a backoff protocol as in the well known Ethernet or CSMA/CD (Carrier Sense Multiple Access/Collison Detect protocol). An improved solution of the arbitration problem is for a node to count a number of "quiet slots" after detecting the absence of carrier on the bus, in order to minimize the probability that a data collision will occur when the node

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begins transmitting, where a quiet slot is a predetermined time interval, and a fixed number of quiet slots is assigned to each node. An even further improvement in the arbitration protocol makes use of a re-assignment of the fixed number of quiet slots assigned to each node, where the re-assignment is done according to rules designed to reduce bandwidth of the bus used in counting quiet slots. Web site: http://www.delphion.com/details?pn=US05239630__ •

Speech pattern correction device for deaf and voice-impaired Inventor(s): Davis; Stephen A. (Coral Springs, FL) Assignee(s): Davis, Van Nortwick & Company (Gainesville, FL) Patent Number: 5,146,502 Date filed: February 26, 1990 Abstract: A portable voice or speech aid enabling a deaf or voice impaired user to make sounds into a microphone to output intelligible speech through a built-in speaker or to a text display screen. Excerpt(s): This invention relates to computer aided devices enabling deaf or voiceimpaired people to communicate. Computer aid devices for deaf or voice-impaired persons fall into two categories. The first type avoids the need for the production of speech. For example, a computer can interpret hand positions signed by deaf people, or can allow deaf people to type messages which can be transmitted over telephone lines. The second type of device teaches the deaf to speak understandably. But in using the second category, the voice impaired person may not be able to learn to speak understandably, and a deaf person may find learning difficult and time consuming. For both deaf and voice impaired, the user effort may be extensive and results may be embarrassing. However, it is important to recognize that deaf or voice-impaired people can utter sounds in a reasonably consistent fashion. Web site: http://www.delphion.com/details?pn=US05146502__

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Stapedial-saccular strut and method Inventor(s): Lenhardt; Martin L. (Hayes, VA) Assignee(s): Sound Techniques Systems, LLC (Arlington, VA) Patent Number: 6,368,267 Date filed: October 14, 1999 Abstract: A system and method for an auditory inner ear prosthesis. A strut connects the medial surface of the stape footplate with the wall of the saccule. The strut spans the 1.5 mm vestibule between the saccule and the stapes. The strut is a synthetic elastic connector constructed of biocompatible material such as sylastic. The strut converts eardrum or ossicle vibration into saccular stimulation. The saccule is a large otic receptor organ capable of coding sound, and plays a role in reptilian hearing. For human ears, the saccule is so isolated from the eardrum and related elements that sound stimulation is ineffective. The strut allows for better stimulation by direct coupling. In cases of deafness, the saccule can serve as an alternative ear.

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Excerpt(s): The present invention relates to a system and method for an auditory ear prosthesis. In particular, the present invention relates to the construction of a strut for connecting the medial surface of the stape footplate with the wall of the saccule. One type of conventional hearing aid provides for an air-conduction amplifying system such that a microphone picks up air conduction sounds, amplifies them and presents them in the ear canals as an air conduction signal to the eardrum. Such hearing aids offer a small frequency range as well as a small dynamic range of intensity. Another type of conventional hearing aid is called a bone conduction hearing aid, which has been developed for users where the conventional air-conduction hearing aid is not satisfactory in improving hearing for those users. A bone conduction hearing aid is attached to the head of the user, and the output from a microphone pick-up is amplified and fed into a device which causes bone vibration. These devices operate over a small dynamic range and are designed principally for persons whose middle ears could not be surgically repaired or for very young children who have abnormalities of the middle ear that cannot be surgically repaired until they are older. As such, bone conduction hearing aids are rarely used. Web site: http://www.delphion.com/details?pn=US06368267__ •

Tactile stimulation device for use by a deaf person Inventor(s): Coudon; Jean-Max (La Buchetterie, 6 Avenue Hildegarde, 49240 Avrille, FR) Assignee(s): none reported Patent Number: 6,628,195 Date filed: November 7, 2000 Abstract: The tactile stimulation device designed to be used by a deaf person includes, in particular, an electroacoustic transducer. The invention is noteworthy in that the device consists of a case which can be held in one hand and which has a sensor associated with the electroacoustic transducer, which sensor is designed to enable turning the device on by touching said sensor. Excerpt(s): The present invention concerns a tactile stimulation device capable of transforming an audio signal into vibrations which can be sensed by the skin of a person. The field of the invention is of special interest to deaf persons or to persons with a hearing deficiency whose auditory apparatus is deficient or nonfunctional. In order for a deaf person to be able to understand a speaker, the most commonly known method consists in that this person learns to read lips. Web site: http://www.delphion.com/details?pn=US06628195__

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Telecommunication device for the deaf with automatic self-identification Inventor(s): Colwell; Kevin (Middleton, WI), Engelke; Robert M. (Madison, WI), Schultz; Ronald W. (Madison, WI) Assignee(s): Ultratec, Inc. (Madison, WI) Patent Number: 5,325,417 Date filed: May 20, 1992 Abstract: A telecommunication device for the deaf includes a self-identification sequence which automatically commences upon the initiation of communication with

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the device. The self-identification sequence consists of a series of characters which may be separated by pauses and the device listens during the pauses to see if TDD communication has been established. The purpose of the character string is so that a receiving station can identify, with a low rate of error, a calling TDD and therefore switch the incoming call to a TDD equipped operator. This capability is particularly useful for emergency service operators (911 services) which must be able to handle incoming TDD calls with a maximum of efficiency and a minimum of error. Excerpt(s): The present invention relates to a telecommunication device for the deaf which is capable of automatically identifying itself to the party on the other end of the line. Persons who are deaf or hearing-impaired who cannot hear well enough to use the telephone commonly make use of communication terminals specifically constructed and designed to enable such persons to converse over the telephone lines. Such devices are referred to as telecommunication devices for the deaf or TDD and include both a keyboard and a display connected to the telephone through a modem (modulator/demodulator). The modem is typically built into the TDD and either directly wired to a telephone line or coupled through an acoustic coupler to a normal telephone handset. The TDD is capable of transmitting information over a telephone line by means of coded tones to another similar TDD connected at the opposite end of the telephone line through another modem. There are several protocols by which electronic devices are capable of communicating through analog lines, such as telephone lines. The most common used in the industry is referred to as ASCII (American Standard Code for Information Interchange), and is commonly used for information interchange between computers. However, historically, TDD have operated on a different protocol, referred to as Baudot. There is both a Baudot code and a protocol of communication referred to as Baudot/Weitbrecht. Web site: http://www.delphion.com/details?pn=US05325417__ •

Telephone communications system for the deaf Inventor(s): Meyer; Jerome Charles (1512 Ben Roe Ave., Los Altos, CA 94022) Assignee(s): none reported Patent Number: 4,012,599 Date filed: July 29, 1975 Abstract: A self-powered, portable, hand-held communicator and system provides a deaf user with a visual display of conversational information transmitted to him with a standard pushbutton telephone. A highly efficient and easily remembered encoding scheme is featured wherein the alphabetic character encoding utilizes the standard markings on the telephone pushbuttons and the relative positional location of the pushbuttons. Rapid tactile transmission of messages by a user is enhanced and user fatigue minimized by requiring only a single pushbutton stroke per hand to transmit any alphabetic character. User fatigue is additionally minimized by distributing pushbutton actuation requirements for normal conversation among a plurality of the fingers of each hand and by assigning control functions to pushbuttons having positions spatially and visually related to the encoding function they perform and to the user's finger positions during message transmission. Excerpt(s): This invention relates to an apparatus for enabling deaf persons to converse over ordinary pushbutton telephone instruments and more particularly to a communicator and system employing an encoding technique and control key selection

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designed to minimize user fatigue and to enhance rate of word transmission during a normal conversation between deaf users. Various systems have been disclosed for communicating data by using the pushbutton telephone set as a communications instrument. Flanagan et al in U.S. Pat. No. 3,675,513 describes a pushbutton telephoneteletypewriter communications system wherein encoded teletypewriter functions can be sent from either a special typewriter or a standard pushbutton telephone over telephone lines to a receiving typewriter for producing a permanent record of information received. Flanagan utilizes an encoding scheme requiring as many as four pushbutton strokes for selection of alphabetic characters. Multiple actuation is required of selected control keys for character selection. Steury in U.S Pat. No. 3,870,821 describes an improvement in pushbutton telephone communications systems of the general type described by Flanagan wherein a code chart display for indicating the particular set of characters, symbols or function signals is required. Selection and transmission of a desired alpha character can require three pushbutton actuations if an alpha portion of an alphanumeric message is transmitted by a device constructed as taught by Steury. It is an object of this invention to provide an apparatus to deaf users for communicating over standard pushbutton telephones wherein a visual indication of a received or transmitted alphabetic character is provided and transmission of an alphabetic character requires a maximum of only two pushbutton strokes. Web site: http://www.delphion.com/details?pn=US04012599__ •

Telephone for the deaf and method of using same Inventor(s): Liebermann; Raanan (79 Bayard Ave., North Haven, CT 06473) Assignee(s): none reported Patent Number: 5,982,853 Date filed: May 23, 1996 Abstract: An electronic communications system for the deaf includes a video apparatus for observing and digitizing the facial, body and hand and finger signing motions of a deaf person, an electronic translator for translating the digitized signing motions into words and phrases, and an electronic output for the words and phrases. The video apparatus desirably includes both a video camera and a video display which will display signing motions provided by translating spoken words of a hearing person into digitized images. The system may function as a translator by outputting the translated words and phrases as synthetic speech at the deaf person's location for another person at that location, and that person's speech may be picked up, translated, and displayed as signing motions on a display in the video apparatus. Excerpt(s): The present invention relates to electronic apparatus for communication by the deaf, and, more particularly, to such apparatus which enables the deaf person to communicate through use of sign language. Deaf people are employed in almost every occupational field. They drive cars, get married, buy homes, and have children, much like everyone else. Because of many inherent communication difficulties, most deaf people are more comfortable when associating with other deaf people. They tend to marry deaf people whom they have met at schools for the deaf or through deaf clubs. Most deaf couples have hearing children who learn sign language early in life to communicate with their parents. Many deaf people tend to have special electronics and telecommunications equipment in their homes. Captioning decoders may be on their televisions, and electrical hook-ups may flash lights to indicate when the baby is crying, the doorbell is ringing, or the alarm clock is going off. However, deaf persons have

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substantial difficulties in communicating with persons at remote locations. One technique which is employed utilizes a teletype machine for use by the deaf person to transmit his message and also to receive messages, and the person with whom the deaf person is communicating also has such a teletype machine so that there is an effective connection directly between them. In another method, the deaf person utilizes a teletype machine, but the person who is communicating with the deaf person is in contact with a communications center where a person reads the transmission to the hearing person over the telephone and receives the telephone message from the hearing person and transmits that information on the teletype machine to the deaf person. Obviously, this teletype based system is limited and requires the deaf person to be able to manipulate a teletype machine and to understand effectively the written information which he or she receives on the teletype machine. Processing rapidly received written information is not always effective with those who have been profoundly deaf for extended periods of time. Moreover, a system based upon such teletype transmissions is generally relatively slow. Web site: http://www.delphion.com/details?pn=US05982853__

Patent Applications on Deafness As of December 2000, U.S. patent applications are open to public viewing.10 Applications are patent requests which have yet to be granted. (The process to achieve a patent can take several years.) The following patent applications have been filed since December 2000 relating to deafness: •

Alterations in the long QT syndrome genes KVLQT1 and SCN5A and methods for detecting same Inventor(s): Keating, Mark T.; (Brookline, MA), Splawski, Igor; (Allston, MA) Correspondence: Rothwell, Figg, Ernst & Manbeck, P.C.; 555 13th Street, N.W.; Suite 701, East Tower; Washington; DC; 20004; US Patent Application Number: 20020061524 Date filed: April 24, 2001 Abstract: Long QT Syndrome (LQTS) is a cardiovascular disorder characterized by prolongation of the QT interval on electrocardiogram and presence of syncope, seizures and sudden death. Five genes have been implicated in Romano-Ward syndrome, the autosomal dominant form of LQTS. These genes are KVLQT1, HERG, SCN5A, KCNE1 and KCNE2. Mutations in KVLQt1 and KCNE1 also cause the Jervell and Lange-Nielsen syndrome, a form of LQTS associated with deafness, a phenotypic abnormality inherited in an autosomal recessive fashion. Mutational analyses were used to screen 262 unrelated individuals with LQTS for mutations in the five defined genes. A total of 134 mutations were observed of which eighty were novel. Excerpt(s): The present invention is related to provisional application Ser. No. 60/190,057 filed Mar. 17, 2000, and is also related to provisional application Ser. No. 60/147,488 filed Aug. 9, 1999, both of which are incorporated herein by reference. Long QT Syndrome (LQTS) is a cardiovascular disorder characterized by prolongation of the QT interval on electrocardiogram and presence of syncope, seizures and sudden death,

10

This has been a common practice outside the United States prior to December 2000.

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usually in young, otherwise healthy individuals (Jervell and Lange-Nielsen, 1957; Romano et al., 1963; Ward, 1964). The clinical features of LQTS result from episodic ventricular tachyarrhythmias, such as torsade de pointes and ventricular fibrillation (Schwartz et al., 1975; Moss et al., 1991). Two inherited forms of LQTS exist. The more common form, Romano-Ward syndrome (RW), is not associated with other phenotypic abnormalities and is inherited as an autosomal dominant trait with variable penetrance (Roman et al., 1963; Ward, 1964). Jervell and Lange-Nielsen syndrome (JLN) is characterized by the presence of deafness, a phenotypic abnormality inherited as an autosomal recessive trait (Jervell and Lange-Nielsen, 1957). LQTS can also be acquired, usually as a result of pharmacologic therapy. In previous studies, we mapped LQTS loci to chromosomes 11p15.5 (LQT1) (Keating et al., 1991), 7 q35-36 (LQT2) (Jiang et al., 1994) and LQT3 to 3p21-24 (Jiang et al., 1994). A fourth locus (LQT4) was mapped to 4q25-27 (Schott et al., 1995). Five genes have been implicated in Romano-Ward syndrome, the autosomal dominant form of LQTS. These genes are KVLQT1 (LQT1) (Wang Q. et al., 1996a), HERG (LQT2) (Curran et al., 1995), SCN5A (LQT3) (Wang et al., 1995a), and two genes located at 21q22-KCNE1 (LQT5) (Splawski et al., 1997a) and KCNE2 (LQT6) (Abbott et al., 1999). Mutations in KVLQT1 and KCNE1 also cause the Jervell and Lange-Nielsen syndrome, a form of LQTS associated with deafness, a phenotypic abnormality inherited in an autosomal recessive fashion. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Diagnostic test kits Inventor(s): Gersdorff, Michel; (Brussels, BE), Rousseau, Guy; (Brussels, BE), Tomasi, Jean-Paul; (Brussels, BE) Correspondence: Trask Britt; P.O. Box 2550; Salt Lake City; UT; 84110; US Patent Application Number: 20030082171 Date filed: October 16, 2002 Abstract: The invention provides a method for the treatment of prophylaxis of autoimmune inner ear disease, in particular, deafness caused by autoimmune inner ear disease, which comprises administering to a host in need thereof a vaccine comprising peripheral myelin protein zero. The invention further provides diagnostic methods and kits derived from the protein. Excerpt(s): This application is a divisional of Ser. No. 09/611,908, filed Jul. 7, 2000, which is a continuation of U.S. application Ser. No. 09/125,754, filed Sep. 29, 1998, abandoned, which is a 371 of International Application Serial No. PCT/EP97/01003, filed Feb. 28, 1997, published as PCT International Publication Number WO 97/32598 on Sep. 12, 1997, the contents of which are incorporated by this reference. This invention relates to novel vaccine and pharmaceutical formulations and to their manufacture and use in the treatment of deafness. In particular, the invention relates to the use of the protein known as major peripheral myelin protein zero (abbreviated to MPP, Pzero or Po) in a vaccine. The invention further relates to the use of the protein in diagnostic methods and to a kit for use in carrying out such methods. There is a high incidence of inner ear diseases such as progressive sensorineural hearing loss, sudden deafness, otosclerosis and Meniere's disease. The etiology of these inner ear diseases remains unclear or unknown. However, evidence now suggests that certain inner ear diseases, including those above, appear to be of autoimmune origin. In patients with inner ear disease, several attempts have been made to identify specific antigens with which circulating antibodies and active lymphocytes react. Antibodies against type II collagen and heat

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shock protein p70 have been described in patients suffering from idiopathic inner ear disease (e.g. Yoo et al., Science 1982, 217, 1153-1155). Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Dosage forms useful for modifying conditions and functions associated with hearing loss and/or tinnitus Inventor(s): Pearson, Don C.; (Lakewood, WA), Richardson, Kenneth T.; (Anchorage, AK) Correspondence: M. Henry Heines; Townsend And Townsend And Crew Llp; Two Embarcadero Center, 8th Floor; San Francisco; CA; 94111-3834; US Patent Application Number: 20020061870 Date filed: January 19, 2001 Abstract: The invention defines interdependent biofactors and biomolecules, and clinically useful formulations that are comprised of them. The active agents are demonstrated to be complementary in their physiologic functions especially as these relate to the quenching of free radicals and to the support of endothelial physiology, the reduction of hyperinsulinemia and improvements in vascular health. The active components of the invention are selected for inclusion in precise combinations specifically because they improve these various conditions and physiological functions, and by so doing reduce a variety of risks associated with hearing loss and tinnitus. The resulting enhancement of general systemic vascular health, improvement in local VIII.sup.th nerve vascular health, modulation of conditions surrounding blood fluid dynamics, the consequences of hyperinsulinemia, and improvements in free radical defenses, all reduce the potential for cochlear hair cell death and VIII.sup.th nerve atrophy, and the hearing loss and possible deafness that accompany them. Excerpt(s): This application is related to United States Provisional Patent Application No. 60/178,487, filed Jan. 27, 2000, and claims all benefits legally available therefrom. Provisional Patent Application No. 60/178,487 is hereby incorporated by reference for all purposes capable of being served thereby. This invention is in the field of pharmacology and relates specifically to the improvement of clinical conditions associated with symptomatic or presymptomatic hearing loss and/or tinnitus and the reduction of risks associated with their onset. The ear of humans consists of three parts: the outer, middle and inner ear. The outer ear consists of the external ear and the auditory canal. The external ear modifies sound waves and the air-filled auditory canal conducts the sound waves to the middle ear, which consists of the tympanic membrane, or eardrum; the eustachian tube; and three tiny bones called the hammer, anvil, and stirrup. Membranes and bone surround the middle ear with the eustachian tube connecting it to the pharynx, equalizing the air pressure between the middle ear and the atmosphere. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

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Method for diagnosing and treating dentinogenesis imperfecta type II by using dentin sialophsphoprotein (dspp)gene and its encoding product Inventor(s): Hu, Landian; (Shanghai, CN), Kong, Xiangyin; (Shanghai, CN), Xiao, Shangxi; (Shanghai, CN), Yu, Chuan; (Shanghai, CN), Zhao, Guoping; (Shanghai, CN) Correspondence: Merchant & Gould PC; P.O. Box 2903; Minneapolis; MN; 55402-0903; US Patent Application Number: 20030180280 Date filed: March 5, 2003 Abstract: The invention has disclosed a method for diagnosis of dentinogenesis imperfecta type II (DGI-II) and/or dentinogenesis imperfecta type II with deafness (DGI-II with deafness). Said method comprises the steps of detecting the DSPP gene, transcript and/or protein in said subject and comparing it with the normal DSPP gene, transcript and/or protein to determine whether there is any variation, wherein said variation indicates that the possibility of suffering DGI-II and/or DGI-II with deafness in said subject is higher than the normal population. The present invention also discloses the method and pharmaceutical composition for treating DGI-II and/or DGI-II with deafness. Excerpt(s): This invention relates to both biological engineering and medical fields. In particular, it relates to a method of diagnosing and treating dentinogenesis imperfecta type II using human dentin sialophosphoprotein or DSPP gene and the coded product, and a pharmaceutical composition containing DSPP gene and/or protein. The odontoblasts produce the dentin, which consists in mature tooth or the tooth during tooth development phase. During dentinogenesis, the odontoblasts form dentinal tubules. Dentin cell processes in these tubules make dentin a living tissue. During the primary stage of dentinogenesis, the odontoblasts synthesize, secrete and reabsorb the dentin matrix components. Protein synthesis occurs within cells. Exocytosis and endocytosis occurs mainly in cell processes. The first material formed is unmineralized mantle dentin matrix, mainly including collagen secreted by cells and non-collagenous components. The fasciculata collagen fibers congregate to a ball structure. Due to the continual increase of new fibrils, collagen becomes closer and closer. As a result, these prophase collagen fibers change into collagen fibers. Thus predentin characterized by collagen matrix is formed. Later, the mineralization crystals gradually deposited to become dentin at some distance away from cells. The mature dentin contains more inorganic minerals than the bone. 65 wt. % of dentin are minerals, mostly hydroxyapatite crystals. Organic materials are 20%, mainly collagenous proteins and non-collagenous proteins. These collagens offer braces to the deposition of hydroxyapatite plate like crystalline. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

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More protein-protein interactions in the inner ear Inventor(s): Boeda, Batiste; (Paris, FR), Daviet, Laurent; (Paris, FR), El-Amraoui, Aziz; (Paris, FR), Legrain, Pierre; (Paris, FR), Petit, Christine; (Le Plessis Robinson, FR) Correspondence: Lerner, David, Littenberg,; Krumholz & Mentlik; 600 South Avenue West; Westfield; NJ; 07090; US Patent Application Number: 20030148381 Date filed: June 21, 2002

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Abstract: The present invention relates to protein-protein interactions involved in deafness or in hearing disorders and/or diseases. More specifically, the present invention relates to complexes of polypeptides or polynucleotides encoding the polypeptides, fragments of the polypeptides, antibodies to the complexes, Selected Interacting Domains (SID.RTM.) which are identified due to the protein-protein interactions, methods for screening drugs for agents which modulate the interaction of proteins and pharmaceutical compositions that are capable of modulating the proteinprotein interactions. Excerpt(s): This application claims priority from U.S. provisional application No. 60/299,848 filed Jun. 21, 2001 and European application No. 02290277.9 filed Feb. 5, 2002. Most biological processes involve specific protein-protein interactions. Proteinprotein interactions enable two or more proteins to associate. A large number of noncovalent bonds form between the proteins when two protein surfaces are precisely matched. These bonds account for the specificity of recognition. Thus, protein-protein interactions are involved, for example, in the assembly of enzyme subunits, in antibodyantigen recognition, in the formation of biochemical complexes, in the correct folding of proteins, in the metabolism of proteins, in the transport of proteins, in the localization of proteins, in protein turnover, in first translation modifications, in the core structures of viruses and in signal transduction. General methodologies to identify interacting proteins or to study these interactions have been developed. Among these methods are the two-hybrid system originally developed by Fields and co-workers and described, for example, in U.S. Pat. Nos. 5,283,173, 5,468,614 and 5,667,973, which are hereby incorporated by reference. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Mutation within the connexin 26 gene responsible for prelingual non-syndromic deafness and method of detection Inventor(s): Denoyelle-Gryson, Francoise; (Sceaux, FR), Guesdon, Jean-Luc; (Sevres, FR), Marlin-Duvernois, Sandrine; (Colombes, FR), Petit, Christine; (Le Plessis-Robinson, FR), Weil, Dominique; (Paris, FR) Correspondence: Finnegan, Henderson, Farabow, Garrett & Dunner; Llp; 1300 I Street, NW; Washington; DC; 20005; US Patent Application Number: 20030170676 Date filed: October 23, 2002 Abstract: A purified polynucleotide having a chain of nucleotides corresponding to a mutated sequence, which in a wild form encodes a polypeptide implicated in hereditary sensory defect, wherein said mutated purified polynucleotide presents a mutation responsible for prelingual non-syndromic deafness selected from the group consisting of a specific deletion of at least one nucleotide. Excerpt(s): The present invention concerns a mutation responsible for autosomal prelingual non-syndromic deafness and a method for the detection of this hereditary sensory defect for homozygous and heterozygous individuals. The invention concerns more particularly a specific deletion of at least one nucleotide in the connexin 26 (Cx 26) gene and especially in a guanosine rich region, notably between the nucleotides 27 and 32. The invention is also directed to the use of polynucleotide, or fragments thereof, for example as tools useful for the in vitro detection of a mutation of a gene belonging to the Cx26 gene family. Profound or severe prelingual deafness affects one child in a

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thousand in developed countries (Morton N E. Genetic epidemiology of hearing impairment. In Genetics of hearing impairment. (The New York Acad Sci, New York 1991; 630:16-31). It is a major handicap as it impedes language acquisition. According to studies performed in a U.S. population of children with non-syndromic (isolated) prelingual deafness and in whom an obvious environmental cause has been excluded, it is estimated that up to two-thirds of the cases have a genetic basis (Marazita M L, Ploughman L M, Rawlings B, Remington E, Arnos K S, Nance W E. Genetic epidemiological studies of early-onset deafness in the U.S. school-age population. Am J Med Genet 1993; 46:486-91). These forms are mainly sensorineural and are almost exclusively monogenic. The major mode of inheritance is autosomal recessive (DFNB), involving 72% to 85% of cases, this fraction increasing to 90% when only profound deafness is taken into account. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Prevention of cisplatin induced deafness Inventor(s): Ehrsson, Hans; (Stockholm, SE), Ekborn, Andreas; (Stockholm, SE), Laurell, Goran; (Stockholm, SE), Miller, Josef; (Ann Arbor, MI) Correspondence: Medlen & Carroll, Llp; Suite 350; 101 Howard Street; San Francisco; CA; 94105; US Patent Application Number: 20030180388 Date filed: January 16, 2003 Abstract: The present invention relates to compositions and methods for the protection and restoration of hearing. In particular, the present invention relates to methods and compositions for the prevention of chemical (e.g., cisplatin) induced deafness. The present invention thus provides methods of improving the outcome of subjects treated with cisplatin. Excerpt(s): This application claims priority to U.S. provisional patent application serial No. 60/349,801, filed Jan. 17, 2002 and U.S. provisional patent application serial No. 60/351,662, filed Jan. 25, 2002. The present invention relates to compositions and methods for the protection and restoration of hearing. In particular, the present invention relates to methods and compositions for the prevention of chemical (e.g., cisplatin) induced deafness. Hearing impairment is the United State's number one disability. It has been estimated to compromise the quality of life and communication in more than 30 million Americans, and approximately 1 billion individuals worldwide. With the increase in longevity in life and the association of hearing impairment with aging, this disability is increasing in incidence and prevalence. In children, it severely affects education and future employment opportunities. In a working individual, it compromises the quality of life, job satisfaction, and productivity; and in the elderly, it leads to isolation and increased medical costs. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

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System and method of teleconferencing with the deaf or hearing-impaired Inventor(s): Basson, Sara H.; (White Plains, NY), Epstein, Edward Adam; (Putnam Valley, NY), Fairweather, Peter G.; (Yorktown Heights, NY), Kanevsky, Dimitri; (Ossining, NY) Correspondence: Paul J. Farrell; Dilworth & Barrese, Llp; 333 Earle Ovington Boulevard; Uniondale; NY; 11553; US Patent Application Number: 20020069069 Date filed: December 1, 2000 Abstract: A system and method is provided for real time teleconferencing, where one of the participants is deaf or hearing-impaired. In one aspect of the system and method, each participant has an Automatic Speech Recognition (ASR) system and a chat service system, such as AOL Instant Messenger.TM. Each participant may have a different type of ASR system, as well as a different type of chat service system. It is not necessary that the deaf or hearing-impaired participant have an ASR system. For each participant, the participant's ASR system transcribes the speech of the participant and provides it to the participant's chat service system, which translates the transcribed text into the chat service message in the format of the participant's chat service system. An Integration Server receives all the participant's chat messages, which have various formats, and translates them into the format used by the chat service system of the deaf or hearingimpaired participant, thus allowing her to see the transcribed text of the conversation between the other participants. In addition, the deaf or hearing-impaired participant inputs text to her chat service system, which creates a formatted message text. The Integration Server receives this formatted chat message and translates it into the formats of the remaining participant's chat service systems, so that the remaining participants see the comments of the deaf or hearing-impaired participant. In other aspects, the teleconferencing system and method is used to connect disparate chat service systems, without necessarily including a deaf or hearing-impaired participant. Excerpt(s): This invention relates to a system and method for providing the deaf or hearing impaired to participate in a teleconference in general, and, in particular, to a system and method that provides a platform which allows various Automatic Speech Recognition (ASR) systems to communicate over various chat messenger systems so that a deaf or hearing-impaired person may receive a transcribed text of the teleconference. Presently, there are several methods for the deaf and hearing-impaired to interpret those speaking to them. One method is having a live interpreter, who listens to the conversation and translates the spoken words into sign language, and vice-versa. Another method is having a live stenographer, who listens to the conversation and types the spoken words so that they may be viewed in real-time and responded to. Yet another method is a stenographic, or Automatic Speech Recognition (ASR), program, which transcribes the spoken words in the same manner as a live transcriber. However, teleconferencing presents particular problems to the deaf and hearing impaired. The logistics of providing one or more live interpreters or translators would be both complex and expensive. Although using a computer program, i.e. ASR, is cheaper than an interpreter/stenographer, ASR has its own problems. If the ASR program is speakerdependent, it requires a certain amount of time to be trained to the intonations of each individual speaker. This would require multiple speaker profiles to be stored in order that the ASR program could deal with the audio input from the multiple participants in a teleconference. These profiles would use up more and more storage space. The ASR system itself would need to be more complex, because it would need to deal with several different audio streams being received simultaneously.

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Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Transcanal, transtympanic cochlear implant system for the rehabilitation of deafness and tinnitus Inventor(s): Boylston, Byron Lee; (Woodstock, GA), Goldsmith, Manning Miles; (Savannah, GA) Correspondence: Jason A. Bernstein; Bernstein & Assoicates, P.C.; 6600 Peachtree Dunwoody Road, N.E.; Embassy Row 400, Suite 495; Atlanta; GA; 30328-1649; US Patent Application Number: 20020099421 Date filed: January 22, 2002 Abstract: A transcanal, transtympanic cochlear implant system for implantation comprising a molded insert for removable positioning in the auditory canal of the human ear and an insulated receiver coil, preferably in a generally circular or looped form, where the receiver coil receives electromagnetic signals, in a first embodiment, or radio frequency signals, in a second embodiment, through inductive coupling to a solenoid coil within the molded insert. Excerpt(s): This application claims benefit of copending provisional patent application No. 60/263,600 filed Jan. 23, 2001, entitled TRANSCANAL COCHLEAR IMPLANT, which is incorporated herein. The present invention is directed to the field of cochlear implants for patients with hearing impairment and/or tinnitus, more particularly to a transcanal, transtympanic cochlear implant system that requires a minimum of surgical intrusion that may be performed at a physician's office under local anesthesia. The present invention relates to a transcanal, transtympanic cochlear implant system ideally suited for those profoundly deaf, where conventional amplifying hearing aids are of limited or no value to those suffering the hearing impairment. That is to say, with maximum gain delivered by the most powerful hearing aids, these profoundly deaf individuals cannot hear sound and hence cannot discriminate and understand speech. In addition, there are an estimated 200-300 million people who have various patterns of severe sensorineural hearing loss, which are imperfectly rehabilitated via hearing aids. An example of such is so called "ski-sloped hearing loss," where there is near normal hearing in the low to middle frequency range, but the hearing drops out dramatically in the higher frequencies. For these types of hearing loss, amplification is ineffective, because the cochlea cannot perform its transductive function of converting the mechanical energy of sound to the electrical current, which is ultimately perceived as sound by the brain. The inner ear structures responsible for this transductive function are known as hair cells, and the electrical currents, which they produce in response to the mechanical stimulation by sound, are known as cochlear microphonics. When these hair cells are sufficiently damaged in the above mentioned scenarios, no amount of amplification will be effective. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

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Use of riluzole in the treating acoustic traumas Inventor(s): Randle, John; (Brookline, MA), Stutzmann, Jean-Marie; (Villecresnes, FR) Correspondence: Aventis Pharmaceuticals, INC.; Patents Department; Route 202-206, P.O. Box 6800; Bridgewater; NJ; 08807-0800; US Patent Application Number: 20020004516 Date filed: June 15, 2001 Abstract: The invention concerns the use of riluzole or one of its pharmaceutically acceptable salts for preventing and/or treating acoustic traumas and, in particular, different types of deafness and tinnitus. Excerpt(s): This application is a continuation of International application No. PCT/FR99/03,108, filed Dec. 13, 1999; which claims the benefit of priority of French Patent Application No. 98/15,834, filed Dec. 15, 1998. The present invention relates to the use of riluzole or one of its pharmaceutically acceptable salts in the prevention and/or treatment of acoustic traumas and, in particular, of deafness and of tinnitus. Riluzole (2-amino-6-trifluoromethoxy-benzothiazole) is marketed for the treatment of amyotrophic lateral sclerosis. This compound is also useful as an anticonvulsant, an anxiolytic and a hypnotic (EP 50551), in the treatment of schizophrenia (EP 305276), in the treatment of sleep disorders and of depression (EP 305277), in the treatment of cerebrovascular disorders and as an anesthetic (EP 282971), in the treatment of spinal, cranial and craniospinal traumas (WO 94/13288), as a radio restorative (WO 94/15600), in the treatment of Parkinson's disease (WO 94/15601), in the treatment of neuro-AIDS (WO 94/20103), in the treatment of mitochondrial diseases (WO 95/19170). All of these references are herein incorporated by reference in their entirety. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

Keeping Current In order to stay informed about patents and patent applications dealing with deafness, you can access the U.S. Patent Office archive via the Internet at the following Web address: http://www.uspto.gov/patft/index.html. You will see two broad options: (1) Issued Patent, and (2) Published Applications. To see a list of issued patents, perform the following steps: Under “Issued Patents,” click “Quick Search.” Then, type “deafness” (or synonyms) into the “Term 1” box. After clicking on the search button, scroll down to see the various patents which have been granted to date on deafness. You can also use this procedure to view pending patent applications concerning deafness. Simply go back to http://www.uspto.gov/patft/index.html. Select “Quick Search” under “Published Applications.” Then proceed with the steps listed above.

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CHAPTER 7. BOOKS ON DEAFNESS Overview This chapter provides bibliographic book references relating to deafness. In addition to online booksellers such as www.amazon.com and www.bn.com, excellent sources for book titles on deafness include the Combined Health Information Database and the National Library of Medicine. Your local medical library also may have these titles available for loan.

Book Summaries: Federal Agencies The Combined Health Information Database collects various book abstracts from a variety of healthcare institutions and federal agencies. To access these summaries, go directly to the following hyperlink: http://chid.nih.gov/detail/detail.html. You will need to use the “Detailed Search” option. To find book summaries, use the drop boxes at the bottom of the search page where “You may refine your search by.” Select the dates and language you prefer. For the format option, select “Monograph/Book.” Now type “deafness” (or synonyms) into the “For these words:” box. You should check back periodically with this database which is updated every three months. The following is a typical result when searching for books on deafness: •

Psychotherapy with Deaf and Hard-of-hearing Persons: A Systemic Model, Second Edition Source: Framingham, MA. Boston University. Contact: Available from Lawrence Erlbaum Associates, Inc. 10 Industrial Avenue, Mahwah, NJ 07430-2262. 800-926-6579. Fax: 201-760-3753. E-mail: [email protected]. Web site: www.erlbaum.com. Fed ID: 22-2043137. PRICE: $49.95 plus shipping and handling. ISBN: 0-8058-4375-2 (cloth). 248pp. Summary: In this 2nd edition, the author elaborates on his original biopsychosocial model of the effective assessment and treatment of deaf and hard-of-hearing clients in individual and family therapy. He examines the influences of larger networks on the individual and vice versa, and illuminates the overt and covert conflicts among family members, school, vocational rehabilitation personnel, and friends that often exacerbate problems. Spiritual issues are addressed, and theory is balanced with practical advice.

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Keys to Raising a Deaf Child Source: [New York]: Barron's. 1999. 202 p. Contact: Available from Barron's Educational Series, Inc. 250 Wireless Boulevard, Hauppauge, NY 11788. (800) 645-3476 ext. 204, 214, 215. Fax (516) 434-3217. E-mail: [email protected]. Website: www.barronseduc.com. PRICE: $6.95 plus shipping and handling. ISBN: 0764107232. Summary: In this book, two educators (one of them a parent of two deaf children) offer advice and encouragement on helping children adapt to deafness. The authors recommend the bimodal communication approach, which entails having the child, parents, and other non deaf family members combine sign language and speech as the first step to normal communication. The authors also offer general advice on parenting. Topics include coping strategies, bonding and communicating with a deaf infant, parents as teachers, extended family members, connecting brothers and sisters, collaborating with professionals and others(medical, educational, members of the Deaf community), schools and classrooms, parent rights regarding education of their children, educational placement, the etiology of hearing loss, hearing tests, hearing aids, using amplification, cochlear implants, choices and decisions, speech and language, strategies for communication, linguistic creativity, listening strategies, language fluency, balancing speech and sign, teaching vocabulary, grammatical competence, social language, academic language, discourse competence, supporting literacy, and reading skills. The authors emphasize ideas that will help parents foster a nurturing and language rich environment for a deaf or hard of hearing child. Throughout the book, the authors stress that deaf and hearing children are more alike than they are different. The book concludes with a section of common questions and answers, a glossary of terms, a list of recommended reading, a list of resources for parents, and a subject index.

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Deafened People: Adjustment and Support Source: Toronto, Ontario: University of Toronto Press. 2000. 248 p. Contact: Available from University of Toronto Press Inc. 5201 Dufferin Street, North York, Ontario M3H 5T8. (800) 565-9523. Fax (416) 667-7832. E-mail: [email protected]. Website: www.utpress.utoronto.ca. Price: $21.95 plus shipping and handling. ISBN: 0802083730. Summary: It is estimated that there are currently 1.9 million deafened people living in North America, people who could once hear naturally or with amplification but who have become deaf and are now unable to rely on hearing to comprehend spoken information. This book addresses the process of adjustment to, and acceptance of, deafness as an adult. The authors demonstrate that deafness is not merely a medical condition; it is a social disability that affects the person, the family, the social circle, and the work group. By describing the psychosocial experience of acquired deafness as a process of adjustment, the authors demonstrate that acceptance of deafness is a process with practical, social, and emotional implications. The authors also provide a guide to self help techniques of proven value to deafened people. Drawing on their own experiences as deaf professionals, the authors explore questions such as how deafness occurs, how relationships (professional and personal) can be affected by progressive deafness, and how and where to find peer support. Section 1 describes the process of adjustment, while Section 2 offers a practical guide to a successful method of establishing a self help support network, with reference to such organizations as the Association of Late Deafened Adults. The book combines medical background,

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professional advice, information on resources, and personal examples. A list of references and a subject index conclude the book. •

Rehabilitation of Late-Deafened Adults: Modular Program Manual Source: St. Louis, MO: Mosby-Year Book, Inc. 1993. 223 p. Contact: Available from Mosby-Year Book, Inc. 11830 Westline Industrial Drive, St. Louis, MO 63146. (800) 433-3803; Fax (800) 535-9935. PRICE: $32.95 plus shipping and handling. ISBN: 0801677882. Summary: The book provides an overview of the practical issues involved in providing services to deaf and hearing-impaired veterans and other late-deafened adults. Six chapters cover the philosophy of rehabilitation of the late-deafened adult; the impact of deafness on daily living; speech perception training, including methods and samples of auditory, vibrotactile, and electrical stimulation; methods and samples of speechreading; voice and resonance; and applications (home and work environments) and resources. The authors, members of a cochlear implant program team, describe procedures found to be useful with persons who were not implant candidates but for whom the isolation and communicative impact of severe or profound late-onset deafness had imposed considerable obstacles. Each chapter includes references, and a subject index concludes the volume.

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Legal Rights: The Guide for Deaf and Hard of Hearing People Source: Washington, DC: Gallaudet University Press. 2000. 264 p. Contact: Available from Gallaudet University Press, c/o Chicago Distribution Center. 11030 South Langley Avenue, Chicago, Illinois, 60628. Toll-free: (800) 621-2736. Toll-free TTY: (888) 630-9347. Web site: gupress.gallaudet.edu. ISBN 1-56368-091. PRICE: $29.95 plus shipping, tax, and handling. Available in soft cover. Summary: The fifth edition of Legal Rights has been updated with new information about state and federal laws and regulations regarding the rights of people who are deaf or hard-of-hearing. It provides guidelines for communicating with people who are deaf or hard-of-hearing and explains the provisions of the Americans with Disabilities Act, the Telecommunications Act of 1996, the Rehabilitation Act, the Individuals with Disabilities Education Act, and relevant Supreme Court decisions. Laws related to healthcare and social services, employment, architectural barriers, the legal system, and television also are discussed. The appendices contain lists of publications about deafness and education, state services, online legal resources, and nongovernmental organizations. A model bar association position on deaf clients and model hospital policy are included.

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Understanding Deafness and the Rehabilitation Process Source: Needham Heights, MA: Allyn and Bacon. 1994. 320 p. Contact: Available from Allyn and Bacon. 160 Gould Street, Needham Heights, MA 02194-2310. (617) 455-1200. PRICE: $44.95 plus shipping and handling. ISBN: 0205156282. Summary: The purpose of this book is to help vocational rehabilitation counselors and counselors-in-training to better understand deafness, people who are deaf, and the many challenges that they face in preparing themselves for and finding jobs. The book begins with a brief overview of hearing loss and the development of people who are

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deaf. This is followed by an in-depth look at the effects of deafness on language and communication, cognition, and affective development. Next the authors examine the critical task of helping individuals who are deaf make the transition from school to work. Other chapters cover general assessment; how the outcomes of assessment are applied in vocational guidance for workers who are deaf; personal adjustment issues; therapeutic interventions, including individual and group counseling with cognitive behavior therapy, and family intervention with a systems approach; technological and personal support services; and job placement. Each chapter includes extensive references and a subject index concludes the volume. •

The Deaf Child in the Family and At School: Essays in Honor of Kathryn P. MeadowOrlans Source: Mahwah, NJ: Lawerence Erlbaum Associates, Inc. Contact: Available from Lawerence Erlbaum Associates, Inc. 10 Industrial Avenue, Mahwah, NJ 07430-2262. 800-926-6579. Fax: 201-760-3753. E-mail: [email protected]. Web site: www.erlbaum.com. Fed ID: 22-2043137. PRICE: $37.50 plus shipping and handling. ISBN: 0-8058-3221-1 (paper). Also available in cloth: $69.95 plus shipping and handling. ISBN: 0-8058-3220-3. 336pp. Summary: This book brings together leading researchers and interventionists trained in a variety of disciplines, and representing a variety of perspectives, to synthesize what is known about the complex factors affecting the development of deaf and hard-of-hearing children in their diverse environments. The focus throughout is on family interaction and the strengths and needs of the child, and the role of education and other interventions in supporting family and child growth. References.

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Educating the Deaf: Psychology, Principles, and Practices. 4th ed Source: Boston, MA: Houghton Mifflin Company. 1996. 420 p. Contact: Available from Houghton Mifflin Company. 222 Berkeley Street, Boston, MA 02116. (800) 225-1464; http://www.hmco.com. PRICE: $73.16 plus shipping and handling. ISBN: 0395357810. Summary: This book is designed to serve as a primary source of information on deafness. Information is presented in fourteen chapters: an overview of the education of the deaf; historical perspectives from prehistoric times to the nineteenth century; historical perspectives from the United States in the Nineteenth Century; the causes, prevention, and treatment for deafness; deaf individuals with additional conditions, families with deaf members, notably interpersonal relations from diagnosis to adulthood; deafness and cognitive functioning; deafness and mental health; American Sign Language and manual communication; early intervention, infant, and preschool programs; teaching and training techniques for grammar and speech; the development of reading and writing; elementary and secondary education; and postsecondary education and the economic status of deaf individuals. The epilogue in the latest edition includes information on school achievement and classroom placement, instruction and curriculum, and the deaf community and ASL. Extensive references, and author and subject indices conclude the volume.

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Journey into the Deaf-World Source: San Diego, CA: Dawn Sign Press. 1996. 513 p.

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Contact: Available from Dawn Sign Press. 6130 Nancy Ridge Drive, San Diego, CA 92121-3223. Voice-TTY (619)625-0600. Fax (619) 625-2336. E-mail: [email protected]. Website: www.dawnsign.com. PRICE: $24.95 plus shipping and handling. ISBN: 0915035634. Summary: This book offers an insight into the Deaf world, a language minority culture in the United States that is some million strong. The authors address issues including an introduction to Deaf culture, a brief history of sign language and Deaf culture, how Deaf children are raised and educated, what sign language and Deaf culture can offer to Deaf children and hearing people, what can be learned from Deaf societies in other lands, language and literacy, bilingual and bicultural education, differences versus disabilities, how technology can help or hinder Deaf people, and how Deaf people integrate into the larger society. The book reviews relevant research and contains extensive excerpts from illustrative first person accounts. The book concludes with a series of chapter notes, a list of references, a subject index, and information about the authors. The book is illustrated with black and white photographs. 550 references. •

Literacy Learning for Children Who Are Deaf or Hard of Hearing Source: Washington, DC: Alexander Graham Bell Association for the Deaf and Hard of Hearing. 2000. 159 p. Contact: Available from Alexander Graham Bell Association for the Deaf and Hard of Hearing. 3417 Volta Place, NW, Washington, DC 20007-2778. Voice (202) 337-5220. TTY (202) 337-5221. Fax (202) 337-8314. Website: www.agbell.org. PRICE: $27.95 plus shipping and handling. Summary: This book offers parents of children who are diagnosed with deafness or hearing impairment a guide to the choices they will be making and suggestions for supporting their child's achievement of literacy. The book begins with an introduction focusing on how new amplification technologies are changing everything about how deafness is managed. Topics in the introduction include the types of hearing loss, general causes of hearing loss, an audiometric perspective of the severity or degree of hearing impairment, the acoustic filter effect of hearing impairment, computer analogy and amplification technology, distance hearing, how to understand the audiogram, amplification technologies, hearing aids, FM units, sound field FM systems, cochlear implants, auditory verbal communication, evaluating intervention or amplification technology, and listening skill development. The book then presents eight chapters covering how hearing is related to literacy, current research and understanding on reading and writing, how to help the child learn spoken language, how to help the child learn to read in natural ways, writing skills and how to achieve them, and the role of parents, therapists and teachers at various levels of the child's schooling. The book concludes with a list of references, a subject index, and three appendices: a holistic scale for evaluating writing (K-12), favorite books for different ages, and a list of auditory verbal centers. 79 references.

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Multicultural Issues in Deafness Source: White Plains, NY: Longman Publishing Group. 1993. 204 p. Contact: Available from Longman Publishing Group. 10 Bank Street, White Plains, NY 10606. (800) 322-1377; Fax (800) 333-3328. PRICE: $29.00 plus shipping and handling. ISBN: 080130752X.

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Summary: This book on multicultural issues in Deafness focuses on four special populations of Deaf children: those from African-American, American Indian, Hispanic, and Asian/Pacific Island families. These children are considered from both educational and cultural perspectives. Ten chapters cover Deaf culture and cultures, home-school communication and staff development, addressing the needs of each of the four populations under consideration, and multiculturalism. This text calls for a childcentered curriculum based on a fundamental acceptance of differences in communication and interpretation. Multicultural teaching and learning is described as an ongoing, dynamic process. Each chapter includes extensive references and resource listings. (AA-M). •

American Deaf Culture: An Anthology Source: Burtonsville, MD: Sign Media, Inc. 1989. 210 p. Contact: Available from Sign Media, Inc. 4020 Blackburn Lane, Burtonsville, MD 208661167. Voice/TTY (800) 475-4756; Voice (301) 421-0268; TTY (301) 421-4460; Fax (301) 4210270; http://www.signmedia.com. PRICE: $17.95 plus shipping and handling. ISBN: 0932130097. Summary: This book presents a collection of classic articles selected to provide a variety of perspectives on the language and culture of Deaf people in American. Deaf and hearing scholars and writers explore topics including Deaf cultural values, American Sign Language, social interaction in the deaf community, the ethnography of communication, Deaf education, folklore, and the power struggle of Deaf people to take control of their cultural destiny. Authors include Carol Padden, Ben Bahan, Barbara Kannapell, Shanny Mow, William C. Stokoe, Susan D. Rutherford, Stephanie Hall, Veda Charrow, Ronnie Wilbur, Tom Humphries, Better Martin, Terry Coye, Sherman Wilcox, and James Woodward. The pieces are a mixture of scholarly analysis and first-person accounts of Deafness in the United States. The text is designed for students and researchers of Deaf culture and American Sign Language, educators, and sign language interpreters. 130 references. (AA-M).

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You and Your Deaf Child: A Self-Help Guide for Parents of Deaf and Hard of Hearing Children. 2nd ed Source: Washington, DC: Clerc Books, Gallaudet University Press. 1997. 240 p. Contact: Available from Gallaudet University Press. Chicago Distribution Center, 11030 South Langley Avenue, Chicago, IL 60628. (800) 621-2736. PRICE: $19.95. ISBN: 1563680602. Summary: This book presents eleven chapters intended for hearing parents of children who are deaf and hard of hearing. After an introductory chapter, chapters 2 and 3 include material on feelings about hearing loss and a discussion of how to cope with these feelings. Chapters 4 and 5 introduce the topic of family communication, focus on nonverbal communication, and discuss family communication as it relates to family unity. Chapter 6 provides information about typical child development and how hearing loss and the family's communication system affects the child's behavior. Chapters 7 and 8 include material about managing the child's behavior through general limit setting or plans designed to prevent behavior problems. Developing plans for solving future problems is discussed in Chapter 9. Chapter 10 covers general topics of interest regarding hearing loss, such as issues related to technological assistance, choice of appropriate education placement, and an introduction to the Deaf community and culture. Appendices offer a list of resources and suggested readings for parents. Chapter

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2 through 8 follow a similar format that includes an introductory section, a 'Skill Development' section, a section called 'Parent and Child' that provides interactions for use of the concepts, 'Points to Remember' (a summary), and 'Activities for Practice.' A subject index concludes the volume. 157 references. •

Spoken Communication for Students Who Are Deaf or Hard-of-Hearing: A Multidisciplinary Approach Source: Hillsboro, Oregon. 2002. Butte Publications, Inc. ISBN: 1-884362-54-0. 159pp. Contact: Available from Butte Publications, Inc. P.O. Box 1328, Hillsboro, OR 97123. 866312-8883. Catalog order No. 2540. PRICE: $39.00 plus shipping and handling. www.buttepublications.com. Summary: This book supports the instructional best practice of using multidisciplinary team approach to develop spoken communication skills in deaf or hard-of-hearing students. Teachers, speech therapists, parents, school district personnel and the student all working together within the classroom setting to establish, develop and support spoken communication skills. The text supports the premise that spoken language development is possible regardless of the type and degree of hearing loss or the educational program's philosophy. The text includes pictures, forms, springboard discussions, experiments, and practical ideas for use in school and at home.

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Full Face: A Correspondence About Becoming Deaf in Mid-Life Source: Hillsboro, OR: Butte Publications, Inc. 1997. 144 p. Contact: Available from Butte Publications, Inc. P.O. Box 1328, Hillsboro, OR 971231328. (800) 330-9791; Fax (503) 693-9526. PRICE: $9.95 plus shipping and handling. ISBN: 1884362214. Summary: This book uses a fictional character, David Reilly, to share practical information, humorous anecdotes, encouragement, and insights into the psychological and spiritual aspects of becoming deaf in midlife. The author notes in her introduction that David Reilly was created out of half a dozen people, both men and women, struggling to make sense of deafness at midlife, which can bewilder, discourage, and isolate people. The author uses a fictitious correspondence between herself and David Reilly to explore the issues that adults may face with midlife deafness. The book concludes with a brief list of recommended readings.

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Study of American Deaf Folklore Source: Burtonsville, MD: Sign Media, Inc. 1993. 170 p. Contact: Available from Sign Media, Inc. 4020 Blackburn Lane, Burtonsville, MD 208661167. Voice/TTY (800) 475-4756; Voice (301) 421-0268; TTY (301) 421-4460; Fax (301) 4210270; http://signmedia.com. PRICE: $19.95 plus shipping and handling. ISBN: 0932130178. Summary: This book, intended for health professionals, educators, and the general public, is part of a series of dissertations on issues in the Deaf community. This dissertation examines how the American Deaf community can be studied and better understood, both as a cultural group and as a linguistic minority group, through its folklore. In the process, the author examines the different functions that folklore serves in the community: folklore acts as a metaphor for the group's experience; transmits group customs, values, and behavior norms; serves as an educational tool for specific

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competencies; and establishes and maintains group identity. The author examines the concept of 'sign play' and presents a number of traditional forms from the Deaf Community, including legends, jokes, skits, tall tales, and slurred name signs. The author also demonstrates that folklore serves to provide avenues for creative expression and to validate the worth of the group and their culture. 178 references. (AA-M). •

Psychology of Deafness Source: New York, NY: Longman, 1990, 292 p. Contact: Available from Harris Communications, 6541 City West Parkway, Eden Prairie, MN 55344-3248. (612)946-0921. (800)825-6758. (612)946-0922 TDD/TT. (612)946-0924 Fax. PRICE: $44.95. ISBN 0801302322. Summary: This book, written for professionals, is based on the premise that deafness is a psychological variable which influences the behavior of deaf persons so that their life experience differs in some consistent ways from that of those who are not deaf. Its purpose is to improve service delivery to the Deaf community. The characteristics of the American Deaf community are described as well as the etiologies of deafness. The historical and linguistic roots of American Sign Language (ASL) are identified; other forms of communication used by deaf people are also included. The book describes in detail the stress that deafness imposes on individuals and their families, and presents information aimed at reducing misdiagnosis with deaf children.

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Dina the Deaf Dinosaur Source: Stamford, CT: Hannacroiz Creek Books. 1998. 32 p. Contact: Available from Hannacroiz Creek Books. 1127 High Ridge Road, Number 110, Stamford, CT 06905. (203) 321-8674; Fax (203) 968-0193; E-mail: [email protected]; http://www.HannacroixCreekBooks.com. PRICE: $19.95 plus shipping and handling. ISBN: 1889262048. Summary: This children's book tells the story of a deaf dinosaur who ran away from home because her parents would not learn sign language. She befriends an owl, a mole, and a chipmunk. The story shows how sign language can help communication between creatures (people) who are hearing and those who are deaf. The story emphasizes that communication is the beginning of love. The creatures that the dinosaur befriends are willing to learn sign language, beginning with their names and fingerspelling and going on to learn some basic family and activity signs. The story ends when the dinosaur's parents come looking for her and accept that they must learn sign language in order for her to come home. The book is illustrated with watercolor paintings of the animals and the woodland setting. The illustrations also depict numerous signs and fingerspelled words. The book includes a list of suggested readings and related children's books, and the addresses of four related resource organizations.

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Deafness. 5th ed Source: London, England: Whurr Publishers Ltd. 1993. 339 p. Contact: Available from Singular Publishing Group, Inc. 401 West 'A' Street, Suite 325, San Diego, CA 92101-7904. (800) 521-8545 or (619) 238-6777. Fax (800) 774-8398 or (619) 238-6789. E-mail: [email protected]. Website: www.singpub.com. PRICE: $63.50 plus shipping and handling. ISBN: 1565935179.

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Summary: This collection of readings is written for health professionals and educators whose professional work may bring them into close contact with individuals who are deaf. Chapters cover the normal ear and hearing, the diagnosis of deafness, conductive deafness, deafness in children, acquired sensorineural deafness (hearing loss due to hereditary factors, infections, iatrogenic factors, trauma, and presbycusis), non-organic hearing loss, tinnitus, psychological aspects of hearing loss, hearing aids and other assistive devices, implants (cochlear and bone-conduction), and auditory rehabilitation. The book includes a glossary of abbreviations and acronyms, and a subject index. A list of resource organizations reflects the British perspective of the text. 68 references. (AAM). •

GLAD Directory of Resources for Deaf and Hard-of-Hearing People: 2001 Edition Source: Greater Los Angeles Council on Deafness, Inc. Los Angeles, CA. 2001. Contact: Available from Greater Los Angeles Council on Deafness, Inc. 2222 Laverna Avenue, Los Angeles, CA 90041. Voice/TDD: (323) 478-8000. FAX: (323) 550-4225. Email: [email protected]. Web site: http://www.gladinc.org. PRICE: $12.00 members; $17.00 non-members plus, $3.00 shipping per book. Summary: This directory contains resources for deaf and hard-of-hearing individuals compiled from a previous Greater Los Angeles Council on Deafness, Inc. (GLAD) directory, Internet resources, GLAD information files, and other related directories. Each listing includes the organization name, mailing and electronic addresses, telephone numbers (TTY and/or voice), contact name, and a brief description of services.

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HIV/AIDS Resources for the Deaf Directory Contact: University of California Center on Deafness, Deaf AIDS Support Services, 3333 California St Ste 10, San Francisco, CA, 94143-1208, (415) 476-4980, http://www.uccd.org/AIDS.html. Summary: This directory presents information about resources in California, and specifically the San Francisco Bay Area, that provide educational, medical, social, support, and interpreting services to the deaf community. The entries are organized alphabetically under each topic. Topics include deaf community AIDS service providers; information, counseling, and social services providers; AIDS services throughout California; national hotline resources; medical services providers; and private practice referrals for deaf individuals.

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Gallaudet Encyclopedia of Deaf People and Deafness Source: New York, NY: McGraw-Hill Book Company, 1987, 3 volumes. Contact: Available from TAB/McGraw-Hill, Blue Ridge Summit, PA 17294. (800)2331128. PRICE: $385.00. ISBN 0070792291. Summary: This encyclopedia is written for persons with hearing impairments. The volumes pertain both to an identifiable group of people, those with hearing impairments, and to a physical attribute, deafness. The information has been selected from studies in the sciences, the social sciences, and the humanities. The 273 entries in the encyclopedia are arranged alphabetically in continuous sequence in three volumes. Many of the entries are divided into sections and subsections about particular aspects of the topic. An index is included. Bibliographies and cross-references accompany most entries. Illustrations accompany the text.

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Growing Together: Information for Parents of Deaf and Hard of Hearing Children Source: Washington, DC: National Deaf Education Network and Clearinghouse, Laurent Clerc National Deaf Education Center. 1991. 105 p. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. PRICE: $8.00 plus shipping and handling. Code Number 569. Summary: This handbook was development to help answer questions that parents of deaf and hard of hearing children often ask. The handbook includes several fact sheets that discuss in detail deafness, education, and communication, as well as question and answer sheets that cover various topics of interest to parents. It was originally sold as a packet of individual publications. These publications are bound together so that the publications which complement and supplement each other remain intact as a helpful handbook. Topic Sheets include: when a hearing loss is diagnosed; accepting the deaf or hard of hearing child in the family; discipline, behavior, and emotional development; sexuality; preparing for the world of work; amplification and assistive devices for deaf and hard of hearing children; working with schools and professionals; questions parents can ask in evaluating an education program; and books for parents of deaf and hard of hearing children. Fact sheets are provided on: deafness; educating deaf children; how deaf people communicate; growing up without hearing; the ear and hearing; educating the hearing impaired child; and genetics and deafness. Each of the items are illustrated and offer suggested readings and additional resources.

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Nuts and Bolts Guide to College Success for Deaf and Hard of Hearing Students Source: Knoxville, TN: Postsecondary Education Consortium (PEC). 2000. 155 p. Contact: Available from PEPNet Resource Center. National Center on Deafness, California State University, Northridge, 18111 Nordhoff Street, Northridge, CA 913308267. Voice/TTY (888) 684-4695 or (818) 677-2611. Fax (818) 677-4899. Website: www.pepnet.org. PRICE: Single copy free. Summary: This manual offers a comprehensive, detailed guide to the services and strategies that will help a student who is deaf or hearing impaired to succeed in the college setting. The introductory material reminds readers of the high rate of drop out for college students and stresses that students who avail themselves of disabilities services at their college or university will greatly increase their chances of success. The manual then offers information on self advocacy, precollege and transition times, financial aid, accommodations and disability services, academic issues, and campus life. The manual includes detailed fact sheets on communication tips in different settings, charts to plot and plan one's progress before and through college, descriptions of specific financial aid programs and scholarships, explanations of equipment that can be used for classroom support, suggestions for working with interpreters and other support personnel, record keeping information (for transcripts, health records, and personal identification items), suggestions for developing study skills, career planning, coping with dorm living, extracurricular activities, and how to discover and use different campus services. The manual offers many pages of self inventories and guides for the student to complete. The manual concludes with a section on resources, including many web sites and support groups, and a glossary of important terms.

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Honoring Our Past, Creating Our Future Together. Proceedings of the 16th National Convention of the Registry of Interpreters for the Deaf Source: Silver Spring, MD: RID Publications. 1999. 276 p. Contact: Available from Registry of Interpreters for the Deaf (RID), Inc. 8630 Fenton Street, Suite 324, Silver Spring, MD 20910. Voice/TTY (301) 608-0050. Fax (301) 608-0508. Website: www.rid.org. PRICE: $16.95 plus shipping and handling for members; $24.95 plus shipping and handling for nonmembers. ISBN: 0916883272. Summary: This monograph offers the proceedings of the 16th National Convention of the Registry of Interpreters for the Deaf, which was held in August 1999 in Boston, Massachusetts. The proceedings include twelve presentations, covering organizational change and strategies with multicultural students, interpreters, and consumers; interpreting in Jewish settings; program accreditation; implications of vision loss on the interpreting process; practitioner research; scripture and analysis and interpretation; the issue of Deafness as a disability; a practical approach to cultural adjustments; a national curriculum for interpreters working with persons who are Deaf-Blind; the role and credentials of Deaf teachers in sign language interpreter education; the perceptions of Deafness and language learning of incoming ASL students; and the mutual relevance of ethnography and American Sign Language and English interpreting. Most chapters offer diagrams and references. The Proceedings were prepared prior to the Convention, to be handed out to all attendees. The monograph includes a few pages of blank lines for notes.

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Perspectives on Deafness Source: Silver Spring, MD: National Association of the Deaf. 1991. 174 p. Contact: Available from National Association of the Deaf (NAD). 814 Thayer Avenue, Silver Spring, MD 20910-4500. Voice-TTY (301) 587-6282; Fax (301) 587-1791. PRICE: $19.95 plus shipping and handling. Summary: This monograph presents a collection of thought-provoking articles, stories, poems, and essays that offers a broad spectrum of views, information, and experiences of both deaf and hearing persons involved with Deaf people. Thirty-two chapters cover topics including the Swedish Federation of the Deaf; perceptions on political activism; the use of cued speech; bilingual-bicultural education; freedom of choice; the evolution of a hard-of-hearing person; the importance of a cultural identity; Deaf adults in society; the perspective of hearing parents of Deaf children; Deaf teachers of the Deaf; perceptions of Deaf people in a nation on the threshold of educating Deaf children; civil rights; cochlear implants; TTY manufacture in the U.S.; the Deaf community in the 21st Century; educational options for Deaf children; and the historical, cultural, psychological, and educational aspects of American Sign Language. Also included are numerous poems. Photographs and brief biographical information for each contributor are included.

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Deafness: Life and Culture Source: Spring, MD: National Association of the Deaf (NAD). 1994. 139 p. Contact: Available from National Association of the Deaf (NAD). 814 Thayer Avenue, Silver Spring, MD 20910-4500. Voice (301) 587-6282; TTY (301) 587-6283; Fax (301) 5874873. PRICE: $19.95 plus shipping and handling; $15.96 for members plus shipping and handling. Item Number DE017.

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Summary: This monograph presents a varied selection of articles and poetry covering a broad range of topics, all related to some aspect of deafness. Topics covered include the Black, Deaf community; national organizations of the Deaf; becoming bicultural; disabling images; Austrian culture and deafness; the Americans With Disabilities Act (ADA); cross-cultural research; the women's movement and the Deaf social movement; people with adult-onset deafness; growing up hearing in a Deaf family; Deaf superiority through humor; oppression and Deaf people; Deafness in Canada and the U.S.; the impact of captioning; self-advocacy; bilingual and bicultural education; and Deaf culture education. •

Viewpoints on Deafness Source: Silver Spring, MD: National Association of the Deaf (NAD). 1992. 168 p. Contact: Available from National Association of the Deaf (NAD). Bookstore, 814 Thayer Avenue, Silver Spring, MD 20910-4500. Voice (301) 587-6282; TTY (301) 587-6283; Fax (301) 587-4873. PRICE: $19.95 plus shipping and handling. Also available from Harris Communications. 15159 Technology Drive, Eden Prairie, MN 55344-2277. Voice (800) 825-6758 or (612) 906-1180; TTY (800) 825-9187 or (612) 906-1198; Fax (612) 906-1099. PRICE: $22.95 plus shipping and handling. Item Number B287. Summary: This monograph presents articles and poetry reprinted from the periodical, Deaf American. Essays and viewpoints are presented on the history of the Deaf in Russia; diversity within the Deaf population; equal access for Deaf teachers in Texas; community issues and political activism; the viewpoint of a Deaf child; defense of English-like signing on a television program; communication and Deaf children; discrimination against Deaf people; the right to be a Deaf student at universities; the Black Deaf experience; intellectual oppression of Black Deaf children; the folly of believing that Deaf children can speak; cochlear implants; Deaf adolescents; problems in educating Deaf children; Deaf cultural studies; the bilingual and bicultural approach to communication; early American Sign Language (ASL) training for hearing families with Deaf children; building coalitions between the Black and the Deaf communities; the politics of Deafness; and the language aspect of curricula.

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Mask of Benevolence: Disabling the Deaf Community. 2nd ed Source: [San Diego, CA]: Dawn Sign Press. 1999. 360 p. Contact: Available from Dawn Sign Press. 6130 Nancy Ridge Drive, San Diego, CA 92121-3223. Voice-TTY (858)625-0600. Fax (858) 625-2336. E-mail: [email protected]. Website: www.dawnsign.com. PRICE: $11.95 plus shipping and handling. ISBN: 158121009. Summary: This new edition of Harlan Lane's classic text introduces readers to the gulf that separates the Deaf minority from the hearing world. This edition, with new information on the science and ethics of cochlear implants, sheds a forceful light on the mistreatment of the Deaf minority by a hearing establishment that resists understanding and awareness. Topics covered include representations of deaf people, the infirmity model of deafness, the cultural model of deafness, the colonization of African and deaf communities, paternalism, representation and power, the role of the oppressed, language bigotry and Deaf communities, the education of deaf children, mainstreaming, Deaf people without a Deaf community, hearing parents of deaf children, bilingual education and Deaf power, the politics of Deaf education, the risks and limitations of childhood cochlear implants, and the science and ethics of cochlear implantation. The book concludes with extensive footnotes and a detailed subject index.

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Americans with Disabilities Act: Responsibilities for Postsecondary Institutions Serving Deaf and Hard of Hearing Students. Questions and Answers. 2nd ed Source: St. Paul, MN: Midwest Center for Postsecondary Outreach, St. Paul Technical College. 1998. 57 p. Contact: Available from PEPNet Resource Center. National Center on Deafness, California State University, Northridge, 18111 Nordhoff Street, Northridge, CA 913308267. Voice/TTY (888) 684-4695 or (818) 677-2611. Fax (818) 677-4899. Website: www.pepnet.org. PRICE: $20.00. Summary: This report answers questions that were remaining at the end of two satellite conferences, held in May 1997 and March 1998, on 'The Americans with Disabilities Act: Responsibilities for Postsecondary Institutions Serving Deaf and Hard of Hearing Students.' The questions are organized by topic areas: literacy, auxiliary services (interpreting, notetaking, and captioning), vocational rehabilitation, administration, residence halls, and equipment. The conferences were hosted by the Midwest Center for Postsecondary Outreach, one of four national regional centers that comprise the Postsecondary Education Programs Network (PEPNet) that are available to help educators who are working with people who are deaf or hard of hearing. These centers work primarily at the institutional level to increase and enhance postsecondary educational opportunities for individuals who are deaf and hard of hearing. This document provides attendees of the conferences and other readers with technical assistance to some of the questions posed to the presenters. The authors caution that the information provided should not be considered legal advice, but merely serve as guidance in assisting providers, colleges and universities at large, and others in understanding the obligation of institutions of higher learning to serving students who are deaf and hard of hearing. Extensive footnotes offering legal precedents and references are provided.

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Deaf Plus: A Multicultural Perspective Source: San Diego, CA: Dawn Sign Press. 2000. 288 p. Contact: Available from Dawn Sign Press. 6130 Nancy Ridge Drive, San Diego, CA 92121-3223. (800) 549-5350. Voice/TTY (858) 625-0600. Fax (858) 625-2336. Website: www.dawnsign.com. PRICE: $18.95 plus shipping and handling. ISBN: 1581210175. Summary: This text offers a collection of essays that provide information about the community that surrounds Deaf children from diverse backgrounds. This community includes the children themselves, their families, and teachers. The authors support the contention that multiple cultures in the classroom are a positive thing, and that diversity within Deaf culture is enriching and enhancing. The text calls for critically important changes in teacher preparation to create the leadership needed as the community strives to best serve Deaf children. Eleven chapters cover building a multicultural curriculum, assessment issues, emerging literacy, an Asian Pacific perspective on Deafness, immigrant and refugee children who are Deaf, transnational regional considerations ('on the border'), meeting the needs of Hispanic or Latino Deaf students, school support services, exploring students' personal cultures, and teacher expectations and their implications. Each chapter is written by an expert in the field and concludes with a reference list; a subject index concludes the text.

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Language and Deafness. 3rd ed Source: San Diego, CA: Singular Publishing Group. 2001. 692 p.

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Contact: Available from Thomson Learning Group. P.O. Box 6904, Florence, KY 41022. (800) 842-3636. Fax (606) 647-5963. Website: www.singpub.com. PRICE: $59.95 plus shipping and handling. ISBN: 1565939999. Summary: This text offers an indepth introduction to language development in children and adolescents who are deaf or hard of hearing. In addition to focusing on children with severe to profound hearing impairment, the book also offers some coverage of the language acquisition of individuals with less severe hearing losses, particularly in the chapter on the development of oral English skills. Twelve chapters cover an introduction to language and deafness, language functions and structures, language acquisition, primary language development, orality [speech, audition, and speechreading], signed systems, American Sign Language, the development of reading and writing (script literacy), bilingualism and second language learning, language instruction, language assessment, and language and communication approaches (a concluding chapter). Each chapter includes a summary, a list of comprehension questions for readers, a list of challenge questions, and a list of further readings. The sign language references are illustrated with black and white photographs. The textbook concludes with a reference list and a detailed subject index. 1,063 references. •

Profound Deafness and Speech Communication Source: San Diego, CA: Singular Publishing Group, Inc. 1995. 606 p. Contact: Available from Singular Publishing Group, Inc. 401 West 'A' Street, Suite 325, San Diego, CA 92101-7904. (800) 521-8545 or (619) 238-6777. Fax (800) 774-8398 or (619) 238-6789. E-mail: [email protected]. Website: www.singpub.com. PRICE: $75.00 plus shipping and handling. ISBN: 156593492X. Summary: This textbook addresses profound deafness and speech communication, focusing on tactile aids, cochlear implants, speech perception and testing, and speech production. 26 chapters cover the history of sensory aids for deaf people; the Tadoma method; design fundamentals for electrotactile devices (the Tickle Talker Case Study); tactile aid usage in young deaf children; the historical perspective on cochlear implants; speech production and perception in children and adults using cochlear implants; speech related to pure tone audiograms; speech perception tests; the characteristics of a skilled speechreader; the evaluation of speech tracking results; principles for a complete description of the phonological system of deaf children; the use of sensory aids for teaching speech to deaf children; speech pattern elements in assessment, training, and prosthetic provision; the speech visualization system; and computer-based training programs. Each chapter includes references, and a subject index concludes the volume.

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Understanding Deafness Socially: Continuities in Research and Theory. 2nd ed Source: Springfield, IL: Charles C. Thomas Publishers, Ltd. 1996. 166 p. Contact: Available from Charles C. Thomas Publishers, Ltd. 2600 South First Street, Springfield, IL 62794-9265. Voice (800) 258-8980 or (217) 789-8980; Fax (217) 789-9130. PRICE: $42.95 (cloth); $27.95 (paper); plus shipping and handling. ISBN: 0398065691 (cloth); 0398065705 (paper). Summary: This textbook considers the lives of Deaf people within a social context. The book includes research on deafness and the demographics of deafness. One chapter is devoted to Deaf people in today's workplace, including the use of the ADA and mediation to eliminate barriers to participation. Other chapters describe educational and occupational achievements of prevocationally Deaf adults, socialization of Deaf children

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and youth, and the use of American Sign Language (ASL) to support Deaf children's English literacy. The book discusses the social orientation toward Deaf and hearing peers among Deaf adolescents. The final chapter reviews the Deaf President Now protest at Gallaudet University and its consequences. Each chapter concludes with an extensive reference list. •

Politics of Visual Language: Deafness, Language Choice, and Political Socialization Source: [Toronto, Ontario]: Carleton University Press. 1999. 112 p. Contact: Available from CUP Services. 750 Cascadilla Street, P.O. Box 6525, Ithaca, NY 14851. (607) 277-2211. Fax (607) 277-6292. Website: www.temagami.carleton.ca/cupress. PRICE: $24.95 plus shipping and handling. ISBN: 0886293510. Summary: This textbook is a study of the political socialization of children who are deaf. The author notes that while the battle lines have been drawn by the proponents of oralism versus manualism and their hearing supporters, two linguistic dilemmas facing Deaf people remain constant: a conscious choice is always made for them as to the way they will be taught, and either method of language acquisition results in a form of marginalization. Five chapters cover political socialization and marginalization, an historical overview of the deaf, socializing agencies, a comparative group study, and reflections and recommendations. Specific topics include socialization and political socialization, theoretical frameworks of political socialization, theories of political marginalization, neglect of the disabled, agencies for examination, history of the Deaf, the languages of the Deaf classroom, mainstreaming, Deaf culture, the family, power relationships and the pattern of marginalization, language choice as political choice, the schools and peer group, role models, social maturation and peer groups, quality and ends of education, the cochlear implants rally, family relations and language or identity factors, and language as a tool of Deaf political socialization. The text concludes with a bibliography of additional resources. 193 references.

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Deafness and Vision Disorders: Anatomy and Physiology, Assessment Procedures, Ocular Anomalies, and Educational Implications Source: Springfield, IL: Charles C. Thomas Publisher, Ltd. 1999. 503 p. Contact: Available from Charles C. Thomas Publisher, Ltd. 2600 South First Street, Springfield, IL 62794-9265. (800) 258-8980 or (217) 789-8980. Fax (217) 789-9130. PRICE: $74.95 plus shipping and handling. ISBN: 039806945X. Summary: This textbook is designed to help students preparing for work in the field of deafness to understand and incorporate an awareness of vision disorders in the deaf population. Among other components, the book contains chapters on the anatomy and physiology of the visual mechanism; assessment procedures and recommended test instruments and equipment; common and less common visual and ocular (eye) anomalies often found among deaf students; methods for altering the educational environment to make academic success more likely; an extensive, detailed glossary of vision, eye related and other terminology used within the text; and a list of acronyms and abbreviations in common use among physicians and researchers dealing with vision and or deafness. Within the book, the information concerning the congenital anomalies, functional defects, and pathologic ocular conditions most often found within a deaf student population was obtained from eleven years of research unobtrusively conducted within the NTID Eye and Ear Clinic between August 1984 and May 1995. Each chapter includes references and the textbook concludes with an author index, a

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subject index, and an index of tables. The book also includes full color plates of the anatomy of the eye. 73 tables. •

Literacy and Deafness: The Development of Reading, Writing, and Literative Thought Source: Needham Heights, MA: Allyn and Bacon. 1998. 367 p. Contact: Available from Allyn and Bacon. 160 Gould Street, Needham Heights, MA 02494. (781) 455-1250. Website: www.abacon.com. PRICE: $55.00 plus shipping and handling. ISBN: 0205175767. Summary: This textbook offers a comprehensive study of the acquisition of English literacy skills in children and adolescents with severe to profound hearing impairment. Topics include an overview of the major aspects of literacy and deafness, including major perspectives on literacy, the role of teaching, and deafness; the research on literacy among hearing students, and literacy and deaf students; the development of writing in both hearing and deaf students; the major tenets of the literary critical perspective, including considerations for bilingualism of ASL (American Sign Language) and English. The textbook's topics also include the theory and research on second language development of English literacy; the teaching of literacy in English; reform in assessment, focusing on alternative measures of achievement; the interrelations of metatheory, theory, research, and practice; the reciprocal relations between word identification and comprehension and between the conversational and written forms of a phonetic language such as English; and the contribution of a well and early developed language to the subsequent development of literate thought. Each chapter offers suggestions for further reading. The text concludes with a section of comprehension questions for each chapter, a lengthy reference list, and a subject index. 541 references.

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Global Perspectives on the Education of the Deaf in Selected Countries Source: Hillsboro, OR: Butte Publications, Inc. 1999. 423 p. Contact: Available from Butte Publications, Inc. P.O. Box 1328, Hillsboro, OR 971231328. (800) 330-9791. Fax (503) 693-9526. Website: www.buttepublications.com. PRICE: $49.00 plus shipping and handling. ISBN: 1884362362. Summary: This textbook presents chapters by authors from 27 countries that detail the development, current state, and future directions of the education of the deaf. Topics covered in each chapter include: an historical review of the individuals and events that shaped the course of the education of the deaf in that country, the extent and kind of educational services for the deaf, parent education and guidance availability and opportunities, communication philosophy and methodologies followed, the type of sign system used, the academic performance of deaf children, vocational and career education and opportunities, postsecondary educational opportunities for deaf individuals, college or university programs to prepare teachers of deaf students, certification standards for teachers of the deaf, current issues in the education of the deaf, future directions in the education of the deaf, and demographic data. Countries included are: Australia, Canada, Czech Republic and Slovakia, Denmark, Egypt, El Salvador, France, Germany, Ghana, Greece, Hungary, India, Israel, Japan, Lebanon, Nepal, The Netherlands, Nigeria, Norway, Poland, Puerto Rico, Saudia Arabia, Spain, Sweden, Thailand, and Zimbabwe. The final chapter provides a summary of findings and perspectives in each topic area and a look at current directions and future trends in the education of the deaf in each country.

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Book Summaries: Online Booksellers Commercial Internet-based booksellers, such as Amazon.com and Barnes&Noble.com, offer summaries which have been supplied by each title’s publisher. Some summaries also include customer reviews. Your local bookseller may have access to in-house and commercial databases that index all published books (e.g. Books in Print). IMPORTANT NOTE: Online booksellers typically produce search results for medical and non-medical books. When searching for “deafness” at online booksellers’ Web sites, you may discover non-medical books that use the generic term “deafness” (or a synonym) in their titles. The following is indicative of the results you might find when searching for “deafness” (sorted alphabetically by title; follow the hyperlink to view more details at Amazon.com): •

A Deaf Adult Speaks Out by Leo M. Jacobs (2002); ISBN: 0930323610; http://www.amazon.com/exec/obidos/ASIN/0930323610/icongroupinterna

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A Guide to Planning and Support for Individuals Who Are Deafblind by J. M. McInnes (Editor) (1999); ISBN: 0802042422; http://www.amazon.com/exec/obidos/ASIN/0802042422/icongroupinterna

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Advances in Cognition, Education, and Deafness by David S. Martin (Editor) (2002); ISBN: 1563681102; http://www.amazon.com/exec/obidos/ASIN/1563681102/icongroupinterna

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An Intellectual Look at American Sign Language: Clear Thinking on American Sign Language, English and Deaf Education by Thomas J. Balkany (Editor), et al (2001); ISBN: 0963781375; http://www.amazon.com/exec/obidos/ASIN/0963781375/icongroupinterna

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Angels and Outcasts: An Anthology of Deaf Characters in Literature by Trent Batson (Editor), et al (2002); ISBN: 0930323173; http://www.amazon.com/exec/obidos/ASIN/0930323173/icongroupinterna

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Are My Prayers Falling on Deaf Ears? by Andrew Steinmann (1997); ISBN: 0529107422; http://www.amazon.com/exec/obidos/ASIN/0529107422/icongroupinterna

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As in Every Deafness by Graham Foust (2003); ISBN: 0971005982; http://www.amazon.com/exec/obidos/ASIN/0971005982/icongroupinterna

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Ballantyne's Deafness by John Graham (Editor), et al (2001); ISBN: 1861561709; http://www.amazon.com/exec/obidos/ASIN/1861561709/icongroupinterna

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Beethoven in Person: His Deafness, Illnesses, and Death (Contributions to the Study of Music and Dance) by Peter J. Davies (Author) (2001); ISBN: 0313315876; http://www.amazon.com/exec/obidos/ASIN/0313315876/icongroupinterna

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Bigger Dreams: A Two Act Play About Deaf Politician Gary Malkowski by Richard Medugno (2003); ISBN: 1410725375; http://www.amazon.com/exec/obidos/ASIN/1410725375/icongroupinterna

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Bilingualism and Identity in Deaf Communities (The Sociolinguistics in Deaf Communities Series, V. 6) by Melanie Metzger (Editor) (2000); ISBN: 1563680955; http://www.amazon.com/exec/obidos/ASIN/1563680955/icongroupinterna

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Breaking the Silence: The Education of the Deaf in Ireland, 1816-1996 by Edward J. Crean (1997); ISBN: 0952920603; http://www.amazon.com/exec/obidos/ASIN/0952920603/icongroupinterna

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CACDP Deaf Awareness Tutor Training Pack (1999); ISBN: 095199509X; http://www.amazon.com/exec/obidos/ASIN/095199509X/icongroupinterna

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Can You Hear a Rainbow?: The Story of a Deaf Boy Named Chris (Rehabilitation Institute of Chicago Learning Book) by Jamee Riggio Heelan, Nicola Simmonds (Illustrator) (2002); ISBN: 1561452688; http://www.amazon.com/exec/obidos/ASIN/1561452688/icongroupinterna

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Can't Hear, Can't Benefit: A Survey of Deaf People's Experiences of Claiming Disability Living Allowance (2001); ISBN: 0900634928; http://www.amazon.com/exec/obidos/ASIN/0900634928/icongroupinterna

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Choices in Deafness: A Parents' Guide to Communication Options by Sue, Ph.d Schwartz (Editor), Schwarz (2003); ISBN: 0933149859; http://www.amazon.com/exec/obidos/ASIN/0933149859/icongroupinterna

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Communicating With Deaf Children by Tom Bertling (Editor), et al (2002); ISBN: 0963781383; http://www.amazon.com/exec/obidos/ASIN/0963781383/icongroupinterna

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Communication Disorders Sourcebook: Basic Information About Deafness and Hearing Loss, Speech and Language Disorders, Voice Disorders, Balance and Vestibular Disorders, and Disorders of (Health Reference Series, Vol 11) by Linda M. Ross (Editor) (1996); ISBN: 078080077X; http://www.amazon.com/exec/obidos/ASIN/078080077X/icongroupinterna

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Communication Therapy: An Integrated Approach to Aural Rehabilitation With Deaf and Hard of Hearing Adolescents and Adults by Mary June Moseley (Editor), Scott J. Bally (Editor) (1996); ISBN: 1563680548; http://www.amazon.com/exec/obidos/ASIN/1563680548/icongroupinterna

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Context, Cognition, and Deafness by M. Diane Clark (Editor), et al (2002); ISBN: 1563681056; http://www.amazon.com/exec/obidos/ASIN/1563681056/icongroupinterna

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Crying Hands: Eugenics and Deaf People in Nazi Germany by Horst Biesold, et al (2002); ISBN: 1563680777; http://www.amazon.com/exec/obidos/ASIN/1563680777/icongroupinterna

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Culturally Affirmative Psychotherapy With Deaf Persons by Neil S. Glickman (Editor), Michael A. Harvey (Editor) (1996); ISBN: 0805814884; http://www.amazon.com/exec/obidos/ASIN/0805814884/icongroupinterna

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Dancing Without Music: Deafness in America by Beryl Lieff Benderly (2002); ISBN: 0930323599; http://www.amazon.com/exec/obidos/ASIN/0930323599/icongroupinterna

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Deaf & Hard of Hearing (Teacher License Examination, T-11) by Jack Rudman (1997); ISBN: 0837380111; http://www.amazon.com/exec/obidos/ASIN/0837380111/icongroupinterna

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Deaf & Sober: Journeys Through Recovery by Betty G. Miller, Nancy Creighton (Illustrator) (1998); ISBN: 0913072869; http://www.amazon.com/exec/obidos/ASIN/0913072869/icongroupinterna

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Deaf and Disabled, or Deafness Disabled?: Towards a Human Rights Perspective (Disability, Human Rights, and Society) by Mairian Corker (1998); ISBN: 0335196993; http://www.amazon.com/exec/obidos/ASIN/0335196993/icongroupinterna

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Deaf Children in China by Alison Callaway (2002); ISBN: 1563680858; http://www.amazon.com/exec/obidos/ASIN/1563680858/icongroupinterna

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Deaf Culture: A to Z by Walter Paul Kelley (2003); ISBN: 0972956905; http://www.amazon.com/exec/obidos/ASIN/0972956905/icongroupinterna

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Deaf Elephants by William Benton (2002); ISBN: 0764920235; http://www.amazon.com/exec/obidos/ASIN/0764920235/icongroupinterna

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Deaf Girls Rule by Wendy Tiefenbacher (Editor), Wendy Teifenbacher (Editor) (2002); ISBN: 156368117X; http://www.amazon.com/exec/obidos/ASIN/156368117X/icongroupinterna

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Deaf People in the Holocaust: The Extraordinary Story by Jane Alpert (Editor) (2003); ISBN: 0967769752; http://www.amazon.com/exec/obidos/ASIN/0967769752/icongroupinterna

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Deafened People: Adjustment and Support by Kathryn Woodcock, Miguel Aguayo (2000); ISBN: 0802083730; http://www.amazon.com/exec/obidos/ASIN/0802083730/icongroupinterna

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Deafness by Elaine landau (Author) (1997); ISBN: 0805029931; http://www.amazon.com/exec/obidos/ASIN/0805029931/icongroupinterna

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Deafness and Education in the Uk: Research Perspectives by Clare Gallaway (Editor), Alys Young (Editor) (2003); ISBN: 1861563698; http://www.amazon.com/exec/obidos/ASIN/1861563698/icongroupinterna

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Deafness and Ethnicity: Services, Policy and Politics (1998); ISBN: 1861340885; http://www.amazon.com/exec/obidos/ASIN/1861340885/icongroupinterna

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Deafness and Vision Disorders: Anatomy and Physiology, Assessment Procedures, Ocular Anomalies, and Educational Implications by Donald D. Johnson (1999); ISBN: 039806945X; http://www.amazon.com/exec/obidos/ASIN/039806945X/icongroupinterna

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Directory of Services: For Deaf and Hard of Hearing People 2002/2003 (2001); ISBN: 0900634855; http://www.amazon.com/exec/obidos/ASIN/0900634855/icongroupinterna

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Early Childhood Deafness by Ellen Kurtzer-White (Editor), David Luterman (Editor) (2001); ISBN: 0912752610; http://www.amazon.com/exec/obidos/ASIN/0912752610/icongroupinterna

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Educating the Deaf : Psychology, Principles, and Practices by Donald Moores (Author) (2001); ISBN: 061804289X; http://www.amazon.com/exec/obidos/ASIN/061804289X/icongroupinterna

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Educational and Developmental Aspects of Deafness by Donald F. Moores (Editor), et al (2002); ISBN: 0930323521; http://www.amazon.com/exec/obidos/ASIN/0930323521/icongroupinterna

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Effective Inclusion of Deaf Pupils into Mainstream Schools (Education Guidelines Project) (2000); ISBN: 090063474X; http://www.amazon.com/exec/obidos/ASIN/090063474X/icongroupinterna

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Ethics in Mental Health and Deafness by Virginia Gutman (Editor) (2002); ISBN: 156368120X; http://www.amazon.com/exec/obidos/ASIN/156368120X/icongroupinterna

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Family Communication Folio: A Folio of Articles from Perspectives in Education and Deafness by Shawn N. Mahshie (Editor) (1997); ISBN: 0880952156; http://www.amazon.com/exec/obidos/ASIN/0880952156/icongroupinterna

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Forth and Back: Coping With Deafness by Katie Ricci Franzosa (2001); ISBN: 0759629595; http://www.amazon.com/exec/obidos/ASIN/0759629595/icongroupinterna

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Four from E.S.T. Marathon '99: All About Al/Deaf Day/Dreamtime for Alice/Goodbye Oscar by Cherie Vogelstein, et al (2001); ISBN: 0822217325; http://www.amazon.com/exec/obidos/ASIN/0822217325/icongroupinterna

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Full Face: A Correspondence About Becoming Deaf in Mid-Life by Claire H. Blatchford, Clare Blatchford (1997); ISBN: 1884362214; http://www.amazon.com/exec/obidos/ASIN/1884362214/icongroupinterna

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Gaillard in Deaf America: A Portrait of the Deaf Community, 1917 (Deaf Classics Series) by Henri Gaillard, et al (2002); ISBN: 1563681226; http://www.amazon.com/exec/obidos/ASIN/1563681226/icongroupinterna

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George Dalgarno on Universal Language: The Art of Signs (1661), the Deaf and Dumb Man's Tutor (1680),and the Unpublished Papers by George Dalgarno, et al (2001); ISBN: 0198237324; http://www.amazon.com/exec/obidos/ASIN/0198237324/icongroupinterna

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Handbook of Services for the Deaf and the Hard-of-Hearing: A Bridge to Accessibility by John Adams (Author), Pamela Rohring (Author) (2003); ISBN: 0120441411; http://www.amazon.com/exec/obidos/ASIN/0120441411/icongroupinterna

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Helen Keller, Public Speaker : Sightless But Seen, Deaf But Heard by Lois J. Einhorn (Author) (1998); ISBN: 0313286434; http://www.amazon.com/exec/obidos/ASIN/0313286434/icongroupinterna

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If Blind Guys Wear Sunglasses, Why Don't Deaf People Wear Ear Muffs by Blind Mike Danger (2003); ISBN: 075962254X; http://www.amazon.com/exec/obidos/ASIN/075962254X/icongroupinterna

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Industrial Diseases Series: Deafness (MacDuff Series Practice Guides) by Alistair MacDuff QC (1997); ISBN: 1858111234; http://www.amazon.com/exec/obidos/ASIN/1858111234/icongroupinterna

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Inner Lives of Deaf Children: Interviews and Analysis by Martha Sheridan, et al (2002); ISBN: 1563681021; http://www.amazon.com/exec/obidos/ASIN/1563681021/icongroupinterna

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International Bibliography of Sign Language (International Studies on Sign Language and Communication of the Deaf, Vol 21) by Guido H. G. Joachim, et al (1999); ISBN: 3927731331; http://www.amazon.com/exec/obidos/ASIN/3927731331/icongroupinterna

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Issues in Deaf Education by Susan Gregory (Editor), et al (1998); ISBN: 1853465127; http://www.amazon.com/exec/obidos/ASIN/1853465127/icongroupinterna

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Issues Unresolved: New Perspectives on Language and Deaf Education by Amatzia Weisel (Editor), Israe International Congress on Education for the Deaf 1995 Tel Aviv (1998); ISBN: 156368067X; http://www.amazon.com/exec/obidos/ASIN/156368067X/icongroupinterna

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Know That: Quotes from Deaf Women for a Positive Life by Tina Jo Breindel (Compiler) (2000); ISBN: 1581210124; http://www.amazon.com/exec/obidos/ASIN/1581210124/icongroupinterna

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201

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Language Development of Deaf and Hard-of-hearing Children: Beyond the Great Debate by Christine Yoshinaga-Itano (Editor) (2004); ISBN: 1565932714; http://www.amazon.com/exec/obidos/ASIN/1565932714/icongroupinterna

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Lend Me Your Ear: Rhetorical Constructions of Deafness by Brenda Jo Brueggemann (2002); ISBN: 1563680793; http://www.amazon.com/exec/obidos/ASIN/1563680793/icongroupinterna

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Life After Deafness: A Resource Book for Late Deafened Adults (2001); ISBN: 0969924607; http://www.amazon.com/exec/obidos/ASIN/0969924607/icongroupinterna

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Living Legends: Six Stories About Successful Deaf People by Darlene Toole (1996); ISBN: 1884362133; http://www.amazon.com/exec/obidos/ASIN/1884362133/icongroupinterna

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Living With a Deaf Dog: A Book of Advice, Facts and Experiences About Canine Deafness by Susan Cope Becker, Andrew Caylor (Illustrator) (1997); ISBN: 0966005805; http://www.amazon.com/exec/obidos/ASIN/0966005805/icongroupinterna

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Living With Hearing Loss: The Sourcebook for Deafness and Hearing Disorders (The Facts for Life Series) by Carol Turkington, Allen E. Sussman (2000); ISBN: 0816041407; http://www.amazon.com/exec/obidos/ASIN/0816041407/icongroupinterna

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Looking Back: A Reader on the History of Deaf Communities and Their Sign Languages (International Studies on Sign Language and Communication of the) by Renate Fischer (Editor), Harlan Lane (Editor) (2002); ISBN: 3927731323; http://www.amazon.com/exec/obidos/ASIN/3927731323/icongroupinterna

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Lost Senses: Deafness and Blindness by John Kitto (2003); ISBN: 076615937X; http://www.amazon.com/exec/obidos/ASIN/076615937X/icongroupinterna

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Love Songs of the Tone-Deaf by Asher Brauner (1999); ISBN: 0967086108; http://www.amazon.com/exec/obidos/ASIN/0967086108/icongroupinterna

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Mental Health and Deafness by Peter Hindley (Editor), Nick Kitson (Editor) (1998); ISBN: 1897635397; http://www.amazon.com/exec/obidos/ASIN/1897635397/icongroupinterna

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Mental Health Care of Deaf People: A Culturally Affirmative Approach by Neil S. Glickman (Editor), Sanjay Gulati (Editor) (2003); ISBN: 0805844694; http://www.amazon.com/exec/obidos/ASIN/0805844694/icongroupinterna

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Metaphor Stories for Deaf Children by Robert K. Rittenhouse (1999); ISBN: 1884362338; http://www.amazon.com/exec/obidos/ASIN/1884362338/icongroupinterna

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Movers and Shakers: Deaf People Who Changed the World by Cathryn Carroll, et al (1998); ISBN: 0915035650; http://www.amazon.com/exec/obidos/ASIN/0915035650/icongroupinterna

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My Sense of Silence: Memoirs of a Childhood With Deafness by Lennard J. Davis (2000); ISBN: 0252025334; http://www.amazon.com/exec/obidos/ASIN/0252025334/icongroupinterna

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On His Deafness and Other Melodies Unheard by Robert Panara (1997); ISBN: 0963401653; http://www.amazon.com/exec/obidos/ASIN/0963401653/icongroupinterna

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Panic and Deaf: Two Modern Satires (Fiction from Modern China) by Hsiao-Sheng Liang, et al (2001); ISBN: 0824822501; http://www.amazon.com/exec/obidos/ASIN/0824822501/icongroupinterna

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Psychological Perspectives on Deafness by Marc Marschark (Editor), M. Diane Clark (Editor) (1999); ISBN: 0805827102; http://www.amazon.com/exec/obidos/ASIN/0805827102/icongroupinterna

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Psychosocial Aspects of Deafness by Nanci A. Scheetz (Author) (2003); ISBN: 0205343473; http://www.amazon.com/exec/obidos/ASIN/0205343473/icongroupinterna

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Reading to Deaf Children: Learning from Deaf Adults (A Manual for Parents and Teachers) by David R. Schleper, Shawn N. Mahshie (Editor) (1997); ISBN: 0880952121; http://www.amazon.com/exec/obidos/ASIN/0880952121/icongroupinterna

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Relations of Language and Thought : The View from Sign Language and Deaf Children (Counterpoints) by Marc Marschark (Editor), et al (1997); ISBN: 0195100573; http://www.amazon.com/exec/obidos/ASIN/0195100573/icongroupinterna

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Self-Advocacy for Students Who Are Deaf or Hard of Hearing by Kristina M., Phd. English (1999); ISBN: 9990440948; http://www.amazon.com/exec/obidos/ASIN/9990440948/icongroupinterna

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Sign Me Alice & Laurent Clerc: A Profile/Two Deaf Plays by Gilbert C. Eastman, et al (1997); ISBN: 091503560X; http://www.amazon.com/exec/obidos/ASIN/091503560X/icongroupinterna

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Signs of Resistance: American Deaf Cultural History, 1900 to World War II (History of Disability) by Susan Burch (2002); ISBN: 0814798918; http://www.amazon.com/exec/obidos/ASIN/0814798918/icongroupinterna

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Social Security Acts 1975 to 1990: Prescribed Disease - Occupational Deafness - Time Limit for Claim - Retrospective Legislation - Relevant Change of Circumstances (Decisions of the Commissioners: R(I) 1994/2) (1997); ISBN: 0117625035; http://www.amazon.com/exec/obidos/ASIN/0117625035/icongroupinterna

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Sudden Deafness: A Controversial Issue (Orl Nova, 5) by Jean-Philippe Guyot (Editor) (2000); ISBN: 3805570910; http://www.amazon.com/exec/obidos/ASIN/3805570910/icongroupinterna

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Tactile Sign Language: Turn Taking and Questions in Signed Conversations of DeafBlind People (International Studies on Sign Language and Communication of the Deaf,volume 38) by Johanna Mesch, Wolfgang Duchting (2002); ISBN: 3927731803; http://www.amazon.com/exec/obidos/ASIN/3927731803/icongroupinterna

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The Courage to Live: Donna Gustavel's Triumph Over Cerebral Palsy and Deafness by Dan Brannan (1997); ISBN: 0965022846; http://www.amazon.com/exec/obidos/ASIN/0965022846/icongroupinterna

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The Deaf Child in the Family and at School: Essays in Honor of Kathryn P. MeadowOrlans by Kathryn P. Meadow-Orlans (Editor), et al (1999); ISBN: 0805832211; http://www.amazon.com/exec/obidos/ASIN/0805832211/icongroupinterna

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The Deaf Mute Howls by Albert Ballin (2002); ISBN: 1563680734; http://www.amazon.com/exec/obidos/ASIN/1563680734/icongroupinterna

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The Deaf Way II Anthology: A Literary Collection by Deaf and Hard of Hearing Writers by Tonya M. Stremlau (Editor) (2002); ISBN: 1563681277; http://www.amazon.com/exec/obidos/ASIN/1563681277/icongroupinterna

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The Deaf Way: Perspectives from the International Conference on Deaf Culture by Carol J. Erting (Editor), et al (2002); ISBN: 1563680262; http://www.amazon.com/exec/obidos/ASIN/1563680262/icongroupinterna

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The Employment Situation and Experiences of Deaf and Hard of Hearing People: Research into Deafness and Employment (2002); ISBN: 0900634952; http://www.amazon.com/exec/obidos/ASIN/0900634952/icongroupinterna

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The Encyclopedia of Deafness and Hearing Disorders by Carol Turkington, Allen E., Phd Sussman (2000); ISBN: 081604046X; http://www.amazon.com/exec/obidos/ASIN/081604046X/icongroupinterna

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The Experience of Employers: Research into Deafness and Employment (2002); ISBN: 0900634944; http://www.amazon.com/exec/obidos/ASIN/0900634944/icongroupinterna

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The Hands Are the Head of the Mouth: The Mouth As Articulator in Sign Languages (International Studies on Sign Language and the Communication of the Deaf, v. 39.) by Penny Boyes Braem (Editor), et al (2002); ISBN: 3927731838; http://www.amazon.com/exec/obidos/ASIN/3927731838/icongroupinterna

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The Healing of Rodolphe Grivel Congenital Deaf-Mute - 1927: Congenital Deaf-Mute by Fabre D'Olivet, et al (1997); ISBN: 1564594998; http://www.amazon.com/exec/obidos/ASIN/1564594998/icongroupinterna

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The Impact of a Unique Cooperative American University, Usaid Funded SpeechLanguage Pathologist, Audiologist, and Deaf Educator B.S. Degree Program (Mellen Studies in Education, V. 67) by Franklin H. Silverman, Robert D. Moulton (2002); ISBN: 0773471898; http://www.amazon.com/exec/obidos/ASIN/0773471898/icongroupinterna

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The Legend of Five Great Deaf Ghost Stories by James Gillies (2003); ISBN: 1403369763; http://www.amazon.com/exec/obidos/ASIN/1403369763/icongroupinterna

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The Politics of Deafness by Owen Wrigley (2002); ISBN: 1563680521; http://www.amazon.com/exec/obidos/ASIN/1563680521/icongroupinterna

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The Politics of Visual Language: Deafness, Language Choice and Political Socialization by James Roots (1999); ISBN: 0886293456; http://www.amazon.com/exec/obidos/ASIN/0886293456/icongroupinterna

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The World of Deaf Infants: A Longitudinal Study (Perspectives on Deafness) by Kathryn P. Meadow-Orlans, et al (2004); ISBN: 0195147901; http://www.amazon.com/exec/obidos/ASIN/0195147901/icongroupinterna

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The Young Deaf Child by David Luterman, et al (1999); ISBN: 0912752467; http://www.amazon.com/exec/obidos/ASIN/0912752467/icongroupinterna

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You and Your Deaf Child: A Self-Help Guide for Parents of Deaf and Hard of Hearing Children by John W. Adams (2002); ISBN: 1563680602; http://www.amazon.com/exec/obidos/ASIN/1563680602/icongroupinterna

The National Library of Medicine Book Index The National Library of Medicine at the National Institutes of Health has a massive database of books published on healthcare and biomedicine. Go to the following Internet site,

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http://locatorplus.gov/, and then select “Search LOCATORplus.” Once you are in the search area, simply type “deafness” (or synonyms) into the search box, and select “books only.” From there, results can be sorted by publication date, author, or relevance. The following was recently catalogued by the National Library of Medicine:11 •

A handbook of readings in education of the deaf and postschool implications. Author: Fusfeld, Irving S.; Year: 1955; Springfield, Ill., Thomas [1967]

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Catalog of captioned films for the deaf. Author: Carpenter, Anita Cecile Arsenault,; Year: 1957; [Washington] Office of Education, 1967

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Conquering childhood deafness; a new technique for overcoming hearing problems in infants and children. Report on ten years of HEAR Foundation achievements. Author: Griffiths, Ciwa.; Year: 1965; New York, Exposition Press [c1967]

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Conversation with the deaf. Author: Sutcliffe, T. H.; Year: 1965; London, Royal National Institute for the Deaf [1966]

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Deafness and public responsibility; the provision of hearing aids. Author: Gregory, Peter.; Year: 1955; London, Bell [c1964]

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Deafness in childhood. Edited with a preface by Freeman McConnell and Paul H. Ward. Author: McConnell, Freeman E.,; Year: 1964; [Nashville] Vanderbilt Univ. Press, 1967

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Final report on temporary deafness following exposure to loud tones and noise, Sept. 30, 1943 [by] Hallowell Davis [and others]. Author: United States. Office of Scientific Research and Development. Committee on Medical Research.; Year: 1961; Stockholm, 1950

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Interpreting for deaf people; a report of a workshop on interpreting, Governor Baxter State School for the Deaf, Portland, Maine, July 7-27, 1965. Stephen P. Quigley, editor and chairman, Joseph P. Young, co-chairman. Author: Quigley, Stephen P. (Stephen Patrick),; Year: 1963; Washington, Vocational Rehabilitation Administration [1966]

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Noise, hearing and deafness. Author: Beales, Philip H.; Year: 1966; London, Joseph [1965]

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Non-verbal intelligence tests for deaf and hearing subjects; Snijders-Oomen nonverbal scale S. O. N., by J. Th. Snijders and N. Snijders-Oomen. Author: Snijders, J. Th. (Johannes Theodorus),; Year: 1967; Groningen, Wolters, 1959

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On occupational deafness from white noise. Author: Bernabei, Luigi.; Year: 1963; Chicago, Beltone Institute for Hearing Research, 1955

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Philosophy of the deaf; a symposium on hearing and hearing loss. Author: Australian Association for Better Hearing.; Year: 1964; Melbourne [1962]

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Report on findings related to communication and basic minimum services for deafblind persons for use throughout the world to the World Assembly of the World Council for the Welfare of the Blind, Rome, Italy, July 1959. Author: World Council for the Welfare of the Blind. Committee on Services for the Deaf-Blind.; Year: 1967; [Brooklyn, c1959]

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In addition to LOCATORPlus, in collaboration with authors and publishers, the National Center for Biotechnology Information (NCBI) is currently adapting biomedical books for the Web. The books may be accessed in two ways: (1) by searching directly using any search term or phrase (in the same way as the bibliographic database PubMed), or (2) by following the links to PubMed abstracts. Each PubMed abstract has a "Books" button that displays a facsimile of the abstract in which some phrases are hypertext links. These phrases are also found in the books available at NCBI. Click on hyperlinked results in the list of books in which the phrase is found. Currently, the majority of the links are between the books and PubMed. In the future, more links will be created between the books and other types of information, such as gene and protein sequences and macromolecular structures. See http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Books.

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Research in deafness in children. Author: Fisch, L.; Year: 1964; London, National Deaf Children's Society [1964]

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The deaf child, by Edith Whetnall and D. B. Fry. Author: Whetnall, Edith.; Year: 1959; London, Heinemann [1964]

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The deaf in Britain: a general outline. Author: National Institute for the Deaf, London.; Year: 1965; London, 1961

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The effect of experimentally induced frustration on indirect aggressive responses of deaf and hearing boys. Author: Fuchs, Arnold J.; Year: 1958; [Garden City, N. Y.] 1963

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The efficacy of nasopharyngeal irradiation for the prevention of deafness in children [by] John E. Bordley and William G. Hardy. Author: Bordley, John E.; Year: 1964; [Uppsala, Almqvist; Wiksells, 1955]

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The future of the welfare officer to the deaf. Author: Nixon, Frank Horsfall,; Year: 1964; London, Royal National Institute for the Deaf, 1932

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The psychology of deafness; sensory deprivation, learning, and adjustment. Author: Myklebust, Helmer R.; Year: 1962; New York, Grune; Stratton [c1964]

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Tour and observations of schools for the deaf in North America [by] Eric Stanley Greenaway. International Congress on the Modern Educational Treatment of Deafness, held at University of Manchester, July 15-23, 1958, by Phyllis Watson. Reports published under the auspices of Canadian Association of the Deaf. Author: Greenaway, Eric Stanley.; Year: 1965; [Agincourt, Ont., 1958]

Chapters on Deafness In order to find chapters that specifically relate to deafness, an excellent source of abstracts is the Combined Health Information Database. You will need to limit your search to book chapters and deafness using the “Detailed Search” option. Go to the following hyperlink: http://chid.nih.gov/detail/detail.html. To find book chapters, use the drop boxes at the bottom of the search page where “You may refine your search by.” Select the dates and language you prefer, and the format option “Book Chapter.” Type “deafness” (or synonyms) into the “For these words:” box. The following is a typical result when searching for book chapters on deafness: •

Hearing Impairment and Deafness Source: in Gething, L. Person to Person: A Guide for Professionals Working with People with Disabilities. 3rd ed. Baltimore, MD: Paul H. Brookes Publishing Company. 1997. p. 139-170. Contact: Available from Paul H. Brookes Publishing Company. P.O. Box 10624, Baltimore, MD 21285-0624. (800) 638-3775. Fax (410) 337-8539. E-mail: [email protected]. Website: www.brookespublishing.com. PRICE: $39.00 plus shipping and handling. ISBN: 1557663203. Summary: Hearing loss is a generic term used to describe impairment that may range from mild to profound. Hearing loss can occur in high or low frequencies and can affect one or both ears, to the same or different extents. This chapter on hearing loss and deafness is from a manual for professionals working with people with disabilities. The author stresses that it is important to make distinctions between deafness, hearing impairment, and being hard of hearing; definitions for each category are provided. The author then discusses issues that affect living with hearing loss, including the extent of

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the loss, prelingual versus postlingual hearing loss, and associated consequences such as difficulty in locating sound, problems with tinnitus (ringing or buzzing in the ears), and dizziness or balance problems. The author considers the presence of other disabilities with hearing loss and the options for communicating with people who have hearing loss, including oral communication, manual communication, and written communication. Other topics covered are the Deaf community, personal adjustment to a hearing loss, sexuality, parenting, family, community living, access to community activities and services, education, employment, appropriate interpersonal language and behavior, strategies for interaction, anatomy of the ear, the mechanism of hearing, the causes of hearing loss, incidence and prevalence, diagnostic considerations, support services, hearing aids and other assistive devices, and additional costs that may be incurred by someone with a hearing loss. The chapter concludes with a list of resource organizations in Australia, Canada, New Zealand, the United Kingdom, and the United States. 7 figures. 6 tables. 27 references. •

Meeting the Needs of Hispanic-Latino Deaf Students Source: in Christensen, K. and Delgado, G., eds. Deaf Plus: A Multicultural Perspective. San Diego, CA: Dawn Sign Press. 2000. p. 149-198. Contact: Available from Dawn Sign Press. 6130 Nancy Ridge Drive, San Diego, CA 92121-3223. (800) 549-5350. Voice/TTY (858) 625-0600. Fax (858) 625-2336. Website: www.dawnsign.com. PRICE: $18.95 plus shipping and handling. ISBN: 1581210175. Summary: Hispanic or Latino students are the largest racial or ethnic minority in deaf education, yet resources to meet their needs are limited. This chapter on this population is from a textbook that offers a collection of essays that provide information about the community that surrounds Deaf children from diverse backgrounds. The authors support the contention that multiple cultures in the classroom are a positive thing, and that diversity within Deaf culture is enriching and enhancing. In this chapter, the author begins with a review of the history of Hispanics and Latinos in the United States, including the history of education of this population and the growth of linguistic diversity. The author then focuses on strategies to meet the needs of Hispanic and Latino deaf children, including programs and personnel, language needs, educational and cultural traditions, language dominance and language proficiency, Limited English Proficiency (LEP) and LEP deaf students, the limitations of formal assessments, a team approach to assessment, language assessment instruments, English as a Second Language for Deaf LEP students, immigrant students with limited schooling, students with Spanish literacy, and a literature based approach to teaching. 185 references.

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Early Intervention and Deafness: School/Home Partnerships After Ten Years, How Are They Working Source: in Welch, O.M., ed. Research and Practice in Deafness: Issues and Questions in Education, Psychology, and Vocational Service Provision. Springfield, IL: Charles C Thomas Publisher. 1993. p. 5-27. Contact: Available from Charles C Thomas Publisher. 2600 South First Street, Springfield, IL 62794-9265. (800) 258-8980 or (217) 789-8980; Fax (217) 789-9130. PRICE: $34.95 plus shipping and handling. ISBN: 039805861X. Summary: In this chapter the author reflects on early intervention and deafness and on how well school-home partnerships are working after ten years. The author describes the SKI*HI project, a home-based early intervention program for deaf and hard of hearing children, and how it has worked for the more than 35,000 children who have

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been served using this model. Topics covered include the legislation that formed the basis for early intervention; a description of the three components of SKI*HI, early identification, home intervention, and supportive services; the curriculum areas; the rationale behind SKI*HI; parent training and advising; demographic characteristics of SKI*HI children and families; the effects of home-based programming on SKI*HI children and families; factors that separate the more effective SKI*HI sites from those that are less effective; and implications for future programming. 1 figure. 3 tables. 9 references. (AA-M). •

About Late Deafness Source: in Woodcock, K. and Aguayo, M. Deafened People: Adjustment and Support. Toronto, Ontario: University of Toronto Press. 2000. p. 3-33. Contact: Available from University of Toronto Press Inc. 5201 Dufferin Street, North York, Ontario M3H 5T8. (800) 565-9523. Fax (416) 667-7832. E-mail: [email protected]. Website: www.utpress.utoronto.ca. Price: $21.95 plus shipping and handling. ISBN: 0802083730. Summary: Late deafness can be defined as hearing loss in people who could once hear naturally or with amplification but who have become deaf and are now unable to rely on hearing to comprehend speech. This chapter describing late deafness is from a book that addresses the process of adjustment to, and acceptance of, deafness as an adult. The authors demonstrate that deafness is not merely a medical condition; it is a social disability that affects the individual, the family, the social circle, and the work group. By describing the psychosocial experience of acquired deafness as a process of adjustment, the authors demonstrate that acceptance of deafness is a process with practical, social, and emotional implications. Written predominantly in a question and answer format, this chapter covers hearing evaluation and measurement of hearing loss, how loss of a few sounds affects speech comprehension, the anatomy and physiology of the ear and hearing, the causes of hearing loss and deafness, medical classifications of deafness, the prevalence of different types of hearing loss, and the practical definitions of hearing loss and its impact on every day living. Comments from the authors' experiences are provided in sidebars throughout the chapter. 3 figures. 1 table.

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Common Refractive Errors (Ametropias) and Deafness Source: in Johnson, D.D. Deafness and Vision Disorders: Anatomy and Physiology, Assessment Procedures, Ocular Anomalies, and Educational Implications. Springfield, IL: Charles C. Thomas Publisher, Ltd. 1999. p. 224-264. Contact: Available from Charles C. Thomas Publisher, Ltd. 2600 South First Street, Springfield, IL 62794-9265. (800) 258-8980 or (217) 789-8980. Fax (217) 789-9130. PRICE: $74.95 plus shipping and handling. ISBN: 039806945X. Summary: Refractive errors are functional optical (eye) defects in which the parallel light rays from the environment, after passing through the eye's transparent substances, are not brought to a sharp focus precisely on the retina when the eye is unaccommodated; hence, the retinal images are blurred. This chapter on refractive errors (ametropias) is from a textbook written to help students preparing for work in the field of deafness to understand and incorporate an awareness of vision disorders in the deaf population. The information concerning the congenital anomalies, functional defects, and pathologic ocular conditions most often found within a deaf student population was obtained from eleven years of research unobtrusively conducted within the NTID Eye and Ear Clinic between August 1984 and May 1995 (at the National

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Technical Institute for the Deaf, one of the eight colleges of the Rochester Institute of Technology). This chapter deals specifically with those refractive errors common in this population: myopia (nearsightedness), hyperopia (farsightedness), astigmatism, or a combination thereof. For each condition, the author provides definitions and general comments, classification issues, etiology, and the statistics for the condition in the NTID population. Refractive errors were by far the most common ocular disorders found among NTID deaf students, ranging from approximately 40 percent of the total population of students (n = 3190) entering NTID to about 85 percent of those 756 students seen in the NTID Eye and Ear Clinic. 12 tables. 57 references. •

Use of Sensory Aids for Teaching Speech to Children who are Deaf Source: in Plant, G.; Spens, K.E. Profound Deafness and Speech Communication. San Diego, CA: Singular Publishing Group, Inc. 1995. p. 461-491. Contact: Available from Singular Publishing Group, Inc. 401 West 'A' Street, Suite 325, San Diego, CA 92101-7904. (800) 521-8545 or (619) 238-6777. Fax (800) 774-8398 or (619) 238-6789. E-mail: [email protected]. Website: www.singpub.com. PRICE: $75.00 plus shipping and handling. ISBN: 156593492X. Summary: Teaching children who are deaf to speak is a formidable task for both teacher and child. In this chapter, from a text on profound deafness and speech communication, the author discusses the use of sensory aids for teaching speech to children who are deaf. The pedagogical issues involved in teaching speech are used as a framework within which to examine the development, evaluation, and use of existing technology. The author examines the issues inherent in developing speech in children with minimal auditory capability; describes pedagogical issues associated with using sensory aids for speech training with deaf children; examines existing technologies and the evidence for their clinical efficacy; and discusses future needs. 2 figures. 62 references.

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Clinical Course of Tinnitus in Patients with Sudden Deafness Source: in Hazell, J., ed. Proceedings of the Sixth International Tinnitus Seminar. London, England: Tinnitus and Hyperacusis Centre. 1999. p. 553-557. Contact: Available from Tinnitus and Hyperacusis Centre. 32 Devonshire Place, London, W1N 1PE, United Kingdom. Fax 44 + (0) 207 486 2218. E-mail: [email protected]. Website: www.tinnitus.org. PRICE: Contact publisher for price. ISBN: 0953695700. Also available on CD-ROM. Summary: This article reports on a study in which the clinical course of tinnitus was examined in 90 patients with idiopathic sudden deafness (SD) and this was compared with the recovery of hearing. The article is from a lengthy document that reprints the proceedings of the Sixth International Tinnitus Seminar, held in Cambridge, United Kingdom, in September 1999 and hosted by the British Society of Audiology. In this study, approximately 50 percent of patients experienced persistent tinnitus during the first week after the onset of SD and this decreased gradually by approximately 10 percent beyond the fifth week. In contrast, the proportion of tinnitus free patients increased from 20 to 40 percent during the same period. The tinnitus with a pitch matching low frequency or broad band noise was more frequently observed in SD than in other otological diseases. Approximately 60 percent of the patients experienced a change in pitch or timbre of tinnitus over time. In the groups with a good hearing recovery, decreasing and temporally increasing types of loudness change were predominant, whereas in patients with a poor hearing recovery, fluctuating, constant, and tinnitus free types were most frequently observed. 4 figures. 1 reference.

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Research and Practice in Deafness: Issues and Questions in Education, Psychology, and Vocational Service Provision Source: Springfield, IL: Charles C Thomas Publisher. 1993. 310 p. Contact: Available from Charles C Thomas Publisher. 2600 South First Street, Springfield, IL 62794-9265. (800) 258-8980 or (217) 789-8980; Fax (217) 789-9130. PRICE: $34.95 plus shipping and handling. ISBN: 039805861X. Summary: This book examines questions related to education, psychology, adult literacy, transition, and programming for low functioning and incarcerated youth who are deaf. The book is divided into four sections. Section I, Part I deals with early intervention home/school partnerships, deaf and hard of hearing students with mild additional disabilities, and children who are deaf-blind; Part II focuses on elementary, secondary, and post-secondary education. Section II examines issues in psychology that assume particular importance for persons who are deaf or hard of hearing. Section III focuses on habilitation and rehabilitation issues, as well as on the unique role of community-based centers for adults who are deaf and hard of hearing. Section IV offers two perspectives on future research in the field, in the areas of cross-cultural research and on a paradigm derived from epistemology. Each chapter, written by experts in the field, includes extensive references; a brief subject index concludes the volume.

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Unresolved Issues in the Provision of Mental Health Services to People who are Deaf Source: in Welch, O.M., ed. Research and Practice in Deafness: Issues and Questions in Education, Psychology, and Vocational Service Provision. Springfield, IL: Charles C Thomas Publisher. 1993. p. 211-229. Contact: Available from Charles C Thomas Publisher. 2600 South First Street, Springfield, IL 62794-9265. (800) 258-8980 or (217) 789-8980; Fax (217) 789-9130. PRICE: $34.95 plus shipping and handling. ISBN: 039805861X. Summary: This chapter addresses unresolved issues in the provision of mental health services to people who are deaf. The authors note that because effective communication between the mental health professional and the consumer is critical to the counseling process, many of the issues presented in this chapter are especially pertinent in situations involving individuals who use American Sign Language (ASL) as their primary mode of communication. Topics covered include barriers to the provision of accessible and appropriate mental health services; issues related to establishing rapport and a therapeutic alliance in initial interviews; issues impacting the use of interpreters in mental health settings; and issues impacting the delivery of mental health services to deaf people who are members of racial or ethnic minority groups. The authors stress that people who are deaf should be able to access as broad a continuum of programs and services as is available for people who hear. Numerous case examples are presented to illustrate the concepts discussed. 41 references. (AA-M).

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Education and Rehabilitation of Incarcerated Inmates Who Are Deaf Source: in Welch, O.M., ed. Research and Practice in Deafness: Issues and Questions in Education, Psychology, and Vocational Service Provision. Springfield, IL: Charles C Thomas Publisher. 1993. p. 147-167. Contact: Available from Charles C Thomas Publisher. 2600 South First Street, Springfield, IL 62794-9265. (800) 258-8980 or (217) 789-8980; Fax (217) 789-9130. PRICE: $34.95 plus shipping and handling. ISBN: 039805861X.

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Summary: This chapter describes a comprehensive approach to educational and rehabilitation assessment and programming of incarcerated inmates who have disabilities, focusing particularly on those who are deaf. Topics covered include the goals and purposes of rehabilitation for all inmates; rehabilitation of inmates with disabilities; the model program in place at the Texas Department of Criminal Justice (TDCJ) that provides educational and vocational programs; legislation addressing this issue; inmate assessment, including general considerations, initial data gathering, analysis of past employment, educational assessment, psychological assessment, and vocational evaluation; the environmental skills assessment, including the living, learning, and working environments, and the rehabilitation diagnosis; and rehabilitation programming. 36 references. (AA-M). •

Deaf and Hard of Hearing Students with Mild Additional Disabilities Source: in Welch, O.M., ed. Research and Practice in Deafness: Issues and Questions in Education, Psychology, and Vocational Service Provision. Springfield, IL: Charles C Thomas Publisher. 1993. p. 29-47. Contact: Available from Charles C Thomas Publisher. 2600 South First Street, Springfield, IL 62794-9265. (800) 258-8980 or (217) 789-8980; Fax (217) 789-9130. PRICE: $34.95 plus shipping and handling. ISBN: 039805861X. Summary: This chapter discusses issues that are central to understanding the educational needs of students who are deaf or hearing impaired and who have mild additional disabilities. These issues include concerns related to prevalence, definition, characteristic behaviors, identification, assessment and intervention. The authors explore these issues, focusing on the three most frequently reported mild additional disabilities among deaf and hard of hearing children: learning disabilities, emotional and/or behavioral problems and mental retardation. The authors stress that determining criteria for identifying this population demands thoughtful interdisciplinary assessment, including the comparison of formal and informal data to classroom performance. 37 references. (AA-M).

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Habilitation and Rehabilitation of Persons Who are Deaf: A Conundrum Source: in Welch, O.M., ed. Research and Practice in Deafness: Issues and Questions in Education, Psychology, and Vocational Service Provision. Springfield, IL: Charles C Thomas Publisher. 1993. p. 233-257. Contact: Available from Charles C Thomas Publisher. 2600 South First Street, Springfield, IL 62794-9265. (800) 258-8980 or (217) 789-8980; Fax (217) 789-9130. PRICE: $34.95 plus shipping and handling. ISBN: 039805861X. Summary: This chapter examines the issues that hamper the coordinated and effective delivery of rehabilitation services to persons who are deaf and hard of hearing. Topics covered include who is to be served; who is best qualified to provide vocational rehabilitation services to this population; what model should be used in providing these services; how future deafness rehabilitation professionals should be trained; and the relationship between deafness rehabilitation and the fields of general rehabilitation and deaf education. 17 references.

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Hereditary Hearing Loss and Deafness Source: in Massimini, K., ed. Genetic Disorders Sourcebook. 2nd ed. Detroit, MI: Omnigraphics. 2000. p. 605-617.

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Contact: Available from Omnigraphics. 615 Griswold Street, Detroit, MI 48226. (313) 961-1340. Fax (313) 961-1383. Website: www.omnigraphics.com. PRICE: $78.00 plus shipping and handling. ISBN: 0780802411. Summary: This chapter from a Genetic Disorders Sourcebook covers hereditary hearing loss and deafness. Several hundred genes are known to cause hereditary hearing loss and deafness. The genetic forms of hearing loss are diagnosed by otologic, audiologic, and physical examination, family history, ancillary testing (such as computed tomography, or CT, examination of the temporal bone), and DNA based testing. DNA based genetic tests are available for the diagnosis of branchiootorenal (BOR) syndrome, Pendred syndrome, dilated vestibular aqueduct syndrome, DNFB1, and DFNB4; testing for deafness causing mutations in the GHB2 gene (connexin 26) plays a prominent role in diagnosis and genetic counseling. The authors cover definitions, categories of hearing loss, syndrome hearing loss, manners of inheritance, Usher syndrome, Pendred syndrome, Jervell and Lange Nielsen syndrome, Waardenburg syndrome, BOR syndrome, Stickler syndrome, neurofibromatosis, Alport syndrome, genetic counseling, special issues in counseling Deaf persons, and patient care management. The chapter concludes with a listing of resources through which readers can obtain additional information. •

Theoretical and Applied Insights from Multimedia Facilitation of Communication Skills in Children with Autism, Deaf Children, and Children with Other Disabilities Source: in Adamson, L.B.; Romski, A.R., eds. Communication and Language Acquisition: Discoveries from Atypical Development. Baltimore, MD: Paul H. Brookes Publishing Company. 1997. p. 295-325. Contact: Available from Paul H. Brookes Publishing Company. P.O. Box 10624, Baltimore, MD 21285-0624. (800) 638-3775 or (410) 337-9580. Fax (410) 337-8539. E-mail: [email protected]. Website: www.brookespublishing.com. PRICE: $44.00 plus shipping and handling. ISBN: 1557662797. Summary: This chapter is from a text that explores research on atypical communication and language development as a source of knowledge about how children become accomplished communicators. The authors of this chapter discuss their studies of advances in reading and writing under multimedia literacy instruction in children with disabilities. The children in the studies ranged in age from 5 year olds who are beginning to read to 10 to 16 year olds with serious delays in reading and writing skills. Their disabilities included autism, deafness, motor disabilities, dyslexia, and attention deficit hyperactivity disorder (ADHD). Regardless of age and disability, these children worked with multimedia computer software designed to facilitate skills in the text mode by patterning text sentences together rapidly with other media channels, including graphics, voice, and sign language. The first section in the chapter provides the theoretical basis for the multimedia procedures, including the application of Rare Event Learning (REL) theory. The next section reviews results of field research for children's advances in reading and writing and for related teacher and child variables. Most of the results involve children with deafness and other hearing impairments and children with autism. 6 tables. 75 references.

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Receptive Vocabulary Development of Infants and Toddlers Who Are Deaf or Hard of Hearing Source: Volta Review. 100(5): 29-52. 2000.

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Contact: Available from Alexander Graham Bell Association for the Deaf and Hard of Hearing. Subscription Department, 3417 Volta Place, NW, Washington, DC 20007-2778. Voice/TTY (202) 337-5220. Website: www.agbell.org. Summary: This chapter is from a text that provides information on the language, speech, and social emotional development of very young children who are deaf or hard of hearing. In this chapter, the authors report on a study that examined receptive vocabulary scores on the Words and Gestures subtest of the MacArthur Communicative Development Inventory (CDI); the study population was a group of 8 to 22 month old infants and toddlers with hearing loss (n = 168). Results of analyses demonstrated significant correlations between the children's receptive vocabulary scores and other CDI language subscales, as well as other measures of language, aspects of nonverbal cognition, and an assessment of symbolic play skills. Demographic variables of the sample (including gender, mode of communication, age of identification, disability status, ethnicity, level of maternal education, and degree of hearing loss) were found not to be significantly correlated with children's receptive vocabulary scores. Normative data for receptive vocabulary development, based on the CDI, are presented so that 8 to 22 month old deaf or hard of hearing children with hearing parents can be compared with their peers. The authors note that the majority of children in this study were identified with hearing loss before 6 months. It is possible that, though still delayed in comparison with normal hearing children, the average receptive vocabulary development of this group is more advanced than would have been expected prior to development of universal newborn hearing screening programs. 2 figures. 6 tables. 57 references. •

Literacy, Literate Thought, and Deafness: A Brief Synthesis Source: in Paul, P.V. Introduction to Literacy and Deafness. Needham Heights, MA: Allyn and Bacon. 1998. p. 299-308. Contact: Available from Allyn and Bacon. 160 Gould Street, Needham Heights, MA 02494. (781) 455-1250. Website: www.abacon.com. PRICE: $55.00 plus shipping and handling. ISBN: 0205175767. Summary: This chapter is from a textbook that offers a comprehensive study of the acquisition of English literacy skills in children and adolescents with severe to profound hearing impairment. In this chapter, the author offers some reflections and directions concerning the development of literacy and literate thought in children and youth who are deaf. If English text based literacy is a major, realistic goal, it is argued that educators need to decide if the basic fundamentals of literacy can be developed by students with severe to profound hearing impairment, especially in light of what is known about literacy development and instruction. The chapter reemphasizes several issues relative to the development of literacy and literate thought: the interrelations of metatheory, theory, research, and practice; the reciprocal relations between word identification and comprehension and between the conversational and written forms of a phonetic language such as English; and the contributions of a well and early developed language to the subsequent development of literate thought. 4 references.

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Vision Problems of the Deaf and Educational Implications: Ancillary Clinic and Educational Support Services Source: in Johnson, D.D. Deafness and Vision Disorders: Anatomy and Physiology, Assessment Procedures, Ocular Anomalies, and Educational Implications. Springfield, IL: Charles C. Thomas Publisher, Ltd. 1999. p. 315-350.

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Contact: Available from Charles C. Thomas Publisher, Ltd. 2600 South First Street, Springfield, IL 62794-9265. (800) 258-8980 or (217) 789-8980. Fax (217) 789-9130. PRICE: $74.95 plus shipping and handling. ISBN: 039806945X. Summary: This chapter is from a textbook written to help students preparing for work in the field of deafness to understand and incorporate an awareness of vision disorders in the deaf population. This chapter discusses the educational implications and vision problems of the deaf, focusing on the ancillary clinic and educational support services. Information within the book concerning the congenital anomalies, functional defects, and pathologic ocular conditions most often found within a deaf student population was obtained from eleven years of research unobtrusively conducted within the NTID Eye and Ear Clinic between August 1984 and May 1995 (at the National Technical Institute for the Deaf, one of the eight colleges of the Rochester Institute of Technology). The first part of the chapter deals with the treatment plans requested by the NTID consulting ophthalmologists as a result of their findings. Treatment options discussed include observation and identification, visual field tests, new prescription for glasses, personal and family counseling, maintain same prescription for glasses, prescription for safety glasses, obtain previous records, get fundus or disc photographs, get electroretinograms (ERG), off campus diagnostic referral, get low vision evaluation (LVE), determine RP status, see contact lens specialist, obtain prescription for medications, declare legal blindness, and get Amsler grid test. The second portion of the chapter provides suggestions for modification of the educational environment or use by the student of special low vision aids to more adequately insure academic success for those deaf students with noncorrectable visual problems. These educational support services may include use of low vision aids (LVAs) such as magnifying lenses in spectacles, hand held or stand magnifiers, telescopic devices, and closed circuit television (CCTB) reading devices. Other services may include preferential seating or classroom modifications such as enlarged print on transparencies, nonglare lighting, and enlarged print passouts of the instructor's lecture outline. The author presents a number of case illustrations to demonstrate how these educational modifications are used by students at NTID to enhance their chances for academic success. 5 tables. 15 references. •

Cochlear Implants and Tactile Devices for Students with Profound Deafness Source: in Roeser, R.J.; Downs, M.P., eds. Auditory Disorders in School Children: The Law, Identification, Remediation. New York, NY: Thieme Medical Publishers, Inc. 1995. p. 271-290. Contact: Available from Thieme Medical Publishers, Inc. 333 Seventh Avenue, New York, NY 10001. (800) 782-3488 or (212) 760-0888; Fax (212) 947-1112; E-mail: [email protected]; http://www.thieme.com. PRICE: $45.00 plus shipping and handling. ISBN: 0865775508. Summary: This chapter on cochlear implants and tactile devices for students with profound deafness is from a textbook that brings together experts from all disciplines to present ideas on how to provide help for school children with auditory disorders. The basic tenet of audiological management for children with hearing impairment is to provide amplification as soon as possible to promote communication skills. However, for those children who do not receive benefit from conventional amplification, the use of cochlear implants and tactile devices may be indicated. Topics covered include determining the need for cochlear implants or tactile aids; safety, effectiveness, and labeling, including the role of the Food and Drug Administration (FDA); features and technology of a cochlear implant; cochlear implant candidacy requirements; risks and

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benefits of cochlear implants; cochlear implants in children; types of tactile aids; type and location of stimulation; single versus multichannel tactile aids; functional differences between hearing aids, cochlear implants, and tactile instruments; sound awareness and detection; and training strategies with cochlear implants and tactile devices. One sidebar presents a brief case study. 3 figures. 6 tables. 52 references. •

Deafness and Alcohol Abuse Source: in Lala, F.J.J., Jr. Counseling the Deaf Substance Abuser. Chicago, IL: Adams Press. 1998. p. 76-138. Contact: Available from Midas Management Company. P.O. Box 27740, Las Vegas, NV 89126-1740. PRICE: $28.95 plus shipping and handling. ISBN: 0966375300. Summary: This chapter on deafness and alcohol abuse is from a book intended to focus attention on the problem of substance abuse in the Deaf community. It attempts to help people affected by addiction, and provide preventive information and incentives to preclude the development of alcoholism and substance abuse in the next generation. Originally published as the author's dissertation, the book states that up to 35 percent of people with significant hearing impairment have abused substances, including alcohol. This is almost double the estimated rate of comparable abuse among people who do not have impaired hearing. This chapter presents a broad overview on alcoholism as it relates to deafness. The author discusses basic information about alcoholism; reviews both short and long term physical, mental, emotional, and social effects of alcohol abuse; outlines current thought regarding the etiology and epidemiology of alcoholism, including predisposing influences, such as genetics; discusses sociocultural and psychological interweaving factors which can contribute to or maintain alcohol abuse; and provides a listing of some variables that may influence an individual's willingness to seek treatment for alcohol addiction. 2 tables.

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Introduction to Language and Deafness Source: in Paul, P.V. Language and Deafness. 3rd ed. San Diego, CA: Singular Publishing Group. 2001. p. 1-32. Contact: Available from Thomson Learning Group. P.O. Box 6904, Florence, KY 41022. (800) 842-3636. Fax (606) 647-5963. Website: www.singpub.com. PRICE: $59.95 plus shipping and handling. ISBN: 1565939999. Summary: This chapter on language and deafness is from a textbook that offers an indepth introduction to language development in children and adolescents who are deaf or hard of hearing. This introductory chapter provides an overview of language and deafness, including acquisition and instructional issues such as exposure, use, and representation, and acquisition of more than one language; philosophical perspectives on deafness, including those based on clinical descriptions and those based on cultural descriptions; and the use of metatheory, paradigm, and language. The authors give readers a general framework for learning more about these sometimes conflicting approaches to language development in children who are deaf. The chapter includes a list of comprehension questions (to test the reader's understanding of the chapter), a list of challenge questions (which can be answered only with additional thinking and research), and a group of suggested readings for further study. 6 tables. 5 references.

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Deaf and Hard of Hearing Source: in American College of Sports Medicine. ASCM's Exercise Management for Persons with Chronic Disease and Disabilities. Champaign, IL: Human Kinetics. 1997. p. 233-236. Contact: Available from Human Kinetics. P.O. Box 5076, Champaign, IL 61825-5076. (800) 747-4457 or (217) 351-5076; http://www.humankinetics.com/. PRICE: $39.00 each. ISBN: 0873227980. Item number: BACS0798. Summary: This chapter on people with hearing loss is from an exercise management text that outlines exercise strategies for people with chronic disease and disabilities. The author provides an overview of the pathophysiology of hearing loss, the effects on the exercise response, the effects of exercise training, management and medications, recommendations for exercise testing, recommendations for exercise programming, and special considerations, particularly related to communicating with clients who are deaf or hearing impaired. The author stresses that regular exercise by people with hearing loss produces the same positive physiological and skill benefits as for individuals with no hearing loss. Additional benefits include more opportunities to improve socialization skills, practice and improvement in balance, and improving self image, confidence, and spatial orientation. One chart summarizes the special considerations of working with this population. 1 table. 8 references.

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Reading-Comprehension Perspective: Research on Students Who Are Deaf Source: in Paul, P.V. Introduction to Literacy and Deafness. Needham Heights, MA: Allyn and Bacon. 1998. p. 61-92. Contact: Available from Allyn and Bacon. 160 Gould Street, Needham Heights, MA 02494. (781) 455-1250. Website: www.abacon.com. PRICE: $55.00 plus shipping and handling. ISBN: 0205175767. Summary: This chapter on reading comprehension research in deaf students is from a textbook that offers a comprehensive study of the acquisition of English literacy skills in children and adolescents with severe to profound hearing impairment. The author presents and synthesizes research on literacy and deafness within the framework of reading comprehension models, particularly cognitive information processing models. Initially, the author discusses reading comprehension achievement of students who are deaf. The author's perspective dates back to the first use of formal tests, which provides historical background on the magnitude of the problem. Subsequently, a summary of results is discussed relative to two categories: text based variables and reader based variables. The chapter ends with an in depth discussion of Hanson's classic question: whether reading is different for deaf persons than for hearing persons. 4 tables. 6 references.

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Current and Future Directions in Research on Speech Intelligibility Assessment of Persons who are Deaf Source: in Gagne, J.P.; Tye-Murray, N., eds. Research in Audiological Rehabilitation: Current Trends and Future Directions. Minneapolis, MN: American Academy of Rehabilitative Audiology. 1994. p. 237-249. Contact: Available from Academy of Rehabilitative Audiology. Circulation Manager, P.O. Box 26532, Minneapolis, MN 55426. (612) 885-0095. PRICE: Free for members; $17.50 for non-members; plus shipping and handling.

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Summary: This chapter on the assessment of speech intelligibility in people who are deaf is from an audiological rehabilitation monograph from the Academy of Rehabilitative Audiology. Major themes concern the relative importance of factors affecting speech intelligibility, the relationship of physical speech parameters to perceptual speech intelligibility measures, and the reliability and validity of various approaches to measuring speech intelligibility. The authors suggest future research directions, predicated on the implications of current research, for computer-based measurement of both physical speech parameters and their ultimate use for speech intelligibility estimation. The research findings discussed in this chapter indicate the importance of careful attention to methodological issues in the assessment of speech intelligibility. 33 references. (AA-M). •

Long-Term Effects of Deafness and Chronic Electrical Stimulation of the Cochlea Source: in Waltzman, S.B. and Cohen, N.L., eds. Cochlear Implants. New York, NY: Thieme. 2000. p. 31-41. Contact: Available from Thieme. 333 Seventh Avenue, New York, NY 10001. (800) 7823488. Fax (212) 947-0108. E-mail: [email protected]. PRICE: $89.00 plus shipping and handling. ISBN: 0865778825. Summary: This chapter on the long term effects of deafness and chronic electrical stimulation of the cochlea is from a book that incorporates original chapters on relevant topics with related papers. These papers were presented at the Fifth International Cochlear Implant Conference held in New York, in May, 1997. The book provides professionals involved in the field of cochlear implants with a comprehensive reference text. All aspects of implantation are covered including the effects of electrical stimulation, processing capabilities, preoperative criteria, medical, surgical and radiological issues, results and postoperative programming, rehabilitation, and education. The authors of this chapter note that, with the number of very young children now receiving cochlear implants, it is important to determine the consequences of implantation and stimulation with the highly abnormal input delivered by a cochlear implant upon the deafened, developing auditory system. The authors report on their studies that have been conducted in cats that are neonatally deafened by administration of an ototoxic drug. The deafened animals receive implants unilaterally at the time of weaning, and the electrical signals delivered by the implant provide the initial and sole input to the developing auditory system. The authors summarize the results of research on the morphologic and physiologic consequences of chronic electrical stimulation in these neonatally deafened animals and discuss some of the implications relevant to pediatric cochlear implants. 7 figures. 54 references.

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Profound Deafness Source: in Tyler, R.S. Cochlear Implants: Audiological Foundations. San Diego, CA: Singular Publishing Group, Inc. 1993. p. 1-33. Contact: Available from Singular Publishing Group, Inc. 401 West 'A' Street, Suite 325, San Diego, CA 92101-7904. (800) 521-8545 or (619) 238-6777. Fax (800) 774-8398 or (619) 238-6789. E-mail: [email protected]. Website: www.singpub.com. PRICE: $51.50 plus shipping and handling. ISBN: 1879105810. Summary: This chapter, from a book about cochlear implants, provides an introduction to profound deafness. The author begins with a detailed description of the varied terminology used throughout the years in this area. The author then discusses the auditory capacities of profoundly deaf persons, including amplification issues for the

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profoundly deaf; speechreading as an additional source of information; auditory capacity versus auditory performance; categories of profound deafness; the limited predictive power of pure tone threshold; the need for auditory performance measures; a comparison of persons with hearing aids versus cochlear implants; and auditory criteria for implant candidacy. The chapter continues with a discussion of the nonauditory characteristics of the profoundly deaf and their relevance to cochlear implants, including age at onset of profound deafness; current age; duration of profound deafness; educational environment; cultural identity; groupings; tactile aids; combining implants and hearing aids; progressive hearing loss; improved aids and implants; and the need for more actuarial data and for prediction of implant outcome. 4 figures. 89 references. •

Cognitive Development and Deafness Source: in Nowell, R.C.; Marshak, L.E., eds. Understanding Deafness and the Rehabilitation Process. Needham Heights, MA: Allyn and Bacon. 1994. p. 35-49. Contact: Available from Allyn and Bacon. 160 Gould Street, Needham Heights, MA 02194-2310. (617) 455-1200. PRICE: $44.95 plus shipping and handling. ISBN: 0205156282. Summary: This chapter, from a book on deafness and the rehabilitation process, provides an overview on cognition and deafness. The author begins with an historical perspective on the views of the cognitive abilities of deaf persons and then explores the issues related to this topic within the research and educational communities. Current issues discussed include organic versus educational cognitive disabilities, neuroscience, cognitive style, nonverbal cognitive abilities, language and cognition, and assessment of cognitive performance. The chapter concludes with a set of recommendations and directions for future actions for all persons interested in a constructive life for deaf learners. 45 references.

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Effects of Cochlear Implantation on the Young Deaf Child Source: in Uziel, A.S.; Mondain, M., eds. Cochlear Implants in Children: Advances in Otorhinolaryngology, Volume 50. Basel, Switzerland: S. Karger AG. 1995. p. 125-128. Contact: Available from S. Karger AG. 26 West Avon Road, P.O. Box 529, Framington, CT 06085. (800) 828-5479 or (203) 675-7834. PRICE: $99.50 plus shipping and handling, unless prepaid. ISBN: 3805560958. Summary: This chapter, from a text on cochlear implants in children, reports on an investigation to study the effects of early implantation (shorter length of deafness) on speech recognition in children who are congenitally/prelingually deaf. The authors note that the majority of studies to date have reported results on deaf children with a mean age at time of implantation of approximately 6 years. In this study, the authors included 14 children who received the Nucleus multichannel cochlear implant and have used the device for at least 2 years. None of the subjects exhibited more than pattern perception with conventional amplification and a plateuing of development of auditory and linguistic skills was evident prior to implantation. The authors report that all subjects in this study demonstrate open-set speech recognition, use oral/aural communication, attend age-appropriate nursery or mainstream schools, and continue to improve over time. They also briefly discuss the additional variables that may impact on the success of the cochlear implant, including adequate device programming, remapping, rehabilitation and training, and level of parental involvement. 2 figures. 3 references.

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Quick Guide for the Deaf Community Source: in National Association of the Deaf. The Red Notebook. Silver Spring, MD: National Association for the Deaf. 1994. 41 p. Contact: Available from National Association of the Deaf. Bookstore, 814 Thayer Avenue, Silver Spring, MD 20910-4500. Voice (301) 587-6282; TTY (301) 587-6283; Fax (301) 587-4873. PRICE: $36.00 plus shipping and handling. Item Number RS007. Summary: This document is the 1994 Spring/Summer update to the Red Notebook, a loose-leaf publication for both the Deaf and library communities. The Red Notebook also serves as a forum to encourage individuals and interest groups from the Deaf community to communicate their perspectives on various issues and concerns in one united voice to the library community. Information is presented in four sections: libraries and librarians; auxiliary aids, including communication tips and accessibility; diverse populations; and hearing disabilities. The update provides extensive information for and about deaf people, as well as resources and organizations through which readers can get additional information.

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Facing the Realities of Deafness Source: in Cornett, R.O. and Daisey, M.E. Cued Speech Resource Book for Parents of Deaf Children. 2nd ed. Cleveland, OH: National Cued Speech Association. 2001. p. 1-16. Contact: Available from National Cued Speech Association. 23970 Hermitage Road, Cleveland, OH 44122-4008. Voice/TTY (800) 459-3529 or (216) 292-6213. E-mail: [email protected]. PRICE: $37.50 for members; $39.50 for nonmembers, plus shipping and handling. ISBN: 0963316419. Summary: This introductory chapter is from a book that offers an overview of cued speech, the use of hand cues with speech that permits the deaf child to learn the English language. The book includes information and advice for parents who have decided to use Cued Speech with a child who is deaf or hard of hearing, or are considering doing so. This chapter is addressed to parents who have just learned that they have a hearing impaired child. The authors feel that such parents should begin by taking a careful look at what typically happens to parents of a deaf child and what typically happens to the child. At the same time, the authors stress that it does not have to be this way. The authors discuss the effects of inadequate communication, the implications of different methods of communication, the emotional stages that parents go through, problems associated with post-lingual (after some language has been achieved) hearing loss, and typical performance of deaf children. The authors conclude that most hearing impaired children have speech and speechreading skills inadequate for effective communication with the general public, and they fall (and remain) far behind their hearing peers in language, reading, and other academic areas. The authors end the chapter by noting that Cued Speech offers a solution to these problems.

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Common Ocular Disorders Found Among Deaf NTID Students Source: in Johnson, D.D. Deafness and Vision Disorders: Anatomy and Physiology, Assessment Procedures, Ocular Anomalies, and Educational Implications. Springfield, IL: Charles C. Thomas Publisher, Ltd. 1999. p. 95-223. Contact: Available from Charles C. Thomas Publisher, Ltd. 2600 South First Street, Springfield, IL 62794-9265. (800) 258-8980 or (217) 789-8980. Fax (217) 789-9130. PRICE: $74.95 plus shipping and handling. ISBN: 039806945X.

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Summary: This lengthy chapter is from a textbook written to help students preparing for work in the field of deafness to understand and incorporate an awareness of vision disorders in the deaf population. This chapter discusses common ocular disorders. Information within the book concerning the congenital anomalies, functional defects, and pathologic ocular conditions most often found within a deaf student population was obtained from eleven years of research unobtrusively conducted within the NTID Eye and Ear Clinic between August 1984 and May 1995 (at the National Technical Institute for the Deaf, one of the eight colleges of the Rochester Institute of Technology). This chapter deals specifically with those eleven common visual pathologies and aberrant visual conditions most often encountered among the young and more chronologically mature deaf adult students within the NTID college population. The author also hopes to promote an awareness of those vision problems which are also likely to be found in greater numbers within the deaf population in general. Failure to identify and attend to these problems may not only impact on the learning process, but on communication, mobility, recreation, social interaction, and vocational pursuits as well. The conditions covered are rubella oculopathy, strabismus, amblyopia, inherited color vision deficiency, retinitis pigmentosa (Usher syndrome), cataracts or aphakia, nystagmus, microphthalmos, glaucoma, ocular albinism, and ptosis (blepharoptosis). The chapter demonstrates that a large number of deaf people have concomitant visual problems, many of which are noncorrectable or progressive in nature. 43 tables. 130 references.

Directories In addition to the references and resources discussed earlier in this chapter, a number of directories relating to deafness have been published that consolidate information across various sources. The Combined Health Information Database lists the following, which you may wish to consult in your local medical library:12 •

All About Deafness: Where to Turn for Answers to Questions About Hearing Loss Source: Rochester, NY: Salavatore J. Parlato. 1995. 76 p. Contact: Available from Dolores Parlato. 248 Seville Drive, Rochester, NY 14617. PRICE: $9.95 each. Also available from Gallaudet University Bookstore. 800 Florida Avenue, NE, Washington, DC 20002. Voice (202) 651-5380; Voice/TTY (800) 451-1073; Fax (202) 651-5489. PRICE: $7.95 plus shipping and handling. Summary: This directory lists organizations committed to serving Americans with hearing loss. Organizations are listed in twenty-two categories: the Americans with Disabilities Act, assistive listening devices, associations, book publishers, captioned media, computers, education, employment, financial assistance, foundations, health (including AIDS), hearing aids and cochlear implants, interpreters, mental health, periodicals, rehabilitation, religion, research, speech and hearing, TTY telecommunications, reference materials, and mega-centers. The entry for each organization includes only the organization name, address, and telephone number.

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You will need to limit your search to “Directory” and “deafness” using the "Detailed Search" option. Go directly to the following hyperlink: http://chid.nih.gov/detail/detail.html. To find directories, use the drop boxes at the bottom of the search page where “You may refine your search by.” For publication date, select “All Years.” Select your preferred language and the format option “Directory.” Type “deafness” (or synonyms) into the “For these words:” box. You should check back periodically with this database as it is updated every three months.

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Directory of National Organizations Of and For Deaf and Hard of Hearing People Source: Washington, DC: National Deaf Education Network and Clearinghouse, Laurent Clerc National Deaf Education Center. 1998. [8 p.]. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. PRICE: Single copy free. Code Number 184. Summary: This directory lists the major professional, service, and consumer organizations for deaf and hard of hearing people in the United States. Listed alphabetically by name of the organization, each entry includes the address, telephone number(s), and executive director. Each entry also includes a brief annotation describing the organization and its publications. Organizations are also coded to identify their main focus in areas including: consumer concerns and/or advocacy, education, funding sources, information and/or referral services, medicine, professional organizations, recreation, religion, research, self-help or support groups, and social organizations. The directory also includes a full listing of the state commissions or state offices on deafness for each of the fifty states, the District of Columbia, and Puerto Rico.

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Life After Deafness: A Resources Book for Late-Deafened Adults Source: Ottawa, Ontario: Canadian Hard of Hearing Association (CHHA). 1995. 183 p. Contact: Available from Canadian Hard of Hearing Association (CHHA). 2435 Holly Lane, Suite 205, Ottawa, Ontario K1V 7P2. Voice (613) 526-1584 or (800) 623-8068; TTY (613) 526-1584; Fax (613) 526-4718. PRICE: $20.00 plus shipping and handling. Summary: This resource book is designed to help people with acquired deafness meet and overcome the challenges they face. The book provides over 600 listings related to health and rehabilitation, assistive communication devices, education, employment, organizations, and information services. The book is divided into four sections: a health and rehabilitation section contains recommendations for the care and treatment of patients who are newly deafened, including a short section on cochlear implants; a communication and assistive devices section introduces a variety of assistive devices and communication skills used by late-deafened adults; a section on coping in a hearing world contains information and advice for readers on functioning on a day-to-day basis; and an information section lists information about organizations, books, periodicals, and videos related to people who are deaf, deafened, or hard of hearing. A short bibliography of books about Deaf history and culture by Deaf and deafened authors who share their feelings, philosophies, and experiences is also included. Appendices provide comprehensive listings of Canadian provincial and federal programs and resources, U.S., and international resources.

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CHAPTER 8. MULTIMEDIA ON DEAFNESS Overview In this chapter, we show you how to keep current on multimedia sources of information on deafness. We start with sources that have been summarized by federal agencies, and then show you how to find bibliographic information catalogued by the National Library of Medicine.

Video Recordings An excellent source of multimedia information on deafness is the Combined Health Information Database. You will need to limit your search to “Videorecording” and “deafness” using the “Detailed Search” option. Go directly to the following hyperlink: http://chid.nih.gov/detail/detail.html. To find video productions, use the drop boxes at the bottom of the search page where “You may refine your search by.” Select the dates and language you prefer, and the format option “Videorecording (videotape, videocassette, etc.).” Type “deafness” (or synonyms) into the “For these words:” box. The following is a typical result when searching for video recordings on deafness: •

Voices in a Deaf Theater Source: Fanlight Productions. 1996. ISBN 1-57295-229-6. 24 minutes; Closed-captioned VHS; color. Contact: Fanlight Productions Media Library. Post Office Box 1084, Harriman, NY 10926. 800-343-5540; Fax: 201-652-1973. E-mail: [email protected]; Web site: www.fanlight.com. Order No. QA-229. PRICE: $199.00. Summary: This documentary follows a mixed cast of deaf and hearing actors as they prepare to stage Tennessee Williams' The Glass Menagerie. As they rehearse, each group experiences the culture of the other. The video offers viewers a window into the deaf world, the expressive language of the deaf, as well as an opportunity to witness the way two groups, with a common goal but very different communication tools, can bridge their language barrier. Study Guide included

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AIDS in the Deaf Community/Deaf in the AIDS Community : Creating Partnerships Contact: University of California San Francisco, Center on Deafness, 3333 California St Ste 10, San Francisco, CA, 94118, (415) 476-4980. Summary: This video discusses how organizations for deaf persons and health service providers and program staff can work together to improve care for deaf people with the human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS). The video states that in many cities there is a lack of services available to deaf persons with HIV/AIDS, thus making it difficult for providers or program staff to communicate effectively with patients or clients. This lack of services has also made it more difficult to pass on new information concerning HIV prevention and treatments to the deaf community as a whole. The video makes recommendations that are designed to help programs without services specifically for deaf persons to communicate more effectively with their non-hearing clients. The video provides guidelines for the viewers about how they can provide for the deaf in their existing programs and services. The video examines the impact of HIV/AIDS on the deaf community in the United States (US), and the reasons why some deaf people may be reluctant to use services or programs, even those offered specifically for them. It advises the viewers on how health care providers and HIV/AIDS programs can create partnerships with local deaf organizations to overcome these communication, education, attitudinal, and treatment barriers.

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Beyond the Surface: Implementing ADA in Postsecondary Institutions Serving Deaf and Hard of Hearing Students Source: St. Paul, MN: Midwest Center for Postsecondary Outreach, St. Paul Technical College. 1998. (videocassette). Contact: Available from PEPNet Resource Center. National Center on Deafness, California State University, Northridge, 18111 Nordhoff Street, Northridge, CA 913308267. Voice/TTY (888) 684-4695 or (818) 677-2611. Fax (818) 677-4899. Website: www.pepnet.org. PRICE: $20.00. Summary: This video program depicts a satellite teleconference, held in March 1998, on 'Implementing The Americans with Disabilities Act in Postsecondary Institutions Serving Deaf and Hard of Hearing Students.' The program features Jeanne M. Kincaid, a specialist in disability and law in education, with three other panelists, Dr. Edwin Franklin, Marta Belsky, and Dr. Frederick Schroeder. The conference was hosted by the Midwest Center for Postsecondary Outreach, one of four national regional centers that comprise the Postsecondary Education Programs Network (PEPNet) that are available to help educators who are working with people who are deaf or hard of hearing. These centers work primarily at the institutional level to increase and enhance postsecondary educational opportunities for individuals who are deaf and hard of hearing. The program discusses how to find, evaluate, manage and budget for interpreting services, the role of vocational rehabilitation in providing auxiliary services in postsecondary educational settings, and techniques that can be used to increase the diversity of a particular campus by including deaf or hard of hearing students. The panelists answer questions from the teleconference and studio audience. Dr. Kincaid emphasizes that there are ways to effectively manage the requirements of the ADA and Section 504 that are cost effective and yet provide quality services to the students. The information provided should not be considered legal advice, but should merely serve as guidance in assisting providers, colleges, universities at large, and others in understanding the obligation institutions of higher learning have to serve students who are deaf and hard

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of hearing. The video features open captioning, voice over for the people using sign language, and a sign language interpreter window for people who are voicing. •

Focus on Faculty: Effective Pedagogy with Students Who Are Deaf and Hard of Hearing Source: Northridge, CA: Western Region Outreach Center and Consortia, National Center on Deafness, California State University, Northridge. 1998. (videocassette). Contact: Available from PEPNet Resource Center. National Center on Deafness, California State University, Northridge, 18111 Nordhoff Street, Northridge, CA 913308267. Voice/TTY (888) 684-4695 or (818) 677-2611. Fax (818) 677-4899. Website: www.pepnet.org. PRICE: $20.00. Summary: This videotape features a satellite teleconference class on working with deaf students in a mainstream college environment. The conference was hosted by the Postsecondary Education Programs Network (PEPNet) (four regional centers) that are available to help educators who are working with people who are deaf or hard of hearing. These centers work primarily at the institutional level to increase and enhance postsecondary educational opportunities for individuals who are deaf and hard of hearing. The program begins with a brief history of mainstreaming, then introduces the goal of dispelling the myths and misunderstandings that faculty may have regarding deaf students in their classes. Topics include the varied background of deaf students, the problem of English as a second language, evaluating student presentations (particularly when presented through an interpreter), grading the content of a student's work rather than correcting the language structure (except in English classes), using different testing strategies, the use of email and other technologies in educational methods, teaching mathematics, the differences between deaf and hard of hearing students, and how to encourage more deaf and hard of hearing students to attend mainstream colleges and universities. The program is fully captioned and includes voice over for people who use sign language and a sign language interpreter for people who use voice.

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AIDS in the Deaf Community-Deaf in the AIDS Community: Creating Partnerships Source: Salem, OR: Sign Enhancers, Inc. 1996. (videocassette). Contact: Available from Sign Enhancers, Inc. 10568 Southeast Washington Street, Portland, OR 97216-2809. TTY/Voice (800) 767-4461. Fax (503) 257-3013. PRICE: $49.95 plus shipping and handling. Number MH-6. Summary: This videotape is designed to help care providers understand the concerns of Deaf people who are HIV-positive. The program illustrates a wide variety of concerns, focusing on how to provide services to Deaf people with AIDS. Topics include the use of American Sign Language (ASL), the psychosocial aspects of having AIDS, how to get support and access to support services and groups, working with an interpreter, the use of adaptive devices, HIV-related complications (particularly vision loss), HIV-related hearing loss, confronting barriers to care, the stigma associated with HIV-positive status, problems in awareness and educating the Deaf community about AIDS, the need to utilize a variety of communication options (writing, TTY, sign language, speechreading, gesturing), how to determine if an interpreter is needed, how to find funding for interpreting services, confidentiality issues, communication barriers in service delivery, and creating partnerships between Deaf agencies and organizations and service delivery organizations, including health care and mental health care agencies. The videotape is narrated by a Deaf man with AIDS who uses ASL; voiceover is provided. The video features a variety of people, predominantly Deaf people, ranging from consumers, to

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social workers, to mental health professionals, to advocates. Voice over and sign language are provided throughout the program; the video is also close-captioned. •

Meeting the Challenge: Working with Deaf People in Recovery Source: Salem, OR: Sign Enhancers, Inc. 1994. (videocassette). Contact: Available from Sign Enhancers, Inc. 10568 Southeast Washington Street, Portland, OR 97216-2809. TTY/Voice (800) 767-4461. Fax (503) 257-3013. PRICE: $49.95 plus shipping and handling; $224.00 for Mental Health and Recovery Video Package. Number MH-2. Summary: This videotape program is designed to familiarize drug and alcohol service providers with individuals who are Deaf or hard of hearing. The narrator, Mike Eisele, helps therapists and counselors empathize with Deaf people. Topics covered include Deaf language and culture; barriers to getting services, notably language barriers; and the role and proper use of interpreters. The tape is in American Sign Language, with voice over and closed captioning. (AA-M).

•

I Can: Stories of Deaf and Hard of Hearing People in Recovery Source: Salem, OR: Sign Enhancers, Inc. 1994. (videocassette). Contact: Available from Sign Enhancers, Inc. 10568 Southeast Washington Street, Portland, OR 97216-2809. TTY/Voice (800) 767-4461. Fax (503) 257-3013. PRICE: $49.95 plus shipping and handling; $224.00 for Mental Health and Recovery Video Package. Number MH-3. Summary: This videotape program provides an intimate look at six recovery drug addicts and alcoholics who are Deaf or hard of hearing. The tape profiles two women and four men; three of whom are alcoholic and three of whom are drug addicts, each have been in recovery for varying lengths of time. The videotape is designed for prevention programs, counseling programs, and for individuals and family members who are new to the recovery process. The tape is in American Sign Language, with voice over and closed-captioning.

Bibliography: Multimedia on Deafness The National Library of Medicine is a rich source of information on healthcare-related multimedia productions including slides, computer software, and databases. To access the multimedia database, go to the following Web site: http://locatorplus.gov/. Select “Search LOCATORplus.” Once in the search area, simply type in deafness (or synonyms). Then, in the option box provided below the search box, select “Audiovisuals and Computer Files.” From there, you can choose to sort results by publication date, author, or relevance. The following multimedia has been indexed on deafness: •

"It's okay to be deaf, Denise" [videorecording] Source: Michael Reese Hospital and Medical Center; Year: 1975; Format: Videorecording; Chicago: The Hospital; [Los Angeles: for loan or sale by House Ear Institute], c1975

•

AIDS in the deaf community, deaf in the AIDS community [videorecording]: creating partnerships Source: [presented by] the University of California Center on Deafness; Year: 1996; Format: Videorecording; San Francisco: The Center, 1996

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•

Communicating with the deaf [videorecording]: a piece of cake Source: a presentation of Films for the Humanities & Sciences; Centre for Deafness Studies and Research, Griffith University; Year: 1996; Format: Videorecording; Princeton, N.J.: Films for the Humanities & Sciences, c1996

•

Meeting the challenge [videorecording]: working with deaf people in recovery Source: [presented by] University of California, Center on Deafness; Year: 1991; Format: Videorecording; [San Francisco, Calif.]: The Center, c1991

•

The Deaf patient: language and written communication [videorecording] Source: United Hospitals, inc., Dept. of Health Education; [produced by] UHI-TV; Year: 1977; Format: Videorecording; St. Paul, Minn.: The Dept, c1977

•

The International Conference on Deaf History [videorecording] Source: produced by Sign Media, Inc. & Gallaudet University; Year: 1992; Format: Videorecording; Burtonsville, Md.: Sign Media, c1992

•

Understanding diversity in the deaf community [videorecording]: Jessica M. Lee Source: produced by the Region X Interpreter Education Center in the Regional Resource Center on Deafness (RRCD) at Western Oregon University; Year: 1999; Format: Videorecording; [Stillwater, Okla.: NCRTM], 1999

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CHAPTER 9. PERIODICALS AND NEWS ON DEAFNESS Overview In this chapter, we suggest a number of news sources and present various periodicals that cover deafness.

News Services and Press Releases One of the simplest ways of tracking press releases on deafness is to search the news wires. In the following sample of sources, we will briefly describe how to access each service. These services only post recent news intended for public viewing. PR Newswire To access the PR Newswire archive, simply go to http://www.prnewswire.com/. Select your country. Type “deafness” (or synonyms) into the search box. You will automatically receive information on relevant news releases posted within the last 30 days. The search results are shown by order of relevance. Reuters Health The Reuters’ Medical News and Health eLine databases can be very useful in exploring news archives relating to deafness. While some of the listed articles are free to view, others are available for purchase for a nominal fee. To access this archive, go to http://www.reutershealth.com/en/index.html and search by “deafness” (or synonyms). The following was recently listed in this archive for deafness: •

Chromosome linked to deafness, lymphomas sequenced Source: Reuters Health eLine Date: July 09, 2003

•

Gene found for blind, deaf syndrome in children Source: Reuters Health eLine Date: April 23, 2003

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•

Cochlear implants benefit kids with CMV-related deafness, despite other deficits Source: Reuters Medical News Date: September 24, 2002

•

Guidelines set on genetic tests for infant deafness Source: Reuters Health eLine Date: June 06, 2002

•

Cochlear implants effective for profound deafness from GJB2 gene mutation Source: Reuters Medical News Date: May 24, 2002

•

Hyperbaric oxygen shows promise as initial therapy for sudden deafness Source: Reuters Medical News Date: March 29, 2002

•

Cochlear implantation feasible in very young deaf children Source: Reuters Medical News Date: January 22, 2002

•

Delayed cochlear implantation can benefit congenitally deaf patients Source: Reuters Medical News Date: May 14, 2001

•

UK stem cell research gives hope to the chronically deaf Source: Reuters Industry Breifing Date: March 29, 2001

•

FDA clears Bionic Ear System for deafness Source: Reuters Medical News Date: March 22, 2001

•

FDA clears Advance Bionics Corporation's Bionic Ear System for deafness Source: Reuters Industry Breifing Date: March 22, 2001

•

Researchers discover gene that suppresses recessive deafness Source: Reuters Medical News Date: December 25, 2000

•

US considering standards of care for deaf Source: Reuters Health eLine Date: November 23, 2000

•

HCFA considering standards of care for deaf and hard of hearing Source: Reuters Medical News Date: November 17, 2000

•

FDA okays brainstem implant for deafness Source: Reuters Health eLine Date: October 24, 2000

•

Cochlear's brain-stem implant cleared to treat deafness in NF2 patients Source: Reuters Industry Breifing Date: October 23, 2000

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Deafness, labyrinthitis in AIDS patient may have been due to CMV encephalitis Source: Reuters Medical News Date: August 11, 2000

Periodicals and News

•

Cochlear implant aids word perception in deaf children Source: Reuters Health eLine Date: August 04, 2000

•

Cochlear implants help deaf children learn speech Source: Reuters Health eLine Date: March 14, 2000

•

Percentage of deafness associated with a genetic etiology increasing Source: Reuters Medical News Date: October 01, 1999

•

Gene helps predict risk for deafness in offspring Source: Reuters Health eLine Date: September 29, 1999

•

Early cochlear implants may help deaf children hear Source: Reuters Health eLine Date: September 09, 1999

•

Gene mutation linked to inherited deafness Source: Reuters Health eLine Date: June 24, 1999

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Much inherited severe deafness in US attributable to GJB2 mutations Source: Reuters Medical News Date: June 21, 1999

•

Gene common cause of inherited deafness Source: Reuters Health eLine Date: March 01, 1999

•

Auditory center converts to visual processor in brains of the deaf Source: Reuters Medical News Date: December 07, 1998

•

Variants of GJB2 account for most nonsyndromic deafness in Ashkenazi Jews Source: Reuters Medical News Date: November 19, 1998

•

Mutations in MYO15 gene appear to cause hereditary deafness Source: Reuters Medical News Date: May 29, 1998

•

More clues to hereditary deafness Source: Reuters Health eLine Date: May 28, 1998

•

Tectorial Membrane Defect Implicated In Some Cases Of Nonsyndromic Deafness Source: Reuters Medical News Date: April 29, 1998

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Link Between Craniosynostosis Mutation And Familial Deafness Syndrome Identified Source: Reuters Medical News Date: March 23, 1998

•

Gene Mutation Linked To Deafness Source: Reuters Health eLine Date: March 23, 1998

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Many Deaf Patients Can "Hear" Ultrasound Source: Reuters Medical News Date: February 19, 1998

•

New Director Appointed To National Institute on Deafness Source: Reuters Medical News Date: February 11, 1998

•

Gene Mutations Tied To Deafness Source: Reuters Health eLine Date: February 06, 1998

•

Connexin-26 Gene Implicated In Sporadic Congenital Deafness Source: Reuters Medical News Date: February 06, 1998

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Deafness Gene Discovered Source: Reuters Health eLine Date: December 01, 1997

•

Costa Rican Deafness Gene May Shed Light On Genetics Of Hearing Source: Reuters Medical News Date: November 17, 1997

•

Genetic Mutations That Cause Isolated Deafness Identified Source: Reuters Medical News Date: June 03, 1997

•

Mutation In Gap-Junction Protein Linked To Non-Syndromic Deafness Source: Reuters Medical News Date: May 01, 1997

•

Bird Ear Research May Help Human Deafness Source: Reuters Health eLine Date: October 08, 1996

•

Deafness And Heart Disease May Share A Common Genetic Component Source: Reuters Medical News Date: December 01, 1995 The NIH

Within MEDLINEplus, the NIH has made an agreement with the New York Times Syndicate, the AP News Service, and Reuters to deliver news that can be browsed by the public. Search news releases at http://www.nlm.nih.gov/medlineplus/alphanews_a.html. MEDLINEplus allows you to browse across an alphabetical index. Or you can search by date at the following Web page: http://www.nlm.nih.gov/medlineplus/newsbydate.html. Often, news items are indexed by MEDLINEplus within its search engine. Business Wire Business Wire is similar to PR Newswire. To access this archive, simply go to http://www.businesswire.com/. You can scan the news by industry category or company name.

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Market Wire Market Wire is more focused on technology than the other wires. To browse the latest press releases by topic, such as alternative medicine, biotechnology, fitness, healthcare, legal, nutrition, and pharmaceuticals, access Market Wire’s Medical/Health channel at http://www.marketwire.com/mw/release_index?channel=MedicalHealth. Or simply go to Market Wire’s home page at http://www.marketwire.com/mw/home, type “deafness” (or synonyms) into the search box, and click on “Search News.” As this service is technology oriented, you may wish to use it when searching for press releases covering diagnostic procedures or tests. Search Engines Medical news is also available in the news sections of commercial Internet search engines. See the health news page at Yahoo (http://dir.yahoo.com/Health/News_and_Media/), or you can use this Web site’s general news search page at http://news.yahoo.com/. Type in “deafness” (or synonyms). If you know the name of a company that is relevant to deafness, you can go to any stock trading Web site (such as http://www.etrade.com/) and search for the company name there. News items across various news sources are reported on indicated hyperlinks. Google offers a similar service at http://news.google.com/. BBC Covering news from a more European perspective, the British Broadcasting Corporation (BBC) allows the public free access to their news archive located at http://www.bbc.co.uk/. Search by “deafness” (or synonyms).

Newsletters on Deafness Find newsletters on deafness using the Combined Health Information Database (CHID). You will need to use the “Detailed Search” option. To access CHID, go to the following hyperlink: http://chid.nih.gov/detail/detail.html. Limit your search to “Newsletter” and “deafness.” Go to the bottom of the search page where “You may refine your search by.” Select the dates and language that you prefer. For the format option, select “Newsletter.” Type “deafness” (or synonyms) into the “For these words:” box. The following list was generated using the options described above: •

Advances in the Genetics of Deafness Source: Advances in the Genetics of Deafness: A Bulletin of the HHIRR. 1(1): 1-8. Spring 1994. Contact: Available from National Research Register for Hereditary Hearing Loss, Boys Town National Research Hospital (BTNRH). 555 North 30th Street, Omaha, NE 68131. (800) 320-1171. PRICE: $2.00 for each back issue. Summary: This document is the premier issue of a newsletter on the genetics of deafness, a bulletin of the Hereditary Hearing Impairment Resource Registry (HHIRR). The bulletin is designed to inform researchers in the genetics of the human auditory system about advances and opportunities for research in this area. The main article

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discusses the HHIRR, focusing on its purpose, information collection activities, data management concerns, and research collaboration activities. The newsletter also includes a literature review, with materials grouped under five major categories: reviews, clinical reports, genetic epidemiology, gene localization, and molecular genetics. Other segments include a calendar of related meetings; a questions, comments, and feedback section; and a call for readers to join the mailing list of the HHIRR. •

Hearing Advocate: The Deafness Research Foundation Source: New York, NY: Deafness Research Foundation (DRF). Spring 1998. 8 p. Contact: Available from Deafness Research Foundation (DRF). 15 West 39th Street, New York, NY 10018. Voice/TTY (800) 535-3323 or (212) 768-1181. Fax (212) 768-1782. E-mail: [email protected]. Website: village.ios.com/~drf1. Summary: This is a sample issue of the Hearing Advocate, the newsletter of the Deafness Research Foundation (DRF). The main article describes the DRF's 40th anniversary celebration and activities. The article reviews the history of the organization, the National Hearing Research Grant Center, the National Hearing Education and Advocacy Support Center, working with the Deaf community, and future research and granting activities. The newsletter also includes a section describing the DRF's campaign for hearing care among musicians and other musical artists, a letter from the new President and CEO, the address of the DRF website, the recipients chosen for the 1998 Otologic Research Grant Awards, and an article that profiles members of the otolaryngological professions. The newsletter concludes with a list of longterm donors, and suggestions for contributing to the DRF.

Newsletter Articles Use the Combined Health Information Database, and limit your search criteria to “newsletter articles.” Again, you will need to use the “Detailed Search” option. Go directly to the following hyperlink: http://chid.nih.gov/detail/detail.html. Go to the bottom of the search page where “You may refine your search by.” Select the dates and language that you prefer. For the format option, select “Newsletter Article.” Type “deafness” (or synonyms) into the “For these words:” box. You should check back periodically with this database as it is updated every three months. The following is a typical result when searching for newsletter articles on deafness: •

AIDS Hotline Signs on Deaf Consumers Source: Closing the Gap. p. 12-13. October-November 1999. Contact: Available from Office of Minority Health, Office of Public Health and Science, U.S. Department of Health and Human Services. Closing the Gap, OMH-RC, P.O. Box 37337, Washington, DC 20013-7337. (800) 444-6472. Website: www.omhrc.gov. Summary: Deafness or hearing loss comprise the second most common chronic health condition in the United States. More than 28 million Americans have some form of hearing loss, and 80 percent of those have irreversible and permanent hearing damage. Yet the deaf community is an underserved subculture that often relies on other deaf people for health information. This article reports on the AIDS hotline, established by the Centers for Disease Control and Prevention (CDC) to provide confidential and reliable information on HIV, AIDS, and other sexually transmitted diseases (STDs) to

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the deaf community. This article interviews Chad Ludwig, BSW, the senior supervisor of the Hotline TTY service. Topics include special health information needs of the deaf community, barriers to prevention and treatment information faced by deaf people, the most common vehicle for obtaining information in the deaf community, risk factors for HIV and other STDs in the deaf community, discrimination against deaf people, outreach activities, and how to access the Hotline. One sidebar summarizes the services offered through the CDC National AIDS Hotline TTY Service (800-243-7889). •

Theory of Mind: Language and Cognition in Deaf Children Source: The ASHA Leader. December 3, 2002. 7(22). p. 6-7+. Contact: American Speech-Language-Hearing Association. 10801 Rockville Pike, Rockville, MD 20852-3279. (301) 897-0157. TTY: (301) 897-0157. Fax: (301) 571-0457. Web site: http://professional.asha.org/news/. PRICE: Available free online at http://professional.asha.org/news/f021203.cfm. Summary: Theory of mind refers to the ability of a child to understand that people have different thoughts, wants, and beliefs, and a person's behavior can be predicted based on what he or she thinks or believes. Although children develop an understanding of theory of mind at roughly four years of age, research has shown that deaf children can experience significant delays in understanding the concept. This article discusses the concept of theory of mind in regard to deaf children, and reports the results of an NIDCD-funded study that investigates whether deaf children are equally delayed in their theory-of-mind skills for tasks that employ language as well as tasks that do not.

•

Implications of Delay in Detection and Management of Deafness Source: Educational Audiology Review. 15(4): 10-15. Fall 1998. Contact: Available from Educational Audiology Association. 4319 Ehrlich Road, Tampa, FL 33624. (800) 460-7322. Website: www.edaud.org. Summary: This article, first published in the early 1990s, reviews the implications of delay in the detection and management of deafness in children. The author notes that, although there may be controversies in the field of education of the hearing impaired, nearly everyone involved with children with hearing loss can agree on the important role of early detection and intervention, regardless of method used. The author reviews the typical progression of parental concern, general physician assessment of the child, dismissal of the concern, then finally referral of the child for intervention, often after the child's abnormal auditory and verbal development becomes more apparent. The end result of this sequence is that the average child with hearing loss still does not receive his or her first hearing aid until age two or older. The author then discusses the possible consequences of this delay, including the devaluation of hearing and attitudes toward hearing impairments in the U.S. Topics include self-acceptance, auditory sensory deprivation, auditory self-monitoring, the effects of delay in intervention on early linguistic development, and psychosocial implications. The author reiterates that children with hearing loss who are enrolled in early management programs demonstrate superior linguistic skills compared to those who are enrolled later.

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Academic Periodicals covering Deafness Numerous periodicals are currently indexed within the National Library of Medicine’s PubMed database that are known to publish articles relating to deafness. In addition to these sources, you can search for articles covering deafness that have been published by any of the periodicals listed in previous chapters. To find the latest studies published, go to http://www.ncbi.nlm.nih.gov/pubmed, type the name of the periodical into the search box, and click “Go.” If you want complete details about the historical contents of a journal, you can also visit the following Web site: http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi. Here, type in the name of the journal or its abbreviation, and you will receive an index of published articles. At http://locatorplus.gov/, you can retrieve more indexing information on medical periodicals (e.g. the name of the publisher). Select the button “Search LOCATORplus.” Then type in the name of the journal and select the advanced search option “Journal Title Search.”

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CHAPTER 10. RESEARCHING MEDICATIONS Overview While a number of hard copy or CD-ROM resources are available for researching medications, a more flexible method is to use Internet-based databases. Broadly speaking, there are two sources of information on approved medications: public sources and private sources. We will emphasize free-to-use public sources.

U.S. Pharmacopeia Because of historical investments by various organizations and the emergence of the Internet, it has become rather simple to learn about the medications recommended for deafness. One such source is the United States Pharmacopeia. In 1820, eleven physicians met in Washington, D.C. to establish the first compendium of standard drugs for the United States. They called this compendium the U.S. Pharmacopeia (USP). Today, the USP is a nonprofit organization consisting of 800 volunteer scientists, eleven elected officials, and 400 representatives of state associations and colleges of medicine and pharmacy. The USP is located in Rockville, Maryland, and its home page is located at http://www.usp.org/. The USP currently provides standards for over 3,700 medications. The resulting USP DI Advice for the Patient can be accessed through the National Library of Medicine of the National Institutes of Health. The database is partially derived from lists of federally approved medications in the Food and Drug Administration’s (FDA) Drug Approvals database, located at http://www.fda.gov/cder/da/da.htm. While the FDA database is rather large and difficult to navigate, the Phamacopeia is both user-friendly and free to use. It covers more than 9,000 prescription and over-the-counter medications. To access this database, simply type the following hyperlink into your Web browser: http://www.nlm.nih.gov/medlineplus/druginformation.html. To view examples of a given medication (brand names, category, description, preparation, proper use, precautions, side effects, etc.), simply follow the hyperlinks indicated within the United States Pharmacopeia (USP). Below, we have compiled a list of medications associated with deafness. If you would like more information on a particular medication, the provided hyperlinks will direct you to ample documentation (e.g. typical dosage, side effects, drug-interaction risks, etc.). The

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following drugs have been mentioned in the Pharmacopeia and other sources as being potentially applicable to deafness: http://www.nlm.nih.gov/medlineplus/druginfo/uspdi/202911.html •

Systemic - U.S. Brands: Tetramune http://www.nlm.nih.gov/medlineplus/druginfo/uspdi/202911.html

Commercial Databases In addition to the medications listed in the USP above, a number of commercial sites are available by subscription to physicians and their institutions. Or, you may be able to access these sources from your local medical library.

Mosby’s Drug Consult Mosby’s Drug Consult database (also available on CD-ROM and book format) covers 45,000 drug products including generics and international brands. It provides prescribing information, drug interactions, and patient information. Subscription information is available at the following hyperlink: http://www.mosbysdrugconsult.com/.

PDRhealth The PDRhealth database is a free-to-use, drug information search engine that has been written for the public in layman’s terms. It contains FDA-approved drug information adapted from the Physicians’ Desk Reference (PDR) database. PDRhealth can be searched by brand name, generic name, or indication. It features multiple drug interactions reports. Search PDRhealth at http://www.pdrhealth.com/drug_info/index.html. Other Web Sites Drugs.com (www.drugs.com) reproduces the information in the Pharmacopeia as well as commercial information. You may also want to consider the Web site of the Medical Letter, Inc. (http://www.medletter.com/) which allows users to download articles on various drugs and therapeutics for a nominal fee. If you have any questions about a medical treatment, the FDA may have an office near you. Look for their number in the blue pages of the phone book. You can also contact the FDA through its toll-free number, 1-888-INFO-FDA (1-888-463-6332), or on the World Wide Web at www.fda.gov.

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APPENDICES

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APPENDIX A. PHYSICIAN RESOURCES Overview In this chapter, we focus on databases and Internet-based guidelines and information resources created or written for a professional audience.

NIH Guidelines Commonly referred to as “clinical” or “professional” guidelines, the National Institutes of Health publish physician guidelines for the most common diseases. Publications are available at the following by relevant Institute13: •

Office of the Director (OD); guidelines consolidated across agencies available at http://www.nih.gov/health/consumer/conkey.htm

•

National Institute of General Medical Sciences (NIGMS); fact sheets available at http://www.nigms.nih.gov/news/facts/

•

National Library of Medicine (NLM); extensive encyclopedia (A.D.A.M., Inc.) with guidelines: http://www.nlm.nih.gov/medlineplus/healthtopics.html

•

National Cancer Institute (NCI); guidelines available at http://www.cancer.gov/cancerinfo/list.aspx?viewid=5f35036e-5497-4d86-8c2c714a9f7c8d25

•

National Eye Institute (NEI); guidelines available at http://www.nei.nih.gov/order/index.htm

•

National Heart, Lung, and Blood Institute (NHLBI); guidelines available at http://www.nhlbi.nih.gov/guidelines/index.htm

•

National Human Genome Research Institute (NHGRI); research available at http://www.genome.gov/page.cfm?pageID=10000375

•

National Institute on Aging (NIA); guidelines available at http://www.nia.nih.gov/health/

13

These publications are typically written by one or more of the various NIH Institutes.

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•

National Institute on Alcohol Abuse and Alcoholism (NIAAA); guidelines available at http://www.niaaa.nih.gov/publications/publications.htm

•

National Institute of Allergy and Infectious Diseases (NIAID); guidelines available at http://www.niaid.nih.gov/publications/

•

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS); fact sheets and guidelines available at http://www.niams.nih.gov/hi/index.htm

•

National Institute of Child Health and Human Development (NICHD); guidelines available at http://www.nichd.nih.gov/publications/pubskey.cfm

•

National Institute on Deafness and Other Communication Disorders (NIDCD); fact sheets and guidelines at http://www.nidcd.nih.gov/health/

•

National Institute of Dental and Craniofacial Research (NIDCR); guidelines available at http://www.nidr.nih.gov/health/

•

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK); guidelines available at http://www.niddk.nih.gov/health/health.htm

•

National Institute on Drug Abuse (NIDA); guidelines available at http://www.nida.nih.gov/DrugAbuse.html

•

National Institute of Environmental Health Sciences (NIEHS); environmental health information available at http://www.niehs.nih.gov/external/facts.htm

•

National Institute of Mental Health (NIMH); guidelines available at http://www.nimh.nih.gov/practitioners/index.cfm

•

National Institute of Neurological Disorders and Stroke (NINDS); neurological disorder information pages available at http://www.ninds.nih.gov/health_and_medical/disorder_index.htm

•

National Institute of Nursing Research (NINR); publications on selected illnesses at http://www.nih.gov/ninr/news-info/publications.html

•

National Institute of Biomedical Imaging and Bioengineering; general information at http://grants.nih.gov/grants/becon/becon_info.htm

•

Center for Information Technology (CIT); referrals to other agencies based on keyword searches available at http://kb.nih.gov/www_query_main.asp

•

National Center for Complementary and Alternative Medicine (NCCAM); health information available at http://nccam.nih.gov/health/

•

National Center for Research Resources (NCRR); various information directories available at http://www.ncrr.nih.gov/publications.asp

•

Office of Rare Diseases; various fact sheets available at http://rarediseases.info.nih.gov/html/resources/rep_pubs.html

•

Centers for Disease Control and Prevention; various fact sheets on infectious diseases available at http://www.cdc.gov/publications.htm

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NIH Databases In addition to the various Institutes of Health that publish professional guidelines, the NIH has designed a number of databases for professionals.14 Physician-oriented resources provide a wide variety of information related to the biomedical and health sciences, both past and present. The format of these resources varies. Searchable databases, bibliographic citations, full-text articles (when available), archival collections, and images are all available. The following are referenced by the National Library of Medicine:15 •

Bioethics: Access to published literature on the ethical, legal, and public policy issues surrounding healthcare and biomedical research. This information is provided in conjunction with the Kennedy Institute of Ethics located at Georgetown University, Washington, D.C.: http://www.nlm.nih.gov/databases/databases_bioethics.html

•

HIV/AIDS Resources: Describes various links and databases dedicated to HIV/AIDS research: http://www.nlm.nih.gov/pubs/factsheets/aidsinfs.html

•

NLM Online Exhibitions: Describes “Exhibitions in the History of Medicine”: http://www.nlm.nih.gov/exhibition/exhibition.html. Additional resources for historical scholarship in medicine: http://www.nlm.nih.gov/hmd/hmd.html

•

Biotechnology Information: Access to public databases. The National Center for Biotechnology Information conducts research in computational biology, develops software tools for analyzing genome data, and disseminates biomedical information for the better understanding of molecular processes affecting human health and disease: http://www.ncbi.nlm.nih.gov/

•

Population Information: The National Library of Medicine provides access to worldwide coverage of population, family planning, and related health issues, including family planning technology and programs, fertility, and population law and policy: http://www.nlm.nih.gov/databases/databases_population.html

•

Cancer Information: Access to cancer-oriented databases: http://www.nlm.nih.gov/databases/databases_cancer.html

•

Profiles in Science: Offering the archival collections of prominent twentieth-century biomedical scientists to the public through modern digital technology: http://www.profiles.nlm.nih.gov/

•

Chemical Information: Provides links to various chemical databases and references: http://sis.nlm.nih.gov/Chem/ChemMain.html

•

Clinical Alerts: Reports the release of findings from the NIH-funded clinical trials where such release could significantly affect morbidity and mortality: http://www.nlm.nih.gov/databases/alerts/clinical_alerts.html

•

Space Life Sciences: Provides links and information to space-based research (including NASA): http://www.nlm.nih.gov/databases/databases_space.html

•

MEDLINE: Bibliographic database covering the fields of medicine, nursing, dentistry, veterinary medicine, the healthcare system, and the pre-clinical sciences: http://www.nlm.nih.gov/databases/databases_medline.html

14

Remember, for the general public, the National Library of Medicine recommends the databases referenced in MEDLINEplus (http://medlineplus.gov/ or http://www.nlm.nih.gov/medlineplus/databases.html). 15 See http://www.nlm.nih.gov/databases/databases.html.

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•

Toxicology and Environmental Health Information (TOXNET): Databases covering toxicology and environmental health: http://sis.nlm.nih.gov/Tox/ToxMain.html

•

Visible Human Interface: Anatomically detailed, three-dimensional representations of normal male and female human bodies: http://www.nlm.nih.gov/research/visible/visible_human.html The Combined Health Information Database

A comprehensive source of information on clinical guidelines written for professionals is the Combined Health Information Database. You will need to limit your search to one of the following: Brochure/Pamphlet, Fact Sheet, or Information Package, and “deafness” using the “Detailed Search” option. Go directly to the following hyperlink: http://chid.nih.gov/detail/detail.html. To find associations, use the drop boxes at the bottom of the search page where “You may refine your search by.” For the publication date, select “All Years.” Select your preferred language and the format option “Fact Sheet.” Type “deafness” (or synonyms) into the “For these words:” box. The following is a sample result: •

Report on Notetaking for Deaf and Hard of Hearing Students Source: Rochester, NY: Northeast Technical Assistance Center, National Technical Institute for the Deaf, Rochester Institute of Technology. 1997. 17 p. Contact: Available from PEPNet Resource Center. National Center on Deafness, California State University, Northridge, 18111 Nordhoff Street, Northridge, CA 913308267. Voice/TTY (888) 684-4695 or (818) 677-2611. Fax (818) 677-4899. Website: www.pepnet.org. PRICE: Single copy free. Summary: The provision of notetaking services is crucial for most deaf and hard of hearing students at the postsecondary level in classes designed for students with normal hearing. This report on notetaking is one in a series intended to assist postsecondary institutions in developing and maintaining special services of quality as needed by this population. Each of the reports emphasizes the need to remember that deaf and hearing impaired persons as individuals first, and their needs and wishes for special services and other accommodations will vary, as it does in any other population. This report is intended to clarify needs for notetaking services and ways of providing these services. The authors discuss training, supervision, and the responsibilities of those involved in the provision of notetaking, along with suggested policies and procedures for assuring a useful and efficient service to students. Topics include listening and the process of good notetaking, general suggestions for the individual taking notes, what to look for in a good notetaker, the notetaker Code of Ethics, policies regarding the notetaking process (including class assignments, attendance, punctuality, attire, supervision, sharing of notes, evaluations, photocopies versus NCR paper, payroll and pay scale, recruitment, selection, and student grievance processes). Topics also include training notetakers, the role of the notetaker coordinator, paid versus volunteer notetaking, student responsibilities, faculty responsibilities, applications of technology in notetaking (including computer assisted realtime captioning or CART), and relevant laws and regulations. One appendix reprints the Notetaker's Code of Ethics; another offers sample rating and evaluation forms. 13 references.

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AIDS/HIV: Information Sources for People Who Are Deaf or Have Hearing Impairments Contact: National Institute on Deafness and Other Communication Disorders, Information Clearinghouse, 1 Communication Ave, Bethesda, MD, 20892-3456, (800) 241-1044. Summary: This bibliography includes the results of a literature search from the Deafness and Communication Disorders subfile of the Combined Health Information Database. Each record contains the document title, information on publisher and source of the document, availability, and a brief abstract.

•

AIDS/HIV : Information Sources for People Who Are Deaf or Have Hearing Impairments : A Bibliographic Search From the Combined Health Information Database (CHID) Contact: National Institutes of Health, National Institute on Deafness and Other Communication Disorders, 1 Communication Ave, Bethesda, MD, 20892-3456, (800) 2411044, http://www.nih.gov/nidcd. Summary: This bibliography provides a listing of health information resources about the human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) specifically for hearing impaired persons. The materials included in this bibliography address HIV/AIDS among adolescents and the legal issues concerning deaf persons. It provides citations for sources that present the mission and services of the Centers for Disease Control and Prevention's (CDC) National AIDS Clearinghouse (NAC), now known as the CDC National Prevention Information Network (NPIN). The bibliography supplies summaries of materials on safer sex and educational strategies for teaching deaf persons about HIV/AIDS.

•

Journey Through Late Deafness: Results of a Focus Group Study Source: San Diego, CA: California School of Professional Psychology. 1997. 21 p. Contact: Available from California School of Professional Psychology Rehabilitation Research and Training Center on Mental Health for Persons Who are Hard of Hearing or Late Deafened. 6160 Cornerstone Court East, San Diego, CA 92121. E-mail: [email protected]. PRICE: Single copy free. Summary: This document reports on the results of a series of focus groups with late deafened adults and their family members. Questions posed during these groups attempted to get to the heart of significant issues related to hearing loss that strikes in adulthood. Issues include personal adjustment, employment barriers, communication issues, family dynamics, and social factors. This report outlines the process of the focus group study, provides readers with the significant findings of that process, gives recommendations and suggestions regarding future research, and describes a model service delivery system developed with consumer input. The number one concern reiterated by both individuals who were late deafened and family members was communication difficulty: at home, in the workplace, and with friends and other family members. Other concerns of people the focus groups included: dealing with depression and withdrawal, learning coping skills, finding information about deafness, staying positive, socializing, issues of independence, and intimacy. The report concludes with a recommendation for a proposed Individual and Family Life Center for Late Deafened adults. The center would be research-based and would serve a regional area. The initial center would conduct research and provide training in the area of late deafness, and

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would support the development of a regional system of Individual and Family Life Centers. 5 appendices. 9 figures. •

Southwest Regional Conference on AIDS and Deafness. AIDS and Deafness: A Community's Challenge. Dallas, TX, February 17-19, 1989 Contact: Dallas County Deaf AIDS Task Force, 800 N Lancaster, Dallas, TX, 75203, (214) 941-0523. Summary: This program for the Southwest Regional Conference on Acquired immunodeficiency syndrome (AIDS) and Deafness held on February 17-19, 1989 in Dallas lists sessions for people working with the hearing impaired. Presentations review AIDS and Human immunodeficiency virus (HIV) transmission, HIV prevention, and testing and counseling. Health education, spiritual and religious support, and civil rights protection are also on the agenda. Included in the program are several pages of AIDS-related terminology.

•

Report on Tutoring Deaf and Hard of Hearing Students Source: Rochester, NY: Northeast Technical Assistance Center, National Technical Institute for the Deaf, Rochester Institute of Technology. 1997. 15 p. Contact: Available from PEPNet Resource Center. National Center on Deafness, California State University, Northridge, 18111 Nordhoff Street, Northridge, CA 913308267. Voice/TTY (888) 684-4695 or (818) 677-2611. Fax (818) 677-4899. Website: www.pepnet.org. PRICE: Single copy free. Summary: This report on tutoring deaf and hard of hearing students is one in a series intended to assist postsecondary institutions in developing and maintaining special services of quality as needed by this population. Each of the reports emphasizes the need to remember that deaf and hearing impaired persons as individuals first, and their needs and wishes for special services and other accommodations will vary, as it does in any other population. As discussed in this report, tutoring generally refers to the traditional practice of meeting periodically or as the need arises to help a given student acquire or strengthen a particular knowledge or skill. Tutoring ranks with interpreting and notetaking as one in a triad of fundamental academic support services for deaf and hard of hearing students at the postsecondary level, particularly in mainstream settings. The report addresses the indications for tutoring in this population, and discusses the provision of tutoring services. Other topics covered include how to identify students in need of tutoring, how to make students aware of tutoring services, the selection and training of tutors, the expectations of participants from tutoring sessions, quality in tutoring deaf students, tutoring for basic skills and general courses, tutoring support for written assignments, and tutoring formats. Other considerations in providing tutoring services include location, environment, resources, rewarding tutors, and ethical standards. The report concludes with a brief discussion of evaluating the success of a tutoring program, including the use of models and standards. One appendix includes a sample tutoring agreement form and an evaluation form. 1 table. 12 references.

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The NLM Gateway16 The NLM (National Library of Medicine) Gateway is a Web-based system that lets users search simultaneously in multiple retrieval systems at the U.S. National Library of Medicine (NLM). It allows users of NLM services to initiate searches from one Web interface, providing one-stop searching for many of NLM’s information resources or databases.17 To use the NLM Gateway, simply go to the search site at http://gateway.nlm.nih.gov/gw/Cmd. Type “deafness” (or synonyms) into the search box and click “Search.” The results will be presented in a tabular form, indicating the number of references in each database category. Results Summary Category Journal Articles Books / Periodicals / Audio Visual Consumer Health Meeting Abstracts Other Collections Total

Items Found 36039 1363 739 50 7 38198

HSTAT18 HSTAT is a free, Web-based resource that provides access to full-text documents used in healthcare decision-making.19 These documents include clinical practice guidelines, quickreference guides for clinicians, consumer health brochures, evidence reports and technology assessments from the Agency for Healthcare Research and Quality (AHRQ), as well as AHRQ’s Put Prevention Into Practice.20 Simply search by “deafness” (or synonyms) at the following Web site: http://text.nlm.nih.gov.

Coffee Break: Tutorials for Biologists21 Coffee Break is a general healthcare site that takes a scientific view of the news and covers recent breakthroughs in biology that may one day assist physicians in developing treatments. Here you will find a collection of short reports on recent biological discoveries. Each report incorporates interactive tutorials that demonstrate how bioinformatics tools are used as a part of the research process. Currently, all Coffee Breaks are written by NCBI 16

Adapted from NLM: http://gateway.nlm.nih.gov/gw/Cmd?Overview.x.

17

The NLM Gateway is currently being developed by the Lister Hill National Center for Biomedical Communications (LHNCBC) at the National Library of Medicine (NLM) of the National Institutes of Health (NIH). 18 Adapted from HSTAT: http://www.nlm.nih.gov/pubs/factsheets/hstat.html. 19 20

The HSTAT URL is http://hstat.nlm.nih.gov/.

Other important documents in HSTAT include: the National Institutes of Health (NIH) Consensus Conference Reports and Technology Assessment Reports; the HIV/AIDS Treatment Information Service (ATIS) resource documents; the Substance Abuse and Mental Health Services Administration's Center for Substance Abuse Treatment (SAMHSA/CSAT) Treatment Improvement Protocols (TIP) and Center for Substance Abuse Prevention (SAMHSA/CSAP) Prevention Enhancement Protocols System (PEPS); the Public Health Service (PHS) Preventive Services Task Force's Guide to Clinical Preventive Services; the independent, nonfederal Task Force on Community Services’ Guide to Community Preventive Services; and the Health Technology Advisory Committee (HTAC) of the Minnesota Health Care Commission (MHCC) health technology evaluations. 21 Adapted from http://www.ncbi.nlm.nih.gov/Coffeebreak/Archive/FAQ.html.

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staff.22 Each report is about 400 words and is usually based on a discovery reported in one or more articles from recently published, peer-reviewed literature.23 This site has new articles every few weeks, so it can be considered an online magazine of sorts. It is intended for general background information. You can access the Coffee Break Web site at the following hyperlink: http://www.ncbi.nlm.nih.gov/Coffeebreak/.

Other Commercial Databases In addition to resources maintained by official agencies, other databases exist that are commercial ventures addressing medical professionals. Here are some examples that may interest you: •

CliniWeb International: Index and table of contents to selected clinical information on the Internet; see http://www.ohsu.edu/cliniweb/.

•

Medical World Search: Searches full text from thousands of selected medical sites on the Internet; see http://www.mwsearch.com/.

The Genome Project and Deafness In the following section, we will discuss databases and references which relate to the Genome Project and deafness. Online Mendelian Inheritance in Man (OMIM) The Online Mendelian Inheritance in Man (OMIM) database is a catalog of human genes and genetic disorders authored and edited by Dr. Victor A. McKusick and his colleagues at Johns Hopkins and elsewhere. OMIM was developed for the World Wide Web by the National Center for Biotechnology Information (NCBI).24 The database contains textual information, pictures, and reference information. It also contains copious links to NCBI’s Entrez database of MEDLINE articles and sequence information. To search the database, go to http://www.ncbi.nlm.nih.gov/Omim/searchomim.html. Type “deafness” (or synonyms) into the search box, and click “Submit Search.” If too many results appear, you can narrow the search by adding the word “clinical.” Each report will have additional links to related research and databases. In particular, the option “Database Links” will search across technical databases that offer an abundance of information. The following is an example of the results you can obtain from the OMIM for deafness:

22

The figure that accompanies each article is frequently supplied by an expert external to NCBI, in which case the source of the figure is cited. The result is an interactive tutorial that tells a biological story. 23 After a brief introduction that sets the work described into a broader context, the report focuses on how a molecular understanding can provide explanations of observed biology and lead to therapies for diseases. Each vignette is accompanied by a figure and hypertext links that lead to a series of pages that interactively show how NCBI tools and resources are used in the research process. 24 Adapted from http://www.ncbi.nlm.nih.gov/. Established in 1988 as a national resource for molecular biology information, NCBI creates public databases, conducts research in computational biology, develops software tools for analyzing genome data, and disseminates biomedical information--all for the better understanding of molecular processes affecting human health and disease.

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Albinism, Ocular, with Late-onset Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?300650

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Albinism, Ocular, with Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?103470

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Albinism-deafness Syndrome Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?300700

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Arthrogryposis-like Hand Anomaly and Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?108200

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Ataxia, Deafness, and Cardiomyopathy Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?208750

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Ataxia, Fatal X-linked, with Deafness and Loss of Vision Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?301835

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Ataxia-deafness Syndrome, X-linked Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?301790

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Ataxia-deafness-retardation Syndrome Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?208850

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Atherosclerosis, Premature, with Deafness, Nephropathy, Diabetes Mellitus, Photomyoclonus, and Degenerative Neurologic Disease Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?209010

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Atresia of External Auditory Canal and Conduction Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?108760

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Bartter Syndrome, Infantile, with Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?602522

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Brachycephaly, Deafness, Cataract, Microstomia, and Mental Retardation Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601353

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Brachydactyly, Intraventricular Septal Defect, and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?602561

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Bulbar Palsy, Progressive, with Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?211530

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Cataract-ataxia-deafness-retardation Syndrome Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?212710

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Cataracts, Congenital, with Sensorineural Deafness, Down Syndrome-like Facial Appearance, Short Stature, and Mental Retardation Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601088

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Cerebellar Ataxia and Neurosensory Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?212850

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Cerebellar Ataxia, Deafness, and Narcolepsy Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?604121

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Charcot-marie-tooth Disease and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?118300

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Choroideremia with Deafness and Obesity Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?303110

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Cleft Palate, Deafness, and Oligodontia Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?216300

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Corneal Degeneration, Ribbonlike, with Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?121450

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Corneal Dystrophy and Perceptive Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?217400

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Corneal Hypesthesia with Retinal Abnormalities, Sensorineural Deafness, Unusual Facies, Persistent Ductus Arteriosus, and Mental Retardation Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?122430

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Craniofacial-deafness-hand Syndrome Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?122880

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Cutis Verticis Gyrata, Retinitis Pigmentosa, and Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?605685

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Deafness and Onychodystrophy, Dominant Form Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?124480

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Deafness Locus-associated Putative Guanine Nucleotide Exchange Factor Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?606051

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Deafness with Anhidrotic Ectodermal Dysplasia Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?125050

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Deafness with Ear Pits Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?125100

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Deafness, Autosomal Dominant 16 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?603964

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Deafness, Autosomal Dominant 20 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?604717

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Deafness, Autosomal Dominant 39, with Dentinogenesis Imperfecta 1 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?605594

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Deafness, Autosomal Dominant 52 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607683

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 1 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?124900

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 10 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601316

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 11 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601317

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 12 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601842

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 13 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601868

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 15 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?602459

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 17 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?603622

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 18 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?606012

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 2 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600101

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 21 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607017

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 22 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?606346

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 23 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?605192

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 24 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?606282

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 25 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?605583

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 3 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601544

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 30 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?606451

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 36 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?606705

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 4 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600652

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 41 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?608224

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 44 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607453

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 5 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600994

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 6 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600965

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 7 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601412

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 8 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601543

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural 9 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601369

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Deafness, Autosomal Dominant Nonsyndromic Sensorineural, due to Mutation in Myo1a Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607841

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Deafness, Autosomal Recessive Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?220700

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Deafness, Autosomal Recessive Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607197

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Deafness, Autosomal Recessive 12 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601386

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Deafness, Autosomal Recessive 13 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?603098

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Deafness, Autosomal Recessive 14 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?603678

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Deafness, Autosomal Recessive 15 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601869

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Deafness, Autosomal Recessive 16 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?603720

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Deafness, Autosomal Recessive 20 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?604060

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Deafness, Autosomal Recessive 21 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?603629

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Deafness, Autosomal Recessive 22 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607039

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Deafness, Autosomal Recessive 26 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?605428

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Deafness, Autosomal Recessive 27 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?605818

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Deafness, Autosomal Recessive 30 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607101

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Deafness, Autosomal Recessive 31 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607084

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Deafness, Autosomal Recessive 33 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607239

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Deafness, Autosomal Recessive 9 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601071

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Deafness, Childhood-onset Neurosensory, Autosomal Recessive 8 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601072

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Deafness, Cochlear, with Myopia and Intellectual Impairment Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221200

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Deafness, Conductive Stapedial, with Ear Malformation and Facial Palsy Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?124490

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Deafness, Conductive, with Malformed External Ear Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221300

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Deafness, Conductive, with Ptosis and Skeletal Anomalies Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221320

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Deafness, Conductive, with Stapes Fixation Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?304400

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Deafness, Congenital Neurosensory, Autosomal Recessive 10 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?605316

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Deafness, Congenital Neurosensory, Autosomal Recessive 37 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607821

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Deafness, Congenital Neurosensory, Autosomal Recessive 38 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?608219

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Deafness, Congenital Neurosensory, Autosomal Recessive 39 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?608265

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Deafness, Congenital Neurosensory, Autosomal Recessive 40 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?608264

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Deafness, Congenital, and Familial Myoclonic Epilepsy Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?220300

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Deafness, Congenital, and Onychodystrophy, Recessive Form Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?220500

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Deafness, Congenital, and Split Hands and Feet Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?220600

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Deafness, Congenital, with Keratopachydermia and Constrictions of Fingers and Toes Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?124500

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Deafness, Congenital, with Total Albinism Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?220900

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Deafness, Congenital, with Vitiligo and Achalasia Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221350

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Deafness, High-frequency Sensorineural, X-linked Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?304590

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Deafness, Low-frequency Hearing Loss, Mixed Conductive-sensorineural Type Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?124910

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Deafness, Mid-tone Neural Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?124700

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Deafness, Nerve Type, with Mesenteric Diverticula of Small Bowel and Progressive Sensory Neuropathy Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221400

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Deafness, Neural, Congenital Moderate Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221500

•

Deafness, Neural, Early Onset Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221600

•

Deafness, Neural, Progressive Childhood Type Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221650

•

Deafness, Neural, with Atypical Atopic Dermatitis Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221700

•

Deafness, Neurosensory, Autosomal Recessive 1 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?220290

•

Deafness, Neurosensory, Autosomal Recessive 17 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?603010

•

Deafness, Neurosensory, Autosomal Recessive 18 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?602092

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Deafness, Neurosensory, Autosomal Recessive 2 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600060

•

Deafness, Neurosensory, Autosomal Recessive 3 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600316

•

Deafness, Neurosensory, Autosomal Recessive 4 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600791

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Deafness, Neurosensory, Autosomal Recessive 5 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600792

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Deafness, Neurosensory, Autosomal Recessive 6 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600971

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Deafness, Neurosensory, Autosomal Recessive 7 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600974

•

Deafness, Nonsyndromic, Modifier 1 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?605429

•

Deafness, Progressive High-tone Neural Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?124800

•

Deafness, Progressive, with Stapes Fixation Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601449

•

Deafness, Sensorineural, with Peripheral Neuropathy and Arterial Disease Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?124950

•

Deafness, Sensorineural, with Pituitary Dwarfism Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221750

•

Deafness, Unilateral Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?125000

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Deafness, X-linked 2, Sensorineural Congenital Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?304500

•

Deafness, X-linked 4, Congenital Sensorineural Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?300030

•

Deafness, X-linked 6, Progressive Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?300066

•

Deafness-craniofacial Syndrome Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?125230

•

Deafness-hypogonadism Syndrome Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?304350

•

Deafness-oligodontia Syndrome Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221740

•

Deafness--optic Atrophy Syndrome Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?125250

•

Diabetes-deafness Syndrome, Maternally Transmitted Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?520000

•

Dislocated Elbows, Bowed Tibias, Scoliosis, Deafness, Cataract, Microcephaly, and Mental Retardation Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?603133

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Earlobes, Thickened, with Conductive Deafness from Incudostapedial Abnormalities Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?128980

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Ectodermal Dysplasia and Neurosensory Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?224800

•

Emphysema, Congenital, with Deafness, Penoscrotal Web, and Mental Retardation Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?602564

•

Epiphyseal Dysplasia of Femoral Head, Myopia, and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?226950

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Epiphyseal Dysplasia, Multiple, with Myopia and Conductive Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?132450

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Fibromatosis, Gingival, with Progressive Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?135550

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Friedreich Ataxia, So-called, with Optic Atrophy and Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?136600

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Gonadal Dysgenesis, Xx Type, with Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?233400

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Growth Retardation, Deafness, Femoral Epiphyseal Dysplasia, and Lacrimal Duct Obstruction Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601351

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Hirschsprung Disease with Polydactyly, Renal Agenesis, and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?235740

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Histiocytosis with Joint Contractures and Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?602782

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Hyperlipoproteinemia, Type Ii, and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?144300

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Hypoparathyroidism, Sensorineural Deafness, and Renal Dysplasia Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?146255

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Ichthyosiform Erythroderma, Corneal Involvement, and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?242150

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Ichthyosis, Hystrix-like, with Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?602540

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Keratitis-ichthyosis-deafness Syndrome, Autosomal Dominant Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?148210

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Keratoderma, Palmoplantar, with Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?148350

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Klippel-feil Deformity, Conductive Deafness, and Absent Vagina Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?148860

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Klippel-feil Deformity, Deafness, and Facial Asymmetry Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?148870

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Knuckle Pads, Leukonychia, and Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?149200

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Lipodystrophy, Generalized, with Mental Retardation, Deafness, Short Stature, and Slender Bones Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?608154

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Macrothrombocytopenia and Progressive Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?600208

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Mental Retardation with Optic Atrophy, Deafness, and Seizures Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?309555

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Mental Retardation, X-linked, with Growth Retardation, Deafness, Microgenitalism Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?309590

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Metaphyseal Dysostosis, Mental Retardation, and Conductive Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?250420

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Microcephaly-deafness Syndrome Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?156620

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Microtia with Meatal Atresia and Conductive Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?251800

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Mitochondrial Deafness Modifier Gene 1 Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?221745

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Mitral Regurgitation, Conductive Deafness, and Fusion of Cervical Vertebrae and of Carpal and Tarsal Bones Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?157800

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Myoclonic Epilepsy, Congenital Deafness, Macular Dystrophy, and Psychiatric Disorders Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?604363

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Myoclonus, Cerebellar Ataxia, and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?159800

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Nephropathy, Deafness, and Hyperparathyroidism Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?256120

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Nephrosis with Deafness and Urinary Tract and Digital Malformations Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?256200

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Neuropathy, Axonal Motor-sensory, with Deafness and Mental Retardation Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?310490

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Neuropathy, Hereditary Motor and Sensory, with Deafness, Mental Retardation, and Absent Sensory Large Myelinated Fibers Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?214370

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Optic Atrophy, Deafness, Ophthalmoplegia, and Myopathy Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?165490

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Optic Atrophy, Nerve Deafness, and Distal Neurogenic Amyotrophy Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?258650

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Optic Atrophy, Polyneuropathy, and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?311070

•

Phocomelia-ectrodactyly, Ear Malformation, Deafness, and Sinus Arrhythmia Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?171480

and

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Photomyoclonus, Diabetes Mellitus, Deafness, Nephropathy, and Cerebral Dysfunction Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?172500

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Pili Torti and Nerve Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?262000

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Prune Belly Syndrome with Pulmonic Stenosis, Mental Retardation, and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?264140

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Pulmonic Stenosis and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?178651

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Renal Tubular Acidosis with Progressive Nerve Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?267300

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Retinitis Pigmentosa Inversa with Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?268010

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Retinitis Pigmentosa, Deafness, Mental Retardation, and Hypogonadism Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?268020

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Retinitis Pigmentosa-deafness Syndrome 1, Autosomal Dominant Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?601850

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Rod-cone Dystrophy, Sensorineural Deafness, and Fanconi-type Renal Dysfunction Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?268315

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Secretory Diarrhea, Myopathy, and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?607540

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Spastic Paraparesis and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?312910

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Spastic Paraplegia, Sensorineural Deafness, Mental Retardation, and Progressive Nephropathy Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?182690

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Spinocerebellar Ataxia with Blindness and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?271250

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Spondyloepiphyseal Dysplasia, Myopia, and Sensorineural Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?184000

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Thumb, Hypoplastic, with Choroid Coloboma, Poorly Developed Antihelix, and Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?274205

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Tibia, Absence Of, with Congenital Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?275230

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Tune Deafness Web site: http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?191200 Genes and Disease (NCBI - Map)

The Genes and Disease database is produced by the National Center for Biotechnology Information of the National Library of Medicine at the National Institutes of Health. This Web site categorizes each disorder by system of the body. Go to http://www.ncbi.nlm.nih.gov/disease/, and browse the system pages to have a full view of

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important conditions linked to human genes. Since this site is regularly updated, you may wish to revisit it from time to time. The following systems and associated disorders are addressed: •

Cancer: Uncontrolled cell division. Examples: Breast and ovarian cancer, Burkitt lymphoma, chronic myeloid leukemia, colon cancer, lung cancer, malignant melanoma, multiple endocrine neoplasia, neurofibromatosis, p53 tumor suppressor, pancreatic cancer, prostate cancer, Ras oncogene, RB: retinoblastoma, von Hippel-Lindau syndrome. Web site: http://www.ncbi.nlm.nih.gov/disease/Cancer.html

•

Immune System: Fights invaders. Examples: Asthma, autoimmune polyglandular syndrome, Crohn’s disease, DiGeorge syndrome, familial Mediterranean fever, immunodeficiency with Hyper-IgM, severe combined immunodeficiency. Web site: http://www.ncbi.nlm.nih.gov/disease/Immune.html

•

Metabolism: Food and energy. Examples: Adreno-leukodystrophy, atherosclerosis, Best disease, Gaucher disease, glucose galactose malabsorption, gyrate atrophy, juvenile-onset diabetes, obesity, paroxysmal nocturnal hemoglobinuria, phenylketonuria, Refsum disease, Tangier disease, Tay-Sachs disease. Web site: http://www.ncbi.nlm.nih.gov/disease/Metabolism.html

•

Muscle and Bone: Movement and growth. Examples: Duchenne muscular dystrophy, Ellis-van Creveld syndrome, Marfan syndrome, myotonic dystrophy, spinal muscular atrophy. Web site: http://www.ncbi.nlm.nih.gov/disease/Muscle.html

•

Nervous System: Mind and body. Examples: Alzheimer disease, amyotrophic lateral sclerosis, Angelman syndrome, Charcot-Marie-Tooth disease, epilepsy, essential tremor, fragile X syndrome, Friedreich’s ataxia, Huntington disease, Niemann-Pick disease, Parkinson disease, Prader-Willi syndrome, Rett syndrome, spinocerebellar atrophy, Williams syndrome. Web site: http://www.ncbi.nlm.nih.gov/disease/Brain.html

•

Signals: Cellular messages. Examples: Ataxia telangiectasia, Cockayne syndrome, glaucoma, male-patterned baldness, SRY: sex determination, tuberous sclerosis, Waardenburg syndrome, Werner syndrome. Web site: http://www.ncbi.nlm.nih.gov/disease/Signals.html

•

Transporters: Pumps and channels. Examples: Cystic fibrosis, deafness, diastrophic dysplasia, Hemophilia A, long-QT syndrome, Menkes syndrome, Pendred syndrome, polycystic kidney disease, sickle cell anemia, Wilson’s disease, Zellweger syndrome. Web site: http://www.ncbi.nlm.nih.gov/disease/Transporters.html Entrez

Entrez is a search and retrieval system that integrates several linked databases at the National Center for Biotechnology Information (NCBI). These databases include nucleotide sequences, protein sequences, macromolecular structures, whole genomes, and MEDLINE through PubMed. Entrez provides access to the following databases:

Physician Resources

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3D Domains: Domains from Entrez Structure, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=geo

•

Books: Online books, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=books

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Genome: Complete genome assemblies, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Genome

•

NCBI’s Protein Sequence Information Survey Results: Web site: http://www.ncbi.nlm.nih.gov/About/proteinsurvey/

•

Nucleotide Sequence Database (Genbank): Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Nucleotide

•

OMIM: Online Mendelian Inheritance in Man, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=OMIM

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PopSet: Population study data sets, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Popset

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ProbeSet: Gene Expression Omnibus (GEO), Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=geo

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Protein Sequence Database: Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Protein

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PubMed: Biomedical literature (PubMed), Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed

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Structure: Three-dimensional macromolecular structures, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Structure

•

Taxonomy: Organisms in GenBank, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Taxonomy

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To access the Entrez system at the National Center for Biotechnology Information, go to http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&DB=genome, and then select the database that you would like to search. The databases available are listed in the drop box next to “Search.” Enter “deafness” (or synonyms) into the search box and click “Go.” Jablonski’s Multiple Congenital Anomaly/Mental Retardation (MCA/MR) Syndromes Database25 This online resource has been developed to facilitate the identification and differentiation of syndromic entities. Special attention is given to the type of information that is usually limited or completely omitted in existing reference sources due to space limitations of the printed form. At http://www.nlm.nih.gov/mesh/jablonski/syndrome_toc/toc_a.html, you can search across syndromes using an alphabetical index. Search by keywords at http://www.nlm.nih.gov/mesh/jablonski/syndrome_db.html. 25

Adapted from the National Library of Medicine: http://www.nlm.nih.gov/mesh/jablonski/about_syndrome.html.

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The Genome Database26 Established at Johns Hopkins University in Baltimore, Maryland in 1990, the Genome Database (GDB) is the official central repository for genomic mapping data resulting from the Human Genome Initiative. In the spring of 1999, the Bioinformatics Supercomputing Centre (BiSC) at the Hospital for Sick Children in Toronto, Ontario assumed the management of GDB. The Human Genome Initiative is a worldwide research effort focusing on structural analysis of human DNA to determine the location and sequence of the estimated 100,000 human genes. In support of this project, GDB stores and curates data generated by researchers worldwide who are engaged in the mapping effort of the Human Genome Project (HGP). GDB’s mission is to provide scientists with an encyclopedia of the human genome which is continually revised and updated to reflect the current state of scientific knowledge. Although GDB has historically focused on gene mapping, its focus will broaden as the Genome Project moves from mapping to sequence, and finally, to functional analysis. To access the GDB, simply go to the following hyperlink: http://www.gdb.org/. Search “All Biological Data” by “Keyword.” Type “deafness” (or synonyms) into the search box, and review the results. If more than one word is used in the search box, then separate each one with the word “and” or “or” (using “or” might be useful when using synonyms).

26

Adapted from the Genome Database: http://gdbwww.gdb.org/gdb/aboutGDB.html - mission.

259

APPENDIX B. PATIENT RESOURCES Overview Official agencies, as well as federally funded institutions supported by national grants, frequently publish a variety of guidelines written with the patient in mind. These are typically called “Fact Sheets” or “Guidelines.” They can take the form of a brochure, information kit, pamphlet, or flyer. Often they are only a few pages in length. Since new guidelines on deafness can appear at any moment and be published by a number of sources, the best approach to finding guidelines is to systematically scan the Internet-based services that post them.

Patient Guideline Sources The remainder of this chapter directs you to sources which either publish or can help you find additional guidelines on topics related to deafness. Due to space limitations, these sources are listed in a concise manner. Do not hesitate to consult the following sources by either using the Internet hyperlink provided, or, in cases where the contact information is provided, contacting the publisher or author directly. The National Institutes of Health The NIH gateway to patients is located at http://health.nih.gov/. From this site, you can search across various sources and institutes, a number of which are summarized below. Topic Pages: MEDLINEplus The National Library of Medicine has created a vast and patient-oriented healthcare information portal called MEDLINEplus. Within this Internet-based system are “health topic pages” which list links to available materials relevant to deafness. To access this system, log on to http://www.nlm.nih.gov/medlineplus/healthtopics.html. From there you can either search using the alphabetical index or browse by broad topic areas. Recently, MEDLINEplus listed the following when searched for “deafness”:

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•

Other guides Acoustic Neuroma http://www.nlm.nih.gov/medlineplus/acousticneuroma.html Assistive Devices http://www.nlm.nih.gov/medlineplus/assistivedevices.html Ear Disorders http://www.nlm.nih.gov/medlineplus/eardisorders.html Ear Infections http://www.nlm.nih.gov/medlineplus/earinfections.html Hearing Disorders & Deafness http://www.nlm.nih.gov/medlineplus/hearingdisordersdeafness.html Speech & Communication Disorders http://www.nlm.nih.gov/medlineplus/speechcommunicationdisorders.html

Within the health topic page dedicated to deafness, the following was listed: •

General/Overviews Hearing Loss http://www.nlm.nih.gov/medlineplus/tutorials/hearinglossloader.html Hearing Loss Source: Mayo Foundation for Medical Education and Research http://www.mayoclinic.com/invoke.cfm?id=DS00172

•

Diagnosis/Symptoms Five Minute Hearing Test Source: American Academy of Otolaryngology--Head and Neck Surgery http://www.entnet.org/healthinfo/hearing/hearing_test.cfm Hearing Problems: Self-Care Flowcharts Source: American Academy of Family Physicians http://familydoctor.org/flowcharts/508.html Understanding Your Audiogram Source: American Academy of Audiology http://www.audiology.org/consumer/guides/uya.php

•

Treatment Cochlear Implants Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/coch.asp Cochlear Implants: Who Can Get Them? Source: Mayo Foundation for Medical Education and Research http://www.mayoclinic.com/invoke.cfm?id=HQ00441

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Digital Hearing Aids: Current “State-of-the-Art” Source: American Speech-Language-Hearing Association http://www.asha.org/public/hearing/treatment/digital_aid.htm Frequently Asked Questions About Hearing Aids Source: American Academy of Audiology http://www.audiology.org/consumer/guides/hafaq.php Hearing Aids Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/hearingaid.asp Mind Hears: Tuning In with a Cochlear Implant Source: American Speech-Language-Hearing Association http://www.asha.org/public/hearing/treatment/mind_hears.htm Straight Talk from FDA About Hearing Loss and Hearing Aids http://www.fda.gov/opacom/lowlit/hearaid.html What Are Assistive Listening Devices or “ALDs”? Source: American Speech-Language-Hearing Association http://www.asha.org/public/hearing/treatment/assist_tech.htm •

Coping Adult Aural Rehabilitation Source: American Speech-Language-Hearing Association http://www.asha.org/public/hearing/treatment/adult_aur_rehab.htm Communication and Alerting Devices for Deaf and Hard of Hearing People: What's Available Now Source: Gallaudet University, Laurent Clerc National Deaf Education Center http://clerccenter.gallaudet.edu/InfoToGo/418.html Frequently Asked Questions about FCC Provisions for People with Disabilities Source: Federal Communications Commission http://www.fcc.gov/cgb/dro/dtffaq.html Getting Through: Talking to a Person Who is Hard of Hearing Source: American Academy of Audiology http://www.audiology.org/consumer/guides/getthru.php

•

Specific Conditions/Aspects American Sign Language Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/asl.asp Auditory Neuropathy Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/neuropathy.asp Autoimmune Inner Ear Disease (AIED) Source: American Academy of Otolaryngology--Head and Neck Surgery http://www.entnet.org/healthinfo/hearing/autoimmune.cfm

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Captions for Deaf and Hard-of-Hearing Viewers Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/caption.asp Earwax Source: American Academy of Otolaryngology--Head and Neck Surgery http://www.entnet.org/healthinfo/ears/earwax.cfm Hearing Loss and Bone Disorders Source: Osteoporosis and Related Bone Diseases-National Resource Center http://www.osteo.org/newfile.asp?doc=r604i&doctitle=Hearing%2BLoss%2B%252 6%2BBone%2BDisorders&doctype=HTML%2BFact%2BSheet How Deaf People Communicate Source: Gallaudet University, Laurent Clerc National Deaf Education Center http://clerccenter.gallaudet.edu/InfoToGo/492/492-1.html Medication Effects on Hearing Source: American Speech-Language-Hearing Association http://www.asha.org/public/hearing/disorders/med_effects.htm Noise-Induced Hearing Loss Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/noise.asp Otosclerosis Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/otosclerosis.asp Perforated Eardrum Source: American Academy of Otolaryngology--Head and Neck Surgery http://www.entnet.org/healthinfo/ears/perforation.cfm Risk of Bacterial Meningitis in Children with Cochlear Implants Source: National Center on Birth Defects and Developmental Disabilities http://www.cdc.gov/ncbddd/ehdi/cochlear/default.htm Types of Hearing Loss Source: American Speech-Language-Hearing Association http://www.asha.org/public/hearing/disorders/types.htm Use of Vaccines for the Prevention Meningitis in Persons with Cochlear Implants Source: National Immunization Program http://www.cdc.gov/nip/issues/cochlear/cochlear-gen.htm Usher Syndrome Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/usher.asp Waardenburg Syndrome Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/waard.asp •

From the National Institutes of Health Im Lang Vang http://www.nidcd.nih.gov/health/hearing/VietSilence.pdf

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NIHSeniorHealth: Hearing Loss Source: National Institute on Deafness and Other Communication Disorders http://nihseniorhealth.gov/hearingloss/toc.html Ten Ways to Recognize Hearing Loss Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/10ways.asp •

Latest New Hearing Loss Risk May Be Set Before Birth Source: 11/21/2003, Reuters Health http://www.nlm.nih.gov//www.nlm.nih.gov/medlineplus/news/fullstory_14780 .html Methotrexate Not Effective in Maintaining Improvements in Hearing Gained from Prednisone Source: 10/07/2003, National Institute on Deafness and Other Communication Disorders http://www.nih.gov/news/pr/oct2003/nidcd-07.htm

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Pictures/Diagram Atlas of the Body: The Ear Source: American Medical Association http://www.medem.com/MedLB/article_detaillb.cfm?article_ID=ZZZYXNW46JC &sub_cat=198

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Prevention/Screening Choose the Hearing Protection That's Right for You Source: National Institute for Occupational Safety and Health http://www.cdc.gov/niosh/topics/noise/abouthlp/chooseprotection.html Have WISE EARS! For Life: Protect Yourself and Your Family from NoiseInduced Hearing Loss Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/hearing/wiseears.asp Hearing Screening Source: American Speech-Language-Hearing Association http://www.asha.org/public/hearing/testing/ Noise and Hearing Protection Source: American Academy of Otolaryngology--Head and Neck Surgery http://www.entnet.org/healthinfo/hearing/noise_hearing.cfm

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Research Better Communication Needed to Reduce Infants 'Lost to Follow-Up' Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/inside/spr03/pg1.asp

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Children with Cochlear Implants at Increased Risk for Bacterial Meningitis Source: Centers for Disease Control and Prevention http://www.cdc.gov/od/oc/media/pressrel/r030730.htm Early Diagnosis of Usher Syndrome Type 1 Made Possible by New Findings Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/news/releases/03/04_23_03.asp Key to Lifelong Hearing Identified as Continous Self-Renewal of the Sensory Sterocilia Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/news/releases/02/8_21_02.asp Methotrexate Not Effective in Maintaining Improvements in Hearing Gained from Prednisone Source: National Institute on Deafness and Other Communication Disorders http://www.nih.gov/news/pr/oct2003/nidcd-07.htm Mutation of Key Protein Causes Rare Form of Hearing Loss That's Easily Confused with Otosclerosis Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/news/releases/02/8_01_02.asp New 'Deafness' Gene Causes Two Forms of Hearing Loss Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/news/releases/02/3_01_02.asp New Gene Linked to Deafness in Humans, Mice Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/news/releases/02/9_03_02.asp Researchers Use Gene Therapy to Grow New Hair Cells in Mammals Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/news/releases/03/06_05_03.asp 'SWAT'z New? -- A Fly That's Setting the Hearing World Abuzz Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/health/education/news/swatz.asp Teenage Inventor Brings Sign-Translating Glove to NIDCD Source: National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov/news/releases/02/3_19_02.asp You may also choose to use the search utility provided by MEDLINEplus at the following Web address: http://www.nlm.nih.gov/medlineplus/. Simply type a keyword into the search box and click “Search.” This utility is similar to the NIH search utility, with the exception that it only includes materials that are linked within the MEDLINEplus system (mostly patient-oriented information). It also has the disadvantage of generating unstructured results. We recommend, therefore, that you use this method only if you have a very targeted search. The Combined Health Information Database (CHID) CHID Online is a reference tool that maintains a database directory of thousands of journal articles and patient education guidelines on deafness. CHID offers summaries that describe the guidelines available, including contact information and pricing. CHID’s general Web site

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is http://chid.nih.gov/. To search this database, go to http://chid.nih.gov/detail/detail.html. In particular, you can use the advanced search options to look up pamphlets, reports, brochures, and information kits. The following was recently posted in this archive: •

Baby Alarms for Deaf People Source: London, England: Royal National Institute for Deaf People. 1999. 5 p. Contact: Available from RNID Helpline. P.O. Box 16464, London EC1Y 8TT, United Kingdom. 0870 60 50 123. Fax 0171 296 8199. E-mail: [email protected]. Website: www.rnid.org.uk. PRICE: Single copy free. Summary: As with other home equipment for deaf people, there are specially designed baby alarms that use either a vibrating pad or pager, a bright flashing light, or both to alert the user to the sound a baby makes. This fact sheet, from the Royal National Institute for Deaf People (in Britain), reviews the range of devices available for baby alarms for deaf people. The fact sheet discusses dedicated baby alarms, baby alarms with a wired microphone connection, baby alarms that use a radio transmitter and receiver, and multi alerting systems that incorporate a baby alarm. In each section, the fact sheet describes the technology used and offers at least one source for the product (in England). The fact sheet encourages readers to gather information and make informed choices about these technological options. The information on the equipment described in this fact sheet was compiled from material provided by manufacturers and suppliers.

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Overview on Deaf-Blindness Source: Monmouth, OR: DB-LINK, National Information Clearinghouse on Children Who Are Deaf-Blind. 2000. [9 p.]. Contact: Available from DB-LINK. 345 North Monmouth Avenue, Monmouth, OR 97361. Voice (800) 438-9376. TTY (800) 854-7013. Fax (503) 838-8150. E-mail: [email protected]. Website: www.tr.wou.edu/dblink. PRICE: Single copy free. Also available for free at http://www.tr.wou.edu/dblink/ovrview2.htm. Summary: Children who are called deaf-blind are singled out educationally because impairments of sight and hearing require thoughtful and unique educational approaches in order to ensure that children with this disability have the opportunity to reach their full potential. This fact sheet provides an overview of deaf-blindness in children, defined as hearing and visual losses that cause severe communication, developmental, and educational needs, to the point that children cannot be accommodated in special education programs solely for children with deafness or children with blindness or children with multiple disabilities. After a section defining deaf-blindness, the author discusses the causes of deaf-blindness, the numbers of deafblind people (adults and children) in the United States, the challenges facing a person who is deaf-blind, communication strategies and systems for persons who are deafblind, orientation and mobility issues, individualized education, transition and rehabilitation goals, and inclusion of the deaf-blind child in the family. The author stresses that many persons who are deaf-blind have achieved a quality of life that is excellent. The fact sheet concludes with a list of suggested readings and additional resources. 1 table. 3 references.

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Teaching Students Who Are Deaf-Blind Source: Rochester, NY: Northeast Technical Assistance Center, National Technical Institute for the Deaf, Rochester Institute of Technology. 1998. [2 p.].

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Contact: Available from PEPNet Resource Center. National Center on Deafness, California State University, Northridge, 18111 Nordhoff Street, Northridge, CA 913308267. Voice/TTY (888) 684-4695 or (818) 677-2611. Fax (818) 677-4899. Website: www.pepnet.org. PRICE: Single copy free. Summary: Designed for college educators who may have deaf-blind students in their classrooms, this fact sheet offers information about the needs of this population. There is not a formula for addressing these needs because students who are deaf-blind have differing levels of hearing and vision loss. The first step is to talk with the student about the course to determine what modifications are necessary. The fact sheet describes the common types of support services and accommodations used by these students, including interpreters, notetakers, tutors, readers, and assistive listening devices (ALDs). The fact sheet then covers physical classroom accommodations, the use of handouts or ad hoc materials, the use of audiovisual materials, small group discussions and activities, oral presentations, exams and alternative testing methods, field visits and labs, teaching style, and who to contact for assistance. The fact sheet concludes that keeping an open line of communication with the student will ultimately create a positive learning and teaching atmosphere for both instructor and student. The fact sheet is one in a series of informational fact sheets from the Northeast Technical Assistance Center at the Rochester Institute of Technology (National Technical Institute for the Deaf). •

Deafblind Manual Alphabet Source: London, England: Royal National Institute for Deaf People. 1998. [2 p.]. Contact: Available from RNID Helpline. P.O. Box 16464, London EC1Y 8TT, United Kingdom. 0870 60 50 123. Fax 0171-296 8199. E-mail: [email protected]. Website: www.rnid.org.uk. PRICE: Single copy free. Summary: Fingerspelling is one communication method used by people with a severe sight and hearing loss. This fact sheet, from the British Royal National Institute for Deaf People (RNID), describes the British deafblind manual alphabet. Presented on cardstock, the front side shows line drawings of the alphabet; the reverse briefly describes deafblindness and the work of the RNID. The fact sheet emphasizes that communication methods used by deafblind people vary greatly depending on the amount of residual hearing and sight they have. Many people who cannot hear speech or see well enough to lipread or follow visual sign language use some form of tactile communication. The RNID is a membership charity organization that campaigns and lobbies for a better quality of life for deaf and hard of hearing people, raises awareness of deafness and hearing loss, provides direct services, and supports social, medical, and technical research.

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Working with Students Who Are Late-Deafened Source: Rochester, NY: Northeast Technical Assistance Center, National Technical Institute for the Deaf, Rochester Institute of Technology. 1998. [2 p.]. Contact: Available from PEPNet Resource Center. National Center on Deafness, California State University, Northridge, 18111 Nordhoff Street, Northridge, CA 913308267. Voice/TTY (888) 684-4695 or (818) 677-2611. Fax (818) 677-4899. Website: www.pepnet.org. PRICE: Single copy free. Summary: Late deafness refers to deafness that happens postlingually, that is any time after the development of speech and language in a person who has identified with hearing society through schooling, social connections, etc. Students who are late-

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deafened are unable to understand speech without visual aids such as speechreading, sign language, and captioning (although amplification of residual hearing may be used to assist with speechreading). Designed for college educators who may have latedeafened students in their classrooms, this fact sheet offers 16 strategies for working with students who are late-deafened. They cover introducing the student to the class, the basics of computer aided realtime translation (CART) and other communication options, the use of interpreters in the classroom, the issues covered by the Americans with Disabilities Act (ADA) and the Rehabilitation Act (Section 504), notetakers and classroom notetaking, the classroom environment (lighting and sightlines), the use of written English as a backup, regulating cross talk, providing access for out of classroom activities, strategies to help speechreaders, the use of visual aids, and the use of disability support services offered by the educational institution and by other resources (such as the Association of Late Deafened Adults). The fact sheet is one in a series of informational fact sheets from the Northeast Technical Assistance Center at the Rochester Institute of Technology (National Technical Institute for the Deaf). •

NICD: Publications from the National Information Center on Deafness Source: Washington, DC: National Deaf Education Network and Clearinghouse, Laurent Clerc National Deaf Education Center. 1996. 8 p. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. PRICE: Single copy free. Summary: The National Information Center on Deafness (NICD) is a centralized source of information about hearing loss and deafness. This brochure lists the publications available from NICD. The brochure provides a description and price for materials in twelve categories: for and about deaf and hard of hearing people; especially for children and their teachers; assistive devices and hearing aids; careers and employment; communication and sign language; education; Gallaudet University; health and mental health; legal focus; especially for parents; special interest; and free handouts. The brochure includes an order form and ordering information.

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Deaf Culture Source: Rockville, MD: American Speech-Language-Hearing Association (ASHA). 199x. [2 p.]. Contact: Available from American Speech-Language-Hearing Association (ASHA). Product Sales, 10801 Rockville Pike, Rockville, MD 20852. (888) 498-6699. TTY (301) 8970157. Website: www.asha.org. PRICE: $7.00 per single issue. Summary: This article from Let's Talk, a newsletter of the American Speech Language Hearing Association (ASHA), focuses on Deaf culture. The author notes that many members of the Deaf community view their deafness as a unique characteristic that makes them part of a community that shares a common experience, tradition, history, and language. People who are deaf share an identity, not a disease or a disability. The article discusses the role of American Sign Language (ASL), definitions of culture and ethnicity, the impact of technology and education, accommodations and the Americans with Disabilities Act (and other legislation), and new technology that supports people who are deaf or who have hearing aids. The article includes a sidebar listing related organizations.

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Gallaudet Research Institute: A Tradition of Discovery in Deafness Source: Washington, DC: Gallaudet Research Institute. 199x. 5 p. Contact: Available from Gallaudet Research Institute. 800 Florida Avenue, N.E., Washington, DC 20002. (800) 451-8834 or (202) 651-5400. PRICE: Single copy free. Summary: This booklet describes the Gallaudet Research Institute (GRI) and its work and publications. Topics covered include GRI's information sharing role; international collaboration; GRI researchers teaching and collaborating in the Gallaudet University setting; outreach and service activities; University-wide functions; the role of the National Advisory Committee that guides the GRI; and sources of research support. The booklet also describes the eight units in the GRI: the Center for Assessment and Demographic Studies, the Center for Studies in Education and Human Development, the Center for Auditory and Speech Sciences, the Genetic Services Center, the Technology Assessment Program, the Culture and Communication Studies Program, the Mental Health Research Program, and the Scientific Communications Program. The booklet concludes with a list of some of the resources offered by the GRI.

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Mainstreaming Deaf and Hard of Hearing Students: Questions and Answers. Research, Readings, and Resources Source: Washington, DC: National Deaf Education Network and Clearinghouse, Laurent Clerc National Deaf Education Center. 2002. 39 p. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. PRICE: $6.00 plus shipping and handling. Item Number 096. An online version is available at the Clerc Center Web site. Summary: This booklet discusses the practice of mainstreaming children who are deaf and hard of hearing into classes with children who have normal hearing. The author presents mainstreaming as one educational option and suggests some considerations if a mainstream placement has been selected for a particular child. The author defines and explains the basic distinction between the terms 'deaf' and 'hard of hearing'. The booklet provides an annotated bibliography of recommended books about mainstreaming. In addition, the book includes commonly asked questions and a checklist to help parents explore program options, descriptions of current research projects on mainstreaming being conducted at Gallaudet University, and an annotated resource list of national organizations and agencies with projects and information on mainstreaming. The book does not include discussions of deafness and its educational implications, other educational placements, or various communication approaches. (AA-M).

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HiP on Health and Safety Tips. For Deaf or Hard-of-Hearing Students in Grades K-4 Source: Alameda, CA: HiP Publishing Group. 2001. 19 pp. Summary: This booklet for deaf and hard-of-hearing students in kindergarten through fourth grade provides tips for staying healthy and safe. Topics include eating well, caring for eyes and ears, street safety, feelings, recognizing different kinds of touches, and controlling anger. The guide also explains what to do in an accident or emergency. One section describes how to use helping devices for deaf and hard-of-hearing people, such as visual smoke detectors and doorbells, TTYs and telephones, closed-caption television programs, pagers, and the Internet. The last page provides a worksheet that can be used to develop a family emergency plan.

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Altered World: Living with New Deafness Source: Washington, DC: National Deaf Education Network and Clearinghouse, Laurent Clerc National Deaf Education Center. 1998. 52 p. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. PRICE: $3.00 plus shipping and handling. Summary: This booklet provides information for late-deafened people, focusing on the adaptations required for previously hearing people to learn to cope with their hearing loss. The booklet first identifies two crucial aspects to defining this group of people. First, late-deafened people have an acquired hearing loss; for many years they have had normal or nearly normal hearing; and their language, identities, and cultural and educational experiences are those of hearing people. Second, late-deafened people are, in the audiological sense, deaf. That is, even with the best amplification, they cannot continue to use hearing to understand speech. The authors note that this line separating hard of hearing people from deafened people is not clear or objective. People define themselves based on their experience with communication, their comfort with particular coping strategies, and on social and support groups that match their experience and preferences. After this introductory section on definitions, the booklet covers six main areas: crisis, or the diagnostic stage, including the problem of tinnitus; communication adaptations; work, including vocational rehabilitation, and laws that protect people with disabilities; accommodations for home and recreation; getting support; and moving on. The booklet concludes with a resources section. 19 references.

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History Through Deaf Eyes Source: Washington, DC: Office of the President, Gallaudet University. 1998. 32 p. Contact: Available from Gallaudet University. 800 Florida Avenue, N.E., Washington, DC 20002-3695. Voice/TTY (202) 651-5635; Fax (202) 651-5467. PRICE: Single copy free. Summary: This brochure describes a traveling social history exhibition about Americans who are deaf. Developed by Gallaudet University, the exhibit uses objects and images to illustrate shared experiences of family life, education, and work, as well as the divergent ways of people who are deaf and the way they see themselves, communicate, employ and adapt available technology, and determine their own futures. The exhibition places the social history of Americans who deaf within the context of better known aspects of American history. It focuses on the development of a Deaf community and identity, language as both a cohesive and a divisive force, the impact of technological change, and the struggles for self-determination. The brochure describes and shows photographs from the six sections of the exhibition: introduction and orientation to deafness, formation of a community, an oral approach to education, the war and postwar years, civil rights recognition and access, and the information age and technological choices. The principal target audience for the exhibition and its accompanying materials is hearing people who have little contact with individuals who are deaf. The brochure includes information about the curator of the exhibition (Jack R. Gannon) and who to contact to bring the exhibition for display. (AA-M).

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AIDS Education/Services for the Deaf Contact: United Way, Incorporated, 621 S Virgil Ave, Los Angeles, CA, 90005, (213) 7361300.

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Summary: This brochure describes the human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) services provided by the Greater Los Angeles Council on Deafness (GLAD) and the United Way to hearing impaired/deaf persons. The brochure outlines the goals of GLAD and the United Way related to the education of and services for deaf persons concerning HIV/AIDS. It summarizes the educational programs and services such as a walk-in center, a women's outreach program, a deaf youth program, a deaf gay and bisexual men's program, national training, workshops and presentations, support groups and rap sessions, client support services, AESD interpreting services, volunteer programs, a TDD access line, and an orientation to deaf culture. The brochure the resources, and advises the readers to take advantage of GLAD and the United Way's educational programs and services. •

National Institute on Deafness and Other Communication Disorders Source: Bethesda, MD: National Institute on Deafness and Other Communication Disorders (NIDCD). 1997. Contact: Available from National Institute on Deafness and Other Communication Disorders (NIDCD) Information Clearinghouse. 1 Communication Avenue, Bethesda, MD 20892-3456. Voice (800) 241-1044. TTY (800) 241-1055. Fax (301) 907-8830. E-mail: [email protected]. Website: www.nidcd.nih.gov. PRICE: Single copy free. Summary: This brochure describes the National Institute on Deafness and Other Communication Disorders (NIDCD), one of the institutes of the National Institutes of Health. The NIDCD conducts and supports research and research training on normal and disordered mechanisms affecting hearing, balance, smell, taste, voice, speech, and language. The NIDCD also provides information, disseminates materials and conducts forums to educate the public about these seven research areas.

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University of Arkansas: Deafness Rehabilitation Programs Source: Little Rock, AR: Rehabilitation Research and Training Center for Persons who are Deaf or Hard of Hearing, University of Arkansas. 1995. 22 p. Contact: Available from Rehabilitation Research and Training Center for Persons Who are Deaf or Hard of Hearing. University of Arkansas, 4601 West Markham Street, Little Rock, AR 72205. Voice/TTY (501) 686-9691; Fax (501) 686-9698. PRICE: Single copy free. Summary: This brochure describes the programs leading to the Master of Science degree at the University of Arkansas Rehabilitation Research and Training Center for Persons who are Deaf or Hard of Hearing. Two areas of specialization under the deafness rehabilitation program are available: rehabilitation counseling for deafness; and independent living rehabilitation. The brochure describes the general curriculum requirements; the requirements for specialization; additional requirements, including proficiency in American Sign Language (ASL); and the courses themselves. The brochure also lists the teaching faculty; describes the location of the school; and lists common questions and answers. The booklet concludes with a brief discussion of admissions requirements and the application procedure.

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New York Society for the Deaf: Ryan White Case Management Program Source: New York, NY: New York Society for the Deaf. 199X. 2 p. Contact: Available from New York Society for the Deaf. 817 Broadway, 7th Floor, New York, NY 10003. Voice/TTY (212) 777-3900; Fax (212) 777-5740. PRICE: Single copy free.

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Summary: This brochure describes the Ryan White Case Management Program (RWCMP) of the New York Society for the Deaf (NYSD), a program established to ensure that people who are deaf or deaf-blind and have AIDS receive the same quality and range of services as all other people who are directly affected by HIV and AIDS. The brochure describes the history of the program, its three primary components, services offered through the program, and the goals and activities of the NYSD. The brochure stresses that for people who are deaf or deaf-blind, the communication barriers, cultural insensitivity, and lack of accessible services can make it virtually impossible to maneuver the AIDS service system effectively. •

Deaf AIDS Project at UCSF Source: San Francisco, CA: Deaf AIDS Project, University of California Center on Deafness. 199X. 2 p. Contact: Available from Deaf AIDS Project. University of California Center on Deafness, 3333 California Street, Suite 10, San Francisco, CA 94143-1208. Voice (415) 476-4980; TTY (415) 476-7600. PRICE: Single copy free. Summary: This brochure describes the services available through the UCSF Deaf AIDS Project, an organization designed to assist Deaf people or those who have hearing impairments and who also have HIV or AIDS to obtain support services. The brochure describes the Project's activities in the areas of counseling, referral to other support organizations, practical living support, interpreting services, and assistive devices. The brochure stresses the need for special support services for this population and emphasizes the willingness of the Project to work with individuals to address their needs. The Project also provides workshops, consultation, and referrals for other providers and agencies that may need special training in the areas of communication and access issues.

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Teacher Preparation: Deaf Education Source: Monmouth, OR: Regional Resource Center on Deafness, Western Oregon University. 2000. [4 p.]. Contact: Available from Teacher Preparation: Deaf Education Program, Regional Resource Center on Deafness, Western Oregon University. 345 North Monmouth Avenue, Monmouth, OR 97361. Voice/TTY (503) 838-8444. E-mail: [email protected]. PRICE: Single copy free. Summary: This brochure describes the teacher preparation in Deaf Education (TPD) program at Western Oregon University, a program that offers students a solid foundation for making significant contributions to the teaching profession. Course content and training methods are in keeping with current research findings and best practices in deaf education. The goal of the program, which was established in 1960, is to prepare personnel with qualifications to teach in residential schools, self contained classrooms, resource rooms, special classes and itinerant settings; or to function as consultants to teachers with Deaf or hard of hearing students in regular classrooms. The program prepares students who can articulate the rationale for being proficient bilinguals in American Sign Language (ASL) and English and who give evidence of meeting established criteria in each language. All tuition is waived for this master's degree TPD program, based upon continued federal funding. The brochure lists the program faculty and contributing staff and includes a self mailer with which readers can request an application packet for the TPD program.

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Western Region Outreach Center and Consortia: Serving Postsecondary Institutions and Individuals Who Are Deaf and Hard of Hearing Source: Northridge, CA: Western Region Outreach Center and Consortia, National Center on Deafness, California State University, Northridge. 199x. [6 p.]. Contact: Available from PEPNet Resource Center. National Center on Deafness, California State University, Northridge, 18111 Nordhoff Street, Northridge, CA 913308267. Voice/TTY (888) 684-4695 or (818) 677-2611. Fax (818) 677-4899. Website: www.pepnet.org. PRICE: Single copy free. Summary: This brochure describes the Western Region Outreach Center and Consortia (WROCC), a program of the National Center of Deafness at California State at Northridge. WROCC serves the diverse western region of the U.S. and works primarily at the institutional level to increase and enhance postsecondary educational opportunities for individuals who are deaf and hard of hearing. The staff members provide technical assistance, training, and resources to ensure that the access needs of individuals who are deaf or hard of hearing are met in postsecondary education settings. The brochure describes the objectives and services of WROCC, lists information resources, and lists the California Affiliate and Regional Hub Institutions. The contact information for each of the institutions is provided. 2 figures.

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AIDS Education-Services for the Deaf Source: Los Angeles, CA: AIDS Education-Services for the Deaf. 199x. 2 p. Contact: Available from AIDS Education-Services for the Deaf. 2222 Laverna Avenue, Los Angeles, CA 90041. Voice/TTY (213) 478-8005. PRICE: Single copy free. Summary: This brochure describes the work of the AIDS Education/Services for the Deaf (AESD), a project of the Greater Los Angeles Council on Deafness (GLAD). The AESD program is designed by deaf professionals dedicated to providing quality education and HIV-related services to the deaf and hard of hearing community, as well as establishing better access to AIDS care providers. The brochure describes the goals of the AESD program and then outlines the services available, including the walk-in center; women's outreach program; deaf youth program; deaf gay and bisexual men's program; national training, workshops and presentations; support groups and rap sessions; client support services; AESD interpreting services; volunteers; TTY access line; and orientation to the deaf culture.

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Deaf Teens and AIDS Contact: Greater Los Angeles Council on Deafness, AIDS Education/Services for the Deaf, 2222 Laverna Ave, Los Angeles, CA, 90041, (323) 550-4250, http://www.gladinc.org. Summary: This brochure gives the reader information on the transmission and prevention of HIV and notes that just because a person is hearing impaired does not make him/her immune to AIDS. The brochure lists ways in which HIV can and cannot be transmitted, and the best ways to protect oneself against infection. It stresses condom use and not using drugs, and answers questions about HIV-antibody tests.

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Careers in Deafness Source: Washington, DC: National Deaf Education Network and Clearinghouse, Laurent Clerc National Deaf Education Center. 1998. 18 p.

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Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. PRICE: $1.00 plus shipping and handling. Summary: This brochure lists and discusses career opportunities in deafness, providing information for young people who are making plans for education and employment. The brochure discusses professional areas such as social work, in which one can concentrate on clients who are deaf or hard of hearing, as well as career areas that are specific to deaf people such as sign language interpreting. The brochure then describes each of these career options: audiologist, counselor, dormitory or residence program counselor, interpreter, linguist, social worker, speech language pathologist, teacher, parent and infant specialist, and combined specialties. The educational and licensure or certification preparation for each career is also noted. The brochure also discusses the different settings in which these careers may take place. The brochure encourages young people to begin general preparation even before they ve narrowed down a career choice. This preparation can include learning sign language, developing English skills, and volunteering. The brochure concludes with a list of resource organizations through which readers can get more information. •

RT-31: Publications for Serving Persons Who are Deaf or Hard of Hearing Source: Little Rock, AR: Rehabilitation Research and Training Center for Persons Who are Deaf or Hard of Hearing, University of Arkansas. 1997. [2 p.]. Contact: Available from Rehabilitation Research and Training Center for Persons Who are Deaf or Hard of Hearing. University of Arkansas, 4601 West Markham Street, Little Rock, AR 72205. Voice/TTY (501) 686-9691; Fax (501) 686-9698. PRICE: Free. Summary: This brochure lists materials and publications for use in serving persons who are deaf or hard of hearing. Included are training packages on job seeking skills, interpersonal problem solving skills, developing employer confidence, and managing employer development. Publications cover topics including the Black Deaf experience, career education, vision and hearing loss among older adults, late deafened adults, counseling deaf people, opportunities for postsecondary education, facilitating the transition of Deaf adolescents, training interpreters, mainstreaming, deaf-blindness, rehabilitation programs, hearing ear dogs, behavioral interventions for hearing impaired persons, and mental health and deafness. The brochure serves as an order form for these materials; pricing information is included.

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Tips for Communicating with Deaf People Source: Rochester, NY: National Technical Institute for the Deaf (NTID), Rochester Institute of Technology (RIT). 2002. [11 p.]. Contact: Available from National Technical Institute for the Deaf (NTID). Marketing and Communications Department, 52 Lomb Memorial Drive, Rochester, NY 14623-5604. Voice/TTY (585) 475-6906; Fax (585) 475-5623. PRICE: $.40 each plus shipping (from $1.25 for 1-25 copies); bulk orders available. Summary: This brochure offers strategies for communicating with individuals who are deaf (defined as all ranges of hearing impairment, from mild to profound). The brochure notes that several factors affect communication with individuals who are deaf or hard of hearing. The factors may include age at which deafness began, type of deafness, language skills, amount of residual hearing, speechreading skills, and educational and

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cultural background. Tabbed sections provide suggestions for communicating one-toone, in a group setting, through an interpreter, at an interview, at work, in writing, and on the telephone. A final section provides a glossary of terms related to degrees and types of hearing loss, onset of hearing loss, means of communication, types of interpreting, and communication devices. The brochure is illustrated with black and white photographs of people communicating in a variety of settings. •

Perspectives on Deafness: Working with Students Who Are Deaf and Hard of Hearing Source: Northridge, CA: National Center on Deafness, California State University, Northridge. 199x. [20 p.]. Contact: Available from PEPNet Resource Center. National Center on Deafness, California State University, Northridge, 18111 Nordhoff Street, Northridge, CA 913308267. Voice/TTY (888) 684-4695 or (818) 677-2611. Fax (818) 677-4899. Website: www.pepnet.org. PRICE: Single copy free. Summary: This brochure provides an overview of working with students who are deaf or hard of hearing. Designed for college educators who may have deaf or hard of hearing students in their classrooms, the brochure reminds readers that deaf people are first of all people, with all the diversity that any other population demonstrates. The significant consequence of deafness is being cut off from the means of acquiring and transmitting spoken language which most people enjoy; as a result, communication is affected. The brochure discusses the factors which impact on how deaf and hard of hearing individuals interact with their environment and with other people, including natural intelligence, personality, family climate, age at onset of deafness, language background, degree of hearing loss, communication skills, and the instructor's attitude. One section offers a glossary of terms related to the field of deafness. Other sections review communication tips, the use of interpreters in the classroom, and the use of notetakers. The brochure concludes with an interview with a professor who shares his experiences with teaching students who are deaf. The American Sign Language fingerspelling alphabet is also included in one illustration. 1 figure.

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So You've Hired Someone Who is Deaf or Hard of Hearing Source: Washington, DC: American Association of Retired Persons. 1994. 8 p. Contact: Available from American Association of Retired Persons. Disability Initiative, 601 E Street, N.W., Washington, DC 20049. voice: (202) 434-2477; TTY: (202) 434-6554. PRICE: Single copy free; contact directly for bulk pricing. Summary: This brochure provides information for employers who have recently hired an employee who is deaf or hearing impaired. Written in a question-and-answer format, the brochure covers the need for reasonable accommodations; costs of accommodations; the Americans with Disabilities Act; communicating with an employee who is deaf or hearing-impaired; speechreading; how co-workers can establish a working rapport with a person who is deaf or hearing-impaired; getting the attention of an employee who is deaf; terminology; hearing aids; and using an interpreter. One final section covers basic telecommunications device for the Deaf (TDD or TTY) etiquette. The brochure concludes with a list of TTY numbers at the American Association of Retired Persons (AARP). 3 references.

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Communication Access for Deaf and Hard of Hearing: Discover Needs. Recognize Differences Source: Phoenix, AZ: Arizona Council for the Hearing Impaired. 199x. [2 p.]. Contact: Available from Arizona Council for the Hearing Impaired. 1400 West Washington, Phoenix, AZ 85007. Voice/TTY (602) 542-3323. PRICE: Single copy free. Summary: This brochure summarizes recommendations for supporting communications access for people who are deaf or hard of hearing. The brochure emphasizes that this population has the same legal rights to communications access as do people with normal hearing. The brochure describes the differences between communications methods used with people who are deaf versus those who are hard of hearing. The brochure also summarizes general information about deafness and the heterogeneity of the deaf and hard of hearing population. One chart lists the different types of assistive devices, public systems, and interpretation that may be appropriate. A second chart lists considerations of environment, the speaker's voice, and the speaker's technique. The brochure concludes that, in addition to all other considerations, people who are deaf or hard of hearing should have the right to clarification when it is needed. In some circumstances, this may disrupt formal procedural routine, but it is essential if full communication is to be achieved. (AA-M).

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Deaf and Hard of Hearing People Source: London, England: Royal National Institute for Deaf People. 1998. 31 p. Contact: Available from RNID Helpline. P.O. Box 16464, London EC1Y 8TT, United Kingdom. 0870 60 50 123. Fax 0171-296 8199. E-mail: [email protected]. Website: www.rnid.org.uk. PRICE: Single copy free. Summary: This brochure, from the British Royal National Institute for Deaf People (RNID), gives an overview of the issues that concern deaf and hard of hearing people. The brochure discusses the causes of deafness, describing deafness, the degrees of deafness, sound and how it is measured, the anatomy and physiology of the ear, tinnitus (ringing or other sounds in the ear), the Deaf community, communication strategies, deafblindness, speechreading, sign language, fingerspelling, communication support, equipment and supplies, hearing aids, household equipment, television, telephones, educational considerations, employment, and working with people who are deaf. The brochure includes a list of resource organizations in England and a mail in form with which readers can request additional information. The inside back cover of the brochure summarizes the activities of the RNID. The brochure is illustrated with black and white photographs and line drawings.

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National Institute on Deafness and Other Communication Disorders Information Clearinghouse Source: Washington, DC: National Institute on Deafness and Other Communication Disorders (NIDCD) Information Clearinghouse. 1994. 2 p. Contact: Available from National Institute on Deafness and Other Communication Disorders (NIDCD) Information Clearinghouse. 1 Communication Avenue, Bethesda, MD 20892-3456. Voice (800) 241-1044. TTY (800) 241-1055. Fax (301) 907-8830. E-mail: [email protected]. Website: www.nidcd.nih.gov. PRICE: Single copy free. Summary: This fact sheet describes the National Institute on Deafness and Other Communication Disorders (NIDCD) Information Clearinghouse, a national resource

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center for information about hearing, balance, smell, taste, voice, speech, and language for health professionals, patients, industry, and the public. The clearinghouse collects and disseminates information through an information service, a computerized database (the DC subfile on the Combined Health Information Database), and the development and distribution of publications in the above seven areas of human communication. •

Captions For Deaf and Hard-of-Hearing Viewers Source: Bethesda, MD: National Institute on Deafness and Other Communication Disorders (NIDCD), National Institutes of Health (NIH). July 2002. [4 p.]. Contact: Available from NIDCD Information Clearinghouse. 1 Communication Avenue, Bethesda, MD 20892-3456. Voice (800) 241-1044. TTY (800) 241-1055. Fax (301) 907-8830. E-mail: [email protected]. Website: www.nidcd.nih.gov. PRICE: Single copy free. Summary: This fact sheet describes the use of captions, words that are displayed on a television screen that describe the audio or sound portion of a program. Captions allow viewers who are deaf or hard of hearing to follow the dialogue and the action of a program simultaneously. The fact sheet describes how captions are created, the differences between open and closed captions, real time captions, electronic newsroom captions, edited and verbatim captions, rear window captioning, current research, legal factors, captions and the FCC (Federal Communications Commission), requirements for the provision of closed captions, and programs that are exempt from captioning. The fact sheet emphasizes that captions are considered one type of auxiliary aid that may be used to meet the requirements of the Americans With Disabilities Act (ADA, 1990). The fact sheet concludes with a list of information resources related to captioning; Internet sites are listed where available.

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Summary of the Americans with Disabilities Act and Its Specific Implications for Deaf and Hard of Hearing People Source: Washington, DC: National Deaf Education Network and Clearinghouse, Laurent Clerc National Deaf Education Center. 1992. 5 p. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. PRICE: Single copy free. Summary: This fact sheet is based on a presentation at the national conference of the American Deafness and Rehabilitation Association, held in Chicago, Illinois in 1991; the presentation summarized the Americans With Disabilities Act (ADA) and its specific implications for deaf and hard of hearing people. Topics covered include the purpose and scope of the ADA; how the protections of the ADA apply to deaf and hard of hearing people; private employment and the ADA; deaf and hard of hearing people and public services; privately owned public accommodations and the ADA; and telecommunications and the ADA.

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Residential Programs for Deaf-Emotionally Disturbed Children and Adolescents Source: Washington, DC: National Deaf Education Network and Clearinghouse, Laurent Clerc National Deaf Education Center. 2001. 2 p. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202)

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651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. PRICE: $1.00 plus shipping and handling. Item Number 491. An online version is available at the Clerc Center Web site. Summary: This fact sheet lists residential programs for youth who are deaf or hard of hearing and have emotional or behavioral disorders. Readers are cautioned that admission requirements may vary and that they should write directly to the programs for specific details. The addresses and telephone numbers for 22 programs are listed in alphabetical order by state; voice and TTY telephone numbers are also provided. •

Hearing Impairment or Loss (Deafness) Source: in Griffith, H.W. Instructions for Patients. 5th ed. Orlando, FL: W.B. Saunders Company. 1994. p. 202. Contact: Available from W.B. Saunders Company. Order Fulfillment, 6277 Sea Harbor Drive, Orlando, FL 32887. (800) 545-2522; Fax (800) 874-6418. PRICE: $47.50 plus shipping and handling. ISBN: 0721649300. Summary: This fact sheet on hearing impairment or loss is from a compilation of instructions for patients, published in book format. The fact sheet provides information in three sections: basic information, including a description of the condition, frequent signs and symptoms, causes, risk factors, preventive measures, expected outcome, and possible complications; treatment, including general measures, medication, activity guidelines, and diet; and when to contact one's health care provider. The fact sheet is designed to be photocopied and distributed to patients as a reinforcement of oral instructions and as a teaching tool.

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Fact Sheet on HIV/AIDS and the Deaf Community Contact: Greater Los Angeles Council on Deafness, AIDS Education/Services for the Deaf, 2222 Laverna Ave, Los Angeles, CA, 90041, (323) 550-4250, http://www.gladinc.org. Summary: This fact sheet presents data and information on the effects of HIV/AIDS in the deaf community. Quoting a National Coalition on Deafness estimate of 7,000 cases of HIV/AIDS and 300 deaths as of October 1993 among deaf people in the United States, the sheet offers additional statistics from various sources and indicates how little official surveillance exists to monitor rates of infection and deaths from HIV/AIDS in this population. The sheet also emphasizes that deafness is a communication disability, with the deaf community considering itself a minority group. An attached article discusses the need for increased HIV/AIDS education targeted to the deaf community. It points out that knowledge, attitudes, beliefs, and behavior related to HIV/AIDS in the deaf community lag behind the general population. The author describes risk factors among deaf persons and outlines hindrances to reaching this group, including the following: traditional informational avenues frequently fail to reach the deaf community; few educational materials are geared to meet the needs of the deaf population; and most health care providers are unable to communicate with deaf patients.

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About Deafness/Hearing Loss Source: Washington, DC: National Deaf Education Network and Clearinghouse, Laurent Clerc National Deaf Education Center. 2002. 6 p. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202)

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651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. PRICE: $1.00 plus shipping and handling. Item Number 085. Summary: This fact sheet presents general information about deafness and people who are deaf. The fact sheet first defines the four types of hearing loss, each of which can result in different problems and different possibilities for medical and nonmedical remediation. Educational implications for children are discussed and different communication choices are presented, including American Sign Language, fingerspelling, manual English, oral communication, speechreading, cued speech, simultaneous communication, and total communication. One section about the deaf community and adults who are deaf includes lists of organizations of and for deaf people, educational institutions, special devices and services, and suggested readings. 9 references. •

Fact Sheet on HIV-AIDS and the Deaf Community Source: Los Angeles, CA: AIDS Education-Services for the Deaf, Greater Los Angeles Council on Deafness (GLAD). 1993. 4 p. Contact: Available from AIDS Education-Services for the Deaf. Greater Los Angeles Council on Deafness (GLAD), 2222 Laverna Avenue, Los Angeles, CA 90041. Voice/TTY (213) 550-4250. PRICE: Single copy free. Summary: This fact sheet provides basic information about HIV and AIDS and its incidence in the Deaf community, an overview of the Deaf community (and the role of the Americans With Disabilities Act, or ADA, in this population), and information about deafness and AIDS education. There are only estimates of the number of people with HIV in the Deaf community. The fact sheet reminds readers that the Deaf community is different from other disabled groups by virtue of the communication process, not physical handicaps. Demographically, the Deaf community parallels other minority groups in terms of underachievement, unemployment, and underemployment. A successful AIDS education program must demonstrate an understanding of the Deaf community and its unique culture, based on a common language and maintained by a complex social strata. Formal education for many people who are deaf ends at the 12th grade. Upon completion of a secondary education, 30 percent are functionally illiterate and 60 percent read at grade level 5.3 or below. Services and educational material must be geared to meet these educational and literacy needs. The fact sheet challenges people who are deaf to acquire essential information, to educate their local Deaf communities, and to assist general AIDS agencies in providing vital services to the Deaf population.

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Statistics on Deafness Source: London, England: Royal National Institute for Deaf People. 1997. 6 p. Contact: Available from RNID Helpline. P.O. Box 16464, London EC1Y 8TT, United Kingdom. 0870 60 50 123. Fax 0171-296 8199. E-mail: [email protected]. Website: www.rnid.org.uk. PRICE: Single copy free. Summary: This fact sheet, from the British Royal National Institute for Deaf People (RNID), offers basic statistics on deafness. The fact sheet first provides definitions of mild, moderate, severe, and profound deafness, then offers statistics on the numbers of deaf or hard of hearing people in the U.K., deaf or hard of hearing children in the U.K., how age affects hearing, gender differences in hearing, hearing aids, the use of British Sign Language (BSL), lipreading (speechreading), late deafened persons, deafblind persons, tinnitus, the proportion of deaf people who have additional disabilities,

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occupational considerations, and estimated numbers of deaf and hard of hearing adults in different parts of the U.K. 2 tables. •

Doorbells for Deaf People Source: London, England: Royal National Institute for Deaf People. 1999. 9 p. Contact: Available from RNID Helpline. P.O. Box 16464, London EC1Y 8TT, United Kingdom. 0870 60 50 123. Fax 0171 296 8199. E-mail: [email protected]. Website: www.rnid.org.uk. PRICE: Single copy free. Summary: This fact sheet, from the Royal National Institute for Deaf People (in Britain), reviews the range of devices available for doorbells for deaf people. People who are hard of hearing or deaf may not be able to hear the doorbell ring. There are various solutions to this problem, including louder bells, audible extension bells in different rooms, flashing lights, or a doorbell that triggers a vibrating pad or pager. The fact sheet discusses these options in five groups: audible extension bells; wired visual doorbell systems; wireless and cordless audible and visual doorbells; systems that flash or dim the house lights; and doorphones or entryphones. In each section, the fact sheet describes the technology used and offers at least one source for the product (in England). The fact sheet encourages readers to gather information and make informed choices about these technological options. The information on the equipment described in this fact sheet was compiled from material provided by manufacturers and suppliers.

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Deaf Parents and Their Hearing Children Information Packet Source: CODA - Children of Deaf Adults, International. Santa Barbara, CA. Contact: Available from CODA International, Inc. P.O. Box 30715, Santa Barbara, CA 93130-0715. E-mail: [email protected]. Web site: http://codainternational.org. Summary: This information packet for deaf and hard-of-hearing parents raising hearing children includes information about communication and cultural issues and legal issues regarding communication accessibility, addresses the myths surrounding deaf parents, and includes summer camp information, bibliographical referrals, and listings of state and national resources.

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Nerve Deafness and You Source: Washington, DC: Better Hearing Institute. 1997. [4 p.]. Contact: Available from Better Hearing Institute. P.O. Box 1840, Washington, DC 20013. (800) EAR-WELL or (703) 684-3391. Fax (703) 750-9302. E-mail: [email protected]. Website: www.betterhearing.org. PRICE: Single copy free; bulk orders available. Summary: This pamphlet provides information to patients and the general public about sensorineural hearing loss or nerve deafness. The causes and treatments of nerve deafness are discussed.

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What you should know about genetics and deafness Source: Brooklyn, NY: Brooklyn Hospital. n.d. 8 pp. Contact: Available from Karen L. David, M.D. and Penny Steiner-Grossman, M.P.H., Brooklyn Hospital Center, Genetics Unit, 121 DeKalb Avenue, Brooklyn, NY 11201. Telephone: (718) 403-8032. Available at no charge.

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Summary: This patient education brochure answers questions often asked by families with inherited deafness, including causes of deafness, environmental versus inherited deafness, how deafness is inherited, and genetic counseling. •

Homes and Housing for Aged Deaf Persons Source: Washington, DC: National Deaf Education Network and Clearinghouse, Laurent Clerc National Deaf Education Center. 1993. 2 p. Contact: National Deaf Education Network and Clearinghouse. KDES PAS-6, 800 Florida Avenue, NE, Washington, DC 20002-3695. Voice/TTY (800) 526-9105 or (202) 651-5340. Fax (202) 651-5708. E-mail: [email protected]. Website: clerccenter.gallaudet.edu. PRICE: $1.00 plus shipping and handling. Summary: This resource list identifies facilities that serve older deaf persons. Organized by state, the list provides the name and address of each facility, along with telephone number(s) and the names of key administrators. Readers are encouraged to contact each resource directly for information about eligibility requirements, fees, programs, and services that they offer to people with special needs.

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Excellence in Career Counseling: Improving Services for Students Who Are Deaf or Hard of Hearing Source: Torrance, CA: Southern California Outreach Center at El Camino College. 2001. Contact: Postsecondary Education Programs Network (PEPNet) consortium. 18111 Nordhoff Street, Northridge, CA 91330-8267. (818) 677-2145. Toll-free: (888) 684-4695. TTY: (818) 677-2665. Fax: (818) 677-7693. Web site: www.pepnet.org. PRICE: $20.00 plus shipping and handling. Summary: This video provides guidance to career counselors and educators on how to best provide services to students who are deaf or hard-of-hearing. Topics include methods of communication, community resources, and the use of strategies such as internships and job shadowing. A brochure, which accompanies the video, contains a list of important phone numbers, definitions, and general information and tips for career counselors.

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Health Care Access for Consumers Who Are Deaf, Deaf-Blind, or Hard of Hearing Source: a.b.c. Reports. 8(1): 4-5. Winter 1999. Contact: Available from advocates for better communication (a.b.c.). League for the Hard of Hearing, 71 West 23rd Street, New York, NY 10010-4162. (212) 741-7650. Fax (212) 255-4413. TTY (212) 255-1932. E-mail: [email protected]. Website: www.lhh.org. Summary: Under the Americans With Disabilities Act, all health care providers (including doctors, dentists, and hospitals) must communicate effectively with their patients by providing auxiliary aids and services. This fact sheet offers information on access to health care for consumers who are deaf, deaf-blind, or hard of hearing. These auxiliary aids and services may include qualified interpreters, transcripts of services or written material, FM communication, TTYs, telephone amplification, and television caption decoders. The fact sheet encourages readers to educate themselves as to their needs for communication accommodations and their civil rights. Topics covered in the fact sheet include the health care provider's responsibilities, the patient's responsibilities, the use of the international symbol of deafness, how to handle situations in a waiting room, in a hospital, in the radiology department, undergoing a surgical procedure,

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during an eye examination, and at the dental office. The fact sheet stresses that most health care providers are eager to help, but may need to be informed how best to communicate with individuals with hearing loss. •

HiP on Health and Safety Tips. For Deaf or Hard-of-Hearing Students in Grades 5-8 Source: Alameda, CA: HiP Publishing Group. 2001. 19 pp. Summary: Written for deaf and hard-of-hearing students in grades 5-8, this booklet provides tips for staying healthy and safe at home and in the community. Topics include eating well, caring for listening devices, street safety, violence, Internet safety, and accidents and emergencies. The booklet also addresses sensitive issues such as peer pressure, decision-making, feelings, sexual abuse, and drugs and alcohol. Examples of how deaf and hard-of-hearing youth use technologies for safety are included. Worksheets are provided throughout the text. Healthfinder™

Healthfinder™ is sponsored by the U.S. Department of Health and Human Services and offers links to hundreds of other sites that contain healthcare information. This Web site is located at http://www.healthfinder.gov. Again, keyword searches can be used to find guidelines. The following was recently found in this database: •

Deaf or Hard-of-Hearing: Tips for Working With Your Doctor Summary: Tips on how to improve communication at the doctor's office if you are deaf or hard-of-hearing. Source: American Academy of Family Physicians http://www.healthfinder.gov/scripts/recordpass.asp?RecordType=0&RecordID=6128

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Deafness and Hearing Loss Source: National Information Center for Children and Youth with Disabilities, U.S. Department of Education http://www.healthfinder.gov/scripts/recordpass.asp?RecordType=0&RecordID=3408

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Registry of Interpreters for the Deaf Searchable Databases Summary: Here you can search for Registry of Interpreters for the Deaf members or for interpreter agencies or referral services. Source: Registry of Interpreters for the Deaf, Inc. http://www.healthfinder.gov/scripts/recordpass.asp?RecordType=0&RecordID=7744

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Sudden Deafness Summary: This fact sheet describes sudden deafness and its causes, diagnosis, and treatment. Source: National Institute on Deafness and Other Communication Disorders Information Clearinghouse http://www.healthfinder.gov/scripts/recordpass.asp?RecordType=0&RecordID=6679 The NIH Search Utility

The NIH search utility allows you to search for documents on over 100 selected Web sites that comprise the NIH-WEB-SPACE. Each of these servers is “crawled” and indexed on an ongoing basis. Your search will produce a list of various documents, all of which will relate in some way to deafness. The drawbacks of this approach are that the information is not organized by theme and that the references are often a mix of information for professionals and patients. Nevertheless, a large number of the listed Web sites provide useful background information. We can only recommend this route, therefore, for relatively rare or specific disorders, or when using highly targeted searches. To use the NIH search utility, visit the following Web page: http://search.nih.gov/index.html. Additional Web Sources A number of Web sites are available to the public that often link to government sites. These can also point you in the direction of essential information. The following is a representative sample: •

AOL: http://search.aol.com/cat.adp?id=168&layer=&from=subcats

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Family Village: http://www.familyvillage.wisc.edu/specific.htm

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Google: http://directory.google.com/Top/Health/Conditions_and_Diseases/

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Med Help International: http://www.medhelp.org/HealthTopics/A.html

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Open Directory Project: http://dmoz.org/Health/Conditions_and_Diseases/

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Yahoo.com: http://dir.yahoo.com/Health/Diseases_and_Conditions/

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WebMDHealth: http://my.webmd.com/health_topics

Associations and Deafness The following is a list of associations that provide information on and resources relating to deafness: •

Alexander Graham Bell Association for the Deaf, Inc Telephone: (202) 337-5220 Toll-free: (800) 432-7543 Fax: (202) 337-8314 Email: [email protected] Web Site: http://www.agbell.org

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Background: The Alexander Graham Bell Association for the Deaf, Inc. is a not-forprofit organization dedicated to helping hearing-impaired individuals by developing maximal use of residual hearing, speech reading, and speech and language skills and promoting better public understanding of hearing loss. The Association seeks to promote detection of hearing loss in early infancy as well as prompt and continued use of appropriate hearing aids and collaborates on research relating to auditory or verbal communication. Established in 1890 by Alexander Graham Bell, the organization is also committed to working for better educational opportunities for affected children; providing in-service training for teachers; and offering scholarships. In addition, the Association disseminates information on hearing impairment, its causes, and options for remedial treatment and collaborates with physicians, audiologists, speech/language specialists, and educators to promote educational, vocational, and social opportunities for affected individuals. The organization also supports legislation beneficial to affected individuals, conducts international conferences, and has one of the world s largest historical collections of information on deafness. The Association answers inquiries on all areas of hearing impairment and provides a variety of materials. These include textbooks, videotapes, and materials for parents; information on hearing aids, lipreading, and auditory training; a magazine entitled 'VOLTA VOICES'; and a journal called the 'VOLTA REVIEW.'. Relevant area(s) of interest: Deafness •

Deafness Research Foundation Telephone: (212) 599-0027 Toll-free: (800) 535-3323 Fax: (212) 768-1782 Email: [email protected] Background: The Deafness Research Foundation is a national voluntary health organization that offers seed research grants to help solve the problems of deafness and other serious ear disorders. Founded in 1958, the Deafness Research Foundation is committed to increasing public awareness about hearing health. The four primary objectives of the Deafness Research Foundation include fostering innovative research and education into the causes, treatments, and prevention of hearing loss and other dysfunctions of the auditory and balance systems; increasing awareness of measures to prevent hearing loss and the need to support research; creating greater understanding about the effect of hearing loss on people s lives; and increasing the number of scientists who are committed to hearing health. The Deafness Research Foundation also publishes materials including brochures on deafness and a grants policy fact sheet. Relevant area(s) of interest: Deafness

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Dogs for the Deaf, Inc Telephone: (541) 826-9220 Fax: (541) 826-6696 Email: [email protected] Background: Dogs for the Deaf, Inc. is a not-for-profit organization dedicated to providing professionally trained 'hearing dogs' free of charge to qualified applicants nationwide. Established in 1977, Dogs for the Deaf is the oldest and largest hearing dog training center in the United States. Dogs for the Deaf selects dogs from adoption shelters. The dogs are usually mixed breeds, small to medium sized, and eight to 24

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months of age. All dogs are spayed or neutered; thereafter, they begin four to six months of intensive specialized training. The dogs are taught to respond to verbal commands and hand signals and are trained to alert their partners to household sounds such as smoke alarms, doorbells, crying babies, telephones, alarm clocks, etc. Dogs for the Deaf provides educational materials including a general brochure and a regular newsletter entitled 'Canine Listener.' All applicants must meet certain requirements before receiving a dog. Dogs for the Deaf has more than 600 recipients of hearing dogs. All applicants must agree to certain requirements including regular training sessions to keep appropriate obedience and alert responses to sound, a fenced yard or area, and no other dogs in the household. Relevant area(s) of interest: Deafness •

Helen Keller National Center for Deaf-Blind Youths and Adults Telephone: (516) 944-8900 Fax: (516) 944-7302 Email: [email protected] Web Site: http://helenkeller.org/national Background: The mission of the Helen Keller National Center is to enable each person who is deaf-blind to live and work in his or her community of choice. The Center provides comprehensive vocational rehabilitation training at its headquarters in New York, as well as assistance with job and residential placements when the training is completed. It has ten regional offices and more than 40 affiliated agencies, a National Training Team and an Older Adult Program. HKNC is a partner in the National Technical Assistance Consortium for Children and Young Adults who are deaf-blind and with DB-LINK, a clearinghouse for information on children who are deaf-blind. HKNC also maintains a national registry of individuals who are deaf-blind.

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John Tracy Clinic for Preschool Deaf Children Telephone: (213) 748-5481 Toll-free: (800) 522-4582 Fax: (213) 749-1651 Email: jtc.org Web Site: http://www.jtc.org Background: The John Tracy Clinic for Preschool Deaf Children is a not-for-profit organization dedicated to providing worldwide parent-centered services to young children with hearing impairment and offering affected families hope, guidance, and encouragement. The Clinic was founded in 1942 by Louise Tracy, wife of actor Spencer Tracy, to provide educational programs, without charge, to young deaf children and their families. The Clinic offers a variety of programs to serve families in Southern California. Free services for children from birth to age six include hearing screenings in preschools and daycare centers throughout the greater Los Angeles area; complete audiological tests at the Clinic s downtown location for any child suspected of having a hearing loss; classes and support groups for parents of hearing-impaired children; and demonstration home programs to show parents how to use activities in the home to guide their infants or newly diagnosed deaf toddlers in the development of communication skills. Additional services include a demonstration preschool program for children from ages two through five that emphasizes language, speech, cognitive, and social development; a correspondence education program to educate and provide

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emotional support to families from all over the United States and worldwide; and, in conjunction with the University of Southern California, a graduate teacher education program to train new teachers of hearing impaired individuals. The John Tracy Clinic is an affiliate of the National Distance Education Council, the National Association of Private Schools for Exceptional Children, the Alexander Graham Bell Association, the Council on the Education of the Deaf, the Conference of the Educational Administration Serving the Deaf, and the Convention of American Instructors of the Deaf. Educational materials include brochures, a regular newsletter entitled 'Bulletin,' and fact sheets. Relevant area(s) of interest: Deafness •

National Association of the Deaf Telephone: (301) 587-1788 Fax: (301) 587-1791 Email: [email protected] Web Site: http://www.nad.org Background: Established in 1880, the National Association of the Deaf (NAD) is the nation's oldest and largest nonprofit organization safeguarding the accessibility and civil rights of 28 million deaf and hard of hearing Americans across a broad range of areas including education, employment, health care, and telecommunications. The NAD is a federation of 51 state association affiliates, organizational affiliates, and national members. Primary areas of focus include grassroots advocacy and empowerment, policy development and research, legal assistance, captioned media, information and publications, and youth leadership. Relevant area(s) of interest: Deafness

•

Telecommunications for the Deaf, Inc Telephone: (301) 589-3786 Fax: (301) 589-3797 Email: [email protected] Web Site: http://www.tdi-online.org Background: Telecommunications for the Deaf, Inc. (TDI) is a non-profit advocacy organization dedicated to promoting full visual access to information and telecommunications through consumer education and involvement; technical assistance and consulting; application of existing and emerging technologies; networking and collaboration; uniformity of standards; and national policy development and advocacy. The or. The organization serves people who are deaf, hear of hearing, deaf/blind, and/or speech impaired. TDI s main objectives are promoting text telephone compatibility with existing communication devices; increasing toll-free services for TTY users; improving emergency access; and improving communications between TTY and other telephone users through comprehensive telecommunications relay services training. Telecommunications for the Deaf, Inc. also develops, implements, and evaluates new strategies for improving the quality and availability of visual telecommunications; encourages progress in key technologies such as television captioning; creates improved specifications and standards for equipment, telecommunications relay, and emergency services; educates the public about and supports legislation to improve telecommunications services; and builds coalitions of consumers and service providers to meet the needs of the deaf, heard of hearing, and

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speech-impaired. In addition, TDI provides training to industry groups and service providers who need assistance in service implementation. TDI provides a comprehensive overview of deafness and deaf culture; explains the influence of American Sign Language on TTY communication; trains personnel in telecommunications relay and emergency services; and conducts needs assessment studies, manages focus groups, and designs surveys to guide service-improvement projects. Relevant area(s) of interest: Deafness

Finding Associations There are several Internet directories that provide lists of medical associations with information on or resources relating to deafness. By consulting all of associations listed in this chapter, you will have nearly exhausted all sources for patient associations concerned with deafness. The National Health Information Center (NHIC) The National Health Information Center (NHIC) offers a free referral service to help people find organizations that provide information about deafness. For more information, see the NHIC’s Web site at http://www.health.gov/NHIC/ or contact an information specialist by calling 1-800-336-4797. Directory of Health Organizations The Directory of Health Organizations, provided by the National Library of Medicine Specialized Information Services, is a comprehensive source of information on associations. The Directory of Health Organizations database can be accessed via the Internet at http://www.sis.nlm.nih.gov/Dir/DirMain.html. It is composed of two parts: DIRLINE and Health Hotlines. The DIRLINE database comprises some 10,000 records of organizations, research centers, and government institutes and associations that primarily focus on health and biomedicine. To access DIRLINE directly, go to the following Web site: http://dirline.nlm.nih.gov/. Simply type in “deafness” (or a synonym), and you will receive information on all relevant organizations listed in the database. Health Hotlines directs you to toll-free numbers to over 300 organizations. You can access this database directly at http://www.sis.nlm.nih.gov/hotlines/. On this page, you are given the option to search by keyword or by browsing the subject list. When you have received your search results, click on the name of the organization for its description and contact information. The Combined Health Information Database Another comprehensive source of information on healthcare associations is the Combined Health Information Database. Using the “Detailed Search” option, you will need to limit

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your search to “Organizations” and “deafness”. Type the following hyperlink into your Web browser: http://chid.nih.gov/detail/detail.html. To find associations, use the drop boxes at the bottom of the search page where “You may refine your search by.” For publication date, select “All Years.” Then, select your preferred language and the format option “Organization Resource Sheet.” Type “deafness” (or synonyms) into the “For these words:” box. You should check back periodically with this database since it is updated every three months. The National Organization for Rare Disorders, Inc. The National Organization for Rare Disorders, Inc. has prepared a Web site that provides, at no charge, lists of associations organized by health topic. You can access this database at the following Web site: http://www.rarediseases.org/search/orgsearch.html. Type “deafness” (or a synonym) into the search box, and click “Submit Query.”

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APPENDIX C. FINDING MEDICAL LIBRARIES Overview In this Appendix, we show you how to quickly find a medical library in your area.

Preparation Your local public library and medical libraries have interlibrary loan programs with the National Library of Medicine (NLM), one of the largest medical collections in the world. According to the NLM, most of the literature in the general and historical collections of the National Library of Medicine is available on interlibrary loan to any library. If you would like to access NLM medical literature, then visit a library in your area that can request the publications for you.27

Finding a Local Medical Library The quickest method to locate medical libraries is to use the Internet-based directory published by the National Network of Libraries of Medicine (NN/LM). This network includes 4626 members and affiliates that provide many services to librarians, health professionals, and the public. To find a library in your area, simply visit http://nnlm.gov/members/adv.html or call 1-800-338-7657.

Medical Libraries in the U.S. and Canada In addition to the NN/LM, the National Library of Medicine (NLM) lists a number of libraries with reference facilities that are open to the public. The following is the NLM’s list and includes hyperlinks to each library’s Web site. These Web pages can provide information on hours of operation and other restrictions. The list below is a small sample of

27

Adapted from the NLM: http://www.nlm.nih.gov/psd/cas/interlibrary.html.

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libraries recommended by the National Library of Medicine (sorted alphabetically by name of the U.S. state or Canadian province where the library is located)28: •

Alabama: Health InfoNet of Jefferson County (Jefferson County Library Cooperative, Lister Hill Library of the Health Sciences), http://www.uab.edu/infonet/

•

Alabama: Richard M. Scrushy Library (American Sports Medicine Institute)

•

Arizona: Samaritan Regional Medical Center: The Learning Center (Samaritan Health System, Phoenix, Arizona), http://www.samaritan.edu/library/bannerlibs.htm

•

California: Kris Kelly Health Information Center (St. Joseph Health System, Humboldt), http://www.humboldt1.com/~kkhic/index.html

•

California: Community Health Library of Los Gatos, http://www.healthlib.org/orgresources.html

•

California: Consumer Health Program and Services (CHIPS) (County of Los Angeles Public Library, Los Angeles County Harbor-UCLA Medical Center Library) - Carson, CA, http://www.colapublib.org/services/chips.html

•

California: Gateway Health Library (Sutter Gould Medical Foundation)

•

California: Health Library (Stanford University Medical Center), http://wwwmed.stanford.edu/healthlibrary/

•

California: Patient Education Resource Center - Health Information and Resources (University of California, San Francisco), http://sfghdean.ucsf.edu/barnett/PERC/default.asp

•

California: Redwood Health Library (Petaluma Health Care District), http://www.phcd.org/rdwdlib.html

•

California: Los Gatos PlaneTree Health Library, http://planetreesanjose.org/

•

California: Sutter Resource Library (Sutter Hospitals Foundation, Sacramento), http://suttermedicalcenter.org/library/

•

California: Health Sciences Libraries (University of California, Davis), http://www.lib.ucdavis.edu/healthsci/

•

California: ValleyCare Health Library & Ryan Comer Cancer Resource Center (ValleyCare Health System, Pleasanton), http://gaelnet.stmarysca.edu/other.libs/gbal/east/vchl.html

•

California: Washington Community Health Resource Library (Fremont), http://www.healthlibrary.org/

•

Colorado: William V. Gervasini Memorial Library (Exempla Healthcare), http://www.saintjosephdenver.org/yourhealth/libraries/

•

Connecticut: Hartford Hospital Health Science Libraries (Hartford Hospital), http://www.harthosp.org/library/

•

Connecticut: Healthnet: Connecticut Consumer Health Information Center (University of Connecticut Health Center, Lyman Maynard Stowe Library), http://library.uchc.edu/departm/hnet/

28

Abstracted from http://www.nlm.nih.gov/medlineplus/libraries.html.

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•

Connecticut: Waterbury Hospital Health Center Library (Waterbury Hospital, Waterbury), http://www.waterburyhospital.com/library/consumer.shtml

•

Delaware: Consumer Health Library (Christiana Care Health System, Eugene du Pont Preventive Medicine & Rehabilitation Institute, Wilmington), http://www.christianacare.org/health_guide/health_guide_pmri_health_info.cfm

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Delaware: Lewis B. Flinn Library (Delaware Academy of Medicine, Wilmington), http://www.delamed.org/chls.html

•

Georgia: Family Resource Library (Medical College of Georgia, Augusta), http://cmc.mcg.edu/kids_families/fam_resources/fam_res_lib/frl.htm

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Georgia: Health Resource Center (Medical Center of Central Georgia, Macon), http://www.mccg.org/hrc/hrchome.asp

•

Hawaii: Hawaii Medical Library: Consumer Health Information Service (Hawaii Medical Library, Honolulu), http://hml.org/CHIS/

•

Idaho: DeArmond Consumer Health Library (Kootenai Medical Center, Coeur d’Alene), http://www.nicon.org/DeArmond/index.htm

•

Illinois: Health Learning Center of Northwestern Memorial Hospital (Chicago), http://www.nmh.org/health_info/hlc.html

•

Illinois: Medical Library (OSF Saint Francis Medical Center, Peoria), http://www.osfsaintfrancis.org/general/library/

•

Kentucky: Medical Library - Services for Patients, Families, Students & the Public (Central Baptist Hospital, Lexington), http://www.centralbap.com/education/community/library.cfm

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Kentucky: University of Kentucky - Health Information Library (Chandler Medical Center, Lexington), http://www.mc.uky.edu/PatientEd/

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Louisiana: Alton Ochsner Medical Foundation Library (Alton Ochsner Medical Foundation, New Orleans), http://www.ochsner.org/library/

•

Louisiana: Louisiana State University Health Sciences Center Medical LibraryShreveport, http://lib-sh.lsuhsc.edu/

•

Maine: Franklin Memorial Hospital Medical Library (Franklin Memorial Hospital, Farmington), http://www.fchn.org/fmh/lib.htm

•

Maine: Gerrish-True Health Sciences Library (Central Maine Medical Center, Lewiston), http://www.cmmc.org/library/library.html

•

Maine: Hadley Parrot Health Science Library (Eastern Maine Healthcare, Bangor), http://www.emh.org/hll/hpl/guide.htm

•

Maine: Maine Medical Center Library (Maine Medical Center, Portland), http://www.mmc.org/library/

•

Maine: Parkview Hospital (Brunswick), http://www.parkviewhospital.org/

•

Maine: Southern Maine Medical Center Health Sciences Library (Southern Maine Medical Center, Biddeford), http://www.smmc.org/services/service.php3?choice=10

•

Maine: Stephens Memorial Hospital’s Health Information Library (Western Maine Health, Norway), http://www.wmhcc.org/Library/

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•

Manitoba, Canada: Consumer & Patient Health Information Service (University of Manitoba Libraries), http://www.umanitoba.ca/libraries/units/health/reference/chis.html

•

Manitoba, Canada: J.W. Crane Memorial Library (Deer Lodge Centre, Winnipeg), http://www.deerlodge.mb.ca/crane_library/about.asp

•

Maryland: Health Information Center at the Wheaton Regional Library (Montgomery County, Dept. of Public Libraries, Wheaton Regional Library), http://www.mont.lib.md.us/healthinfo/hic.asp

•

Massachusetts: Baystate Medical Center Library (Baystate Health System), http://www.baystatehealth.com/1024/

•

Massachusetts: Boston University Medical Center Alumni Medical Library (Boston University Medical Center), http://med-libwww.bu.edu/library/lib.html

•

Massachusetts: Lowell General Hospital Health Sciences Library (Lowell General Hospital, Lowell), http://www.lowellgeneral.org/library/HomePageLinks/WWW.htm

•

Massachusetts: Paul E. Woodard Health Sciences Library (New England Baptist Hospital, Boston), http://www.nebh.org/health_lib.asp

•

Massachusetts: St. Luke’s Hospital Health Sciences Library (St. Luke’s Hospital, Southcoast Health System, New Bedford), http://www.southcoast.org/library/

•

Massachusetts: Treadwell Library Consumer Health Reference Center (Massachusetts General Hospital), http://www.mgh.harvard.edu/library/chrcindex.html

•

Massachusetts: UMass HealthNet (University of Massachusetts Medical School, Worchester), http://healthnet.umassmed.edu/

•

Michigan: Botsford General Hospital Library - Consumer Health (Botsford General Hospital, Library & Internet Services), http://www.botsfordlibrary.org/consumer.htm

•

Michigan: Helen DeRoy Medical Library (Providence Hospital and Medical Centers), http://www.providence-hospital.org/library/

•

Michigan: Marquette General Hospital - Consumer Health Library (Marquette General Hospital, Health Information Center), http://www.mgh.org/center.html

•

Michigan: Patient Education Resouce Center - University of Michigan Cancer Center (University of Michigan Comprehensive Cancer Center, Ann Arbor), http://www.cancer.med.umich.edu/learn/leares.htm

•

Michigan: Sladen Library & Center for Health Information Resources - Consumer Health Information (Detroit), http://www.henryford.com/body.cfm?id=39330

•

Montana: Center for Health Information (St. Patrick Hospital and Health Sciences Center, Missoula)

•

National: Consumer Health Library Directory (Medical Library Association, Consumer and Patient Health Information Section), http://caphis.mlanet.org/directory/index.html

•

National: National Network of Libraries of Medicine (National Library of Medicine) provides library services for health professionals in the United States who do not have access to a medical library, http://nnlm.gov/

•

National: NN/LM List of Libraries Serving the Public (National Network of Libraries of Medicine), http://nnlm.gov/members/

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•

Nevada: Health Science Library, West Charleston Library (Las Vegas-Clark County Library District, Las Vegas), http://www.lvccld.org/special_collections/medical/index.htm

•

New Hampshire: Dartmouth Biomedical Libraries (Dartmouth College Library, Hanover), http://www.dartmouth.edu/~biomed/resources.htmld/conshealth.htmld/

•

New Jersey: Consumer Health Library (Rahway Hospital, Rahway), http://www.rahwayhospital.com/library.htm

•

New Jersey: Dr. Walter Phillips Health Sciences Library (Englewood Hospital and Medical Center, Englewood), http://www.englewoodhospital.com/links/index.htm

•

New Jersey: Meland Foundation (Englewood Hospital and Medical Center, Englewood), http://www.geocities.com/ResearchTriangle/9360/

•

New York: Choices in Health Information (New York Public Library) - NLM Consumer Pilot Project participant, http://www.nypl.org/branch/health/links.html

•

New York: Health Information Center (Upstate Medical University, State University of New York, Syracuse), http://www.upstate.edu/library/hic/

•

New York: Health Sciences Library (Long Island Jewish Medical Center, New Hyde Park), http://www.lij.edu/library/library.html

•

New York: ViaHealth Medical Library (Rochester General Hospital), http://www.nyam.org/library/

•

Ohio: Consumer Health Library (Akron General Medical Center, Medical & Consumer Health Library), http://www.akrongeneral.org/hwlibrary.htm

•

Oklahoma: The Health Information Center at Saint Francis Hospital (Saint Francis Health System, Tulsa), http://www.sfh-tulsa.com/services/healthinfo.asp

•

Oregon: Planetree Health Resource Center (Mid-Columbia Medical Center, The Dalles), http://www.mcmc.net/phrc/

•

Pennsylvania: Community Health Information Library (Milton S. Hershey Medical Center, Hershey), http://www.hmc.psu.edu/commhealth/

•

Pennsylvania: Community Health Resource Library (Geisinger Medical Center, Danville), http://www.geisinger.edu/education/commlib.shtml

•

Pennsylvania: HealthInfo Library (Moses Taylor Hospital, Scranton), http://www.mth.org/healthwellness.html

•

Pennsylvania: Hopwood Library (University of Pittsburgh, Health Sciences Library System, Pittsburgh), http://www.hsls.pitt.edu/guides/chi/hopwood/index_html

•

Pennsylvania: Koop Community Health Information Center (College of Physicians of Philadelphia), http://www.collphyphil.org/kooppg1.shtml

•

Pennsylvania: Learning Resources Center - Medical Library (Susquehanna Health System, Williamsport), http://www.shscares.org/services/lrc/index.asp

•

Pennsylvania: Medical Library (UPMC Health System, Pittsburgh), http://www.upmc.edu/passavant/library.htm

•

Quebec, Canada: Medical Library (Montreal General Hospital), http://www.mghlib.mcgill.ca/

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•

South Dakota: Rapid City Regional Hospital Medical Library (Rapid City Regional Hospital), http://www.rcrh.org/Services/Library/Default.asp

•

Texas: Houston HealthWays (Houston Academy of Medicine-Texas Medical Center Library), http://hhw.library.tmc.edu/

•

Washington: Community Health Library (Kittitas Valley Community Hospital), http://www.kvch.com/

•

Washington: Southwest Washington Medical Center Library (Southwest Washington Medical Center, Vancouver), http://www.swmedicalcenter.com/body.cfm?id=72

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ONLINE GLOSSARIES The Internet provides access to a number of free-to-use medical dictionaries. The National Library of Medicine has compiled the following list of online dictionaries: •

ADAM Medical Encyclopedia (A.D.A.M., Inc.), comprehensive medical reference: http://www.nlm.nih.gov/medlineplus/encyclopedia.html

•

MedicineNet.com Medical Dictionary (MedicineNet, Inc.): http://www.medterms.com/Script/Main/hp.asp

•

Merriam-Webster Medical Dictionary (Inteli-Health, Inc.): http://www.intelihealth.com/IH/

•

Multilingual Glossary of Technical and Popular Medical Terms in Eight European Languages (European Commission) - Danish, Dutch, English, French, German, Italian, Portuguese, and Spanish: http://allserv.rug.ac.be/~rvdstich/eugloss/welcome.html

•

On-line Medical Dictionary (CancerWEB): http://cancerweb.ncl.ac.uk/omd/

•

Rare Diseases Terms (Office of Rare Diseases): http://ord.aspensys.com/asp/diseases/diseases.asp

•

Technology Glossary (National Library of Medicine) - Health Care Technology: http://www.nlm.nih.gov/nichsr/ta101/ta10108.htm

Beyond these, MEDLINEplus contains a very patient-friendly encyclopedia covering every aspect of medicine (licensed from A.D.A.M., Inc.). The ADAM Medical Encyclopedia can be accessed at http://www.nlm.nih.gov/medlineplus/encyclopedia.html. ADAM is also available on commercial Web sites such as drkoop.com (http://www.drkoop.com/) and Web MD (http://my.webmd.com/adam/asset/adam_disease_articles/a_to_z/a).

Online Dictionary Directories The following are additional online directories compiled by the National Library of Medicine, including a number of specialized medical dictionaries: •

Medical Dictionaries: Medical & Biological (World Health Organization): http://www.who.int/hlt/virtuallibrary/English/diction.htm#Medical

•

MEL-Michigan Electronic Library List of Online Health and Medical Dictionaries (Michigan Electronic Library): http://mel.lib.mi.us/health/health-dictionaries.html

•

Patient Education: Glossaries (DMOZ Open Directory Project): http://dmoz.org/Health/Education/Patient_Education/Glossaries/

•

Web of Online Dictionaries (Bucknell University): http://www.yourdictionary.com/diction5.html#medicine

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DEAFNESS DICTIONARY The definitions below are derived from official public sources, including the National Institutes of Health [NIH] and the European Union [EU]. Abdomen: That portion of the body that lies between the thorax and the pelvis. [NIH] Aberrant: Wandering or deviating from the usual or normal course. [EU] Ablation: The removal of an organ by surgery. [NIH] Abscess: Accumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection. [NIH] Acceptor: A substance which, while normally not oxidized by oxygen or reduced by hydrogen, can be oxidized or reduced in presence of a substance which is itself undergoing oxidation or reduction. [NIH] Accommodation: Adjustment, especially that of the eye for various distances. [EU] Acetylcholine: A neurotransmitter. Acetylcholine in vertebrates is the major transmitter at neuromuscular junctions, autonomic ganglia, parasympathetic effector junctions, a subset of sympathetic effector junctions, and at many sites in the central nervous system. It is generally not used as an administered drug because it is broken down very rapidly by cholinesterases, but it is useful in some ophthalmological applications. [NIH] Acidosis: A pathologic condition resulting from accumulation of acid or depletion of the alkaline reserve (bicarbonate content) in the blood and body tissues, and characterized by an increase in hydrogen ion concentration. [EU] Acoustic: Having to do with sound or hearing. [NIH] Actin: Essential component of the cell skeleton. [NIH] Action Potentials: The electric response of a nerve or muscle to its stimulation. [NIH] Activities of Daily Living: The performance of the basic activities of self care, such as dressing, ambulation, eating, etc., in rehabilitation. [NIH] Acuity: Clarity or clearness, especially of the vision. [EU] Acyl: Chemical signal used by bacteria to communicate. [NIH] Adaptability: Ability to develop some form of tolerance to conditions extremely different from those under which a living organism evolved. [NIH] Adaptation: 1. The adjustment of an organism to its environment, or the process by which it enhances such fitness. 2. The normal ability of the eye to adjust itself to variations in the intensity of light; the adjustment to such variations. 3. The decline in the frequency of firing of a neuron, particularly of a receptor, under conditions of constant stimulation. 4. In dentistry, (a) the proper fitting of a denture, (b) the degree of proximity and interlocking of restorative material to a tooth preparation, (c) the exact adjustment of bands to teeth. 5. In microbiology, the adjustment of bacterial physiology to a new environment. [EU] Adjustment: The dynamic process wherein the thoughts, feelings, behavior, and biophysiological mechanisms of the individual continually change to adjust to the environment. [NIH] Adolescence: The period of life beginning with the appearance of secondary sex characteristics and terminating with the cessation of somatic growth. The years usually referred to as adolescence lie between 13 and 18 years of age. [NIH]

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Adrenal Glands: Paired glands situated in the retroperitoneal tissues at the superior pole of each kidney. [NIH] Adverse Effect: An unwanted side effect of treatment. [NIH] Aerobic: In biochemistry, reactions that need oxygen to happen or happen when oxygen is present. [NIH] Affinity: 1. Inherent likeness or relationship. 2. A special attraction for a specific element, organ, or structure. 3. Chemical affinity; the force that binds atoms in molecules; the tendency of substances to combine by chemical reaction. 4. The strength of noncovalent chemical binding between two substances as measured by the dissociation constant of the complex. 5. In immunology, a thermodynamic expression of the strength of interaction between a single antigen-binding site and a single antigenic determinant (and thus of the stereochemical compatibility between them), most accurately applied to interactions among simple, uniform antigenic determinants such as haptens. Expressed as the association constant (K litres mole -1), which, owing to the heterogeneity of affinities in a population of antibody molecules of a given specificity, actually represents an average value (mean intrinsic association constant). 6. The reciprocal of the dissociation constant. [EU] Agar: A complex sulfated polymer of galactose units, extracted from Gelidium cartilagineum, Gracilaria confervoides, and related red algae. It is used as a gel in the preparation of solid culture media for microorganisms, as a bulk laxative, in making emulsions, and as a supporting medium for immunodiffusion and immunoelectrophoresis. [NIH]

Agarose: A polysaccharide complex, free of nitrogen and prepared from agar-agar which is produced by certain seaweeds (red algae). It dissolves in warm water to form a viscid solution. [NIH] Age of Onset: The age or period of life at which a disease or the initial symptoms or manifestations of a disease appear in an individual. [NIH] Agonists: Drugs that trigger an action from a cell or another drug. [NIH] Air-Conduction: The transmission of sound through the external and middle ear to the internal ear. [NIH] Airway: A device for securing unobstructed passage of air into and out of the lungs during general anesthesia. [NIH] Albinism: General term for a number of inherited defects of amino acid metabolism in which there is a deficiency or absence of pigment in the eyes, skin, or hair. [NIH] Alexia: The inability to recognize or comprehend written or printed words. [NIH] Algorithms: A procedure consisting of a sequence of algebraic formulas and/or logical steps to calculate or determine a given task. [NIH] Alkaline: Having the reactions of an alkali. [EU] Alkalosis: A pathological condition that removes acid or adds base to the body fluids. [NIH] Alleles: Mutually exclusive forms of the same gene, occupying the same locus on homologous chromosomes, and governing the same biochemical and developmental process. [NIH] Alopecia: Absence of hair from areas where it is normally present. [NIH] Alternative medicine: Practices not generally recognized by the medical community as standard or conventional medical approaches and used instead of standard treatments. Alternative medicine includes the taking of dietary supplements, megadose vitamins, and herbal preparations; the drinking of special teas; and practices such as massage therapy,

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magnet therapy, spiritual healing, and meditation. [NIH] Amblyopia: A nonspecific term referring to impaired vision. Major subcategories include stimulus deprivation-induced amblyopia and toxic amblyopia. Stimulus deprivationinduced amblopia is a developmental disorder of the visual cortex. A discrepancy between visual information received by the visual cortex from each eye results in abnormal cortical development. Strabismus and refractive errors may cause this condition. Toxic amblyopia is a disorder of the optic nerve which is associated with alcoholism, tobacco smoking, and other toxins and as an adverse effect of the use of some medications. [NIH] Amino Acid Sequence: The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining protein conformation. [NIH] Amino Acids: Organic compounds that generally contain an amino (-NH2) and a carboxyl (COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins. [NIH] Amino Acids: Organic compounds that generally contain an amino (-NH2) and a carboxyl (COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins. [NIH] Ammonia: A colorless alkaline gas. It is formed in the body during decomposition of organic materials during a large number of metabolically important reactions. [NIH] Amniotic Fluid: Amniotic cavity fluid which is produced by the amnion and fetal lungs and kidneys. [NIH] Amplification: The production of additional copies of a chromosomal DNA sequence, found as either intrachromosomal or extrachromosomal DNA. [NIH] Amyloidosis: A group of diseases in which protein is deposited in specific organs (localized amyloidosis) or throughout the body (systemic amyloidosis). Amyloidosis may be either primary (with no known cause) or secondary (caused by another disease, including some types of cancer). Generally, primary amyloidosis affects the nerves, skin, tongue, joints, heart, and liver; secondary amyloidosis often affects the spleen, kidneys, liver, and adrenal glands. [NIH] Anaemia: A reduction below normal in the number of erythrocytes per cu. mm., in the quantity of haemoglobin, or in the volume of packed red cells per 100 ml. of blood which occurs when the equilibrium between blood loss (through bleeding or destruction) and blood production is disturbed. [EU] Anaesthesia: Loss of feeling or sensation. Although the term is used for loss of tactile sensibility, or of any of the other senses, it is applied especially to loss of the sensation of pain, as it is induced to permit performance of surgery or other painful procedures. [EU] Anal: Having to do with the anus, which is the posterior opening of the large bowel. [NIH] Analog: In chemistry, a substance that is similar, but not identical, to another. [NIH] Analogous: Resembling or similar in some respects, as in function or appearance, but not in origin or development;. [EU] Anaphylatoxins: The family of peptides C3a, C4a, C5a, and C5a des-arginine produced in the serum during complement activation. They produce smooth muscle contraction, mast cell histamine release, affect platelet aggregation, and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from strongest to weakest is C5a, C3a, C4a, and C5a des-arginine. The latter is the so-called "classical" anaphylatoxin but shows no spasmogenic activity though it contains some chemotactic ability. [NIH] Anatomical: Pertaining to anatomy, or to the structure of the organism. [EU]

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Anemia: A reduction in the number of circulating erythrocytes or in the quantity of hemoglobin. [NIH] Anesthesia: A state characterized by loss of feeling or sensation. This depression of nerve function is usually the result of pharmacologic action and is induced to allow performance of surgery or other painful procedures. [NIH] Angina: Chest pain that originates in the heart. [NIH] Angina Pectoris: The symptom of paroxysmal pain consequent to myocardial ischemia usually of distinctive character, location and radiation, and provoked by a transient stressful situation during which the oxygen requirements of the myocardium exceed the capacity of the coronary circulation to supply it. [NIH] Animal model: An animal with a disease either the same as or like a disease in humans. Animal models are used to study the development and progression of diseases and to test new treatments before they are given to humans. Animals with transplanted human cancers or other tissues are called xenograft models. [NIH] Anions: Negatively charged atoms, radicals or groups of atoms which travel to the anode or positive pole during electrolysis. [NIH] Anomalies: Birth defects; abnormalities. [NIH] Anterior Cerebral Artery: Artery formed by the bifurcation of the internal carotid artery. Branches of the anterior cerebral artery supply the caudate nucleus, internal capsule, putamen, septal nuclei, gyrus cinguli, and surfaces of the frontal lobe and parietal lobe. [NIH] Antibacterial: A substance that destroys bacteria or suppresses their growth or reproduction. [EU] Antibiotic: A drug used to treat infections caused by bacteria and other microorganisms. [NIH]

Antibodies: Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the antigen that induced their synthesis in cells of the lymphoid series (especially plasma cells), or with an antigen closely related to it. [NIH] Antibody: A type of protein made by certain white blood cells in response to a foreign substance (antigen). Each antibody can bind to only a specific antigen. The purpose of this binding is to help destroy the antigen. Antibodies can work in several ways, depending on the nature of the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen. [NIH] Anticoagulant: A drug that helps prevent blood clots from forming. Also called a blood thinner. [NIH] Anticonvulsant: An agent that prevents or relieves convulsions. [EU] Antigen: Any substance which is capable, under appropriate conditions, of inducing a specific immune response and of reacting with the products of that response, that is, with specific antibody or specifically sensitized T-lymphocytes, or both. Antigens may be soluble substances, such as toxins and foreign proteins, or particulate, such as bacteria and tissue cells; however, only the portion of the protein or polysaccharide molecule known as the antigenic determinant (q.v.) combines with antibody or a specific receptor on a lymphocyte. Abbreviated Ag. [EU] Antigen-Antibody Complex: The complex formed by the binding of antigen and antibody molecules. The deposition of large antigen-antibody complexes leading to tissue damage causes immune complex diseases. [NIH] Anti-inflammatory: Having to do with reducing inflammation. [NIH]

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Antineoplastic: Inhibiting or preventing the development of neoplasms, checking the maturation and proliferation of malignant cells. [EU] Antioxidant: A substance that prevents damage caused by free radicals. Free radicals are highly reactive chemicals that often contain oxygen. They are produced when molecules are split to give products that have unpaired electrons. This process is called oxidation. [NIH] Anuria: Inability to form or excrete urine. [NIH] Anus: The opening of the rectum to the outside of the body. [NIH] Anxiolytic: An anxiolytic or antianxiety agent. [EU] Aphakia: Absence of crystalline lens totally or partially from field of vision, from any cause except after cataract extraction. Aphakia is mainly congenital or as result of lens dislocation and subluxation. [NIH] Aphasia: A cognitive disorder marked by an impaired ability to comprehend or express language in its written or spoken form. This condition is caused by diseases which affect the language areas of the dominant hemisphere. Clinical features are used to classify the various subtypes of this condition. General categories include receptive, expressive, and mixed forms of aphasia. [NIH] Aplasia: Lack of development of an organ or tissue, or of the cellular products from an organ or tissue. [EU] Apnea: A transient absence of spontaneous respiration. [NIH] Aponeurosis: Tendinous expansion consisting of a fibrous or membranous sheath which serves as a fascia to enclose or bind a group of muscles. [NIH] Apoptosis: One of the two mechanisms by which cell death occurs (the other being the pathological process of necrosis). Apoptosis is the mechanism responsible for the physiological deletion of cells and appears to be intrinsically programmed. It is characterized by distinctive morphologic changes in the nucleus and cytoplasm, chromatin cleavage at regularly spaced sites, and the endonucleolytic cleavage of genomic DNA (DNA fragmentation) at internucleosomal sites. This mode of cell death serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth. [NIH] Aptitude: The ability to acquire general or special types of knowledge or skill. [NIH] Aqueous: Having to do with water. [NIH] Arachidonic Acid: An unsaturated, essential fatty acid. It is found in animal and human fat as well as in the liver, brain, and glandular organs, and is a constituent of animal phosphatides. It is formed by the synthesis from dietary linoleic acid and is a precursor in the biosynthesis of prostaglandins, thromboxanes, and leukotrienes. [NIH] Arrhythmia: Any variation from the normal rhythm or rate of the heart beat. [NIH] Arterial: Pertaining to an artery or to the arteries. [EU] Arteries: The vessels carrying blood away from the heart. [NIH] Arteriovenous: Both arterial and venous; pertaining to or affecting an artery and a vein. [EU] Aseptic: Free from infection or septic material; sterile. [EU] Astigmatism: A condition in which the surface of the cornea is not spherical; causes a blurred image to be received at the retina. [NIH] Astrocytes: The largest and most numerous neuroglial cells in the brain and spinal cord. Astrocytes (from "star" cells) are irregularly shaped with many long processes, including those with "end feet" which form the glial (limiting) membrane and directly and indirectly

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contribute to the blood brain barrier. They regulate the extracellular ionic and chemical environment, and "reactive astrocytes" (along with microglia) respond to injury. Astrocytes have high- affinity transmitter uptake systems, voltage-dependent and transmitter-gated ion channels, and can release transmitter, but their role in signaling (as in many other functions) is not well understood. [NIH] Asymptomatic: Having no signs or symptoms of disease. [NIH] Ataxia: Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharnyx, larnyx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or peripheral nerve diseases. Motor ataxia may be associated with cerebellar diseases; cerebral cortex diseases; thalamic diseases; basal ganglia diseases; injury to the red nucleus; and other conditions. [NIH] Atmospheric Pressure: The pressure at any point in an atmosphere due solely to the weight of the atmospheric gases above the point concerned. [NIH] Atresia: Lack of a normal opening from the esophagus, intestines, or anus. [NIH] Atrioventricular: Pertaining to an atrium of the heart and to a ventricle. [EU] Atrium: A chamber; used in anatomical nomenclature to designate a chamber affording entrance to another structure or organ. Usually used alone to designate an atrium of the heart. [EU] Atrophy: Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes. [NIH] Atypical: Irregular; not conformable to the type; in microbiology, applied specifically to strains of unusual type. [EU] Audiologist: Study of hearing including treatment of persons with hearing defects. [NIH] Audiology: The study of hearing and hearing impairment. [NIH] Audiometry: The testing of the acuity of the sense of hearing to determine the thresholds of the lowest intensity levels at which an individual can hear a set of tones. The frequencies between 125 and 8000 Hz are used to test air conduction thresholds, and the frequencies between 250 and 4000 Hz are used to test bone conduction thresholds. [NIH] Audiometry, Speech: Measurement of the ability to hear speech under various conditions of intensity and noise interference using sound-field as well as earphones and bone oscillators. [NIH]

Audition: The sense of hearing. [NIH] Auditory: Pertaining to the sense of hearing. [EU] Auditory Cortex: Area of the temporal lobe concerned with hearing. [NIH] Auditory nerve: The eight cranial nerve; also called vestibulocochlear nerve or acoustic nerve. [NIH] Auditory Perception: The process whereby auditory stimuli are selected, organized and interpreted by the organism; includes speech discrimination. [NIH] Aural: Pertaining to or perceived by the ear, as an aural stimulus. [EU] Autacoids: A chemically diverse group of substances produced by various tissues in the body that cause slow contraction of smooth muscle; they have other intense but varied pharmacologic activities. [NIH] Autonomic: Self-controlling; functionally independent. [EU]

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Autoradiography: A process in which radioactive material within an object produces an image when it is in close proximity to a radiation sensitive emulsion. [NIH] Autosuggestion: Suggestion coming from the subject himself. [NIH] Avian: A plasmodial infection in birds. [NIH] Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body. [NIH] Bacteria: Unicellular prokaryotic microorganisms which generally possess rigid cell walls, multiply by cell division, and exhibit three principal forms: round or coccal, rodlike or bacillary, and spiral or spirochetal. [NIH] Bacterial Physiology: Physiological processes and activities of bacteria. [NIH] Bactericidal: Substance lethal to bacteria; substance capable of killing bacteria. [NIH] Bacteriophage: A virus whose host is a bacterial cell; A virus that exclusively infects bacteria. It generally has a protein coat surrounding the genome (DNA or RNA). One of the coliphages most extensively studied is the lambda phage, which is also one of the most important. [NIH] Bacteriostatic: 1. Inhibiting the growth or multiplication of bacteria. 2. An agent that inhibits the growth or multiplication of bacteria. [EU] Bacterium: Microscopic organism which may have a spherical, rod-like, or spiral unicellular or non-cellular body. Bacteria usually reproduce through asexual processes. [NIH] Basal Ganglia: Large subcortical nuclear masses derived from the telencephalon and located in the basal regions of the cerebral hemispheres. [NIH] Basal Ganglia Diseases: Diseases of the basal ganglia including the putamen; globus pallidus; claustrum; amygdala; and caudate nucleus. Dyskinesias (most notably involuntary movements and alterations of the rate of movement) represent the primary clinical manifestations of these disorders. Common etiologies include cerebrovascular disease; neurodegenerative diseases; and craniocerebral trauma. [NIH] Base: In chemistry, the nonacid part of a salt; a substance that combines with acids to form salts; a substance that dissociates to give hydroxide ions in aqueous solutions; a substance whose molecule or ion can combine with a proton (hydrogen ion); a substance capable of donating a pair of electrons (to an acid) for the formation of a coordinate covalent bond. [EU] Base Sequence: The sequence of purines and pyrimidines in nucleic acids and polynucleotides. It is also called nucleotide or nucleoside sequence. [NIH] Basement Membrane: Ubiquitous supportive tissue adjacent to epithelium and around smooth and striated muscle cells. This tissue contains intrinsic macromolecular components such as collagen, laminin, and sulfated proteoglycans. As seen by light microscopy one of its subdivisions is the basal (basement) lamina. [NIH] Basilar Membrane: A membrane that stretches from the spiral lamina to the basilar crest consisting of an inner and an outer part. The inner part supports the spiral organ of Corti. [NIH]

Behavior Therapy: The application of modern theories of learning and conditioning in the treatment of behavior disorders. [NIH] Benign: Not cancerous; does not invade nearby tissue or spread to other parts of the body. [NIH]

Bewilderment: Impairment or loss of will power. [NIH] Bilateral: Affecting both the right and left side of body. [NIH] Bile: An emulsifying agent produced in the liver and secreted into the duodenum. Its

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composition includes bile acids and salts, cholesterol, and electrolytes. It aids digestion of fats in the duodenum. [NIH] Biochemical: Relating to biochemistry; characterized by, produced by, or involving chemical reactions in living organisms. [EU] Biogenesis: The origin of life. It includes studies of the potential basis for life in organic compounds but excludes studies of the development of altered forms of life through mutation and natural selection, which is evolution. [NIH] Biological response modifier: BRM. A substance that stimulates the body's response to infection and disease. [NIH] Biological Transport: The movement of materials (including biochemical substances and drugs) across cell membranes and epithelial layers, usually by passive diffusion. [NIH] Biopsy: Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body. [NIH] Biosynthesis: The building up of a chemical compound in the physiologic processes of a living organism. [EU] Biotechnology: Body of knowledge related to the use of organisms, cells or cell-derived constituents for the purpose of developing products which are technically, scientifically and clinically useful. Alteration of biologic function at the molecular level (i.e., genetic engineering) is a central focus; laboratory methods used include transfection and cloning technologies, sequence and structure analysis algorithms, computer databases, and gene and protein structure function analysis and prediction. [NIH] Bladder: The organ that stores urine. [NIH] Blast phase: The phase of chronic myelogenous leukemia in which the number of immature, abnormal white blood cells in the bone marrow and blood is extremely high. Also called blast crisis. [NIH] Blepharoptosis: Drooping of the upper lid due to deficient development or paralysis of the levator palpebrae muscle. [NIH] Blood Coagulation: The process of the interaction of blood coagulation factors that results in an insoluble fibrin clot. [NIH] Blood pressure: The pressure of blood against the walls of a blood vessel or heart chamber. Unless there is reference to another location, such as the pulmonary artery or one of the heart chambers, it refers to the pressure in the systemic arteries, as measured, for example, in the forearm. [NIH] Blood vessel: A tube in the body through which blood circulates. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins. [NIH] Blood Viscosity: The internal resistance of the blood to shear forces. The in vitro measure of whole blood viscosity is of limited clinical utility because it bears little relationship to the actual viscosity within the circulation, but an increase in the viscosity of circulating blood can contribute to morbidity in patients suffering from disorders such as sickle cell anemia and polycythemia. [NIH] Blood Volume: Volume of circulating blood. It is the sum of the plasma volume and erythrocyte volume. [NIH] Blot: To transfer DNA, RNA, or proteins to an immobilizing matrix such as nitrocellulose. [NIH]

Body Fluids: Liquid components of living organisms. [NIH] Body Mass Index: One of the anthropometric measures of body mass; it has the highest

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correlation with skinfold thickness or body density. [NIH] Bone Conduction: Sound transmission through the bones of the skull to the inner ear. [NIH] Bone Marrow: The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells. [NIH] Bone scan: A technique to create images of bones on a computer screen or on film. A small amount of radioactive material is injected into a blood vessel and travels through the bloodstream; it collects in the bones and is detected by a scanner. [NIH] Bone-conduction: The means by which sound can reach the inner ear and be heard without traveling via the air in the ear canal or meatus. [NIH] Bowel: The long tube-shaped organ in the abdomen that completes the process of digestion. There is both a small and a large bowel. Also called the intestine. [NIH] Bowel Movement: Body wastes passed through the rectum and anus. [NIH] Brachial: All the nerves from the arm are ripped from the spinal cord. [NIH] Brachial Plexus: The large network of nerve fibers which distributes the innervation of the upper extremity. The brachial plexus extends from the neck into the axilla. In humans, the nerves of the plexus usually originate from the lower cervical and the first thoracic spinal cord segments (C5-C8 and T1), but variations are not uncommon. [NIH] Brachytherapy: A collective term for interstitial, intracavity, and surface radiotherapy. It uses small sealed or partly-sealed sources that may be placed on or near the body surface or within a natural body cavity or implanted directly into the tissues. [NIH] Brain Infarction: The formation of an area of necrosis in the brain, including the cerebral hemispheres (cerebral infarction), thalami, basal ganglia, brain stem (brain stem infarctions), or cerebellum secondary to an insufficiency of arterial or venous blood flow. [NIH] Brain Ischemia: Localized reduction of blood flow to brain tissue due to arterial obtruction or systemic hypoperfusion. This frequently occurs in conjuction with brain hypoxia. Prolonged ischemia is associated with brain infarction. [NIH] Brain Stem: The part of the brain that connects the cerebral hemispheres with the spinal cord. It consists of the mesencephalon, pons, and medulla oblongata. [NIH] Brain Stem Infarctions: Infarctions that occur in the brain stem which is comprised of the midbrain, pons, and medulla. There are several named syndromes characterized by their distinctive clinical manifestations and specific sites of ischemic injury. [NIH] Branch: Most commonly used for branches of nerves, but applied also to other structures. [NIH]

Breakdown: A physical, metal, or nervous collapse. [NIH] Bronchi: The larger air passages of the lungs arising from the terminal bifurcation of the trachea. [NIH] Bronchial: Pertaining to one or more bronchi. [EU] Bronchitis: Inflammation (swelling and reddening) of the bronchi. [NIH] Bronchopulmonary: Pertaining to the lungs and their air passages; both bronchial and pulmonary. [EU] Bronchopulmonary Dysplasia: A chronic lung disease appearing in certain newborn infants

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treated for respiratory distress syndrome with mechanical ventilation and elevated concentration of inspired oxygen. [NIH] Bullous: Pertaining to or characterized by bullae. [EU] Cadherins: A group of functionally related glycoproteins responsible for the calciumdependent cell-to-cell adhesion mechanism. They are divided into subclasses E-, P-, and Ncadherins, which are distinct in immunological specificity and tissue distribution. They promote cell adhesion via a homophilic mechanism. These compounds play a role in the construction of tissues and of the whole animal body. [NIH] Calcium: A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes. [NIH] Calmodulin: A heat-stable, low-molecular-weight activator protein found mainly in the brain and heart. The binding of calcium ions to this protein allows this protein to bind to cyclic nucleotide phosphodiesterases and to adenyl cyclase with subsequent activation. Thereby this protein modulates cyclic AMP and cyclic GMP levels. [NIH] Carbohydrate: An aldehyde or ketone derivative of a polyhydric alcohol, particularly of the pentahydric and hexahydric alcohols. They are so named because the hydrogen and oxygen are usually in the proportion to form water, (CH2O)n. The most important carbohydrates are the starches, sugars, celluloses, and gums. They are classified into mono-, di-, tri-, polyand heterosaccharides. [EU] Carbon Dioxide: A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. [NIH] Carcinogenic: Producing carcinoma. [EU] Carcinogens: Substances that increase the risk of neoplasms in humans or animals. Both genotoxic chemicals, which affect DNA directly, and nongenotoxic chemicals, which induce neoplasms by other mechanism, are included. [NIH] Cardiac: Having to do with the heart. [NIH] Cardiomyopathy: A general diagnostic term designating primary myocardial disease, often of obscure or unknown etiology. [EU] Cardiovascular: Having to do with the heart and blood vessels. [NIH] Cardiovascular Agents: Agents that affect the rate or intensity of cardiac contraction, blood vessel diameter, or blood volume. [NIH] Cardiovascular disease: Any abnormal condition characterized by dysfunction of the heart and blood vessels. CVD includes atherosclerosis (especially coronary heart disease, which can lead to heart attacks), cerebrovascular disease (e.g., stroke), and hypertension (high blood pressure). [NIH] Career Choice: Selection of a type of occupation or profession. [NIH] Carotene: The general name for a group of pigments found in green, yellow, and leafy vegetables, and yellow fruits. The pigments are fat-soluble, unsaturated aliphatic hydrocarbons functioning as provitamins and are converted to vitamin A through enzymatic processes in the intestinal wall. [NIH] Carrier State: The condition of harboring an infective organism without manifesting symptoms of infection. The organism must be readily transmissable to another susceptible

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host. [NIH] Case report: A detailed report of the diagnosis, treatment, and follow-up of an individual patient. Case reports also contain some demographic information about the patient (for example, age, gender, ethnic origin). [NIH] Case series: A group or series of case reports involving patients who were given similar treatment. Reports of case series usually contain detailed information about the individual patients. This includes demographic information (for example, age, gender, ethnic origin) and information on diagnosis, treatment, response to treatment, and follow-up after treatment. [NIH] Cataract: An opacity, partial or complete, of one or both eyes, on or in the lens or capsule, especially an opacity impairing vision or causing blindness. The many kinds of cataract are classified by their morphology (size, shape, location) or etiology (cause and time of occurrence). [EU] Cations: Postively charged atoms, radicals or groups of atoms which travel to the cathode or negative pole during electrolysis. [NIH] Caudal: Denoting a position more toward the cauda, or tail, than some specified point of reference; same as inferior, in human anatomy. [EU] Caudate Nucleus: Elongated gray mass of the neostriatum located adjacent to the lateral ventricle of the brain. [NIH] Cell: The individual unit that makes up all of the tissues of the body. All living things are made up of one or more cells. [NIH] Cell Adhesion: Adherence of cells to surfaces or to other cells. [NIH] Cell Adhesion Molecules: Surface ligands, usually glycoproteins, that mediate cell-to-cell adhesion. Their functions include the assembly and interconnection of various vertebrate systems, as well as maintenance of tissue integration, wound healing, morphogenic movements, cellular migrations, and metastasis. [NIH] Cell Count: A count of the number of cells of a specific kind, usually measured per unit volume of sample. [NIH] Cell Cycle: The complex series of phenomena, occurring between the end of one cell division and the end of the next, by which cellular material is divided between daughter cells. [NIH] Cell Death: The termination of the cell's ability to carry out vital functions such as metabolism, growth, reproduction, responsiveness, and adaptability. [NIH] Cell Differentiation: Progressive restriction of the developmental potential and increasing specialization of function which takes place during the development of the embryo and leads to the formation of specialized cells, tissues, and organs. [NIH] Cell Division: The fission of a cell. [NIH] Cell Lineage: The developmental history of cells as traced from the first division of the original cell or cells in the embryo. [NIH] Cell membrane: Cell membrane = plasma membrane. The structure enveloping a cell, enclosing the cytoplasm, and forming a selective permeability barrier; it consists of lipids, proteins, and some carbohydrates, the lipids thought to form a bilayer in which integral proteins are embedded to varying degrees. [EU] Cell Movement: The movement of cells from one location to another. [NIH] Cell Physiology: Characteristics and physiological processes of cells from cell division to cell death. [NIH]

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Cell Polarity: Orientation of intracellular structures especially with respect to the apical and basolateral domains of the plasma membrane. Polarized cells must direct proteins from the Golgi apparatus to the appropriate domain since tight junctions prevent proteins from diffusing between the two domains. [NIH] Cell proliferation: An increase in the number of cells as a result of cell growth and cell division. [NIH] Cell Respiration: The metabolic process of all living cells (animal and plant) in which oxygen is used to provide a source of energy for the cell. [NIH] Cell Survival: The span of viability of a cell characterized by the capacity to perform certain functions such as metabolism, growth, reproduction, some form of responsiveness, and adaptability. [NIH] Cellulose: A polysaccharide with glucose units linked as in cellobiose. It is the chief constituent of plant fibers, cotton being the purest natural form of the substance. As a raw material, it forms the basis for many derivatives used in chromatography, ion exchange materials, explosives manufacturing, and pharmaceutical preparations. [NIH] Central Nervous System: The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges. [NIH] Cerebellar: Pertaining to the cerebellum. [EU] Cerebellum: Part of the metencephalon that lies in the posterior cranial fossa behind the brain stem. It is concerned with the coordination of movement. [NIH] Cerebral: Of or pertaining of the cerebrum or the brain. [EU] Cerebral hemispheres: The two halves of the cerebrum, the part of the brain that controls muscle functions of the body and also controls speech, emotions, reading, writing, and learning. The right hemisphere controls muscle movement on the left side of the body, and the left hemisphere controls muscle movement on the right side of the body. [NIH] Cerebral Infarction: The formation of an area of necrosis in the cerebrum caused by an insufficiency of arterial or venous blood flow. Infarcts of the cerebrum are generally classified by hemisphere (i.e., left vs. right), lobe (e.g., frontal lobe infarction), arterial distribution (e.g., infarction, anterior cerebral artery), and etiology (e.g., embolic infarction). [NIH]

Cerebral Palsy: Refers to a motor disability caused by a brain dysfunction. [NIH] Cerebrovascular: Pertaining to the blood vessels of the cerebrum, or brain. [EU] Cerebrovascular Disorders: A broad category of disorders characterized by impairment of blood flow in the arteries and veins which supply the brain. These include cerebral infarction; brain ischemia; hypoxia, brain; intracranial embolism and thrombosis; intracranial arteriovenous malformations; and vasculitis, central nervous system. In common usage, the term cerebrovascular disorders is not limited to conditions that affect the cerebrum, but refers to vascular disorders of the entire brain including the diencephalon; brain stem; and cerebellum. [NIH] Cerebrum: The largest part of the brain. It is divided into two hemispheres, or halves, called the cerebral hemispheres. The cerebrum controls muscle functions of the body and also controls speech, emotions, reading, writing, and learning. [NIH] Cervical: Relating to the neck, or to the neck of any organ or structure. Cervical lymph nodes are located in the neck; cervical cancer refers to cancer of the uterine cervix, which is the lower, narrow end (the "neck") of the uterus. [NIH] Character: In current usage, approximately equivalent to personality. The sum of the relatively fixed personality traits and habitual modes of response of an individual. [NIH]

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Chemotactic Factors: Chemical substances that attract or repel cells or organisms. The concept denotes especially those factors released as a result of tissue injury, invasion, or immunologic activity, that attract leukocytes, macrophages, or other cells to the site of infection or insult. [NIH] Child Development: The continuous sequential physiological and psychological maturing of the child from birth up to but not including adolescence. It includes healthy responses to situations, but does not include growth in stature or size (= growth). [NIH] Chloride Channels: Cell membrane glycoproteins selective for chloride ions. [NIH] Cholesterol: The principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils. [NIH] Cholinergic: Resembling acetylcholine in pharmacological action; stimulated by or releasing acetylcholine or a related compound. [EU] Chorioretinitis: Inflammation of the choroid in which the sensory retina becomes edematous and opaque. The inflammatory cells and exudate may burst through the sensory retina to cloud the vitreous body. [NIH] Choroid: The thin, highly vascular membrane covering most of the posterior of the eye between the retina and sclera. [NIH] Chromatin: The material of chromosomes. It is a complex of DNA, histones, and nonhistone proteins (chromosomal proteins, non-histone) found within the nucleus of a cell. [NIH] Chromosomal: Pertaining to chromosomes. [EU] Chromosome: Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes. [NIH] Chronic: A disease or condition that persists or progresses over a long period of time. [NIH] Chronic Disease: Disease or ailment of long duration. [NIH] Chronic lymphocytic leukemia: A slowly progressing disease in which too many white blood cells (called lymphocytes) are found in the body. [NIH] Chronic myelogenous leukemia: CML. A slowly progressing disease in which too many white blood cells are made in the bone marrow. Also called chronic myeloid leukemia or chronic granulocytic leukemia. [NIH] Chronic phase: Refers to the early stages of chronic myelogenous leukemia or chronic lymphocytic leukemia. The number of mature and immature abnormal white blood cells in the bone marrow and blood is higher than normal, but lower than in the accelerated or blast phase. [NIH] Chronic renal: Slow and progressive loss of kidney function over several years, often resulting in end-stage renal disease. People with end-stage renal disease need dialysis or transplantation to replace the work of the kidneys. [NIH] CIS: Cancer Information Service. The CIS is the National Cancer Institute's link to the public, interpreting and explaining research findings in a clear and understandable manner, and providing personalized responses to specific questions about cancer. Access the CIS by calling 1-800-4-CANCER, or by using the Web site at http://cis.nci.nih.gov. [NIH] Cisplatin: An inorganic and water-soluble platinum complex. After undergoing hydrolysis, it reacts with DNA to produce both intra and interstrand crosslinks. These crosslinks appear to impair replication and transcription of DNA. The cytotoxicity of cisplatin correlates with cellular arrest in the G2 phase of the cell cycle. [NIH] Civil Rights: Legal guarantee protecting the individual from attack on personal liberties, right to fair trial, right to vote, and freedom from discrimination on the basis of race,

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religion, national origin, age, or gender. [NIH] Clamp: A u-shaped steel rod used with a pin or wire for skeletal traction in the treatment of certain fractures. [NIH] Clinical Medicine: The study and practice of medicine by direct examination of the patient. [NIH]

Clinical study: A research study in which patients receive treatment in a clinic or other medical facility. Reports of clinical studies can contain results for single patients (case reports) or many patients (case series or clinical trials). [NIH] Clinical trial: A research study that tests how well new medical treatments or other interventions work in people. Each study is designed to test new methods of screening, prevention, diagnosis, or treatment of a disease. [NIH] Clone: The term "clone" has acquired a new meaning. It is applied specifically to the bits of inserted foreign DNA in the hybrid molecules of the population. Each inserted segment originally resided in the DNA of a complex genome amid millions of other DNA segment. [NIH]

Cloning: The production of a number of genetically identical individuals; in genetic engineering, a process for the efficient replication of a great number of identical DNA molecules. [NIH] Cochlea: The part of the internal ear that is concerned with hearing. It forms the anterior part of the labyrinth, is conical, and is placed almost horizontally anterior to the vestibule. [NIH]

Cochlear: Of or pertaining to the cochlea. [EU] Cochlear Diseases: Diseases of the cochlea, the part of the inner ear that is concerned with hearing. [NIH] Cochlear Duct: Spiral tube in the bony canal of the cochlea, lying on its outer wall between the scala vestibuli and scala tympani. [NIH] Cochlear Implantation: Surgical insertion of an electronic device implanted beneath the skin with electrodes to the cochlear nerve to create sound sensation in persons with sensorineural deafness. [NIH] Cochlear Implants: Electronic devices implanted beneath the skin with electrodes to the cochlear nerve to create sound sensation in persons with sensorineural deafness. [NIH] Cochlear Nerve: The cochlear part of the 8th cranial nerve (vestibulocochlear nerve). The cochlear nerve fibers originate from neurons of the spiral ganglion and project peripherally to cochlear hair cells and centrally to the cochlear nuclei (cochlear nucleus) of the brain stem. They mediate the sense of hearing. [NIH] Cochlear Nucleus: The brain stem nucleus that receives the central input from the cochlear nerve. The cochlear nucleus is located lateral and dorsolateral to the inferior cerebellar peduncles and is functionally divided into dorsal and ventral parts. It is tonotopically organized, performs the first stage of central auditory processing, and projects (directly or indirectly) to higher auditory areas including the superior olivary nuclei, the medial geniculi, the inferior colliculi, and the auditory cortex. [NIH] Codon: A set of three nucleotides in a protein coding sequence that specifies individual amino acids or a termination signal (codon, terminator). Most codons are universal, but some organisms do not produce the transfer RNAs (RNA, transfer) complementary to all codons. These codons are referred to as unassigned codons (codons, nonsense). [NIH] Coenzyme: An organic nonprotein molecule, frequently a phosphorylated derivative of a water-soluble vitamin, that binds with the protein molecule (apoenzyme) to form the active

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enzyme (holoenzyme). [EU] Cofactor: A substance, microorganism or environmental factor that activates or enhances the action of another entity such as a disease-causing agent. [NIH] Cognition: Intellectual or mental process whereby an organism becomes aware of or obtains knowledge. [NIH] Cognitive behavior therapy: A system of psychotherapy based on the premise that distorted or dysfunctional thinking, which influences a person's mood or behavior, is common to all psychosocial problems. The focus of therapy is to identify the distorted thinking and to replace it with more rational, adaptive thoughts and beliefs. [NIH] Collagen: A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of skin, connective tissue, and the organic substance of bones and teeth. Different forms of collagen are produced in the body but all consist of three alpha-polypeptide chains arranged in a triple helix. Collagen is differentiated from other fibrous proteins, such as elastin, by the content of proline, hydroxyproline, and hydroxylysine; by the absence of tryptophan; and particularly by the high content of polar groups which are responsible for its swelling properties. [NIH] Collapse: 1. A state of extreme prostration and depression, with failure of circulation. 2. Abnormal falling in of the walls of any part of organ. [EU] Communication Barriers: Those factors, such as language or sociocultural relationships, which interfere in the meaningful interpretation and transmission of ideas between individuals or groups. [NIH] Competency: The capacity of the bacterium to take up DNA from its surroundings. [NIH] Complement: A term originally used to refer to the heat-labile factor in serum that causes immune cytolysis, the lysis of antibody-coated cells, and now referring to the entire functionally related system comprising at least 20 distinct serum proteins that is the effector not only of immune cytolysis but also of other biologic functions. Complement activation occurs by two different sequences, the classic and alternative pathways. The proteins of the classic pathway are termed 'components of complement' and are designated by the symbols C1 through C9. C1 is a calcium-dependent complex of three distinct proteins C1q, C1r and C1s. The proteins of the alternative pathway (collectively referred to as the properdin system) and complement regulatory proteins are known by semisystematic or trivial names. Fragments resulting from proteolytic cleavage of complement proteins are designated with lower-case letter suffixes, e.g., C3a. Inactivated fragments may be designated with the suffix 'i', e.g. C3bi. Activated components or complexes with biological activity are designated by a bar over the symbol e.g. C1 or C4b,2a. The classic pathway is activated by the binding of C1 to classic pathway activators, primarily antigen-antibody complexes containing IgM, IgG1, IgG3; C1q binds to a single IgM molecule or two adjacent IgG molecules. The alternative pathway can be activated by IgA immune complexes and also by nonimmunologic materials including bacterial endotoxins, microbial polysaccharides, and cell walls. Activation of the classic pathway triggers an enzymatic cascade involving C1, C4, C2 and C3; activation of the alternative pathway triggers a cascade involving C3 and factors B, D and P. Both result in the cleavage of C5 and the formation of the membrane attack complex. Complement activation also results in the formation of many biologically active complement fragments that act as anaphylatoxins, opsonins, or chemotactic factors. [EU] Complementary and alternative medicine: CAM. Forms of treatment that are used in addition to (complementary) or instead of (alternative) standard treatments. These practices are not considered standard medical approaches. CAM includes dietary supplements, megadose vitamins, herbal preparations, special teas, massage therapy, magnet therapy, spiritual healing, and meditation. [NIH]

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Complementary medicine: Practices not generally recognized by the medical community as standard or conventional medical approaches and used to enhance or complement the standard treatments. Complementary medicine includes the taking of dietary supplements, megadose vitamins, and herbal preparations; the drinking of special teas; and practices such as massage therapy, magnet therapy, spiritual healing, and meditation. [NIH] Complementation: The production of a wild-type phenotype when two different mutations are combined in a diploid or a heterokaryon and tested in trans-configuration. [NIH] Computational Biology: A field of biology concerned with the development of techniques for the collection and manipulation of biological data, and the use of such data to make biological discoveries or predictions. This field encompasses all computational methods and theories applicable to molecular biology and areas of computer-based techniques for solving biological problems including manipulation of models and datasets. [NIH] Computed tomography: CT scan. A series of detailed pictures of areas inside the body, taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called computerized tomography and computerized axial tomography (CAT) scan. [NIH] Computer Systems: Systems composed of a computer or computers, peripheral equipment, such as disks, printers, and terminals, and telecommunications capabilities. [NIH] Computerized axial tomography: A series of detailed pictures of areas inside the body, taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography (CT scan), or computerized tomography. [NIH] Computerized tomography: A series of detailed pictures of areas inside the body, taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called computerized axial tomography (CAT) scan and computed tomography (CT scan). [NIH] Concomitant: Accompanying; accessory; joined with another. [EU] Conduction: The transfer of sound waves, heat, nervous impulses, or electricity. [EU] Cone: One of the special retinal receptor elements which are presumed to be primarily concerned with perception of light and color stimuli when the eye is adapted to light. [NIH] Confusion: A mental state characterized by bewilderment, emotional disturbance, lack of clear thinking, and perceptual disorientation. [NIH] Conjugated: Acting or operating as if joined; simultaneous. [EU] Connective Tissue: Tissue that supports and binds other tissues. It consists of connective tissue cells embedded in a large amount of extracellular matrix. [NIH] Connective Tissue: Tissue that supports and binds other tissues. It consists of connective tissue cells embedded in a large amount of extracellular matrix. [NIH] Connexins: A group of homologous proteins which form the intermembrane channels of gap junctions. The connexins are the products of an identified gene family which has both highly conserved and highly divergent regions. The variety contributes to the wide range of functional properties of gap junctions. [NIH] Consciousness: Sense of awareness of self and of the environment. [NIH] Constitutional: 1. Affecting the whole constitution of the body; not local. 2. Pertaining to the constitution. [EU] Constriction: The act of constricting. [NIH] Consultation: A deliberation between two or more physicians concerning the diagnosis and

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the proper method of treatment in a case. [NIH] Consumer Organizations: Organized groups of users of goods and services. [NIH] Consumption: Pulmonary tuberculosis. [NIH] Continuum: An area over which the vegetation or animal population is of constantly changing composition so that homogeneous, separate communities cannot be distinguished. [NIH]

Contraindications: Any factor or sign that it is unwise to pursue a certain kind of action or treatment, e. g. giving a general anesthetic to a person with pneumonia. [NIH] Contralateral: Having to do with the opposite side of the body. [NIH] Contrast Sensitivity: The ability to detect sharp boundaries (stimuli) and to detect slight changes in luminance at regions without distinct contours. Psychophysical measurements of this visual function are used to evaluate visual acuity and to detect eye disease. [NIH] Control group: In a clinical trial, the group that does not receive the new treatment being studied. This group is compared to the group that receives the new treatment, to see if the new treatment works. [NIH] Conventional therapy: A currently accepted and widely used treatment for a certain type of disease, based on the results of past research. Also called conventional treatment. [NIH] Conventional treatment: A currently accepted and widely used treatment for a certain type of disease, based on the results of past research. Also called conventional therapy. [NIH] Convulsions: A general term referring to sudden and often violent motor activity of cerebral or brainstem origin. Convulsions may also occur in the absence of an electrical cerebral discharge (e.g., in response to hypotension). [NIH] Cornea: The transparent part of the eye that covers the iris and the pupil and allows light to enter the inside. [NIH] Corneum: The superficial layer of the epidermis containing keratinized cells. [NIH] Coronary: Encircling in the manner of a crown; a term applied to vessels; nerves, ligaments, etc. The term usually denotes the arteries that supply the heart muscle and, by extension, a pathologic involvement of them. [EU] Coronary Circulation: The circulation of blood through the coronary vessels of the heart. [NIH]

Coronary heart disease: A type of heart disease caused by narrowing of the coronary arteries that feed the heart, which needs a constant supply of oxygen and nutrients carried by the blood in the coronary arteries. When the coronary arteries become narrowed or clogged by fat and cholesterol deposits and cannot supply enough blood to the heart, CHD results. [NIH] Coronary Thrombosis: Presence of a thrombus in a coronary artery, often causing a myocardial infarction. [NIH] Corpus: The body of the uterus. [NIH] Corpus Striatum: Striped gray and white matter consisting of the neostriatum and paleostriatum (globus pallidus). It is located in front of and lateral to the thalamus in each cerebral hemisphere. The gray substance is made up of the caudate nucleus and the lentiform nucleus (the latter consisting of the globus pallidus and putamen). The white matter is the internal capsule. [NIH] Cortex: The outer layer of an organ or other body structure, as distinguished from the internal substance. [EU] Cortical: Pertaining to or of the nature of a cortex or bark. [EU]

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Cortices: The outer layer of an organ; used especially of the cerebrum and cerebellum. [NIH] Corticosteroids: Hormones that have antitumor activity in lymphomas and lymphoid leukemias; in addition, corticosteroids (steroids) may be used for hormone replacement and for the management of some of the complications of cancer and its treatment. [NIH] Cortisone: A natural steroid hormone produced in the adrenal gland. It can also be made in the laboratory. Cortisone reduces swelling and can suppress immune responses. [NIH] Cranial: Pertaining to the cranium, or to the anterior (in animals) or superior (in humans) end of the body. [EU] Craniocerebral Trauma: Traumatic injuries involving the cranium and intracranial structures (i.e., brain; cranial nerves; meninges; and other structures). Injuries may be classified by whether or not the skull is penetrated (i.e., penetrating vs. nonpenetrating) or whether there is an associated hemorrhage. [NIH] Criterion: A standard by which something may be judged. [EU] Crossing-over: The exchange of corresponding segments between chromatids of homologous chromosomes during meiosia, forming a chiasma. [NIH] Cues: Signals for an action; that specific portion of a perceptual field or pattern of stimuli to which a subject has learned to respond. [NIH] Curative: Tending to overcome disease and promote recovery. [EU] Cutaneous: Having to do with the skin. [NIH] Cyclic: Pertaining to or occurring in a cycle or cycles; the term is applied to chemical compounds that contain a ring of atoms in the nucleus. [EU] Cyclophosphamide: Precursor of an alkylating nitrogen mustard antineoplastic and immunosuppressive agent that must be activated in the liver to form the active aldophosphamide. It is used in the treatment of lymphomas, leukemias, etc. Its side effect, alopecia, has been made use of in defleecing sheep. Cyclophosphamide may also cause sterility, birth defects, mutations, and cancer. [NIH] Cyst: A sac or capsule filled with fluid. [NIH] Cysteine: A thiol-containing non-essential amino acid that is oxidized to form cystine. [NIH] Cystine: A covalently linked dimeric nonessential amino acid formed by the oxidation of cysteine. Two molecules of cysteine are joined together by a disulfide bridge to form cystine. [NIH]

Cytochrome: Any electron transfer hemoprotein having a mode of action in which the transfer of a single electron is effected by a reversible valence change of the central iron atom of the heme prosthetic group between the +2 and +3 oxidation states; classified as cytochromes a in which the heme contains a formyl side chain, cytochromes b, which contain protoheme or a closely similar heme that is not covalently bound to the protein, cytochromes c in which protoheme or other heme is covalently bound to the protein, and cytochromes d in which the iron-tetrapyrrole has fewer conjugated double bonds than the hemes have. Well-known cytochromes have been numbered consecutively within groups and are designated by subscripts (beginning with no subscript), e.g. cytochromes c, c1, C2, . New cytochromes are named according to the wavelength in nanometres of the absorption maximum of the a-band of the iron (II) form in pyridine, e.g., c-555. [EU] Cytogenetics: A branch of genetics which deals with the cytological and molecular behavior of genes and chromosomes during cell division. [NIH] Cytokine: Small but highly potent protein that modulates the activity of many cell types, including T and B cells. [NIH]

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Cytoplasm: The protoplasm of a cell exclusive of that of the nucleus; it consists of a continuous aqueous solution (cytosol) and the organelles and inclusions suspended in it (phaneroplasm), and is the site of most of the chemical activities of the cell. [EU] Cytoskeleton: The network of filaments, tubules, and interconnecting filamentous bridges which give shape, structure, and organization to the cytoplasm. [NIH] Cytotoxic: Cell-killing. [NIH] Cytotoxicity: Quality of being capable of producing a specific toxic action upon cells of special organs. [NIH] Data Collection: Systematic gathering of data for a particular purpose from various sources, including questionnaires, interviews, observation, existing records, and electronic devices. The process is usually preliminary to statistical analysis of the data. [NIH] Databases, Bibliographic: Extensive collections, reputedly complete, of references and citations to books, articles, publications, etc., generally on a single subject or specialized subject area. Databases can operate through automated files, libraries, or computer disks. The concept should be differentiated from factual databases which is used for collections of data and facts apart from bibliographic references to them. [NIH] De novo: In cancer, the first occurrence of cancer in the body. [NIH] Deamination: The removal of an amino group (NH2) from a chemical compound. [NIH] Defense Mechanisms: Unconscious process used by an individual or a group of individuals in order to cope with impulses, feelings or ideas which are not acceptable at their conscious level; various types include reaction formation, projection and self reversal. [NIH] Degenerative: Undergoing degeneration : tending to degenerate; having the character of or involving degeneration; causing or tending to cause degeneration. [EU] Dehydration: The condition that results from excessive loss of body water. [NIH] Deletion: A genetic rearrangement through loss of segments of DNA (chromosomes), bringing sequences, which are normally separated, into close proximity. [NIH] Dementia: An acquired organic mental disorder with loss of intellectual abilities of sufficient severity to interfere with social or occupational functioning. The dysfunction is multifaceted and involves memory, behavior, personality, judgment, attention, spatial relations, language, abstract thought, and other executive functions. The intellectual decline is usually progressive, and initially spares the level of consciousness. [NIH] Dendrites: Extensions of the nerve cell body. They are short and branched and receive stimuli from other neurons. [NIH] Density: The logarithm to the base 10 of the opacity of an exposed and processed film. [NIH] Dentists: Individuals licensed to practice dentistry. [NIH] Depolarization: The process or act of neutralizing polarity. In neurophysiology, the reversal of the resting potential in excitable cell membranes when stimulated, i.e., the tendency of the cell membrane potential to become positive with respect to the potential outside the cell. [EU] Deprivation: Loss or absence of parts, organs, powers, or things that are needed. [EU] Dermatitis: Any inflammation of the skin. [NIH] Developed Countries: Countries that have reached a level of economic achievement through an increase of production, per capita income and consumption, and utilization of natural and human resources. [NIH] Developing Countries: Countries in the process of change directed toward economic growth, that is, an increase in production, per capita consumption, and income. The process

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of economic growth involves better utilization of natural and human resources, which results in a change in the social, political, and economic structures. [NIH] Diabetes Mellitus: A heterogeneous group of disorders that share glucose intolerance in common. [NIH] Diabetic Retinopathy: Retinopathy associated with diabetes mellitus, which may be of the background type, progressively characterized by microaneurysms, interretinal punctuate macular edema, or of the proliferative type, characterized by neovascularization of the retina and optic disk, which may project into the vitreous, proliferation of fibrous tissue, vitreous hemorrhage, and retinal detachment. [NIH] Diagnostic procedure: A method used to identify a disease. [NIH] Diagnostic Services: Organized services for the purpose of providing diagnosis to promote and maintain health. [NIH] Diarrhea: Passage of excessively liquid or excessively frequent stools. [NIH] Diastolic: Of or pertaining to the diastole. [EU] Diathermy: The induction of local hyperthermia by either short radio waves or highfrequency sound waves. [NIH] Diencephalon: The paired caudal parts of the prosencephalon from which the thalamus, hypothalamus, epithalamus, and subthalamus are derived. [NIH] Diffusion: The tendency of a gas or solute to pass from a point of higher pressure or concentration to a point of lower pressure or concentration and to distribute itself throughout the available space; a major mechanism of biological transport. [NIH] Digestive system: The organs that take in food and turn it into products that the body can use to stay healthy. Waste products the body cannot use leave the body through bowel movements. The digestive system includes the salivary glands, mouth, esophagus, stomach, liver, pancreas, gallbladder, small and large intestines, and rectum. [NIH] Dihydrotestosterone: Anabolic agent. [NIH] Dilatation: The act of dilating. [NIH] Diploid: Having two sets of chromosomes. [NIH] Direct: 1. Straight; in a straight line. 2. Performed immediately and without the intervention of subsidiary means. [EU] Discrimination: The act of qualitative and/or quantitative differentiation between two or more stimuli. [NIH] Dislocation: The displacement of any part, more especially of a bone. Called also luxation. [EU]

Disorientation: The loss of proper bearings, or a state of mental confusion as to time, place, or identity. [EU] Dissociation: 1. The act of separating or state of being separated. 2. The separation of a molecule into two or more fragments (atoms, molecules, ions, or free radicals) produced by the absorption of light or thermal energy or by solvation. 3. In psychology, a defense mechanism in which a group of mental processes are segregated from the rest of a person's mental activity in order to avoid emotional distress, as in the dissociative disorders (q.v.), or in which an idea or object is segregated from its emotional significance; in the first sense it is roughly equivalent to splitting, in the second, to isolation. 4. A defect of mental integration in which one or more groups of mental processes become separated off from normal consciousness and, thus separated, function as a unitary whole. [EU] Distal: Remote; farther from any point of reference; opposed to proximal. In dentistry, used

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to designate a position on the dental arch farther from the median line of the jaw. [EU] Dizziness: An imprecise term which may refer to a sense of spatial disorientation, motion of the environment, or lightheadedness. [NIH] Domesticated: Species in which the evolutionary process has been influenced by humans to meet their needs. [NIH] Dominance: In genetics, the full phenotypic expression of a gene in both heterozygotes and homozygotes. [EU] Dorsal: 1. Pertaining to the back or to any dorsum. 2. Denoting a position more toward the back surface than some other object of reference; same as posterior in human anatomy; superior in the anatomy of quadrupeds. [EU] Dorsum: A plate of bone which forms the posterior boundary of the sella turcica. [NIH] Drive: A state of internal activity of an organism that is a necessary condition before a given stimulus will elicit a class of responses; e.g., a certain level of hunger (drive) must be present before food will elicit an eating response. [NIH] Drug Interactions: The action of a drug that may affect the activity, metabolism, or toxicity of another drug. [NIH] Duct: A tube through which body fluids pass. [NIH] Dura mater: The outermost, toughest, and most fibrous of the three membranes (meninges) covering the brain and spinal cord; called also pachymeninx. [EU] Dyes: Chemical substances that are used to stain and color other materials. The coloring may or may not be permanent. Dyes can also be used as therapeutic agents and test reagents in medicine and scientific research. [NIH] Dynein: A transport protein that normally binds proteins to the microtubule. [NIH] Dyslexia: Partial alexia in which letters but not words may be read, or in which words may be read but not understood. [NIH] Dysplasia: Cells that look abnormal under a microscope but are not cancer. [NIH] Dystonia: Disordered tonicity of muscle. [EU] Dystrophy: Any disorder arising from defective or faulty nutrition, especially the muscular dystrophies. [EU] Ear Diseases: Diseases of the ear, general or unspecified. [NIH] Eardrum: A thin, tense membrane forming the greater part of the outer wall of the tympanic cavity and separating it from the external auditory meatus; it constitutes the boundary between the external and middle ear. [NIH] Ectopic: Pertaining to or characterized by ectopia. [EU] Eczema: A pruritic papulovesicular dermatitis occurring as a reaction to many endogenous and exogenous agents (Dorland, 27th ed). [NIH] Edema: Excessive amount of watery fluid accumulated in the intercellular spaces, most commonly present in subcutaneous tissue. [NIH] Effector: It is often an enzyme that converts an inactive precursor molecule into an active second messenger. [NIH] Efficacy: The extent to which a specific intervention, procedure, regimen, or service produces a beneficial result under ideal conditions. Ideally, the determination of efficacy is based on the results of a randomized control trial. [NIH] Elastin: The protein that gives flexibility to tissues. [NIH]

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Elective: Subject to the choice or decision of the patient or physician; applied to procedures that are advantageous to the patient but not urgent. [EU] Electrocardiogram: Measurement of electrical activity during heartbeats. [NIH] Electrode: Component of the pacing system which is at the distal end of the lead. It is the interface with living cardiac tissue across which the stimulus is transmitted. [NIH] Electrolyte: A substance that dissociates into ions when fused or in solution, and thus becomes capable of conducting electricity; an ionic solute. [EU] Electrons: Stable elementary particles having the smallest known negative charge, present in all elements; also called negatrons. Positively charged electrons are called positrons. The numbers, energies and arrangement of electrons around atomic nuclei determine the chemical identities of elements. Beams of electrons are called cathode rays or beta rays, the latter being a high-energy biproduct of nuclear decay. [NIH] Electrophysiological: Pertaining to electrophysiology, that is a branch of physiology that is concerned with the electric phenomena associated with living bodies and involved in their functional activity. [EU] Embolus: Bit of foreign matter which enters the blood stream at one point and is carried until it is lodged or impacted in an artery and obstructs it. It may be a blood clot, an air bubble, fat or other tissue, or clumps of bacteria. [NIH] Embryo: The prenatal stage of mammalian development characterized by rapid morphological changes and the differentiation of basic structures. [NIH] Embryology: The study of the development of an organism during the embryonic and fetal stages of life. [NIH] Empirical: A treatment based on an assumed diagnosis, prior to receiving confirmatory laboratory test results. [NIH] Emulsion: A preparation of one liquid distributed in small globules throughout the body of a second liquid. The dispersed liquid is the discontinuous phase, and the dispersion medium is the continuous phase. When oil is the dispersed liquid and an aqueous solution is the continuous phase, it is known as an oil-in-water emulsion, whereas when water or aqueous solution is the dispersed phase and oil or oleaginous substance is the continuous phase, it is known as a water-in-oil emulsion. Pharmaceutical emulsions for which official standards have been promulgated include cod liver oil emulsion, cod liver oil emulsion with malt, liquid petrolatum emulsion, and phenolphthalein in liquid petrolatum emulsion. [EU] Encephalitis: Inflammation of the brain due to infection, autoimmune processes, toxins, and other conditions. Viral infections (see encephalitis, viral) are a relatively frequent cause of this condition. [NIH] Encephalitis, Viral: Inflammation of brain parenchymal tissue as a result of viral infection. Encephalitis may occur as primary or secondary manifestation of Togaviridae infections; Herpesviridae infections; Adenoviridae infections; Flaviviridae infections; Bunyaviridae infections; Picornaviridae infections; Paramyxoviridae infections; Orthomyxoviridae infections; Retroviridae infections; and Arenaviridae infections. [NIH] Endemic: Present or usually prevalent in a population or geographical area at all times; said of a disease or agent. Called also endemial. [EU] Endocytosis: Cellular uptake of extracellular materials within membrane-limited vacuoles or microvesicles. Endosomes play a central role in endocytosis. [NIH] Endolymph: The fluid contained in the membranous labyrinth of the ear. [NIH] Endolymphatic Duct: Duct connecting the endolymphatic sac with the membranous

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labyrinth. [NIH] Endolymphatic Sac: The blind pouch at the end of the endolymphatic duct. [NIH] Endotoxins: Toxins closely associated with the living cytoplasm or cell wall of certain microorganisms, which do not readily diffuse into the culture medium, but are released upon lysis of the cells. [NIH] End-stage renal: Total chronic kidney failure. When the kidneys fail, the body retains fluid and harmful wastes build up. A person with ESRD needs treatment to replace the work of the failed kidneys. [NIH] Enhancer: Transcriptional element in the virus genome. [NIH] Environmental Exposure: The exposure to potentially harmful chemical, physical, or biological agents in the environment or to environmental factors that may include ionizing radiation, pathogenic organisms, or toxic chemicals. [NIH] Environmental Health: The science of controlling or modifying those conditions, influences, or forces surrounding man which relate to promoting, establishing, and maintaining health. [NIH]

Enzymatic: Phase where enzyme cuts the precursor protein. [NIH] Enzyme: A protein that speeds up chemical reactions in the body. [NIH] Epidemic: Occurring suddenly in numbers clearly in excess of normal expectancy; said especially of infectious diseases but applied also to any disease, injury, or other healthrelated event occurring in such outbreaks. [EU] Epidemiological: Relating to, or involving epidemiology. [EU] Epidermal: Pertaining to or resembling epidermis. Called also epidermic or epidermoid. [EU] Epidermis: Nonvascular layer of the skin. It is made up, from within outward, of five layers: 1) basal layer (stratum basale epidermidis); 2) spinous layer (stratum spinosum epidermidis); 3) granular layer (stratum granulosum epidermidis); 4) clear layer (stratum lucidum epidermidis); and 5) horny layer (stratum corneum epidermidis). [NIH] Epinephrine: The active sympathomimetic hormone from the adrenal medulla in most species. It stimulates both the alpha- and beta- adrenergic systems, causes systemic vasoconstriction and gastrointestinal relaxation, stimulates the heart, and dilates bronchi and cerebral vessels. It is used in asthma and cardiac failure and to delay absorption of local anesthetics. [NIH] Epithelial: Refers to the cells that line the internal and external surfaces of the body. [NIH] Epithelial Cells: Cells that line the inner and outer surfaces of the body. [NIH] Epithelium: One or more layers of epithelial cells, supported by the basal lamina, which covers the inner or outer surfaces of the body. [NIH] Equipment and Supplies: Expendable and nonexpendable equipment, supplies, apparatus, and instruments that are used in diagnostic, surgical, therapeutic, scientific, and experimental procedures. [NIH] Erythrocytes: Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing hemoglobin whose function is to transport oxygen. [NIH] Esophagus: The muscular tube through which food passes from the throat to the stomach. [NIH]

Esotropia: A form of ocular misalignment characterized by an excessive convergence of the visual axes, resulting in a "cross-eye" appearance. An example of this condition occurs when paralysis of the lateral rectus muscle causes an abnormal inward deviation of one eye on attempted gaze. [NIH]

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Essential Tremor: A rhythmic, involuntary, purposeless, oscillating movement resulting from the alternate contraction and relaxation of opposing groups of muscles. [NIH] Eukaryotic Cells: Cells of the higher organisms, containing a true nucleus bounded by a nuclear membrane. [NIH] Eustachian tube: The middle ear cavity is in communication with the back of the nose through the Eustachian tube, which is normally closed, but opens on swallowing, in order to maintain equal air pressure. [NIH] Evoke: The electric response recorded from the cerebral cortex after stimulation of a peripheral sense organ. [NIH] Evoked Potentials: The electric response evoked in the central nervous system by stimulation of sensory receptors or some point on the sensory pathway leading from the receptor to the cortex. The evoked stimulus can be auditory, somatosensory, or visual, although other modalities have been reported. Event-related potentials is sometimes used synonymously with evoked potentials but is often associated with the execution of a motor, cognitive, or psychophysiological task, as well as with the response to a stimulus. [NIH] Excitability: Property of a cardiac cell whereby, when the cell is depolarized to a critical level (called threshold), the membrane becomes permeable and a regenerative inward current causes an action potential. [NIH] Excitation: An act of irritation or stimulation or of responding to a stimulus; the addition of energy, as the excitation of a molecule by absorption of photons. [EU] Excitatory: When cortical neurons are excited, their output increases and each new input they receive while they are still excited raises their output markedly. [NIH] Excrete: To get rid of waste from the body. [NIH] Exercise Test: Controlled physical activity, more strenuous than at rest, which is performed in order to allow assessment of physiological functions, particularly cardiovascular and pulmonary, but also aerobic capacity. Maximal (most intense) exercise is usually required but submaximal exercise is also used. The intensity of exercise is often graded, using criteria such as rate of work done, oxygen consumption, and heart rate. Physiological data obtained from an exercise test may be used for diagnosis, prognosis, and evaluation of disease severity, and to evaluate therapy. Data may also be used in prescribing exercise by determining a person's exercise capacity. [NIH] Exogenous: Developed or originating outside the organism, as exogenous disease. [EU] Exons: Coding regions of messenger RNA included in the genetic transcript which survive the processing of RNA in cell nuclei to become part of a spliced messenger of structural RNA in the cytoplasm. They include joining and diversity exons of immunoglobulin genes. [NIH]

Exotropia: A form of ocular misalignment where the visual axes diverge inappropriately. For example, medial rectus muscle weakness may produce this condition as the affected eye will deviate laterally upon attempted forward gaze. An exotropia occurs due to the relatively unopposed force exerted on the eye by the lateral rectus muscle, which pulls the eye in an outward direction. [NIH] Extensor: A muscle whose contraction tends to straighten a limb; the antagonist of a flexor. [NIH]

External-beam radiation: Radiation therapy that uses a machine to aim high-energy rays at the cancer. Also called external radiation. [NIH] Extracellular: Outside a cell or cells. [EU] Extracellular Matrix: A meshwork-like substance found within the extracellular space and

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in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere. [NIH] Extracellular Space: Interstitial space between cells, occupied by fluid as well as amorphous and fibrous substances. [NIH] Extraction: The process or act of pulling or drawing out. [EU] Extremity: A limb; an arm or leg (membrum); sometimes applied specifically to a hand or foot. [EU] Facial: Of or pertaining to the face. [EU] Facial Nerve: The 7th cranial nerve. The facial nerve has two parts, the larger motor root which may be called the facial nerve proper, and the smaller intermediate or sensory root. Together they provide efferent innervation to the muscles of facial expression and to the lacrimal and salivary glands, and convey afferent information for taste from the anterior two-thirds of the tongue and for touch from the external ear. [NIH] Family Planning: Programs or services designed to assist the family in controlling reproduction by either improving or diminishing fertility. [NIH] Family Relations: Behavioral, psychological, and social relations among various members of the nuclear family and the extended family. [NIH] Family Therapy: A form of group psychotherapy. It involves treatment of more than one member of the family simultaneously in the same session. [NIH] Farsightedness: The common term for hyperopia. [NIH] Fasciculation: A small local contraction of muscles, visible through the skin, representing a spontaneous discharge of a number of fibres innervated by a single motor nerve filament. [EU]

Fat: Total lipids including phospholipids. [NIH] Fatigue: The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli. [NIH]

Fatty acids: A major component of fats that are used by the body for energy and tissue development. [NIH] Fetus: The developing offspring from 7 to 8 weeks after conception until birth. [NIH] Fibrillation: A small, local, involuntary contraction of muscle, invisible under the skin, resulting from spontaneous activation of single muscle cells or muscle fibres. [EU] Fibroblasts: Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules. [NIH] Fibrosis: Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. [NIH] Fistulas: An abnormal passage from one hollow structure of the body to another, or from a hollow structure to the surface, formed by an abscess, disease process, incomplete closure of a wound, or by a congenital anomaly. [NIH] Fixation: 1. The act or operation of holding, suturing, or fastening in a fixed position. 2. The condition of being held in a fixed position. 3. In psychiatry, a term with two related but distinct meanings : (1) arrest of development at a particular stage, which like regression (return to an earlier stage), if temporary is a normal reaction to setbacks and difficulties but if protracted or frequent is a cause of developmental failures and emotional problems, and (2) a close and suffocating attachment to another person, especially a childhood figure, such

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as one's mother or father. Both meanings are derived from psychoanalytic theory and refer to 'fixation' of libidinal energy either in a specific erogenous zone, hence fixation at the oral, anal, or phallic stage, or in a specific object, hence mother or father fixation. 4. The use of a fixative (q.v.) to preserve histological or cytological specimens. 5. In chemistry, the process whereby a substance is removed from the gaseous or solution phase and localized, as in carbon dioxide fixation or nitrogen fixation. 6. In ophthalmology, direction of the gaze so that the visual image of the object falls on the fovea centralis. 7. In film processing, the chemical removal of all undeveloped salts of the film emulsion, leaving only the developed silver to form a permanent image. [EU] Flatus: Gas passed through the rectum. [NIH] Flexor: Muscles which flex a joint. [NIH] Fluorescence: The property of emitting radiation while being irradiated. The radiation emitted is usually of longer wavelength than that incident or absorbed, e.g., a substance can be irradiated with invisible radiation and emit visible light. X-ray fluorescence is used in diagnosis. [NIH] Focus Groups: A method of data collection and a qualitative research tool in which a small group of individuals are brought together and allowed to interact in a discussion of their opinions about topics, issues, or questions. [NIH] Fold: A plication or doubling of various parts of the body. [NIH] Follow-Up Studies: Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. [NIH]

Forearm: The part between the elbow and the wrist. [NIH] Fovea: The central part of the macula that provides the sharpest vision. [NIH] Friction: Surface resistance to the relative motion of one body against the rubbing, sliding, rolling, or flowing of another with which it is in contact. [NIH] Frontal Lobe: The anterior part of the cerebral hemisphere. [NIH] Functional magnetic resonance imaging: A noninvasive tool used to observe functioning in the brain or other organs by detecting changes in chemical composition, blood flow, or both. [NIH]

Fundus: The larger part of a hollow organ that is farthest away from the organ's opening. The bladder, gallbladder, stomach, uterus, eye, and cavity of the middle ear all have a fundus. [NIH] Fungi: A kingdom of eukaryotic, heterotrophic organisms that live as saprobes or parasites, including mushrooms, yeasts, smuts, molds, etc. They reproduce either sexually or asexually, and have life cycles that range from simple to complex. Filamentous fungi refer to those that grow as multicelluar colonies (mushrooms and molds). [NIH] Gallbladder: The pear-shaped organ that sits below the liver. Bile is concentrated and stored in the gallbladder. [NIH] Gamma Rays: Very powerful and penetrating, high-energy electromagnetic radiation of shorter wavelength than that of x-rays. They are emitted by a decaying nucleus, usually between 0.01 and 10 MeV. They are also called nuclear x-rays. [NIH] Ganglia: Clusters of multipolar neurons surrounded by a capsule of loosely organized connective tissue located outside the central nervous system. [NIH] Ganglion: 1. A knot, or knotlike mass. 2. A general term for a group of nerve cell bodies located outside the central nervous system; occasionally applied to certain nuclear groups within the brain or spinal cord, e.g. basal ganglia. 3. A benign cystic tumour occurring on a

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aponeurosis or tendon, as in the wrist or dorsum of the foot; it consists of a thin fibrous capsule enclosing a clear mucinous fluid. [EU] Gap Junctions: Connections between cells which allow passage of small molecules and electric current. Gap junctions were first described anatomically as regions of close apposition between cells with a narrow (1-2 nm) gap between cell membranes. The variety in the properties of gap junctions is reflected in the number of connexins, the family of proteins which form the junctions. [NIH] Gas: Air that comes from normal breakdown of food. The gases are passed out of the body through the rectum (flatus) or the mouth (burp). [NIH] Gastrin: A hormone released after eating. Gastrin causes the stomach to produce more acid. [NIH]

Gastrointestinal: Refers to the stomach and intestines. [NIH] Gelatin: A product formed from skin, white connective tissue, or bone collagen. It is used as a protein food adjuvant, plasma substitute, hemostatic, suspending agent in pharmaceutical preparations, and in the manufacturing of capsules and suppositories. [NIH] Gels: Colloids with a solid continuous phase and liquid as the dispersed phase; gels may be unstable when, due to temperature or other cause, the solid phase liquifies; the resulting colloid is called a sol. [NIH] Gene: The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein. [NIH]

Gene Expression: The phenotypic manifestation of a gene or genes by the processes of gene action. [NIH] General practitioner: A medical practitioner who does not specialize in a particular branch of medicine or limit his practice to a specific class of diseases. [NIH] Genetic Code: The specifications for how information, stored in nucleic acid sequence (base sequence), is translated into protein sequence (amino acid sequence). The start, stop, and order of amino acids of a protein is specified by consecutive triplets of nucleotides called codons (codon). [NIH] Genetic Counseling: Advising families of the risks involved pertaining to birth defects, in order that they may make an informed decision on current or future pregnancies. [NIH] Genetic Engineering: Directed modification of the gene complement of a living organism by such techniques as altering the DNA, substituting genetic material by means of a virus, transplanting whole nuclei, transplanting cell hybrids, etc. [NIH] Genetic Markers: A phenotypically recognizable genetic trait which can be used to identify a genetic locus, a linkage group, or a recombination event. [NIH] Genetic Techniques: Chromosomal, biochemical, intracellular, and other methods used in the study of genetics. [NIH] Genetic testing: Analyzing DNA to look for a genetic alteration that may indicate an increased risk for developing a specific disease or disorder. [NIH] Genetics: The biological science that deals with the phenomena and mechanisms of heredity. [NIH] Genital: Pertaining to the genitalia. [EU] Genitourinary: Pertaining to the genital and urinary organs; urogenital; urinosexual. [EU] Genotype: The genetic constitution of the individual; the characterization of the genes. [NIH] Germ Cells: The reproductive cells in multicellular organisms. [NIH]

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Gestation: The period of development of the young in viviparous animals, from the time of fertilization of the ovum until birth. [EU] Gestational: Psychosis attributable to or occurring during pregnancy. [NIH] Gestational Age: Age of the conceptus. In humans, this may be assessed by medical history, physical examination, early immunologic pregnancy tests, radiography, ultrasonography, and amniotic fluid analysis. [NIH] Gestures: Movement of a part of the body for the purpose of communication. [NIH] Gland: An organ that produces and releases one or more substances for use in the body. Some glands produce fluids that affect tissues or organs. Others produce hormones or participate in blood production. [NIH] Glomerular: Pertaining to or of the nature of a glomerulus, especially a renal glomerulus. [EU]

Glomerulus: A tiny set of looping blood vessels in the nephron where blood is filtered in the kidney. [NIH] Glucocorticoid: A compound that belongs to the family of compounds called corticosteroids (steroids). Glucocorticoids affect metabolism and have anti-inflammatory and immunosuppressive effects. They may be naturally produced (hormones) or synthetic (drugs). [NIH] Glucose: D-Glucose. A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. [NIH] Glucose Intolerance: A pathological state in which the fasting plasma glucose level is less than 140 mg per deciliter and the 30-, 60-, or 90-minute plasma glucose concentration following a glucose tolerance test exceeds 200 mg per deciliter. This condition is seen frequently in diabetes mellitus but also occurs with other diseases. [NIH] Glutamate: Excitatory neurotransmitter of the brain. [NIH] Glutamic Acid: A non-essential amino acid naturally occurring in the L-form. Glutamic acid (glutamate) is the most common excitatory neurotransmitter in the central nervous system. [NIH]

Glutathione Peroxidase: An enzyme catalyzing the oxidation of 2 moles of glutathione in the presence of hydrogen peroxide to yield oxidized glutathione and water. EC 1.11.1.9. [NIH]

Glycine: A non-essential amino acid. It is found primarily in gelatin and silk fibroin and used therapeutically as a nutrient. It is also a fast inhibitory neurotransmitter. [NIH] Glycoprotein: A protein that has sugar molecules attached to it. [NIH] Goiter: Enlargement of the thyroid gland. [NIH] Gonadal: Pertaining to a gonad. [EU] Gonads: The gamete-producing glands, ovary or testis. [NIH] Governing Board: The group in which legal authority is vested for the control of healthrelated institutions and organizations. [NIH] Grade: The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. Grading systems are different for each type of cancer. [NIH] Grading: A system for classifying cancer cells in terms of how abnormal they appear when examined under a microscope. The objective of a grading system is to provide information about the probable growth rate of the tumor and its tendency to spread. The systems used to

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grade tumors vary with each type of cancer. Grading plays a role in treatment decisions. [NIH]

Graft: Healthy skin, bone, or other tissue taken from one part of the body and used to replace diseased or injured tissue removed from another part of the body. [NIH] Grafting: The operation of transfer of tissue from one site to another. [NIH] Gramicidin: Antibiotic mixture that is one of the two principle components of tyrothricin from Bacillus brevis. Gramicidin C or S is a cyclic, ten-amino acid polypeptide and gramicidins A, B, D, etc., seem to be linear polypeptides. The mixture is used topically for gram-positive organisms. It is toxic to blood, liver, kidneys, meninges, and the olfactory apparatus. [NIH] Gram-positive: Retaining the stain or resisting decolorization by alcohol in Gram's method of staining, a primary characteristic of bacteria whose cell wall is composed of a thick layer of peptidologlycan with attached teichoic acids. [EU] Granulocytes: Leukocytes with abundant granules in the cytoplasm. They are divided into three groups: neutrophils, eosinophils, and basophils. [NIH] Growth: The progressive development of a living being or part of an organism from its earliest stage to maturity. [NIH] Growth factors: Substances made by the body that function to regulate cell division and cell survival. Some growth factors are also produced in the laboratory and used in biological therapy. [NIH] Guinea Pigs: A common name used for the family Caviidae. The most common species is Cavia porcellus which is the domesticated guinea pig used for pets and biomedical research. [NIH]

Habitual: Of the nature of a habit; according to habit; established by or repeated by force of habit, customary. [EU] Hair Cells: Mechanoreceptors located in the organ of Corti that are sensitive to auditory stimuli and in the vestibular apparatus that are sensitive to movement of the head. In each case the accessory sensory structures are arranged so that appropriate stimuli cause movement of the hair-like projections (stereocilia and kinocilia) which relay the information centrally in the nervous system. [NIH] Hammer: The largest of the three ossicles of the ear. [NIH] Handicap: A handicap occurs as a result of disability, but disability does not always constitute a handicap. A handicap may be said to exist when a disability causes a substantial and continuing reduction in a person's capacity to function socially and vocationally. [NIH] Haploid: An organism with one basic chromosome set, symbolized by n; the normal condition of gametes in diploids. [NIH] Haptens: Small antigenic determinants capable of eliciting an immune response only when coupled to a carrier. Haptens bind to antibodies but by themselves cannot elicit an antibody response. [NIH] Health Promotion: Encouraging consumer behaviors most likely to optimize health potentials (physical and psychosocial) through health information, preventive programs, and access to medical care. [NIH] Health Resources: Available manpower, facilities, revenue, equipment, and supplies to produce requisite health care and services. [NIH] Health Services: Services for the diagnosis and treatment of disease and the maintenance of health. [NIH]

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Health Status: The level of health of the individual, group, or population as subjectively assessed by the individual or by more objective measures. [NIH] Hearing aid: A miniature, portable sound amplifier for persons with impaired hearing, consisting of a microphone, audio amplifier, earphone, and battery. [NIH] Hearing Disorders: Conditions that impair the transmission or perception of auditory impulses and information from the level of the ear to the temporal cortices, including the sensorineural pathways. [NIH] Hearing Impaired Persons: Persons with any degree of loss of hearing that has an impact on their activities of daily living or that requires special assistance or intervention. [NIH] Heart attack: A seizure of weak or abnormal functioning of the heart. [NIH] Heartbeat: One complete contraction of the heart. [NIH] Heme: The color-furnishing portion of hemoglobin. It is found free in tissues and as the prosthetic group in many hemeproteins. [NIH] Hemodialysis: The use of a machine to clean wastes from the blood after the kidneys have failed. The blood travels through tubes to a dialyzer, which removes wastes and extra fluid. The cleaned blood then flows through another set of tubes back into the body. [NIH] Hemoglobin: One of the fractions of glycosylated hemoglobin A1c. Glycosylated hemoglobin is formed when linkages of glucose and related monosaccharides bind to hemoglobin A and its concentration represents the average blood glucose level over the previous several weeks. HbA1c levels are used as a measure of long-term control of plasma glucose (normal, 4 to 6 percent). In controlled diabetes mellitus, the concentration of glycosylated hemoglobin A is within the normal range, but in uncontrolled cases the level may be 3 to 4 times the normal conentration. Generally, complications are substantially lower among patients with Hb levels of 7 percent or less than in patients with HbA1c levels of 9 percent or more. [NIH] Hemoglobinuria: The presence of free hemoglobin in the urine. [NIH] Hemorrhage: Bleeding or escape of blood from a vessel. [NIH] Hemostasis: The process which spontaneously arrests the flow of blood from vessels carrying blood under pressure. It is accomplished by contraction of the vessels, adhesion and aggregation of formed blood elements, and the process of blood or plasma coagulation. [NIH]

Hereditary: Of, relating to, or denoting factors that can be transmitted genetically from one generation to another. [NIH] Heredity: 1. The genetic transmission of a particular quality or trait from parent to offspring. 2. The genetic constitution of an individual. [EU] Heterodimers: Zippered pair of nonidentical proteins. [NIH] Heterogeneity: The property of one or more samples or populations which implies that they are not identical in respect of some or all of their parameters, e. g. heterogeneity of variance. [NIH]

Heterogenic: Derived from a different source or species. Also called heterogenous. [NIH] Heterogenous: Derived from a different source or species. Also called heterogenic. [NIH] Heterotropia: One in which the angle of squint remains relatively unaltered on conjugate movement of the eyes. [NIH] Heterozygotes: Having unlike alleles at one or more corresponding loci on homologous chromosomes. [NIH] Histology: The study of tissues and cells under a microscope. [NIH]

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Homeostasis: The processes whereby the internal environment of an organism tends to remain balanced and stable. [NIH] Homogeneous: Consisting of or composed of similar elements or ingredients; of a uniform quality throughout. [EU] Homologous: Corresponding in structure, position, origin, etc., as (a) the feathers of a bird and the scales of a fish, (b) antigen and its specific antibody, (c) allelic chromosomes. [EU] Homozygote: An individual in which both alleles at a given locus are identical. [NIH] Hormonal: Pertaining to or of the nature of a hormone. [EU] Hormone: A substance in the body that regulates certain organs. Hormones such as gastrin help in breaking down food. Some hormones come from cells in the stomach and small intestine. [NIH] Host: Any animal that receives a transplanted graft. [NIH] Housekeeping: The care and management of property. [NIH] Human Development: Continuous sequential changes which occur in the physiological and psychological functions during the individual's life. [NIH] Hybrid: Cross fertilization between two varieties or, more usually, two species of vines, see also crossing. [NIH] Hybridization: The genetic process of crossbreeding to produce a hybrid. Hybrid nucleic acids can be formed by nucleic acid hybridization of DNA and RNA molecules. Protein hybridization allows for hybrid proteins to be formed from polypeptide chains. [NIH] Hydrogen: The first chemical element in the periodic table. It has the atomic symbol H, atomic number 1, and atomic weight 1. It exists, under normal conditions, as a colorless, odorless, tasteless, diatomic gas. Hydrogen ions are protons. Besides the common H1 isotope, hydrogen exists as the stable isotope deuterium and the unstable, radioactive isotope tritium. [NIH] Hydrogen Peroxide: A strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials. [NIH] Hydrolysis: The process of cleaving a chemical compound by the addition of a molecule of water. [NIH] Hydrophilic: Readily absorbing moisture; hygroscopic; having strongly polar groups that readily interact with water. [EU] Hydroxylysine: A hydroxylated derivative of the amino acid lysine that is present in certain collagens. [NIH] Hydroxyproline: A hydroxylated form of the imino acid proline. A deficiency in ascorbic acid can result in impaired hydroxyproline formation. [NIH] Hyperbaric: Characterized by greater than normal pressure or weight; applied to gases under greater than atmospheric pressure, as hyperbaric oxygen, or to a solution of greater specific gravity than another taken as a standard of reference. [EU] Hyperbaric oxygen: Oxygen that is at an atmospheric pressure higher than the pressure at sea level. Breathing hyperbaric oxygen to enhance the effectiveness of radiation therapy is being studied. [NIH] Hyperlipidemia: An excess of lipids in the blood. [NIH] Hyperopia: Farsightedness; ability to see distant objects more clearly than close objects; may be corrected with glasses or contact lenses. [NIH]

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Hypersensitivity: Altered reactivity to an antigen, which can result in pathologic reactions upon subsequent exposure to that particular antigen. [NIH] Hypertension: Persistently high arterial blood pressure. Currently accepted threshold levels are 140 mm Hg systolic and 90 mm Hg diastolic pressure. [NIH] Hypertrophy: General increase in bulk of a part or organ, not due to tumor formation, nor to an increase in the number of cells. [NIH] Hypnotic: A drug that acts to induce sleep. [EU] Hypoglycemia: Abnormally low blood sugar [NIH] Hypogonadism: Condition resulting from or characterized by abnormally decreased functional activity of the gonads, with retardation of growth and sexual development. [NIH] Hypoplasia: Incomplete development or underdevelopment of an organ or tissue. [EU] Hypothyroidism: Deficiency of thyroid activity. In adults, it is most common in women and is characterized by decrease in basal metabolic rate, tiredness and lethargy, sensitivity to cold, and menstrual disturbances. If untreated, it progresses to full-blown myxoedema. In infants, severe hypothyroidism leads to cretinism. In juveniles, the manifestations are intermediate, with less severe mental and developmental retardation and only mild symptoms of the adult form. When due to pituitary deficiency of thyrotropin secretion it is called secondary hypothyroidism. [EU] Hypoxia: Reduction of oxygen supply to tissue below physiological levels despite adequate perfusion of the tissue by blood. [EU] Iatrogenic: Resulting from the activity of physicians. Originally applied to disorders induced in the patient by autosuggestion based on the physician's examination, manner, or discussion, the term is now applied to any adverse condition in a patient occurring as the result of treatment by a physician or surgeon, especially to infections acquired by the patient during the course of treatment. [EU] Ichthyosis: Any of several generalized skin disorders characterized by dryness, roughness, and scaliness, due to hypertrophy of the stratum corneum epidermis. Most are genetic, but some are acquired, developing in association with other systemic disease or genetic syndrome. [NIH] Id: The part of the personality structure which harbors the unconscious instinctive desires and strivings of the individual. [NIH] Idiopathic: Describes a disease of unknown cause. [NIH] Illusion: A false interpretation of a genuine percept. [NIH] Immune function: Production and action of cells that fight disease or infection. [NIH] Immune response: The activity of the immune system against foreign substances (antigens). [NIH]

Immune system: The organs, cells, and molecules responsible for the recognition and disposal of foreign ("non-self") material which enters the body. [NIH] Immunity: Nonsusceptibility to the invasive or pathogenic microorganisms or to the toxic effect of antigenic substances. [NIH]

effects

of

foreign

Immunochemistry: Field of chemistry that pertains to immunological phenomena and the study of chemical reactions related to antigen stimulation of tissues. It includes physicochemical interactions between antigens and antibodies. [NIH] Immunodeficiency: The decreased ability of the body to fight infection and disease. [NIH] Immunodeficiency syndrome: The inability of the body to produce an immune response. [NIH]

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Immunoglobulin: A protein that acts as an antibody. [NIH] Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents. [NIH] Immunologic: The ability of the antibody-forming system to recall a previous experience with an antigen and to respond to a second exposure with the prompt production of large amounts of antibody. [NIH] Immunology: The study of the body's immune system. [NIH] Immunosuppressive: Describes the ability to lower immune system responses. [NIH] Immunosuppressive Agents: Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of suppressor T-cell populations or by inhibiting the activation of helper cells. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of interleukins and other cytokines are emerging. [NIH] Impairment: In the context of health experience, an impairment is any loss or abnormality of psychological, physiological, or anatomical structure or function. [NIH] Implant radiation: A procedure in which radioactive material sealed in needles, seeds, wires, or catheters is placed directly into or near the tumor. Also called [NIH] Implantation: The insertion or grafting into the body of biological, living, inert, or radioactive material. [EU] In situ: In the natural or normal place; confined to the site of origin without invasion of neighbouring tissues. [EU] In Situ Hybridization: A technique that localizes specific nucleic acid sequences within intact chromosomes, eukaryotic cells, or bacterial cells through the use of specific nucleic acid-labeled probes. [NIH] In vitro: In the laboratory (outside the body). The opposite of in vivo (in the body). [NIH] In vivo: In the body. The opposite of in vitro (outside the body or in the laboratory). [NIH] Incus: One of three ossicles of the middle ear. It conducts sound vibrations from the malleus to the stapes. [NIH] Indicative: That indicates; that points out more or less exactly; that reveals fairly clearly. [EU] Indomethacin: A non-steroidal anti-inflammatory agent (NSAID) that inhibits the enzyme cyclooxygenase necessary for the formation of prostaglandins and other autacoids. It also inhibits the motility of polymorphonuclear leukocytes. [NIH] Induction: The act or process of inducing or causing to occur, especially the production of a specific morphogenetic effect in the developing embryo through the influence of evocators or organizers, or the production of anaesthesia or unconsciousness by use of appropriate agents. [EU] Infancy: The period of complete dependency prior to the acquisition of competence in walking, talking, and self-feeding. [NIH] Infarction: A pathological process consisting of a sudden insufficient blood supply to an area, which results in necrosis of that area. It is usually caused by a thrombus, an embolus, or a vascular torsion. [NIH] Infection: 1. Invasion and multiplication of microorganisms in body tissues, which may be clinically unapparent or result in local cellular injury due to competitive metabolism, toxins, intracellular replication, or antigen-antibody response. The infection may remain localized, subclinical, and temporary if the body's defensive mechanisms are effective. A local

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infection may persist and spread by extension to become an acute, subacute, or chronic clinical infection or disease state. A local infection may also become systemic when the microorganisms gain access to the lymphatic or vascular system. 2. An infectious disease. [EU]

Inflammation: A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function. [NIH] Infuse: To pour (a liquid) into something. [EU] Inhalation: The drawing of air or other substances into the lungs. [EU] Initiation: Mutation induced by a chemical reactive substance causing cell changes; being a step in a carcinogenic process. [NIH] Inlay: In dentistry, a filling first made to correspond with the form of a dental cavity and then cemented into the cavity. [NIH] Inner ear: The labyrinth, comprising the vestibule, cochlea, and semicircular canals. [NIH] Innervation: 1. The distribution or supply of nerves to a part. 2. The supply of nervous energy or of nerve stimulus sent to a part. [EU] Inoculum: The spores or tissues of a pathogen that serve to initiate disease in a plant. [NIH] Inorganic: Pertaining to substances not of organic origin. [EU] Inositol: An isomer of glucose that has traditionally been considered to be a B vitamin although it has an uncertain status as a vitamin and a deficiency syndrome has not been identified in man. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1379) Inositol phospholipids are important in signal transduction. [NIH] Inositol Phosphates: Phosphoric acid esters of inositol. They include mono- and polyphosphoric acid esters, with the exception of inositol hexaphosphate which is phytic acid. [NIH] Insight: The capacity to understand one's own motives, to be aware of one's own psychodynamics, to appreciate the meaning of symbolic behavior. [NIH] Insomnia: Difficulty in going to sleep or getting enough sleep. [NIH] Insulin: A protein hormone secreted by beta cells of the pancreas. Insulin plays a major role in the regulation of glucose metabolism, generally promoting the cellular utilization of glucose. It is also an important regulator of protein and lipid metabolism. Insulin is used as a drug to control insulin-dependent diabetes mellitus. [NIH] Insulin-dependent diabetes mellitus: A disease characterized by high levels of blood glucose resulting from defects in insulin secretion, insulin action, or both. Autoimmune, genetic, and environmental factors are involved in the development of type I diabetes. [NIH] Integrins: A family of transmembrane glycoproteins consisting of noncovalent heterodimers. They interact with a wide variety of ligands including extracellular matrix glycoproteins, complement, and other cells, while their intracellular domains interact with the cytoskeleton. The integrins consist of at least three identified families: the cytoadhesin receptors, the leukocyte adhesion receptors, and the very-late-antigen receptors. Each family contains a common beta-subunit combined with one or more distinct alpha-subunits. These receptors participate in cell-matrix and cell-cell adhesion in many physiologically important processes, including embryological development, hemostasis, thrombosis, wound healing, immune and nonimmune defense mechanisms, and oncogenic transformation. [NIH] Intelligence Tests: Standardized tests that measure the present general ability or aptitude for intellectual performance. [NIH]

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Interferon: A biological response modifier (a substance that can improve the body's natural response to disease). Interferons interfere with the division of cancer cells and can slow tumor growth. There are several types of interferons, including interferon-alpha, -beta, and gamma. These substances are normally produced by the body. They are also made in the laboratory for use in treating cancer and other diseases. [NIH] Interferon-alpha: One of the type I interferons produced by peripheral blood leukocytes or lymphoblastoid cells when exposed to live or inactivated virus, double-stranded RNA, or bacterial products. It is the major interferon produced by virus-induced leukocyte cultures and, in addition to its pronounced antiviral activity, it causes activation of NK cells. [NIH] Interleukins: Soluble factors which stimulate growth-related activities of leukocytes as well as other cell types. They enhance cell proliferation and differentiation, DNA synthesis, secretion of other biologically active molecules and responses to immune and inflammatory stimuli. [NIH] Internal Medicine: A medical specialty concerned with the diagnosis and treatment of diseases of the internal organ systems of adults. [NIH] Internal radiation: A procedure in which radioactive material sealed in needles, seeds, wires, or catheters is placed directly into or near the tumor. Also called brachytherapy, implant radiation, or interstitial radiation therapy. [NIH] Interpersonal Relations: The reciprocal interaction of two or more persons. [NIH] Interstitial: Pertaining to or situated between parts or in the interspaces of a tissue. [EU] Intestinal: Having to do with the intestines. [NIH] Intestines: The section of the alimentary canal from the stomach to the anus. It includes the large intestine and small intestine. [NIH] Intoxication: Poisoning, the state of being poisoned. [EU] Intracellular: Inside a cell. [NIH] Intracellular Membranes: Membranes of subcellular structures. [NIH] Intracranial Embolism: The sudden obstruction of a blood vessel by an embolus. [NIH] Intracranial Embolism and Thrombosis: Embolism or thrombosis involving blood vessels which supply intracranial structures. Emboli may originate from extracranial or intracranial sources. Thrombosis may occur in arterial or venous structures. [NIH] Intracranial Hypertension: Increased pressure within the cranial vault. This may result from several conditions, including hydrocephalus; brain edema; intracranial masses; severe systemic hypertension; pseudotumor cerebri; and other disorders. [NIH] Intrinsic: Situated entirely within or pertaining exclusively to a part. [EU] Invasive: 1. Having the quality of invasiveness. 2. Involving puncture or incision of the skin or insertion of an instrument or foreign material into the body; said of diagnostic techniques. [EU]

Involuntary: Reaction occurring without intention or volition. [NIH] Iodine: A nonmetallic element of the halogen group that is represented by the atomic symbol I, atomic number 53, and atomic weight of 126.90. It is a nutritionally essential element, especially important in thyroid hormone synthesis. In solution, it has anti-infective properties and is used topically. [NIH] Ion Channels: Gated, ion-selective glycoproteins that traverse membranes. The stimulus for channel gating can be a membrane potential, drug, transmitter, cytoplasmic messenger, or a mechanical deformation. Ion channels which are integral parts of ionotropic neurotransmitter receptors are not included. [NIH]

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Ion Transport: The movement of ions across energy-transducing cell membranes. Transport can be active or passive. Passive ion transport (facilitated diffusion) derives its energy from the concentration gradient of the ion itself and allows the transport of a single solute in one direction (uniport). Active ion transport is usually coupled to an energy-yielding chemical or photochemical reaction such as ATP hydrolysis. This form of primary active transport is called an ion pump. Secondary active transport utilizes the voltage and ion gradients produced by the primary transport to drive the cotransport of other ions or molecules. These may be transported in the same (symport) or opposite (antiport) direction. [NIH] Ionophores: Chemical agents that increase the permeability of biological or artificial lipid membranes to specific ions. Most ionophores are relatively small organic molecules that act as mobile carriers within membranes or coalesce to form ion permeable channels across membranes. Many are antibiotics, and many act as uncoupling agents by short-circuiting the proton gradient across mitochondrial membranes. [NIH] Ions: An atom or group of atoms that have a positive or negative electric charge due to a gain (negative charge) or loss (positive charge) of one or more electrons. Atoms with a positive charge are known as cations; those with a negative charge are anions. [NIH] Ipsilateral: Having to do with the same side of the body. [NIH] Irradiation: The use of high-energy radiation from x-rays, neutrons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy) or from materials called radioisotopes. Radioisotopes produce radiation and can be placed in or near the tumor or in the area near cancer cells. This type of radiation treatment is called internal radiation therapy, implant radiation, interstitial radiation, or brachytherapy. Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that circulates throughout the body. Irradiation is also called radiation therapy, radiotherapy, and x-ray therapy. [NIH] Ischemia: Deficiency of blood in a part, due to functional constriction or actual obstruction of a blood vessel. [EU] Isomorphism: The name given to the phenomenon whereby two or more minerals crystallize in the same class of the same system of symmetry and develop very similar forms. [NIH] Job Satisfaction: Personal satisfaction relative to the work situation. [NIH] Kb: A measure of the length of DNA fragments, 1 Kb = 1000 base pairs. The largest DNA fragments are up to 50 kilobases long. [NIH] Keratitis: Inflammation of the cornea. [NIH] Kidney Disease: Any one of several chronic conditions that are caused by damage to the cells of the kidney. People who have had diabetes for a long time may have kidney damage. Also called nephropathy. [NIH] Kidney Failure: The inability of a kidney to excrete metabolites at normal plasma levels under conditions of normal loading, or the inability to retain electrolytes under conditions of normal intake. In the acute form (kidney failure, acute), it is marked by uremia and usually by oliguria or anuria, with hyperkalemia and pulmonary edema. The chronic form (kidney failure, chronic) is irreversible and requires hemodialysis. [NIH] Kidney Failure, Acute: A clinical syndrome characterized by a sudden decrease in glomerular filtration rate, often to values of less than 1 to 2 ml per minute. It is usually associated with oliguria (urine volumes of less than 400 ml per day) and is always associated with biochemical consequences of the reduction in glomerular filtration rate such as a rise in blood urea nitrogen (BUN) and serum creatinine concentrations. [NIH]

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Kidney Failure, Chronic: An irreversible and usually progressive reduction in renal function in which both kidneys have been damaged by a variety of diseases to the extent that they are unable to adequately remove the metabolic products from the blood and regulate the body's electrolyte composition and acid-base balance. Chronic kidney failure requires hemodialysis or surgery, usually kidney transplantation. [NIH] Kinetic: Pertaining to or producing motion. [EU] Kink: Deviation from the normal long axis, as in a fractured bone healed out of line. [NIH] Labile: 1. Gliding; moving from point to point over the surface; unstable; fluctuating. 2. Chemically unstable. [EU] Labyrinth: The internal ear; the essential part of the organ of hearing. It consists of an osseous and a membranous portion. [NIH] Labyrinthine: A vestibular nystagmus resulting from stimulation, injury, or disease of the labyrinth. [NIH] Labyrinthitis: Inflammation of the inner ear. [NIH] Lacrimal: Pertaining to the tears. [EU] Lag: The time elapsing between application of a stimulus and the resulting reaction. [NIH] Laminin: Large, noncollagenous glycoprotein with antigenic properties. It is localized in the basement membrane lamina lucida and functions to bind epithelial cells to the basement membrane. Evidence suggests that the protein plays a role in tumor invasion. [NIH] Language Development: The gradual expansion in complexity and meaning of symbols and sounds as perceived and interpreted by the individual through a maturational and learning process. Stages in development include babbling, cooing, word imitation with cognition, and use of short sentences. [NIH] Language Disorders: Conditions characterized by deficiencies of comprehension or expression of written and spoken forms of language. These include acquired and developmental disorders. [NIH] Large Intestine: The part of the intestine that goes from the cecum to the rectum. The large intestine absorbs water from stool and changes it from a liquid to a solid form. The large intestine is 5 feet long and includes the appendix, cecum, colon, and rectum. Also called colon. [NIH] Larva: Wormlike or grublike stage, following the egg in the life cycle of insects, worms, and other metamorphosing animals. [NIH] Larynx: An irregularly shaped, musculocartilaginous tubular structure, lined with mucous membrane, located at the top of the trachea and below the root of the tongue and the hyoid bone. It is the essential sphincter guarding the entrance into the trachea and functioning secondarily as the organ of voice. [NIH] Latent: Phoria which occurs at one distance or another and which usually has no troublesome effect. [NIH] Laterality: Behavioral manifestations of cerebral dominance in which there is preferential use and superior functioning of either the left or the right side, as in the preferred use of the right hand or right foot. [NIH] Lectin: A complex molecule that has both protein and sugars. Lectins are able to bind to the outside of a cell and cause biochemical changes in it. Lectins are made by both animals and plants. [NIH] Legal blindness: In the U.S., (1) visual acuity of 20/200 or worse in the better eye with corrective lenses (20/200 means that a person must be at 20 feet from an eye chart to see

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what a person with normal vision can see at 200 feet) or (2) visual field restricted to 20 d [NIH]

Lens: The transparent, double convex (outward curve on both sides) structure suspended between the aqueous and vitreous; helps to focus light on the retina. [NIH] Lesion: An area of abnormal tissue change. [NIH] Lethal: Deadly, fatal. [EU] Lethargy: Abnormal drowsiness or stupor; a condition of indifference. [EU] Leukemia: Cancer of blood-forming tissue. [NIH] Leukocytes: White blood cells. These include granular leukocytes (basophils, eosinophils, and neutrophils) as well as non-granular leukocytes (lymphocytes and monocytes). [NIH] Library Services: Services offered to the library user. They include reference and circulation. [NIH]

Life cycle: The successive stages through which an organism passes from fertilized ovum or spore to the fertilized ovum or spore of the next generation. [NIH] Life Expectancy: A figure representing the number of years, based on known statistics, to which any person of a given age may reasonably expect to live. [NIH] Ligament: A band of fibrous tissue that connects bones or cartilages, serving to support and strengthen joints. [EU] Ligands: A RNA simulation method developed by the MIT. [NIH] Linkage: The tendency of two or more genes in the same chromosome to remain together from one generation to the next more frequently than expected according to the law of independent assortment. [NIH] Linkage Disequilibrium: Nonrandom association of linked genes. This is the tendency of the alleles of two separate but already linked loci to be found together more frequently than would be expected by chance alone. [NIH] Lip: Either of the two fleshy, full-blooded margins of the mouth. [NIH] Lipid: Fat. [NIH] Lipid Peroxidation: Peroxidase catalyzed oxidation of lipids using hydrogen peroxide as an electron acceptor. [NIH] Lipreading: The process by which an observer comprehends speech by watching the movements of the speaker's lips without hearing the speaker's voice. [NIH] Liver: A large, glandular organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile. [NIH] Liver scan: An image of the liver created on a computer screen or on film. A radioactive substance is injected into a blood vessel and travels through the bloodstream. It collects in the liver, especially in abnormal areas, and can be detected by the scanner. [NIH] Lobe: A portion of an organ such as the liver, lung, breast, or brain. [NIH] Localization: The process of determining or marking the location or site of a lesion or disease. May also refer to the process of keeping a lesion or disease in a specific location or site. [NIH] Localized: Cancer which has not metastasized yet. [NIH] Locomotion: Movement or the ability to move from one place or another. It can refer to humans, vertebrate or invertebrate animals, and microorganisms. [NIH] Longitudinal study: Also referred to as a "cohort study" or "prospective study"; the analytic

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method of epidemiologic study in which subsets of a defined population can be identified who are, have been, or in the future may be exposed or not exposed, or exposed in different degrees, to a factor or factors hypothesized to influence the probability of occurrence of a given disease or other outcome. The main feature of this type of study is to observe large numbers of subjects over an extended time, with comparisons of incidence rates in groups that differ in exposure levels. [NIH] Loop: A wire usually of platinum bent at one end into a small loop (usually 4 mm inside diameter) and used in transferring microorganisms. [NIH] Low vision: Visual loss that cannot be corrected with eyeglasses or contact lenses and interferes with daily living activities. [NIH] Lucida: An instrument, invented by Wollaton, consisting essentially of a prism or a mirror through which an object can be viewed so as to appear on a plane surface seen in direct view and on which the outline of the object may be traced. [NIH] Lumen: The cavity or channel within a tube or tubular organ. [EU] Lymph: The almost colorless fluid that travels through the lymphatic system and carries cells that help fight infection and disease. [NIH] Lymph node: A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Also known as a lymph gland. Lymph nodes are spread out along lymphatic vessels and contain many lymphocytes, which filter the lymphatic fluid (lymph). [NIH]

Lymphatic: The tissues and organs, including the bone marrow, spleen, thymus, and lymph nodes, that produce and store cells that fight infection and disease. [NIH] Lymphatic system: The tissues and organs that produce, store, and carry white blood cells that fight infection and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes and a network of thin tubes that carry lymph and white blood cells. These tubes branch, like blood vessels, into all the tissues of the body. [NIH] Lymphocyte: A white blood cell. Lymphocytes have a number of roles in the immune system, including the production of antibodies and other substances that fight infection and diseases. [NIH] Lymphoid: Referring to lymphocytes, a type of white blood cell. Also refers to tissue in which lymphocytes develop. [NIH] Lymphoma: A general term for various neoplastic diseases of the lymphoid tissue. [NIH] Macrophage: A type of white blood cell that surrounds and kills microorganisms, removes dead cells, and stimulates the action of other immune system cells. [NIH] Macula: A stain, spot, or thickening. Often used alone to refer to the macula retinae. [EU] Magnetic Resonance Imaging: Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. [NIH] Malabsorption: Impaired intestinal absorption of nutrients. [EU] Malformation: A morphologic developmental process. [EU]

defect

resulting

from

an

intrinsically

abnormal

Malignant: Cancerous; a growth with a tendency to invade and destroy nearby tissue and spread to other parts of the body. [NIH] Malnutrition: A condition caused by not eating enough food or not eating a balanced diet. [NIH]

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Malondialdehyde: The dialdehyde of malonic acid. [NIH] Manifest: Being the part or aspect of a phenomenon that is directly observable : concretely expressed in behaviour. [EU] Manual Communication: Method of nonverbal communication utilizing hand movements as speech equivalents. [NIH] Meatus: A canal running from the internal auditory foramen through the petrous portion of the temporal bone. It gives passage to the facial and auditory nerves together with the auditory branch of the basilar artery and the internal auditory veins. [NIH] Medial: Lying near the midsaggital plane of the body; opposed to lateral. [NIH] Mediate: Indirect; accomplished by the aid of an intervening medium. [EU] MEDLINE: An online database of MEDLARS, the computerized bibliographic Medical Literature Analysis and Retrieval System of the National Library of Medicine. [NIH] Megaloblastic: A large abnormal red blood cell appearing in the blood in pernicious anaemia. [EU] Meiosis: A special method of cell division, occurring in maturation of the germ cells, by means of which each daughter nucleus receives half the number of chromosomes characteristic of the somatic cells of the species. [NIH] Melanin: The substance that gives the skin its color. [NIH] Melanocytes: Epidermal dendritic pigment cells which control long-term morphological color changes by alteration in their number or in the amount of pigment they produce and store in the pigment containing organelles called melanosomes. Melanophores are larger cells which do not exist in mammals. [NIH] Melanoma: A form of skin cancer that arises in melanocytes, the cells that produce pigment. Melanoma usually begins in a mole. [NIH] Membrane: A very thin layer of tissue that covers a surface. [NIH] Membrane Fluidity: The motion of phospholipid molecules within the lipid bilayer, dependent on the classes of phospholipids present, their fatty acid composition and degree of unsaturation of the acyl chains, the cholesterol concentration, and temperature. [NIH] Membrane Glycoproteins: Glycoproteins found on the membrane or surface of cells. [NIH] Membrane Proteins: Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors. [NIH] Memory: Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. [NIH] Meningeal: Refers to the meninges, the tissue covering the brain and spinal cord. [NIH] Meninges: The three membranes that cover and protect the brain and spinal cord. [NIH] Meningitis: Inflammation of the meninges. When it affects the dura mater, the disease is termed pachymeningitis; when the arachnoid and pia mater are involved, it is called leptomeningitis, or meningitis proper. [EU] Mental Disorders: Psychiatric illness or diseases manifested by breakdowns in the adaptational process expressed primarily as abnormalities of thought, feeling, and behavior producing either distress or impairment of function. [NIH] Mental Health: The state wherein the person is well adjusted. [NIH]

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Mental Health Services: Organized services to provide mental health care. [NIH] Mental Processes: Conceptual functions or thinking in all its forms. [NIH] Mental Retardation: Refers to sub-average general intellectual functioning which originated during the developmental period and is associated with impairment in adaptive behavior. [NIH]

Mentors: Senior professionals who provide guidance, direction and support to those persons desirous of improvement in academic positions, administrative positions or other career development situations. [NIH] Metabotropic: A glutamate receptor which triggers an increase in production of 2 intracellular messengers: diacylglycerol and inositol 1, 4, 5-triphosphate. [NIH] Metamorphosis: The ontogeny of insects, i. e. the series of changes undergone from egg, through larva and pupa, or through nymph, to adult. [NIH] Metastasis: The spread of cancer from one part of the body to another. Tumors formed from cells that have spread are called "secondary tumors" and contain cells that are like those in the original (primary) tumor. The plural is metastases. [NIH] Metastatic: Having to do with metastasis, which is the spread of cancer from one part of the body to another. [NIH] Methimazole: A thioureylene antithyroid agent that inhibits the formation of thyroid hormones by interfering with the incorporation of iodine into tyrosyl residues of thyroglobulin. This is done by interfering with the oxidation of iodide ion and iodotyrosyl groups through inhibition of the peroxidase enzyme. [NIH] MI: Myocardial infarction. Gross necrosis of the myocardium as a result of interruption of the blood supply to the area; it is almost always caused by atherosclerosis of the coronary arteries, upon which coronary thrombosis is usually superimposed. [NIH] Microbiological: Pertaining to microbiology : the science that deals with microorganisms, including algae, bacteria, fungi, protozoa and viruses. [EU] Microbiology: The study of microorganisms such as fungi, bacteria, algae, archaea, and viruses. [NIH] Microorganism: An organism that can be seen only through a microscope. Microorganisms include bacteria, protozoa, algae, and fungi. Although viruses are not considered living organisms, they are sometimes classified as microorganisms. [NIH] Microphthalmos: Congenital or developmental anomaly in which the eyeballs are abnormally small. [NIH] Microscopy: The application of microscope magnification to the study of materials that cannot be properly seen by the unaided eye. [NIH] Microwaves: That portion of the electromagnetic spectrum lying between UHF (ultrahigh frequency) radio waves and heat (infrared) waves. Microwaves are used to generate heat, especially in some types of diathermy. They may cause heat damage to tissues. [NIH] Migration: The systematic movement of genes between populations of the same species, geographic race, or variety. [NIH] Mineralization: The action of mineralizing; the state of being mineralized. [EU] Minority Groups: A subgroup having special characteristics within a larger group, often bound together by special ties which distinguish it from the larger group. [NIH] Mitochondria: Parts of a cell where aerobic production (also known as cell respiration) takes place. [NIH]

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Mitosis: A method of indirect cell division by means of which the two daughter nuclei normally receive identical complements of the number of chromosomes of the somatic cells of the species. [NIH] Mobility: Capability of movement, of being moved, or of flowing freely. [EU] Modeling: A treatment procedure whereby the therapist presents the target behavior which the learner is to imitate and make part of his repertoire. [NIH] Modems: Equipment that sends digital information over telephone lines. The term Modem is a short form of the phrase modulator-demodulator. [NIH] Modification: A change in an organism, or in a process in an organism, that is acquired from its own activity or environment. [NIH] Modulator: A specific inductor that brings out characteristics peculiar to a definite region. [EU]

Molecular: Of, pertaining to, or composed of molecules : a very small mass of matter. [EU] Molecular Structure: The location of the atoms, groups or ions relative to one another in a molecule, as well as the number, type and location of covalent bonds. [NIH] Molecule: A chemical made up of two or more atoms. The atoms in a molecule can be the same (an oxygen molecule has two oxygen atoms) or different (a water molecule has two hydrogen atoms and one oxygen atom). Biological molecules, such as proteins and DNA, can be made up of many thousands of atoms. [NIH] Monitor: An apparatus which automatically records such physiological signs as respiration, pulse, and blood pressure in an anesthetized patient or one undergoing surgical or other procedures. [NIH] Monoclonal: An antibody produced by culturing a single type of cell. It therefore consists of a single species of immunoglobulin molecules. [NIH] Monogenic: A human disease caused by a mutation in a single gene. [NIH] Morphogenesis: The development of the form of an organ, part of the body, or organism. [NIH]

Morphological: Relating to the configuration or the structure of live organs. [NIH] Morphology: The science of the form and structure of organisms (plants, animals, and other forms of life). [NIH] Motility: The ability to move spontaneously. [EU] Motion Perception: The real or apparent movement of objects through the visual field. [NIH] Motion Sickness: Sickness caused by motion, as sea sickness, train sickness, car sickness, and air sickness. [NIH] Mucinous: Containing or resembling mucin, the main compound in mucus. [NIH] Mucins: A secretion containing mucopolysaccharides and protein that is the chief constituent of mucus. [NIH] Mucus: The viscous secretion of mucous membranes. It contains mucin, white blood cells, water, inorganic salts, and exfoliated cells. [NIH] Muscle Fibers: Large single cells, either cylindrical or prismatic in shape, that form the basic unit of muscle tissue. They consist of a soft contractile substance enclosed in a tubular sheath. [NIH] Muscle Hypertonia: Abnormal increase in skeletal or smooth muscle tone. Skeletal muscle hypertonicity may be associated with pyramidal tract lesions or basal ganglia diseases. [NIH] Muscular Atrophy: Derangement in size and number of muscle fibers occurring with aging,

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reduction in blood supply, or following immobilization, prolonged weightlessness, malnutrition, and particularly in denervation. [NIH] Muscular Dystrophies: A general term for a group of inherited disorders which are characterized by progressive degeneration of skeletal muscles. [NIH] Mustard Gas: Severe irritant and vesicant of skin, eyes, and lungs. It may cause blindness and lethal lung edema and was formerly used as a war gas. The substance has been proposed as a cytostatic and for treatment of psoriasis. It has been listed as a known carcinogen in the Fourth Annual Report on Carcinogens (NTP-85-002, 1985) (Merck, 11th ed). [NIH] Mutagen: Any agent, such as X-rays, gamma rays, mustard gas, TCDD, that can cause abnormal mutation in living cells; having the power to cause mutations. [NIH] Mutagenesis: Process of generating genetic mutations. It may occur spontaneously or be induced by mutagens. [NIH] Myelin: The fatty substance that covers and protects nerves. [NIH] Myocardial Ischemia: A disorder of cardiac function caused by insufficient blood flow to the muscle tissue of the heart. The decreased blood flow may be due to narrowing of the coronary arteries (coronary arteriosclerosis), to obstruction by a thrombus (coronary thrombosis), or less commonly, to diffuse narrowing of arterioles and other small vessels within the heart. Severe interruption of the blood supply to the myocardial tissue may result in necrosis of cardiac muscle (myocardial infarction). [NIH] Myocardium: The muscle tissue of the heart composed of striated, involuntary muscle known as cardiac muscle. [NIH] Myopathy: Any disease of a muscle. [EU] Myopia: That error of refraction in which rays of light entering the eye parallel to the optic axis are brought to a focus in front of the retina, as a result of the eyeball being too long from front to back (axial m.) or of an increased strength in refractive power of the media of the eye (index m.). Called also nearsightedness, because the near point is less distant than it is in emmetropia with an equal amplitude of accommodation. [EU] Myosin: Chief protein in muscle and the main constituent of the thick filaments of muscle fibers. In conjunction with actin, it is responsible for the contraction and relaxation of muscles. [NIH] Myotonic Dystrophy: A condition presenting muscle weakness and wasting which may be progressive. [NIH] Natural selection: A part of the evolutionary process resulting in the survival and reproduction of the best adapted individuals. [NIH] Nausea: An unpleasant sensation in the stomach usually accompanied by the urge to vomit. Common causes are early pregnancy, sea and motion sickness, emotional stress, intense pain, food poisoning, and various enteroviruses. [NIH] NCI: National Cancer Institute. NCI, part of the National Institutes of Health of the United States Department of Health and Human Services, is the federal government's principal agency for cancer research. NCI conducts, coordinates, and funds cancer research, training, health information dissemination, and other programs with respect to the cause, diagnosis, prevention, and treatment of cancer. Access the NCI Web site at http://cancer.gov. [NIH] Nearsightedness: The common term for myopia. [NIH] Necrosis: A pathological process caused by the progressive degradative action of enzymes that is generally associated with severe cellular trauma. It is characterized by mitochondrial

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swelling, nuclear flocculation, uncontrolled cell lysis, and ultimately cell death. [NIH] Need: A state of tension or dissatisfaction felt by an individual that impels him to action toward a goal he believes will satisfy the impulse. [NIH] Needs Assessment: Systematic identification of a population's needs or the assessment of individuals to determine the proper level of services needed. [NIH] Neomycin: Antibiotic complex produced by Streptomyces fradiae. It is composed of neomycins A, B, and C. It acts by inhibiting translation during protein synthesis. [NIH] Neonatal: Pertaining to the first four weeks after birth. [EU] Neonatal Screening: The identification of selected parameters in newborn infants by various tests, examinations, or other procedures. Screening may be performed by clinical or laboratory measures. A screening test is designed to sort out healthy neonates from those not well, but the screening test is not intended as a diagnostic device, rather instead as epidemiologic. [NIH] Neoplasia: Abnormal and uncontrolled cell growth. [NIH] Neoplasm: A new growth of benign or malignant tissue. [NIH] Neoplastic: Pertaining to or like a neoplasm (= any new and abnormal growth); pertaining to neoplasia (= the formation of a neoplasm). [EU] Neostriatum: The phylogenetically newer part of the corpus striatum consisting of the caudate nucleus and putamen. It is often called simply the striatum. [NIH] Nephritis: Inflammation of the kidney; a focal or diffuse proliferative or destructive process which may involve the glomerulus, tubule, or interstitial renal tissue. [EU] Nephropathy: Disease of the kidneys. [EU] Nephrosis: Descriptive histopathologic term for renal disease without an inflammatory component. [NIH] Nephrotic: Pertaining to, resembling, or caused by nephrosis. [EU] Nephrotic Syndrome: Clinical association of heavy proteinuria, hypoalbuminemia, and generalized edema. [NIH] Nerve: A cordlike structure of nervous tissue that connects parts of the nervous system with other tissues of the body and conveys nervous impulses to, or away from, these tissues. [NIH] Nerve Endings: Specialized terminations of peripheral neurons. Nerve endings include neuroeffector junction(s) by which neurons activate target organs and sensory receptors which transduce information from the various sensory modalities and send it centrally in the nervous system. Presynaptic nerve endings are presynaptic terminals. [NIH] Nerve Fibers: Slender processes of neurons, especially the prolonged axons that conduct nerve impulses. [NIH] Nerve Growth Factor: Nerve growth factor is the first of a series of neurotrophic factors that were found to influence the growth and differentiation of sympathetic and sensory neurons. It is comprised of alpha, beta, and gamma subunits. The beta subunit is responsible for its growth stimulating activity. [NIH] Nervous System: The entire nerve apparatus composed of the brain, spinal cord, nerves and ganglia. [NIH] Networks: Pertaining to a nerve or to the nerves, a meshlike structure of interlocking fibers or strands. [NIH] Neural: 1. Pertaining to a nerve or to the nerves. 2. Situated in the region of the spinal axis, as the neutral arch. [EU]

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Neurites: In tissue culture, hairlike projections of neurons stimulated by growth factors and other molecules. These projections may go on to form a branched tree of dendrites or a single axon or they may be reabsorbed at a later stage of development. "Neurite" may refer to any filamentous or pointed outgrowth of an embryonal or tissue-culture neural cell. [NIH] Neuroeffector Junction: The synapse between a neuron (presynaptic) and an effector cell other than another neuron (postsynaptic). Neuroeffector junctions include synapses onto muscles and onto secretory cells. [NIH] Neuroma: A tumor that arises in nerve cells. [NIH] Neuromuscular: Pertaining to muscles and nerves. [EU] Neuromuscular Diseases: A general term encompassing lower motor neuron disease; peripheral nervous system diseases; and certain muscular diseases. Manifestations include muscle weakness; fasciculation; muscle atrophy; spasm; myokymia; muscle hypertonia, myalgias, and musclehypotonia. [NIH] Neuromuscular Junction: The synapse between a neuron and a muscle. [NIH] Neuronal: Pertaining to a neuron or neurons (= conducting cells of the nervous system). [EU] Neurons: The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the nervous system. [NIH] Neuropathy: A problem in any part of the nervous system except the brain and spinal cord. Neuropathies can be caused by infection, toxic substances, or disease. [NIH] Neurophysiology: The scientific discipline concerned with the physiology of the nervous system. [NIH] Neuroretinitis: Inflammation of the optic nerve head and adjacent retina. [NIH] Neurotransmitters: Endogenous signaling molecules that alter the behavior of neurons or effector cells. Neurotransmitter is used here in its most general sense, including not only messengers that act directly to regulate ion channels, but also those that act through second messenger systems, and those that act at a distance from their site of release. Included are neuromodulators, neuroregulators, neuromediators, and neurohumors, whether or not acting at synapses. [NIH] Neurotrophins: A nerve growth factor. [NIH] Neutrons: Electrically neutral elementary particles found in all atomic nuclei except light hydrogen; the mass is equal to that of the proton and electron combined and they are unstable when isolated from the nucleus, undergoing beta decay. Slow, thermal, epithermal, and fast neutrons refer to the energy levels with which the neutrons are ejected from heavier nuclei during their decay. [NIH] Night Blindness: Anomaly of vision in which there is a pronounced inadequacy or complete absence of dark-adaptation. [NIH] Nitrogen: An element with the atomic symbol N, atomic number 7, and atomic weight 14. Nitrogen exists as a diatomic gas and makes up about 78% of the earth's atmosphere by volume. It is a constituent of proteins and nucleic acids and found in all living cells. [NIH] Nonverbal Communication: Transmission of emotions, ideas, and attitudes between individuals in ways other than the spoken language. [NIH] Normotensive: 1. Characterized by normal tone, tension, or pressure, as by normal blood pressure. 2. A person with normal blood pressure. [EU] Notochord: The rod-shaped body, composed of cells derived from the mesoblast and defining the primitive axis of the embryo. In lower vertebrates, it persists throughout life as

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the main axial support of the body, but in higher vertebrates it is replaced by the vertebral column. [NIH] Nuclear: A test of the structure, blood flow, and function of the kidneys. The doctor injects a mildly radioactive solution into an arm vein and uses x-rays to monitor its progress through the kidneys. [NIH] Nuclear Family: A family composed of spouses and their children. [NIH] Nuclei: A body of specialized protoplasm found in nearly all cells and containing the chromosomes. [NIH] Nucleic acid: Either of two types of macromolecule (DNA or RNA) formed by polymerization of nucleotides. Nucleic acids are found in all living cells and contain the information (genetic code) for the transfer of genetic information from one generation to the next. [NIH] Nucleic Acid Hybridization: The process whereby two single-stranded polynucleotides form a double-stranded molecule, with hydrogen bonding between the complementary bases in the two strains. [NIH] Nucleus: A body of specialized protoplasm found in nearly all cells and containing the chromosomes. [NIH] Nursing Care: Care given to patients by nursing service personnel. [NIH] Nymph: The immature stage in the life cycle of those orders of insects characterized by gradual metamorphosis, in which the young resemble the imago in general form of body, including compound eyes and external wings; also the 8-legged stage of mites and ticks that follows the first moult. [NIH] Nystagmus: Rhythmical oscillation of the eyeballs, either pendular or jerky. [NIH] Occipital Lobe: Posterior part of the cerebral hemisphere. [NIH] Ocular: 1. Of, pertaining to, or affecting the eye. 2. Eyepiece. [EU] Oliguria: Clinical manifestation of the urinary system consisting of a decrease in the amount of urine secreted. [NIH] Omnidirectional: Sound source which radiates uniformly in all directions. [NIH] Oncogene: A gene that normally directs cell growth. If altered, an oncogene can promote or allow the uncontrolled growth of cancer. Alterations can be inherited or caused by an environmental exposure to carcinogens. [NIH] Oncogenic: Chemical, viral, radioactive or other agent that causes cancer; carcinogenic. [NIH] On-line: A sexually-reproducing population derived from a common parentage. [NIH] Oocytes: Female germ cells in stages between the prophase of the first maturation division and the completion of the second maturation division. [NIH] Opacity: Degree of density (area most dense taken for reading). [NIH] Ophthalmic: Pertaining to the eye. [EU] Ophthalmologic: Pertaining to ophthalmology (= the branch of medicine dealing with the eye). [EU] Ophthalmology: A surgical specialty concerned with the structure and function of the eye and the medical and surgical treatment of its defects and diseases. [NIH] Opsin: A protein formed, together with retinene, by the chemical breakdown of metarhodopsin. [NIH] Optic Disk: The portion of the optic nerve seen in the fundus with the ophthalmoscope. It is

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formed by the meeting of all the retinal ganglion cell axons as they enter the optic nerve. [NIH]

Optic Nerve: The 2nd cranial nerve. The optic nerve conveys visual information from the retina to the brain. The nerve carries the axons of the retinal ganglion cells which sort at the optic chiasm and continue via the optic tracts to the brain. The largest projection is to the lateral geniculate nuclei; other important targets include the superior colliculi and the suprachiasmatic nuclei. Though known as the second cranial nerve, it is considered part of the central nervous system. [NIH] Orbicularis: A thin layer of fibers that originates at the posterior lacrimal crest and passes outward and forward, dividing into two slips which surround the canaliculi. [NIH] Organelles: Specific particles of membrane-bound organized living substances present in eukaryotic cells, such as the mitochondria; the golgi apparatus; endoplasmic reticulum; lysomomes; plastids; and vacuoles. [NIH] Osmosis: Tendency of fluids (e.g., water) to move from the less concentrated to the more concentrated side of a semipermeable membrane. [NIH] Osmotic: Pertaining to or of the nature of osmosis (= the passage of pure solvent from a solution of lesser to one of greater solute concentration when the two solutions are separated by a membrane which selectively prevents the passage of solute molecules, but is permeable to the solvent). [EU] Ossicle: A small bone. [EU] Ossification: The formation of bone or of a bony substance; the conversion of fibrous tissue or of cartilage into bone or a bony substance. [EU] Osteoblasts: Bone-forming cells which secrete an extracellular matrix. Hydroxyapatite crystals are then deposited into the matrix to form bone. [NIH] Osteolysis: Dissolution of bone that particularly involves the removal or loss of calcium. [NIH]

Otitis: Inflammation of the ear, which may be marked by pain, fever, abnormalities of hearing, hearing loss, tinnitus, and vertigo. [EU] Otitis Media: Inflammation of the middle ear. [NIH] Otolaryngology: A surgical specialty concerned with the study and treatment of disorders of the ear, nose, and throat. [NIH] Otosclerosis: The formation of spongy bone in the labyrinth capsule. The ossicles can become fixed and unable to transmit sound vibrations, thereby causing deafness. [NIH] Ototoxic: Having a deleterious effect upon the eighth nerve, or upon the organs of hearing and balance. [EU] Outer ear: The pinna and external meatus of the ear. [NIH] Oval Window: Fenestra of the vestibule; an oval opening in the medial wall of the middle ear leading into the vestibule. Normally it is covered by the base of the stapes. [NIH] Ovum: A female germ cell extruded from the ovary at ovulation. [NIH] Oxidation: The act of oxidizing or state of being oxidized. Chemically it consists in the increase of positive charges on an atom or the loss of negative charges. Most biological oxidations are accomplished by the removal of a pair of hydrogen atoms (dehydrogenation) from a molecule. Such oxidations must be accompanied by reduction of an acceptor molecule. Univalent o. indicates loss of one electron; divalent o., the loss of two electrons. [EU]

Oxidative Phosphorylation: Electron transfer through the cytochrome system liberating free

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energy which is transformed into high-energy phosphate bonds. [NIH] Oxidative Stress: A disturbance in the prooxidant-antioxidant balance in favor of the former, leading to potential damage. Indicators of oxidative stress include damaged DNA bases, protein oxidation products, and lipid peroxidation products (Sies, Oxidative Stress, 1991, pxv-xvi). [NIH] Oxygen Consumption: The oxygen consumption is determined by calculating the difference between the amount of oxygen inhaled and exhaled. [NIH] Oxygenation: The process of supplying, treating, or mixing with oxygen. No:1245 oxygenation the process of supplying, treating, or mixing with oxygen. [EU] Pachymeningitis: Inflammation of the dura mater of the brain, the spinal cord or the optic nerve. [NIH] Palliative: 1. Affording relief, but not cure. 2. An alleviating medicine. [EU] Palsy: Disease of the peripheral nervous system occurring usually after many years of increased lead absorption. [NIH] Pancreas: A mixed exocrine and endocrine gland situated transversely across the posterior abdominal wall in the epigastric and hypochondriac regions. The endocrine portion is comprised of the Islets of Langerhans, while the exocrine portion is a compound acinar gland that secretes digestive enzymes. [NIH] Pancreatic: Having to do with the pancreas. [NIH] Pancreatic cancer: Cancer of the pancreas, a salivary gland of the abdomen. [NIH] Paralysis: Loss of ability to move all or part of the body. [NIH] Parietal: 1. Of or pertaining to the walls of a cavity. 2. Pertaining to or located near the parietal bone, as the parietal lobe. [EU] Parietal Lobe: Upper central part of the cerebral hemisphere. [NIH] Parotid: The space that contains the parotid gland, the facial nerve, the external carotid artery, and the retromandibular vein. [NIH] Paroxysmal: Recurring in paroxysms (= spasms or seizures). [EU] Particle: A tiny mass of material. [EU] Partnership Practice: A voluntary contract between two or more doctors who may or may not share responsibility for the care of patients, with proportional sharing of profits and losses. [NIH] Patch: A piece of material used to cover or protect a wound, an injured part, etc.: a patch over the eye. [NIH] Pathogenesis: The cellular events and reactions that occur in the development of disease. [NIH]

Pathologic: 1. Indicative of or caused by a morbid condition. 2. Pertaining to pathology (= branch of medicine that treats the essential nature of the disease, especially the structural and functional changes in tissues and organs of the body caused by the disease). [EU] Pathologic Processes: The abnormal mechanisms and forms involved in the dysfunctions of tissues and organs. [NIH] Pathologies: The study of abnormality, especially the study of diseases. [NIH] Pathophysiology: Altered functions in an individual or an organ due to disease. [NIH] Patient Care Management: Generating, planning, organizing, and administering medical and nursing care and services for patients. [NIH]

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Patient Education: The teaching or training of patients concerning their own health needs. [NIH]

Pediatrics: A medical specialty concerned with maintaining health and providing medical care to children from birth to adolescence. [NIH] Pedigree: A record of one's ancestors, offspring, siblings, and their offspring that may be used to determine the pattern of certain genes or disease inheritance within a family. [NIH] Peer Group: Group composed of associates of same species, approximately the same age, and usually of similar rank or social status. [NIH] Pelvic: Pertaining to the pelvis. [EU] Pentoxifylline: A methylxanthine derivative that inhibits phosphodiesterase and affects blood rheology. It improves blood flow by increasing erythrocyte and leukocyte flexibility. It also inhibits platelet aggregation. Pentoxifylline modulates immunologic activity by stimulating cytokine production. [NIH] Peptide: Any compound consisting of two or more amino acids, the building blocks of proteins. Peptides are combined to make proteins. [NIH] Perception: The ability quickly and accurately to recognize similarities and differences among presented objects, whether these be pairs of words, pairs of number series, or multiple sets of these or other symbols such as geometric figures. [NIH] Percutaneous: Performed through the skin, as injection of radiopacque material in radiological examination, or the removal of tissue for biopsy accomplished by a needle. [EU] Perforation: 1. The act of boring or piercing through a part. 2. A hole made through a part or substance. [EU] Perfusion: Bathing an organ or tissue with a fluid. In regional perfusion, a specific area of the body (usually an arm or a leg) receives high doses of anticancer drugs through a blood vessel. Such a procedure is performed to treat cancer that has not spread. [NIH] Perilymph: The fluid contained within the space separating the membranous from the osseous labyrinth of the ear. [NIH] Peripheral Nervous System: The nervous system outside of the brain and spinal cord. The peripheral nervous system has autonomic and somatic divisions. The autonomic nervous system includes the enteric, parasympathetic, and sympathetic subdivisions. The somatic nervous system includes the cranial and spinal nerves and their ganglia and the peripheral sensory receptors. [NIH] Peripheral Nervous System Diseases: Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves. [NIH] Peripheral Neuropathy: Nerve damage, usually affecting the feet and legs; causing pain, numbness, or a tingling feeling. Also called "somatic neuropathy" or "distal sensory polyneuropathy." [NIH] Peripheral vision: Side vision; ability to see objects and movement outside of the direct line of vision. [NIH] Periventricular Leukomalacia: Rare form of epilepsy. [NIH] Pernicious: Tending to a fatal issue. [EU] Peroxidase: A hemeprotein from leukocytes. Deficiency of this enzyme leads to a hereditary disorder coupled with disseminated moniliasis. It catalyzes the conversion of a donor and peroxide to an oxidized donor and water. EC 1.11.1.7. [NIH] Phagocytosis: The engulfing of microorganisms, other cells, and foreign particles by

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phagocytic cells. [NIH] Phallic: Pertaining to the phallus, or penis. [EU] Pharmacologic: Pertaining to pharmacology or to the properties and reactions of drugs. [EU] Pharynx: The hollow tube about 5 inches long that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes to the stomach). [NIH] Phenotype: The outward appearance of the individual. It is the product of interactions between genes and between the genotype and the environment. This includes the killer phenotype, characteristic of yeasts. [NIH] Phenylalanine: An aromatic amino acid that is essential in the animal diet. It is a precursor of melanin, dopamine, noradrenalin, and thyroxine. [NIH] Phosphodiesterase: Effector enzyme that regulates the levels of a second messenger, the cyclic GMP. [NIH] Phospholipases: A class of enzymes that catalyze the hydrolysis of phosphoglycerides or glycerophosphatidates. EC 3.1.-. [NIH] Phospholipids: Lipids containing one or more phosphate groups, particularly those derived from either glycerol (phosphoglycerides; glycerophospholipids) or sphingosine (sphingolipids). They are polar lipids that are of great importance for the structure and function of cell membranes and are the most abundant of membrane lipids, although not stored in large amounts in the system. [NIH] Phosphorus: A non-metallic element that is found in the blood, muscles, nevers, bones, and teeth, and is a component of adenosine triphosphate (ATP; the primary energy source for the body's cells.) [NIH] Phosphorylate: Attached to a phosphate group. [NIH] Phosphorylated: Attached to a phosphate group. [NIH] Phosphorylating: Attached to a phosphate group. [NIH] Phosphorylation: The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety. [NIH] Photoreceptor: Receptor capable of being activated by light stimuli, as a rod or cone cell of the eye. [NIH] Physical Examination: Systematic and thorough inspection of the patient for physical signs of disease or abnormality. [NIH] Physiologic: Having to do with the functions of the body. When used in the phrase "physiologic age," it refers to an age assigned by general health, as opposed to calendar age. [NIH]

Physiology: The science that deals with the life processes and functions of organismus, their cells, tissues, and organs. [NIH] Phytic Acid: Complexing agent for removal of traces of heavy metal ions. It acts also as a hypocalcemic agent. [NIH] Pigment: A substance that gives color to tissue. Pigments are responsible for the color of skin, eyes, and hair. [NIH] Pilot Projects: Small-scale tests of methods and procedures to be used on a larger scale if the pilot study demonstrates that these methods and procedures can work. [NIH] Pilot study: The initial study examining a new method or treatment. [NIH] Pitch: The subjective awareness of the frequency or spectral distribution of a sound. [NIH]

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Placenta: A highly vascular fetal organ through which the fetus absorbs oxygen and other nutrients and excretes carbon dioxide and other wastes. It begins to form about the eighth day of gestation when the blastocyst adheres to the decidua. [NIH] Plants: Multicellular, eukaryotic life forms of the kingdom Plantae. They are characterized by a mainly photosynthetic mode of nutrition; essentially unlimited growth at localized regions of cell divisions (meristems); cellulose within cells providing rigidity; the absence of organs of locomotion; absense of nervous and sensory systems; and an alteration of haploid and diploid generations. [NIH] Plaque: A clear zone in a bacterial culture grown on an agar plate caused by localized destruction of bacterial cells by a bacteriophage. The concentration of infective virus in a fluid can be estimated by applying the fluid to a culture and counting the number of. [NIH] Plasma: The clear, yellowish, fluid part of the blood that carries the blood cells. The proteins that form blood clots are in plasma. [NIH] Plasma cells: A type of white blood cell that produces antibodies. [NIH] Plasticity: In an individual or a population, the capacity for adaptation: a) through gene changes (genetic plasticity) or b) through internal physiological modifications in response to changes of environment (physiological plasticity). [NIH] Plastids: Self-replicating cytoplasmic organelles of plant and algal cells that contain pigments and may synthesize and accumulate various substances. Plastids are used in phylogenetic studies. [NIH] Platelet Activation: A series of progressive, overlapping events triggered by exposure of the platelets to subendothelial tissue. These events include shape change, adhesiveness, aggregation, and release reactions. When carried through to completion, these events lead to the formation of a stable hemostatic plug. [NIH] Platelet Aggregation: The attachment of platelets to one another. This clumping together can be induced by a number of agents (e.g., thrombin, collagen) and is part of the mechanism leading to the formation of a thrombus. [NIH] Platinum: Platinum. A heavy, soft, whitish metal, resembling tin, atomic number 78, atomic weight 195.09, symbol Pt. (From Dorland, 28th ed) It is used in manufacturing equipment for laboratory and industrial use. It occurs as a black powder (platinum black) and as a spongy substance (spongy platinum) and may have been known in Pliny's time as "alutiae". [NIH]

Point Mutation: A mutation caused by the substitution of one nucleotide for another. This results in the DNA molecule having a change in a single base pair. [NIH] Poisoning: A condition or physical state produced by the ingestion, injection or inhalation of, or exposure to a deleterious agent. [NIH] Polycystic: An inherited disorder characterized by many grape-like clusters of fluid-filled cysts that make both kidneys larger over time. These cysts take over and destroy working kidney tissue. PKD may cause chronic renal failure and end-stage renal disease. [NIH] Polyhydramnios: Excess of amniotic fluid greater than 2,000 ml. It is a common obstetrical complication whose major causes include maternal diabetes, chromosomal disorders, isoimmunological disease, congenital abnormalities, and multiple gestations. [NIH] Polymorphic: Occurring in several or many forms; appearing in different forms at different stages of development. [EU] Polymorphism: The occurrence together of two or more distinct forms in the same population. [NIH] Polypeptide: A peptide which on hydrolysis yields more than two amino acids; called

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tripeptides, tetrapeptides, etc. according to the number of amino acids contained. [EU] Polysaccharide: A type of carbohydrate. It contains sugar molecules that are linked together chemically. [NIH] Pons: The part of the central nervous system lying between the medulla oblongata and the mesencephalon, ventral to the cerebellum, and consisting of a pars dorsalis and a pars ventralis. [NIH] Posterior: Situated in back of, or in the back part of, or affecting the back or dorsal surface of the body. In lower animals, it refers to the caudal end of the body. [EU] Postnatal: Occurring after birth, with reference to the newborn. [EU] Postoperative: After surgery. [NIH] Postsynaptic: Nerve potential generated by an inhibitory hyperpolarizing stimulation. [NIH] Post-synaptic: Nerve potential generated by an inhibitory hyperpolarizing stimulation. [NIH] Post-translational: The cleavage of signal sequence that directs the passage of the protein through a cell or organelle membrane. [NIH] Potassium: An element that is in the alkali group of metals. It has an atomic symbol K, atomic number 19, and atomic weight 39.10. It is the chief cation in the intracellular fluid of muscle and other cells. Potassium ion is a strong electrolyte and it plays a significant role in the regulation of fluid volume and maintenance of the water-electrolyte balance. [NIH] Potassium Channels: Cell membrane glycoproteins selective for potassium ions. [NIH] Potentiates: A degree of synergism which causes the exposure of the organism to a harmful substance to worsen a disease already contracted. [NIH] Potentiation: An overall effect of two drugs taken together which is greater than the sum of the effects of each drug taken alone. [NIH] Practice Guidelines: Directions or principles presenting current or future rules of policy for the health care practitioner to assist him in patient care decisions regarding diagnosis, therapy, or related clinical circumstances. The guidelines may be developed by government agencies at any level, institutions, professional societies, governing boards, or by the convening of expert panels. The guidelines form a basis for the evaluation of all aspects of health care and delivery. [NIH] Precursor: Something that precedes. In biological processes, a substance from which another, usually more active or mature substance is formed. In clinical medicine, a sign or symptom that heralds another. [EU] Predisposition: A latent susceptibility to disease which may be activated under certain conditions, as by stress. [EU] Prednisolone: A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states. [NIH] Prednisone: A synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver. [NIH] Pregnancy Tests: Tests to determine whether or not an individual is pregnant. [NIH] Prejudice: A preconceived judgment made without adequate evidence and not easily alterable by presentation of contrary evidence. [NIH] Prenatal: Existing or occurring before birth, with reference to the fetus. [EU] Prenatal Diagnosis: Determination of the nature of a pathological condition or disease in the postimplantation embryo, fetus, or pregnant female before birth. [NIH]

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Presbycusis: Progressive bilateral loss of hearing that occurs in the aged. Syn: senile deafness. [NIH] Presynaptic: Situated proximal to a synapse, or occurring before the synapse is crossed. [EU] Presynaptic Terminals: The distal terminations of axons which are specialized for the release of neurotransmitters. Also included are varicosities along the course of axons which have similar specializations and also release transmitters. Presynaptic terminals in both the central and peripheral nervous systems are included. [NIH] Prevalence: The total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time. [NIH] Private Practice: Practice of a health profession by an individual, offering services on a person-to-person basis, as opposed to group or partnership practice. [NIH] Probe: An instrument used in exploring cavities, or in the detection and dilatation of strictures, or in demonstrating the potency of channels; an elongated instrument for exploring or sounding body cavities. [NIH] Problem Solving: A learning situation involving more than one alternative from which a selection is made in order to attain a specific goal. [NIH] Progeny: The offspring produced in any generation. [NIH] Progesterone: Pregn-4-ene-3,20-dione. The principal progestational hormone of the body, secreted by the corpus luteum, adrenal cortex, and placenta. Its chief function is to prepare the uterus for the reception and development of the fertilized ovum. It acts as an antiovulatory agent when administered on days 5-25 of the menstrual cycle. [NIH] Progression: Increase in the size of a tumor or spread of cancer in the body. [NIH] Progressive: Advancing; going forward; going from bad to worse; increasing in scope or severity. [EU] Progressive Bulbar Palsy: Disease of the peripheral nervous system occurring usually after many years of increased lead absorption. [NIH] Projection: A defense mechanism, operating unconsciously, whereby that which is emotionally unacceptable in the self is rejected and attributed (projected) to others. [NIH] Proline: A non-essential amino acid that is synthesized from glutamic acid. It is an essential component of collagen and is important for proper functioning of joints and tendons. [NIH] Promoter: A chemical substance that increases the activity of a carcinogenic process. [NIH] Prophase: The first phase of cell division, in which the chromosomes become visible, the nucleus starts to lose its identity, the spindle appears, and the centrioles migrate toward opposite poles. [NIH] Prophylaxis: An attempt to prevent disease. [NIH] Prosencephalon: The part of the brain developed from the most rostral of the three primary vesicles of the embryonic neural tube and consisting of the diencephalon and telencephalon. [NIH]

Prospective study: An epidemiologic study in which a group of individuals (a cohort), all free of a particular disease and varying in their exposure to a possible risk factor, is followed over a specific amount of time to determine the incidence rates of the disease in the exposed and unexposed groups. [NIH] Prostaglandin: Any of a group of components derived from unsaturated 20-carbon fatty acids, primarily arachidonic acid, via the cyclooxygenase pathway that are extremely potent mediators of a diverse group of physiologic processes. The abbreviation for prostaglandin is

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PG; specific compounds are designated by adding one of the letters A through I to indicate the type of substituents found on the hydrocarbon skeleton and a subscript (1, 2 or 3) to indicate the number of double bonds in the hydrocarbon skeleton e.g., PGE2. The predominant naturally occurring prostaglandins all have two double bonds and are synthesized from arachidonic acid (5,8,11,14-eicosatetraenoic acid) by the pathway shown in the illustration. The 1 series and 3 series are produced by the same pathway with fatty acids having one fewer double bond (8,11,14-eicosatrienoic acid or one more double bond (5,8,11,14,17-eicosapentaenoic acid) than arachidonic acid. The subscript a or ß indicates the configuration at C-9 (a denotes a substituent below the plane of the ring, ß, above the plane). The naturally occurring PGF's have the a configuration, e.g., PGF2a. All of the prostaglandins act by binding to specific cell-surface receptors causing an increase in the level of the intracellular second messenger cyclic AMP (and in some cases cyclic GMP also). The effect produced by the cyclic AMP increase depends on the specific cell type. In some cases there is also a positive feedback effect. Increased cyclic AMP increases prostaglandin synthesis leading to further increases in cyclic AMP. [EU] Prostaglandins A: (13E,15S)-15-Hydroxy-9-oxoprosta-10,13-dien-1-oic acid (PGA(1)); (5Z,13E,15S)-15-hydroxy-9-oxoprosta-5,10,13-trien-1-oic acid (PGA(2)); (5Z,13E,15S,17Z)-15hydroxy-9-oxoprosta-5,10,13,17-tetraen-1-oic acid (PGA(3)). A group of naturally occurring secondary prostaglandins derived from PGE. PGA(1) and PGA(2) as well as their 19hydroxy derivatives are found in many organs and tissues. [NIH] Prostate: A gland in males that surrounds the neck of the bladder and the urethra. It secretes a substance that liquifies coagulated semen. It is situated in the pelvic cavity behind the lower part of the pubic symphysis, above the deep layer of the triangular ligament, and rests upon the rectum. [NIH] Prosthesis: An artificial replacement of a part of the body. [NIH] Protease: Proteinase (= any enzyme that catalyses the splitting of interior peptide bonds in a protein). [EU] Protein C: A vitamin-K dependent zymogen present in the blood, which, upon activation by thrombin and thrombomodulin exerts anticoagulant properties by inactivating factors Va and VIIIa at the rate-limiting steps of thrombin formation. [NIH] Protein S: The vitamin K-dependent cofactor of activated protein C. Together with protein C, it inhibits the action of factors VIIIa and Va. A deficiency in protein S can lead to recurrent venous and arterial thrombosis. [NIH] Protein Transport: The process of moving proteins from one cellular compartment (including extracellular) to another by various sorting and transport mechanisms such as gated transport, protein translocation, and vesicular transport. [NIH] Proteins: Polymers of amino acids linked by peptide bonds. The specific sequence of amino acids determines the shape and function of the protein. [NIH] Proteinuria: The presence of protein in the urine, indicating that the kidneys are not working properly. [NIH] Proteoglycans: Glycoproteins which have a very high polysaccharide content. [NIH] Proteolytic: 1. Pertaining to, characterized by, or promoting proteolysis. 2. An enzyme that promotes proteolysis (= the splitting of proteins by hydrolysis of the peptide bonds with formation of smaller polypeptides). [EU] Protocol: The detailed plan for a clinical trial that states the trial's rationale, purpose, drug or vaccine dosages, length of study, routes of administration, who may participate, and other aspects of trial design. [NIH]

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Protozoa: A subkingdom consisting of unicellular organisms that are the simplest in the animal kingdom. Most are free living. They range in size from submicroscopic to macroscopic. Protozoa are divided into seven phyla: Sarcomastigophora, Labyrinthomorpha, Apicomplexa, Microspora, Ascetospora, Myxozoa, and Ciliophora. [NIH] Proximal: Nearest; closer to any point of reference; opposed to distal. [EU] Pruritic: Pertaining to or characterized by pruritus. [EU] Psychiatry: The medical science that deals with the origin, diagnosis, prevention, and treatment of mental disorders. [NIH] Psychic: Pertaining to the psyche or to the mind; mental. [EU] Psychology: The science dealing with the study of mental processes and behavior in man and animals. [NIH] Psychotherapy: A generic term for the treatment of mental illness or emotional disturbances primarily by verbal or nonverbal communication. [NIH] Ptosis: 1. Prolapse of an organ or part. 2. Drooping of the upper eyelid from paralysis of the third nerve or from sympathetic innervation. [EU] Public Health: Branch of medicine concerned with the prevention and control of disease and disability, and the promotion of physical and mental health of the population on the international, national, state, or municipal level. [NIH] Public Policy: A course or method of action selected, usually by a government, from among alternatives to guide and determine present and future decisions. [NIH] Publishing: "The business or profession of the commercial production and issuance of literature" (Webster's 3d). It includes the publisher, publication processes, editing and editors. Production may be by conventional printing methods or by electronic publishing. [NIH]

Pulmonary: Relating to the lungs. [NIH] Pulmonary Artery: The short wide vessel arising from the conus arteriosus of the right ventricle and conveying unaerated blood to the lungs. [NIH] Pulmonary Edema: An accumulation of an excessive amount of watery fluid in the lungs, may be caused by acute exposure to dangerous concentrations of irritant gasses. [NIH] Pulse: The rhythmical expansion and contraction of an artery produced by waves of pressure caused by the ejection of blood from the left ventricle of the heart as it contracts. [NIH]

Pupa: An inactive stage between the larval and adult stages in the life cycle of insects. [NIH] Purines: A series of heterocyclic compounds that are variously substituted in nature and are known also as purine bases. They include adenine and guanine, constituents of nucleic acids, as well as many alkaloids such as caffeine and theophylline. Uric acid is the metabolic end product of purine metabolism. [NIH] Putamen: The largest and most lateral of the basal ganglia lying between the lateral medullary lamina of the globus pallidus and the external capsule. It is part of the neostriatum and forms part of the lentiform nucleus along with the globus pallidus. [NIH] Quality of Life: A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral and social environment. [NIH] Quiescent: Marked by a state of inactivity or repose. [EU] Race: A population within a species which exhibits general similarities within itself, but is both discontinuous and distinct from other populations of that species, though not sufficiently so as to achieve the status of a taxon. [NIH]

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Radial Nerve: A major nerve of the upper extremity. In humans the fibers of the radial nerve originate in the lower cervical and upper thoracic spinal cord (usually C5 to T1), travel via the posterior cord of the brachial plexus, and supply motor innervation to extensor muscles of the arm and cutaneous sensory fibers to extensor regions of the arm and hand. [NIH]

Radiation: Emission or propagation of electromagnetic energy (waves/rays), or the waves/rays themselves; a stream of electromagnetic particles (electrons, neutrons, protons, alpha particles) or a mixture of these. The most common source is the sun. [NIH] Radiation therapy: The use of high-energy radiation from x-rays, gamma rays, neutrons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body in the area near cancer cells (internal radiation therapy, implant radiation, or brachytherapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that circulates throughout the body. Also called radiotherapy. [NIH] Radio Waves: That portion of the electromagnetic spectrum beyond the microwaves, with wavelengths as high as 30 KM. They are used in communications, including television. Short Wave or HF (high frequency), UHF (ultrahigh frequency) and VHF (very high frequency) waves are used in citizen's band communication. [NIH] Radioactive: Giving off radiation. [NIH] Radiography: Examination of any part of the body for diagnostic purposes by means of roentgen rays, recording the image on a sensitized surface (such as photographic film). [NIH] Radiolabeled: Any compound that has been joined with a radioactive substance. [NIH] Radiological: Pertaining to radiodiagnostic and radiotherapeutic procedures, and interventional radiology or other planning and guiding medical radiology. [NIH] Radiology: A specialty concerned with the use of x-ray and other forms of radiant energy in the diagnosis and treatment of disease. [NIH] Radiotherapy: The use of ionizing radiation to treat malignant neoplasms and other benign conditions. The most common forms of ionizing radiation used as therapy are x-rays, gamma rays, and electrons. A special form of radiotherapy, targeted radiotherapy, links a cytotoxic radionuclide to a molecule that targets the tumor. When this molecule is an antibody or other immunologic molecule, the technique is called radioimmunotherapy. [NIH] Random Allocation: A process involving chance used in therapeutic trials or other research endeavor for allocating experimental subjects, human or animal, between treatment and control groups, or among treatment groups. It may also apply to experiments on inanimate objects. [NIH] Randomization: Also called random allocation. Is allocation of individuals to groups, e.g., for experimental and control regimens, by chance. Within the limits of chance variation, random allocation should make the control and experimental groups similar at the start of an investigation and ensure that personal judgment and prejudices of the investigator do not influence allocation. [NIH] Randomized: Describes an experiment or clinical trial in which animal or human subjects are assigned by chance to separate groups that compare different treatments. [NIH] Receptor: A molecule inside or on the surface of a cell that binds to a specific substance and causes a specific physiologic effect in the cell. [NIH] Recombinant: A cell or an individual with a new combination of genes not found together in either parent; usually applied to linked genes. [EU]

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Recombination: The formation of new combinations of genes as a result of segregation in crosses between genetically different parents; also the rearrangement of linked genes due to crossing-over. [NIH] Rectum: The last 8 to 10 inches of the large intestine. [NIH] Recurrence: The return of a sign, symptom, or disease after a remission. [NIH] Red Nucleus: A pinkish-yellow portion of the midbrain situated in the rostral mesencephalic tegmentum. It receives a large projection from the contralateral half of the cerebellum via the superior cerebellar peduncle and a projection from the ipsilateral motor cortex. [NIH] Reductase: Enzyme converting testosterone to dihydrotestosterone. [NIH] Refer: To send or direct for treatment, aid, information, de decision. [NIH] Reflective: Capable of throwing back light, images, sound waves : reflecting. [EU] Reflex: An involuntary movement or exercise of function in a part, excited in response to a stimulus applied to the periphery and transmitted to the brain or spinal cord. [NIH] Refraction: A test to determine the best eyeglasses or contact lenses to correct a refractive error (myopia, hyperopia, or astigmatism). [NIH] Refractive Errors: Deviations from the average or standard indices of refraction of the eye through its dioptric or refractive apparatus. [NIH] Refractive Power: The ability of an object, such as the eye, to bend light as light passes through it. [NIH] Regeneration: The natural renewal of a structure, as of a lost tissue or part. [EU] Regimen: A treatment plan that specifies the dosage, the schedule, and the duration of treatment. [NIH] Rehabilitative: Instruction of incapacitated individuals or of those affected with some mental disorder, so that some or all of their lost ability may be regained. [NIH] Reliability: Used technically, in a statistical sense, of consistency of a test with itself, i. e. the extent to which we can assume that it will yield the same result if repeated a second time. [NIH]

Remission: A decrease in or disappearance of signs and symptoms of cancer. In partial remission, some, but not all, signs and symptoms of cancer have disappeared. In complete remission, all signs and symptoms of cancer have disappeared, although there still may be cancer in the body. [NIH] Renal failure: Progressive renal insufficiency and uremia, due to irreversible and progressive renal glomerular tubular or interstitial disease. [NIH] Renal tubular: A defect in the kidneys that hinders their normal excretion of acids. Failure to excrete acids can lead to weak bones, kidney stones, and poor growth in children. [NIH] Renal tubular acidosis: A rare disorder in which structures in the kidney that filter the blood are impaired, producing using that is more acid than normal. [NIH] Research Support: Financial support of research activities. [NIH] Respiration: The act of breathing with the lungs, consisting of inspiration, or the taking into the lungs of the ambient air, and of expiration, or the expelling of the modified air which contains more carbon dioxide than the air taken in (Blakiston's Gould Medical Dictionary, 4th ed.). This does not include tissue respiration (= oxygen consumption) or cell respiration (= cell respiration). [NIH] Respiratory distress syndrome: A lung disease that occurs primarily in premature infants;

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the newborn must struggle for each breath and blueing of its skin reflects the baby's inability to get enough oxygen. [NIH] Restoration: Broad term applied to any inlay, crown, bridge or complete denture which restores or replaces loss of teeth or oral tissues. [NIH] Reticular: Coarse-fibered, netlike dermis layer. [NIH] Retina: The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outer-most layer is pigmented, whereas the inner nine layers are transparent. [NIH] Retinae: A congenital notch or cleft of the retina, usually located inferiorly. [NIH] Retinal: 1. Pertaining to the retina. 2. The aldehyde of retinol, derived by the oxidative enzymatic splitting of absorbed dietary carotene, and having vitamin A activity. In the retina, retinal combines with opsins to form visual pigments. One isomer, 11-cis retinal combines with opsin in the rods (scotopsin) to form rhodopsin, or visual purple. Another, all-trans retinal (trans-r.); visual yellow; xanthopsin) results from the bleaching of rhodopsin by light, in which the 11-cis form is converted to the all-trans form. Retinal also combines with opsins in the cones (photopsins) to form the three pigments responsible for colour vision. Called also retinal, and retinene1. [EU] Retinitis: Inflammation of the retina. It is rarely limited to the retina, but is commonly associated with diseases of the choroid (chorioretinitis) and of the optic nerve (neuroretinitis). The disease may be confined to one eye, but since it is generally dependent on a constitutional factor, it is almost always bilateral. It may be acute in course, but as a rule it lasts many weeks or even several months. [NIH] Retinitis Pigmentosa: Hereditary, progressive degeneration of the neuroepithelium of the retina characterized by night blindness and progressive contraction of the visual field. [NIH] Retinoblastoma: An eye cancer that most often occurs in children younger than 5 years. It occurs in hereditary and nonhereditary (sporadic) forms. [NIH] Retinol: Vitamin A. It is essential for proper vision and healthy skin and mucous membranes. Retinol is being studied for cancer prevention; it belongs to the family of drugs called retinoids. [NIH] Retinopathy: 1. Retinitis (= inflammation of the retina). 2. Retinosis (= degenerative, noninflammatory condition of the retina). [EU] Retrospective: Looking back at events that have already taken place. [NIH] Rheology: The study of the deformation and flow of matter, usually liquids or fluids, and of the plastic flow of solids. The concept covers consistency, dilatancy, liquefaction, resistance to flow, shearing, thixotrophy, and viscosity. [NIH] Rheumatism: A group of disorders marked by inflammation or pain in the connective tissue structures of the body. These structures include bone, cartilage, and fat. [NIH] Rheumatoid: Resembling rheumatism. [EU] Rheumatoid arthritis: A form of arthritis, the cause of which is unknown, although infection, hypersensitivity, hormone imbalance and psychologic stress have been suggested as possible causes. [NIH] Ribosome: A granule of protein and RNA, synthesized in the nucleolus and found in the cytoplasm of cells. Ribosomes are the main sites of protein synthesis. Messenger RNA attaches to them and there receives molecules of transfer RNA bearing amino acids. [NIH] Rigidity: Stiffness or inflexibility, chiefly that which is abnormal or morbid; rigor. [EU]

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Riluzole: A glutamate antagonist that has reported anticonvulsant activity. It has been shown to prolong the survival of patients with amyotrophic lateral sclerosis and has been approved in the United States to treat patients with ALS. [NIH] Risk factor: A habit, trait, condition, or genetic alteration that increases a person's chance of developing a disease. [NIH] Rod: A reception for vision, located in the retina. [NIH] Rubella: An acute, usually benign, infectious disease caused by a togavirus and most often affecting children and nonimmune young adults, in which the virus enters the respiratory tract via droplet nuclei and spreads to the lymphatic system. It is characterized by a slight cold, sore throat, and fever, followed by enlargement of the postauricular, suboccipital, and cervical lymph nodes, and the appearances of a fine pink rash that begins on the head and spreads to become generalized. Called also German measles, roetln, röteln, and three-day measles, and rubeola in French and Spanish. [EU] Saccule: The smaller of the 2 sacs within the vestibule of the ear. [NIH] Saliva: The clear, viscous fluid secreted by the salivary glands and mucous glands of the mouth. It contains mucins, water, organic salts, and ptylin. [NIH] Salivary: The duct that convey saliva to the mouth. [NIH] Salivary glands: Glands in the mouth that produce saliva. [NIH] Saponins: Sapogenin glycosides. A type of glycoside widely distributed in plants. Each consists of a sapogenin as the aglycon moiety, and a sugar. The sapogenin may be a steroid or a triterpene and the sugar may be glucose, galactose, a pentose, or a methylpentose. Sapogenins are poisonous towards the lower forms of life and are powerful hemolytics when injected into the blood stream able to dissolve red blood cells at even extreme dilutions. [NIH] Satellite: Applied to a vein which closely accompanies an artery for some distance; in cytogenetics, a chromosomal agent separated by a secondary constriction from the main body of the chromosome. [NIH] Scala Tympani: The lower tube of the cochlea, extending from the round window to the helicotrema and containing perilymph. [NIH] Scans: Pictures of structures inside the body. Scans often used in diagnosing, staging, and monitoring disease include liver scans, bone scans, and computed tomography (CT) or computerized axial tomography (CAT) scans and magnetic resonance imaging (MRI) scans. In liver scanning and bone scanning, radioactive substances that are injected into the bloodstream collect in these organs. A scanner that detects the radiation is used to create pictures. In CT scanning, an x-ray machine linked to a computer is used to produce detailed pictures of organs inside the body. MRI scans use a large magnet connected to a computer to create pictures of areas inside the body. [NIH] Schizoid: Having qualities resembling those found in greater degree in schizophrenics; a person of schizoid personality. [NIH] Schizophrenia: A mental disorder characterized by a special type of disintegration of the personality. [NIH] Schizotypal Personality Disorder: A personality disorder in which there are oddities of thought (magical thinking, paranoid ideation, suspiciousness), perception (illusions, depersonalization), speech (digressive, vague, overelaborate), and behavior (inappropriate affect in social interactions, frequently social isolation) that are not severe enough to characterize schizophrenia. [NIH] Sclerosis: A pathological process consisting of hardening or fibrosis of an anatomical

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structure, often a vessel or a nerve. [NIH] Screening: Checking for disease when there are no symptoms. [NIH] Secretion: 1. The process of elaborating a specific product as a result of the activity of a gland; this activity may range from separating a specific substance of the blood to the elaboration of a new chemical substance. 2. Any substance produced by secretion. [EU] Secretory: Secreting; relating to or influencing secretion or the secretions. [NIH] Segregation: The separation in meiotic cell division of homologous chromosome pairs and their contained allelomorphic gene pairs. [NIH] Seizures: Clinical or subclinical disturbances of cortical function due to a sudden, abnormal, excessive, and disorganized discharge of brain cells. Clinical manifestations include abnormal motor, sensory and psychic phenomena. Recurrent seizures are usually referred to as epilepsy or "seizure disorder." [NIH] Semen: The thick, yellowish-white, viscid fluid secretion of male reproductive organs discharged upon ejaculation. In addition to reproductive organ secretions, it contains spermatozoa and their nutrient plasma. [NIH] Semicircular canal: Three long canals of the bony labyrinth of the ear, forming loops and opening into the vestibule by five openings. [NIH] Senile: Relating or belonging to old age; characteristic of old age; resulting from infirmity of old age. [NIH] Sensor: A device designed to respond to physical stimuli such as temperature, light, magnetism or movement and transmit resulting impulses for interpretation, recording, movement, or operating control. [NIH] Sensory Deprivation: The absence or restriction of the usual external sensory stimuli to which the individual responds. [NIH] Septicemia: Systemic disease associated with the presence and persistence of pathogenic microorganisms or their toxins in the blood. Called also blood poisoning. [EU] Sequencing: The determination of the order of nucleotides in a DNA or RNA chain. [NIH] Serine: A non-essential amino acid occurring in natural form as the L-isomer. It is synthesized from glycine or threonine. It is involved in the biosynthesis of purines, pyrimidines, and other amino acids. [NIH] Serum: The clear liquid part of the blood that remains after blood cells and clotting proteins have been removed. [NIH] Sex Determination: The biological characteristics which distinguish human beings as female or male. [NIH] Sex Education: Education which increases the knowledge of the functional, structural, and behavioral aspects of human reproduction. [NIH] Sexually Transmitted Diseases: Diseases due to or propagated by sexual contact. [NIH] Sharpness: The apparent blurring of the border between two adjacent areas of a radiograph having different optical densities. [NIH] Shock: The general bodily disturbance following a severe injury; an emotional or moral upset occasioned by some disturbing or unexpected experience; disruption of the circulation, which can upset all body functions: sometimes referred to as circulatory shock. [NIH]

Side effect: A consequence other than the one(s) for which an agent or measure is used, as the adverse effects produced by a drug, especially on a tissue or organ system other than the

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one sought to be benefited by its administration. [EU] Sign Language: A system of hand gestures used for communication by the deaf or by people speaking different languages. [NIH] Signal Transduction: The intercellular or intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GABA-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptormediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway. [NIH] Signs and Symptoms: Clinical manifestations that can be either objective when observed by a physician, or subjective when perceived by the patient. [NIH] Silicon: A trace element that constitutes about 27.6% of the earth's crust in the form of silicon dioxide. It does not occur free in nature. Silicon has the atomic symbol Si, atomic number 14, and atomic weight 28.09. [NIH] Silicon Dioxide: Silica. Transparent, tasteless crystals found in nature as agate, amethyst, chalcedony, cristobalite, flint, sand, quartz, and tridymite. The compound is insoluble in water or acids except hydrofluoric acid. [NIH] Single Person: The unmarried man or woman. [NIH] Sinoatrial Node: The small mass of modified cardiac muscle fibers located at the junction of the superior vena cava and right atrium. Contraction impulses probably start in this node, spread over the atrium and are then transmitted by the atrioventricular bundle to the ventricle. [NIH] Skeletal: Having to do with the skeleton (boney part of the body). [NIH] Skeleton: The framework that supports the soft tissues of vertebrate animals and protects many of their internal organs. The skeletons of vertebrates are made of bone and/or cartilage. [NIH] Skull: The skeleton of the head including the bones of the face and the bones enclosing the brain. [NIH] Sleep apnea: A serious, potentially life-threatening breathing disorder characterized by repeated cessation of breathing due to either collapse of the upper airway during sleep or absence of respiratory effort. [NIH] Small intestine: The part of the digestive tract that is located between the stomach and the large intestine. [NIH] Social Environment: The aggregate of social and cultural institutions, forms, patterns, and processes that influence the life of an individual or community. [NIH] Social Sciences: Disciplines concerned with the interrelationships of individuals in a social environment including social organizations and institutions. Includes Sociology and Anthropology. [NIH] Social Work: The use of community resources, individual case work, or group work to promote the adaptive capacities of individuals in relation to their social and economic

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environments. It includes social service agencies. [NIH] Socialization: The training or molding of an individual through various relationships, educational agencies, and social controls, which enables him to become a member of a particular society. [NIH] Sodium: An element that is a member of the alkali group of metals. It has the atomic symbol Na, atomic number 11, and atomic weight 23. With a valence of 1, it has a strong affinity for oxygen and other nonmetallic elements. Sodium provides the chief cation of the extracellular body fluids. Its salts are the most widely used in medicine. (From Dorland, 27th ed) Physiologically the sodium ion plays a major role in blood pressure regulation, maintenance of fluid volume, and electrolyte balance. [NIH] Software Design: Specifications and instructions applied to the software. [NIH] Solvent: 1. Dissolving; effecting a solution. 2. A liquid that dissolves or that is capable of dissolving; the component of a solution that is present in greater amount. [EU] Somatic: 1. Pertaining to or characteristic of the soma or body. 2. Pertaining to the body wall in contrast to the viscera. [EU] Songbirds: Passeriformes of the suborder, Oscines, in which the flexor tendons of the toes are separate, and the lower syrinx has 4 to 9 pairs of tensor muscles inserted at both ends of the tracheal half rings. They include many commonly recognized birds such as crows, finches, robins, sparrows, and swallows. [NIH] Sound wave: An alteration of properties of an elastic medium, such as pressure, particle displacement, or density, that propagates through the medium, or a superposition of such alterations. [NIH] Spasm: An involuntary contraction of a muscle or group of muscles. Spasms may involve skeletal muscle or smooth muscle. [NIH] Spatial disorientation: Loss of orientation in space where person does not know which way is up. [NIH] Specialist: In medicine, one who concentrates on 1 special branch of medical science. [NIH] Species: A taxonomic category subordinate to a genus (or subgenus) and superior to a subspecies or variety, composed of individuals possessing common characters distinguishing them from other categories of individuals of the same taxonomic level. In taxonomic nomenclature, species are designated by the genus name followed by a Latin or Latinized adjective or noun. [EU] Specificity: Degree of selectivity shown by an antibody with respect to the number and types of antigens with which the antibody combines, as well as with respect to the rates and the extents of these reactions. [NIH] Spectrum: A charted band of wavelengths of electromagnetic vibrations obtained by refraction and diffraction. By extension, a measurable range of activity, such as the range of bacteria affected by an antibiotic (antibacterial s.) or the complete range of manifestations of a disease. [EU] Speech Intelligibility: Ability to make speech sounds that are recognizable. [NIH] Speech Perception: The process whereby an utterance is decoded into a representation in terms of linguistic units (sequences of phonetic segments which combine to form lexical and grammatical morphemes). [NIH] Speech-Language Pathology: The study of speech or language disorders and their diagnosis and correction. [NIH] Sperm: The fecundating fluid of the male. [NIH]

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Sphincter: A ringlike band of muscle fibres that constricts a passage or closes a natural orifice; called also musculus sphincter. [EU] Spinal cord: The main trunk or bundle of nerves running down the spine through holes in the spinal bone (the vertebrae) from the brain to the level of the lower back. [NIH] Spiral Ganglion: The sensory ganglion of the cochlear nerve. The cells of the spiral ganglion send fibers peripherally to the cochlear hair cells and centrally to the cochlear nuclei of the brain stem. [NIH] Spiral Lamina: The bony plate which extends outwards from the modiolus. It is part of the structure which divides trhe cochlea into sections. [NIH] Spleen: An organ that is part of the lymphatic system. The spleen produces lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach. [NIH] Sporadic: Neither endemic nor epidemic; occurring occasionally in a random or isolated manner. [EU] Stabilization: The creation of a stable state. [EU] Staff Development: The process by which the employer promotes staff performance and efficiency consistent with management goals and objectives. [NIH] Staging: Performing exams and tests to learn the extent of the cancer within the body, especially whether the disease has spread from the original site to other parts of the body. [NIH]

Stapes: One of the three ossicles of the middle ear. It transmits sound vibrations from the incus to the internal ear. [NIH] Statistically significant: Describes a mathematical measure of difference between groups. The difference is said to be statistically significant if it is greater than what might be expected to happen by chance alone. [NIH] Steel: A tough, malleable, iron-based alloy containing up to, but no more than, two percent carbon and often other metals. It is used in medicine and dentistry in implants and instrumentation. [NIH] Stem Cells: Relatively undifferentiated cells of the same lineage (family type) that retain the ability to divide and cycle throughout postnatal life to provide cells that can become specialized and take the place of those that die or are lost. [NIH] Sterility: 1. The inability to produce offspring, i.e., the inability to conceive (female s.) or to induce conception (male s.). 2. The state of being aseptic, or free from microorganisms. [EU] Steroid: A group name for lipids that contain a hydrogenated cyclopentanoperhydrophenanthrene ring system. Some of the substances included in this group are progesterone, adrenocortical hormones, the gonadal hormones, cardiac aglycones, bile acids, sterols (such as cholesterol), toad poisons, saponins, and some of the carcinogenic hydrocarbons. [EU] Stimulus: That which can elicit or evoke action (response) in a muscle, nerve, gland or other excitable issue, or cause an augmenting action upon any function or metabolic process. [NIH] Stomach: An organ of digestion situated in the left upper quadrant of the abdomen between the termination of the esophagus and the beginning of the duodenum. [NIH] Strabismus: Deviation of the eye which the patient cannot overcome. The visual axes assume a position relative to each other different from that required by the physiological conditions. The various forms of strabismus are spoken of as tropias, their direction being indicated by the appropriate prefix, as cyclo tropia, esotropia, exotropia, hypertropia, and

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hypotropia. Called also cast, heterotropia, manifest deviation, and squint. [EU] Stress: Forcibly exerted influence; pressure. Any condition or situation that causes strain or tension. Stress may be either physical or psychologic, or both. [NIH] Stria: 1. A streak, or line. 2. A narrow bandlike structure; a general term for such longitudinal collections of nerve fibres in the brain. [EU] Stria Vascularis: A layer of highly vascular pigmented granular cells on the outer wall of the cochlear duct. [NIH] Striatum: A higher brain's domain thus called because of its stripes. [NIH] Stroke: Sudden loss of function of part of the brain because of loss of blood flow. Stroke may be caused by a clot (thrombosis) or rupture (hemorrhage) of a blood vessel to the brain. [NIH] Subacute: Somewhat acute; between acute and chronic. [EU] Subarachnoid: Situated or occurring between the arachnoid and the pia mater. [EU] Subclinical: Without clinical manifestations; said of the early stage(s) of an infection or other disease or abnormality before symptoms and signs become apparent or detectable by clinical examination or laboratory tests, or of a very mild form of an infection or other disease or abnormality. [EU] Subculture: A culture derived from another culture or the aseptic division and transfer of a culture or a portion of that culture (inoculum) to fresh nutrient medium. [NIH] Subcutaneous: Beneath the skin. [NIH] Submandibular: Four to six lymph glands, located between the lower jaw and the submandibular salivary gland. [NIH] Submaxillary: Four to six lymph glands, located between the lower jaw and the submandibular salivary gland. [NIH] Subspecies: A category intermediate in rank between species and variety, based on a smaller number of correlated characters than are used to differentiate species and generally conditioned by geographical and/or ecological occurrence. [NIH] Substance P: An eleven-amino acid neurotransmitter that appears in both the central and peripheral nervous systems. It is involved in transmission of pain, causes rapid contractions of the gastrointestinal smooth muscle, and modulates inflammatory and immune responses. [NIH]

Substrate: A substance upon which an enzyme acts. [EU] Substrate Specificity: A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts. [NIH] Sudden death: Cardiac arrest caused by an irregular heartbeat. The term "death" is somewhat misleading, because some patients survive. [NIH] Superior vena cava: Vein which returns blood from the head and neck, upper limbs, and thorax. It is formed by the union of the two brachiocephalic veins. [NIH] Support group: A group of people with similar disease who meet to discuss how better to cope with their cancer and treatment. [NIH] Suppurative: Consisting of, containing, associated with, or identified by the formation of pus. [NIH] Symphysis: A secondary cartilaginous joint. [NIH] Symptomatic: Having to do with symptoms, which are signs of a condition or disease. [NIH] Synapses: Specialized junctions at which a neuron communicates with a target cell. At

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classical synapses, a neuron's presynaptic terminal releases a chemical transmitter stored in synaptic vesicles which diffuses across a narrow synaptic cleft and activates receptors on the postsynaptic membrane of the target cell. The target may be a dendrite, cell body, or axon of another neuron, or a specialized region of a muscle or secretory cell. Neurons may also communicate through direct electrical connections which are sometimes called electrical synapses; these are not included here but rather in gap junctions. [NIH] Synapsis: The pairing between homologous chromosomes of maternal and paternal origin during the prophase of meiosis, leading to the formation of gametes. [NIH] Synaptic: Pertaining to or affecting a synapse (= site of functional apposition between neurons, at which an impulse is transmitted from one neuron to another by electrical or chemical means); pertaining to synapsis (= pairing off in point-for-point association of homologous chromosomes from the male and female pronuclei during the early prophase of meiosis). [EU] Synaptic Transmission: The communication from a neuron to a target (neuron, muscle, or secretory cell) across a synapse. In chemical synaptic transmission, the presynaptic neuron releases a neurotransmitter that diffuses across the synaptic cleft and binds to specific synaptic receptors. These activated receptors modulate ion channels and/or secondmessenger systems to influence the postsynaptic cell. Electrical transmission is less common in the nervous system, and, as in other tissues, is mediated by gap junctions. [NIH] Synaptic Vesicles: Membrane-bound compartments which contain transmitter molecules. Synaptic vesicles are concentrated at presynaptic terminals. They actively sequester transmitter molecules from the cytoplasm. In at least some synapses, transmitter release occurs by fusion of these vesicles with the presynaptic membrane, followed by exocytosis of their contents. [NIH] Syncope: A temporary suspension of consciousness due to generalized cerebral schemia, a faint or swoon. [EU] Syrinx: A fistula. [NIH] Systemic: Affecting the entire body. [NIH] Systemic disease: Disease that affects the whole body. [NIH] Systolic: Indicating the maximum arterial pressure during contraction of the left ventricle of the heart. [EU] Tape Recording: Recording of information on magnetic or punched paper tape. [NIH] Tectorial Membrane: A gelatinous membrane, attached to the bony spiral lamina, which overlies the hair cells within the cochlea of the inner ear. [NIH] Telangiectasia: The permanent enlargement of blood vessels, causing redness in the skin or mucous membranes. [NIH] Telecommunications: Transmission of information over distances via electronic means. [NIH]

Telencephalon: Paired anteriolateral evaginations of the prosencephalon plus the lamina terminalis. The cerebral hemispheres are derived from it. Many authors consider cerebrum a synonymous term to telencephalon, though a minority include diencephalon as part of the cerebrum (Anthoney, 1994). [NIH] Telomerase: Essential ribonucleoprotein reverse transcriptase that adds telomeric DNA to the ends of eukaryotic chromosomes. Telomerase appears to be repressed in normal human somatic tissues but reactivated in cancer, and thus may be necessary for malignant transformation. EC 2.7.7.-. [NIH] Temporal: One of the two irregular bones forming part of the lateral surfaces and base of the

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skull, and containing the organs of hearing. [NIH] Temporal Lobe: Lower lateral part of the cerebral hemisphere. [NIH] Terminalis: A groove on the lateral surface of the right atrium. [NIH] Terminator: A DNA sequence sited at the end of a transcriptional unit that signals the end of transcription. [NIH] Testosterone: A hormone that promotes the development and maintenance of male sex characteristics. [NIH] Thalamic: Cell that reaches the lateral nucleus of amygdala. [NIH] Thalamic Diseases: Disorders of the centrally located thalamus, which integrates a wide range of cortical and subcortical information. Manifestations include sensory loss, movement disorders; ataxia, pain syndromes, visual disorders, a variety of neuropsychological conditions, and coma. Relatively common etiologies include cerebrovascular disorders; craniocerebral trauma; brain neoplasms; brain hypoxia; intracranial hemorrhages; and infectious processes. [NIH] Thalamus: Paired bodies containing mostly gray substance and forming part of the lateral wall of the third ventricle of the brain. The thalamus represents the major portion of the diencephalon and is commonly divided into cellular aggregates known as nuclear groups. [NIH]

Therapeutics: The branch of medicine which is concerned with the treatment of diseases, palliative or curative. [NIH] Thiamine: 3-((4-Amino-2-methyl-5-pyrimidinyl)methyl)-5-(2methylthiazolium chloride. [NIH]

hydroxyethyl)-4-

Thoracic: Having to do with the chest. [NIH] Threonine: An essential amino acid occurring naturally in the L-form, which is the active form. It is found in eggs, milk, gelatin, and other proteins. [NIH] Threshold: For a specified sensory modality (e. g. light, sound, vibration), the lowest level (absolute threshold) or smallest difference (difference threshold, difference limen) or intensity of the stimulus discernible in prescribed conditions of stimulation. [NIH] Thrombin: An enzyme formed from prothrombin that converts fibrinogen to fibrin. (Dorland, 27th ed) EC 3.4.21.5. [NIH] Thrombomodulin: A cell surface glycoprotein of endothelial cells that binds thrombin and serves as a cofactor in the activation of protein C and its regulation of blood coagulation. [NIH]

Thrombosis: The formation or presence of a blood clot inside a blood vessel. [NIH] Thrombus: An aggregation of blood factors, primarily platelets and fibrin with entrapment of cellular elements, frequently causing vascular obstruction at the point of its formation. Some authorities thus differentiate thrombus formation from simple coagulation or clot formation. [EU] Thyroid: A gland located near the windpipe (trachea) that produces thyroid hormone, which helps regulate growth and metabolism. [NIH] Thyroid Gland: A highly vascular endocrine gland consisting of two lobes, one on either side of the trachea, joined by a narrow isthmus; it produces the thyroid hormones which are concerned in regulating the metabolic rate of the body. [NIH] Thyroid Hormones: Hormones secreted by the thyroid gland. [NIH] Thyrotropin: A peptide hormone secreted by the anterior pituitary. It promotes the growth of the thyroid gland and stimulates the synthesis of thyroid hormones and the release of

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thyroxine by the thyroid gland. [NIH] Tin: A trace element that is required in bone formation. It has the atomic symbol Sn, atomic number 50, and atomic weight 118.71. [NIH] Tinnitus: Sounds that are perceived in the absence of any external noise source which may take the form of buzzing, ringing, clicking, pulsations, and other noises. Objective tinnitus refers to noises generated from within the ear or adjacent structures that can be heard by other individuals. The term subjective tinnitus is used when the sound is audible only to the affected individual. Tinnitus may occur as a manifestation of cochlear diseases; vestibulocochlear nerve diseases; intracranial hypertension; craniocerebral trauma; and other conditions. [NIH] Tissue: A group or layer of cells that are alike in type and work together to perform a specific function. [NIH] Tissue Culture: Maintaining or growing of tissue, organ primordia, or the whole or part of an organ in vitro so as to preserve its architecture and/or function (Dorland, 28th ed). Tissue culture includes both organ culture and cell culture. [NIH] Tissue Distribution: Accumulation of a drug or chemical substance in various organs (including those not relevant to its pharmacologic or therapeutic action). This distribution depends on the blood flow or perfusion rate of the organ, the ability of the drug to penetrate organ membranes, tissue specificity, protein binding. The distribution is usually expressed as tissue to plasma ratios. [NIH] Tomography: Imaging methods that result in sharp images of objects located on a chosen plane and blurred images located above or below the plane. [NIH] Tonicity: The normal state of muscular tension. [NIH] Tooth Preparation: Procedures carried out with regard to the teeth or tooth structures preparatory to specified dental therapeutic and surgical measures. [NIH] Torsion: A twisting or rotation of a bodily part or member on its axis. [NIH] Toxic: Having to do with poison or something harmful to the body. Toxic substances usually cause unwanted side effects. [NIH] Toxicity: The quality of being poisonous, especially the degree of virulence of a toxic microbe or of a poison. [EU] Toxicology: The science concerned with the detection, chemical composition, and pharmacologic action of toxic substances or poisons and the treatment and prevention of toxic manifestations. [NIH] Toxins: Specific, characterizable, poisonous chemicals, often proteins, with specific biological properties, including immunogenicity, produced by microbes, higher plants, or animals. [NIH] Trace element: Substance or element essential to plant or animal life, but present in extremely small amounts. [NIH] Trachea: The cartilaginous and membranous tube descending from the larynx and branching into the right and left main bronchi. [NIH] Traction: The act of pulling. [NIH] Transcriptase: An enzyme which catalyses the synthesis of a complementary mRNA molecule from a DNA template in the presence of a mixture of the four ribonucleotides (ATP, UTP, GTP and CTP). [NIH] Transcription Factors: Endogenous substances, usually proteins, which are effective in the initiation, stimulation, or termination of the genetic transcription process. [NIH]

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Transduction: The transfer of genes from one cell to another by means of a viral (in the case of bacteria, a bacteriophage) vector or a vector which is similar to a virus particle (pseudovirion). [NIH] Transfection: The uptake of naked or purified DNA into cells, usually eukaryotic. It is analogous to bacterial transformation. [NIH] Transgenes: Genes that are introduced into an organism using gene transfer techniques. [NIH]

Translating: Conversion from one language to another language. [NIH] Translation: The process whereby the genetic information present in the linear sequence of ribonucleotides in mRNA is converted into a corresponding sequence of amino acids in a protein. It occurs on the ribosome and is unidirectional. [NIH] Translational: The cleavage of signal sequence that directs the passage of the protein through a cell or organelle membrane. [NIH] Translocate: The attachment of a fragment of one chromosome to a non-homologous chromosome. [NIH] Translocation: The movement of material in solution inside the body of the plant. [NIH] Transmitter: A chemical substance which effects the passage of nerve impulses from one cell to the other at the synapse. [NIH] Transplantation: Transference of a tissue or organ, alive or dead, within an individual, between individuals of the same species, or between individuals of different species. [NIH] Transposons: Discrete genetic elements capable of inserting, in a non-permuted fashion, into the chromosomes of many bacteria. [NIH] Trauma: Any injury, wound, or shock, must frequently physical or structural shock, producing a disturbance. [NIH] Triad: Trivalent. [NIH] Trophic: Of or pertaining to nutrition. [EU] Tryptophan: An essential amino acid that is necessary for normal growth in infants and for nitrogen balance in adults. It is a precursor serotonin and niacin. [NIH] Tuberous Sclerosis: A rare congenital disease in which the essential pathology is the appearance of multiple tumors in the cerebrum and in other organs, such as the heart or kidneys. [NIH] Tumour: 1. Swelling, one of the cardinal signs of inflammations; morbid enlargement. 2. A new growth of tissue in which the multiplication of cells is uncontrolled and progressive; called also neoplasm. [EU] Tympani: The part of the cochlea below the spiral lamina. [NIH] Tympanic membrane: A thin, tense membrane forming the greater part of the outer wall of the tympanic cavity and separating it from the external auditory meatus; it constitutes the boundary between the external and middle ear. [NIH] Type 2 diabetes: Usually characterized by a gradual onset with minimal or no symptoms of metabolic disturbance and no requirement for exogenous insulin. The peak age of onset is 50 to 60 years. Obesity and possibly a genetic factor are usually present. [NIH] Tyrosine: A non-essential amino acid. In animals it is synthesized from phenylalanine. It is also the precursor of epinephrine, thyroid hormones, and melanin. [NIH] Tyrothricin: A polypeptide antibiotic mixture obtained from Bacillus brevis. It consists of a mixture of three tyrocidines (60%) and several gramicidins (20%) and is very toxic to blood,

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liver, kidneys, meninges, and the olfactory apparatus. It is used topically. [NIH] Ultrasonography: The visualization of deep structures of the body by recording the reflections of echoes of pulses of ultrasonic waves directed into the tissues. Use of ultrasound for imaging or diagnostic purposes employs frequencies ranging from 1.6 to 10 megahertz. [NIH] Umbilical Arteries: Either of a pair of arteries originating from the internal iliac artery and passing through the umbilical cord to carry blood from the fetus to the placenta. [NIH] Umbilical Cord: The flexible structure, giving passage to the umbilical arteries and vein, which connects the embryo or fetus to the placenta. [NIH] Umbilical cord blood: Blood from the placenta (afterbirth) that contains high concentrations of stem cells needed to produce new blood cells. [NIH] Unconscious: Experience which was once conscious, but was subsequently rejected, as the "personal unconscious". [NIH] Uncoupling Agents: Chemical agents that uncouple oxidation from phosphorylation in the metabolic cycle so that ATP synthesis does not occur. Included here are those ionophores that disrupt electron transfer by short-circuiting the proton gradient across mitochondrial membranes. [NIH] Underachievement: Performance, usually in school work, poorer than that predicted from aptitude and/or intelligence testing. [NIH] Urea: A compound (CO(NH2)2), formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids. [NIH] Uremia: The illness associated with the buildup of urea in the blood because the kidneys are not working effectively. Symptoms include nausea, vomiting, loss of appetite, weakness, and mental confusion. [NIH] Urethra: The tube through which urine leaves the body. It empties urine from the bladder. [NIH]

Urinary: Having to do with urine or the organs of the body that produce and get rid of urine. [NIH] Urine: Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra. [NIH] Urogenital: Pertaining to the urinary and genital apparatus; genitourinary. [EU] Uterus: The small, hollow, pear-shaped organ in a woman's pelvis. This is the organ in which a fetus develops. Also called the womb. [NIH] Vaccine: A substance or group of substances meant to cause the immune system to respond to a tumor or to microorganisms, such as bacteria or viruses. [NIH] Vacuoles: Any spaces or cavities within a cell. They may function in digestion, storage, secretion, or excretion. [NIH] Vascular: Pertaining to blood vessels or indicative of a copious blood supply. [EU] Vasculitis: Inflammation of a blood vessel. [NIH] VE: The total volume of gas either inspired or expired in one minute. [NIH] Vector: Plasmid or other self-replicating DNA molecule that transfers DNA between cells in nature or in recombinant DNA technology. [NIH] Vein: Vessel-carrying blood from various parts of the body to the heart. [NIH] Venous: Of or pertaining to the veins. [EU]

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Venous blood: Blood that has given up its oxygen to the tissues and carries carbon dioxide back for gas exchange. [NIH] Ventilation: 1. In respiratory physiology, the process of exchange of air between the lungs and the ambient air. Pulmonary ventilation (usually measured in litres per minute) refers to the total exchange, whereas alveolar ventilation refers to the effective ventilation of the alveoli, in which gas exchange with the blood takes place. 2. In psychiatry, verbalization of one's emotional problems. [EU] Ventral: 1. Pertaining to the belly or to any venter. 2. Denoting a position more toward the belly surface than some other object of reference; same as anterior in human anatomy. [EU] Ventricle: One of the two pumping chambers of the heart. The right ventricle receives oxygen-poor blood from the right atrium and pumps it to the lungs through the pulmonary artery. The left ventricle receives oxygen-rich blood from the left atrium and pumps it to the body through the aorta. [NIH] Ventricular: Pertaining to a ventricle. [EU] Ventricular fibrillation: Rapid, irregular quivering of the heart's ventricles, with no effective heartbeat. [NIH] Vertebral: Of or pertaining to a vertebra. [EU] Vertebrobasilar Insufficiency: Localized or diffuse reduction in blood flow through the vertebrobasilar arterial system, which supplies the brain stem; cerebellum; occipital lobe; medial temporal lobe; and thalamus. Characteristic clinical features include syncope; lightheadedness; visual disturbances; and vertigo. brain stem infarctions or other brain infarction may be associated. [NIH] Vertigo: An illusion of movement; a sensation as if the external world were revolving around the patient (objective vertigo) or as if he himself were revolving in space (subjective vertigo). The term is sometimes erroneously used to mean any form of dizziness. [EU] Vesicular: 1. Composed of or relating to small, saclike bodies. 2. Pertaining to or made up of vesicles on the skin. [EU] Vestibular: Pertaining to or toward a vestibule. In dental anatomy, used to refer to the tooth surface directed toward the vestibule of the mouth. [EU] Vestibular Aqueduct: A small bony canal linking the vestibule of the inner ear to the posterior part of the internal surface of the petrous temporal bone. It surrounds the endolymphatic duct. [NIH] Vestibule: A small, oval, bony chamber of the labyrinth. The vestibule contains the utricle and saccule, organs which are part of the balancing apparatus of the ear. [NIH] Vestibulocochlear Nerve: The 8th cranial nerve. The vestibulocochlear nerve has a cochlear part (cochlear nerve) which is concerned with hearing and a vestibular part (vestibular nerve) which mediates the sense of balance and head position. The fibers of the cochlear nerve originate from neurons of the spiral ganglion and project to the cochlear nuclei (cochlear nucleus). The fibers of the vestibular nerve arise from neurons of Scarpa's ganglion and project to the vestibular nuclei. [NIH] Vestibulocochlear Nerve Diseases: Diseases of the vestibular and/or cochlear (acoustic) nerves, which join to form the vestibulocochlear nerve. Vestibular neuritis, cochlear neuritis, and acoustic neuromas are relatively common conditions that affect these nerves. Clinical manifestations vary with which nerve is primarily affected, and include hearing loss, vertigo, and tinnitus. [NIH] Veterinary Medicine: The medical science concerned with the prevention, diagnosis, and treatment of diseases in animals. [NIH]

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Viral: Pertaining to, caused by, or of the nature of virus. [EU] Virus: Submicroscopic organism that causes infectious disease. In cancer therapy, some viruses may be made into vaccines that help the body build an immune response to, and kill, tumor cells. [NIH] Viscosity: A physical property of fluids that determines the internal resistance to shear forces. [EU] Visual Acuity: Acuteness or clearness of vision, especially of form vision, which is dependent mainly on the sharpness of the retinal focus. [NIH] Visual Cortex: Area of the occipital lobe concerned with vision. [NIH] Visual field: The entire area that can be seen when the eye is forward, including peripheral vision. [NIH] Visual Perception: The selecting and organizing of visual stimuli based on the individual's past experience. [NIH] Vitamin A: A substance used in cancer prevention; it belongs to the family of drugs called retinoids. [NIH] Vitreous Body: The transparent, semigelatinous substance that fills the cavity behind the crystalline lens of the eye and in front of the retina. It is contained in a thin hyoid membrane and forms about four fifths of the optic globe. [NIH] Vitreous Hemorrhage: Hemorrhage into the vitreous body. [NIH] Vitro: Descriptive of an event or enzyme reaction under experimental investigation occurring outside a living organism. Parts of an organism or microorganism are used together with artificial substrates and/or conditions. [NIH] Vivo: Outside of or removed from the body of a living organism. [NIH] Vocational Guidance: Systematic efforts to assist individuals in selecting an occupation or suitable employment on the basis of aptitude, education, etc. [NIH] Voltage-gated: It is opened by the altered charge distribution across the cell membrane. [NIH]

War: Hostile conflict between organized groups of people. [NIH] White blood cell: A type of cell in the immune system that helps the body fight infection and disease. White blood cells include lymphocytes, granulocytes, macrophages, and others. [NIH]

Windpipe: A rigid tube, 10 cm long, extending from the cricoid cartilage to the upper border of the fifth thoracic vertebra. [NIH] Withdrawal: 1. A pathological retreat from interpersonal contact and social involvement, as may occur in schizophrenia, depression, or schizoid avoidant and schizotypal personality disorders. 2. (DSM III-R) A substance-specific organic brain syndrome that follows the cessation of use or reduction in intake of a psychoactive substance that had been regularly used to induce a state of intoxication. [EU] Wound Healing: Restoration of integrity to traumatized tissue. [NIH] Xenograft: The cells of one species transplanted to another species. [NIH] X-ray: High-energy radiation used in low doses to diagnose diseases and in high doses to treat cancer. [NIH] X-ray therapy: The use of high-energy radiation from x-rays to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy) or from materials called radioisotopes. Radioisotopes produce radiation and can be

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placed in or near the tumor or in the area near cancer cells. This type of radiation treatment is called internal radiation therapy, implant radiation, interstitial radiation, or brachytherapy. Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that circulates throughout the body. X-ray therapy is also called radiation therapy, radiotherapy, and irradiation. [NIH] Yeasts: A general term for single-celled rounded fungi that reproduce by budding. Brewers' and bakers' yeasts are Saccharomyces cerevisiae; therapeutic dried yeast is dried yeast. [NIH] Zygote: The fertilized ovum. [NIH] Zymogen: Inactive form of an enzyme which can then be converted to the active form, usually by excision of a polypeptide, e. g. trypsinogen is the zymogen of trypsin. [NIH]

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INDEX A Abdomen, 297, 305, 334, 344, 359 Aberrant, 46, 219, 297 Ablation, 43, 46, 297 Abscess, 297, 321 Acceptor, 297, 334, 343 Accommodation, 21, 297, 339 Acetylcholine, 56, 70, 297, 309 Acidosis, 255, 297 Acoustic, 12, 37, 43, 76, 82, 101, 131, 148, 154, 158, 169, 179, 185, 260, 297, 302, 366 Actin, 63, 69, 297, 339 Action Potentials, 45, 297 Activities of Daily Living, 297, 326 Acuity, 151, 297, 302 Acyl, 297, 336 Adaptability, 297, 307, 308 Adaptation, 83, 297, 341, 347 Adjustment, 137, 138, 162, 182, 184, 199, 205, 206, 207, 243, 297 Adolescence, 131, 297, 309, 345 Adrenal Glands, 298, 299 Adverse Effect, 298, 299, 356 Aerobic, 298, 320, 337 Affinity, 78, 298, 302, 358 Agar, 298, 347 Agarose, 62, 298 Age of Onset, 65, 298, 364 Agonists, 45, 298 Air-Conduction, 168, 298 Airway, 298, 357 Albinism, 219, 247, 251, 298 Alexia, 298, 317 Algorithms, 298, 304 Alkaline, 297, 298, 299, 306 Alkalosis, 14, 298 Alleles, 8, 52, 64, 98, 298, 326, 327, 334 Alopecia, 298, 314 Alternative medicine, 231, 298 Amblyopia, 219, 299 Amino Acid Sequence, 299, 300, 323 Amino Acids, 80, 299, 310, 323, 345, 347, 350, 354, 356, 364, 365 Ammonia, 299, 365 Amniotic Fluid, 299, 324, 347 Amplification, 23, 27, 68, 92, 178, 182, 185, 190, 207, 213, 216, 217, 267, 269, 280, 299 Amyloidosis, 89, 299

Anaemia, 114, 115, 299, 336 Anaesthesia, 94, 299, 329 Anal, 40, 299, 322, 334 Analog, 160, 169, 299 Analogous, 159, 299, 364 Anaphylatoxins, 299, 311 Anatomical, 34, 37, 46, 48, 53, 67, 299, 302, 329, 355 Anemia, 256, 300, 304 Anesthesia, 178, 298, 300 Angina, 156, 300 Angina Pectoris, 156, 300 Animal model, 37, 49, 56, 69, 80, 300 Anions, 300, 332 Anomalies, 22, 26, 67, 81, 99, 195, 199, 207, 212, 213, 218, 219, 250, 300 Anterior Cerebral Artery, 300, 308 Antibacterial, 300, 358 Antibiotic, 79, 300, 325, 340, 358, 364 Antibodies, 48, 84, 172, 175, 300, 325, 328, 329, 335, 347 Antibody, 11, 96, 175, 272, 298, 300, 311, 325, 327, 329, 332, 338, 352, 358, 368 Anticoagulant, 300, 350 Anticonvulsant, 179, 300, 355 Antigen, 175, 298, 300, 311, 327, 328, 329, 330 Antigen-Antibody Complex, 300, 311 Anti-inflammatory, 41, 300, 324, 329, 348 Antineoplastic, 301, 314 Antioxidant, 112, 301, 344 Anuria, 301, 332 Anus, 299, 301, 302, 305, 331 Anxiolytic, 179, 301 Aphakia, 219, 301 Aphasia, 140, 301 Aplasia, 121, 301 Apnea, 301 Aponeurosis, 301, 323 Apoptosis, 32, 55, 77, 301 Aptitude, 301, 330, 365, 367 Aqueous, 49, 301, 303, 315, 318, 327, 334 Arachidonic Acid, 301, 349 Arrhythmia, 60, 156, 157, 254, 301 Arterial, 252, 301, 305, 308, 328, 331, 350, 361, 366 Arteries, 301, 304, 308, 313, 337, 339, 365 Arteriovenous, 107, 301, 308

370 Deafness

Aseptic, 301, 359, 360 Astigmatism, 208, 301, 353 Astrocytes, 79, 301 Asymptomatic, 7, 302 Ataxia, 60, 92, 112, 247, 253, 254, 255, 256, 302, 362 Atmospheric Pressure, 302, 327 Atresia, 99, 247, 254, 302 Atrioventricular, 302, 357 Atrium, 302, 357, 362, 366 Atrophy, 173, 252, 253, 254, 256, 302, 341 Atypical, 90, 112, 211, 251, 302 Audiologist, 23, 203, 273, 302 Audiology, 4, 5, 17, 18, 102, 112, 121, 123, 208, 215, 216, 233, 260, 261, 302 Audiometry, 12, 302 Audiometry, Speech, 12, 302 Audition, 106, 194, 302 Auditory Cortex, 29, 39, 105, 123, 125, 302, 310 Auditory nerve, 29, 48, 70, 83, 114, 124, 149, 150, 158, 302, 336 Auditory Perception, 35, 302 Aural, 11, 131, 198, 217, 261, 302 Autacoids, 302, 329 Autonomic, 297, 302, 345 Autoradiography, 31, 41, 303 Autosuggestion, 303, 328 Avian, 31, 32, 303 Axons, 46, 303, 340, 343, 349 B Bacteria, 41, 297, 300, 303, 318, 325, 337, 358, 364, 365 Bacterial Physiology, 297, 303 Bactericidal, 41, 303 Bacteriophage, 303, 347, 364 Bacteriostatic, 41, 303 Bacterium, 303, 311 Basal Ganglia, 302, 303, 305, 322, 338, 351 Basal Ganglia Diseases, 302, 303, 338 Base, 36, 45, 47, 53, 57, 83, 150, 155, 162, 163, 298, 303, 315, 323, 332, 333, 343, 347, 361 Base Sequence, 303, 323 Basement Membrane, 65, 69, 303, 321, 333 Basilar Membrane, 45, 82, 149, 150, 303 Behavior Therapy, 303 Benign, 303, 322, 340, 352, 355 Bewilderment, 303, 312 Bilateral, 4, 7, 17, 73, 95, 102, 107, 108, 112, 124, 303, 349, 354 Bile, 303, 322, 334, 359

Biochemical, 54, 55, 78, 79, 97, 105, 175, 298, 304, 323, 332, 333 Biogenesis, 72, 304 Biological response modifier, 304, 331 Biological Transport, 304, 316 Biopsy, 304, 345 Biosynthesis, 301, 304, 356 Biotechnology, 84, 86, 204, 231, 241, 246, 255, 256, 257, 304 Bladder, 304, 322, 350, 365 Blast phase, 304, 309 Blepharoptosis, 219, 304 Blood Coagulation, 304, 306, 362 Blood pressure, 27, 304, 306, 328, 338, 341, 358 Blood vessel, 304, 305, 306, 308, 324, 331, 332, 334, 335, 345, 360, 361, 362, 365 Blood Viscosity, 156, 304 Blood Volume, 304, 306 Blot, 32, 304 Body Fluids, 298, 304, 317, 358 Body Mass Index, 6, 304 Bone Conduction, 168, 302, 305 Bone Marrow, 304, 305, 309, 335 Bone scan, 305, 355 Bone-conduction, 189, 305 Bowel, 251, 299, 305, 316 Bowel Movement, 305, 316 Brachial, 305, 352 Brachial Plexus, 305, 352 Brachytherapy, 305, 331, 332, 352, 368 Brain Infarction, 305, 366 Brain Ischemia, 305, 308 Brain Stem, 30, 95, 305, 308, 310, 359, 366 Brain Stem Infarctions, 305, 366 Branch, 101, 157, 293, 305, 314, 318, 323, 335, 336, 342, 344, 351, 358, 362 Breakdown, 5, 305, 323, 342 Bronchi, 305, 319, 363 Bronchial, 305 Bronchitis, 156, 305 Bronchopulmonary, 54, 305 Bronchopulmonary Dysplasia, 54, 305 Bullous, 93, 306 C Cadherins, 64, 306 Calcium, 45, 66, 70, 79, 306, 311, 343, 357 Calmodulin, 77, 306 Carbohydrate, 71, 306, 348 Carbon Dioxide, 306, 322, 347, 353, 366 Carcinogenic, 306, 330, 342, 349, 359 Carcinogens, 306, 339, 342

Index 371

Cardiac, 60, 156, 157, 306, 318, 319, 320, 339, 357, 359, 360 Cardiomyopathy, 7, 87, 247, 306 Cardiovascular, 55, 164, 171, 306, 320 Cardiovascular Agents, 164, 306 Cardiovascular disease, 55, 306 Career Choice, 273, 306 Carotene, 306, 354 Carrier State, 12, 306 Case report, 4, 22, 307, 310 Case series, 307, 310 Cataract, 247, 252, 301, 307 Cations, 307, 332 Caudal, 307, 316, 348 Caudate Nucleus, 300, 303, 307, 313, 340 Cell Adhesion, 46, 69, 306, 307, 330 Cell Adhesion Molecules, 46, 307 Cell Count, 72, 307 Cell Cycle, 307, 309 Cell Death, 32, 55, 173, 301, 307, 340 Cell Differentiation, 52, 307, 357 Cell Division, 74, 256, 303, 307, 308, 314, 325, 336, 338, 347, 349, 356 Cell Lineage, 62, 307 Cell membrane, 60, 72, 304, 307, 309, 315, 323, 332, 346, 348, 367 Cell Movement, 36, 307 Cell Physiology, 70, 307 Cell Polarity, 74, 308 Cell proliferation, 32, 308, 331, 357 Cell Respiration, 308, 337, 353 Cell Survival, 32, 83, 308, 325 Cellulose, 308, 347 Central Nervous System, 150, 297, 308, 320, 322, 324, 343, 348 Cerebellar, 107, 247, 254, 302, 308, 310, 353 Cerebellum, 305, 308, 314, 348, 353, 366 Cerebral hemispheres, 303, 305, 308, 361 Cerebral Infarction, 156, 305, 308 Cerebral Palsy, 20, 54, 202, 308 Cerebrovascular, 179, 303, 306, 308, 362 Cerebrovascular Disorders, 179, 308, 362 Cerebrum, 308, 314, 361, 364 Cervical, 254, 305, 308, 352, 355 Character, 155, 156, 169, 170, 187, 300, 308, 315 Chemotactic Factors, 309, 311 Child Development, 95, 186, 309 Chloride Channels, 90, 309 Cholesterol, 13, 304, 309, 313, 336, 359 Cholinergic, 70, 309 Chorioretinitis, 309, 354

Choroid, 255, 309, 354 Chromatin, 301, 309 Chromosomal, 52, 53, 299, 309, 323, 347, 355 Chromosome, 14, 25, 33, 51, 53, 58, 61, 86, 87, 88, 90, 104, 157, 227, 309, 325, 334, 355, 356, 364 Chronic Disease, 215, 309 Chronic lymphocytic leukemia, 309 Chronic myelogenous leukemia, 304, 309 Chronic phase, 13, 309 Chronic renal, 14, 113, 309, 347 CIS, 112, 309, 354 Cisplatin, 176, 309 Civil Rights, 191, 244, 269, 280, 285, 309 Clamp, 43, 56, 310 Clinical Medicine, 310, 348 Clinical study, 50, 310 Clinical trial, 28, 30, 72, 143, 144, 241, 310, 313, 350, 352 Clone, 35, 44, 65, 310 Cloning, 36, 43, 67, 80, 304, 310 Cochlear Diseases, 310, 363 Cochlear Duct, 73, 310, 360 Cochlear Implantation, 8, 9, 14, 17, 18, 40, 46, 70, 102, 192, 217, 228, 310 Cochlear Nerve, 310, 359, 366 Cochlear Nucleus, 45, 102, 124, 310, 366 Codon, 65, 310, 323 Coenzyme, 115, 310 Cofactor, 311, 350, 362 Cognition, 57, 105, 138, 184, 197, 198, 212, 217, 233, 311, 333 Cognitive behavior therapy, 184, 311 Collagen, 65, 172, 174, 303, 311, 321, 323, 347, 349 Collapse, 305, 311, 357 Communication Barriers, 5, 223, 271, 311 Competency, 7, 311 Complement, 59, 78, 160, 190, 299, 311, 312, 323, 330 Complementary and alternative medicine, 119, 127, 311 Complementary medicine, 119, 312 Complementation, 69, 312 Computational Biology, 241, 246, 312 Computed tomography, 211, 312, 355 Computer Systems, 166, 312 Computerized axial tomography, 312, 355 Computerized tomography, 312 Concomitant, 219, 312 Conduction, 168, 247, 302, 312

372 Deafness

Cone, 255, 312, 346 Confusion, 4, 312, 316, 365 Conjugated, 312, 314 Connective Tissue, 305, 311, 312, 321, 322, 323, 335, 354 Connexins, 78, 79, 312, 323 Consciousness, 312, 315, 316, 361 Constitutional, 312, 354 Constriction, 312, 332, 355 Consultation, 271, 312 Consumer Organizations, 220, 313 Consumption, 313, 315, 344 Continuum, 209, 313 Contraindications, ii, 313 Contralateral, 12, 92, 101, 109, 125, 313, 353 Contrast Sensitivity, 125, 313 Control group, 12, 19, 76, 313, 352 Conventional therapy, 313 Conventional treatment, 121, 313 Convulsions, 300, 313 Cornea, 301, 313, 332 Corneum, 313, 319, 328 Coronary, 156, 300, 306, 313, 337, 339 Coronary Circulation, 300, 313 Coronary heart disease, 156, 306, 313 Coronary Thrombosis, 313, 337, 339 Corpus, 313, 340, 349 Corpus Striatum, 313, 340 Cortex, 29, 85, 112, 119, 122, 299, 302, 313, 320, 349, 353 Cortical, 29, 37, 39, 46, 47, 75, 93, 120, 123, 299, 313, 320, 356, 362 Cortices, 314, 326 Corticosteroids, 30, 314, 324, 348 Cortisone, 314, 348 Cranial, 179, 302, 308, 310, 314, 321, 331, 343, 345, 366 Craniocerebral Trauma, 303, 314, 362, 363 Criterion, 44, 314 Crossing-over, 314, 353 Cues, 20, 35, 74, 106, 218, 314 Curative, 314, 362 Cutaneous, 314, 352 Cyclic, 56, 77, 79, 306, 314, 325, 346, 350 Cyclophosphamide, 30, 314 Cyst, 90, 314 Cysteine, 97, 314 Cystine, 314 Cytochrome, 55, 314, 343 Cytogenetics, 314, 355 Cytokine, 54, 314, 345

Cytoplasm, 77, 78, 301, 307, 315, 319, 320, 325, 354, 361 Cytoskeleton, 63, 74, 315, 330 Cytotoxic, 315, 329, 352, 357 Cytotoxicity, 309, 315 D Data Collection, 39, 315, 322 Databases, Bibliographic, 241, 315 De novo, 49, 87, 93, 315 Deamination, 315, 365 Defense Mechanisms, 315, 330 Degenerative, 17, 67, 163, 247, 315, 354 Dehydration, 71, 315 Deletion, 93, 101, 163, 175, 301, 315 Dementia, 156, 315 Dendrites, 315, 341 Density, 305, 315, 342, 358 Dentists, 280, 315 Depolarization, 77, 114, 315, 357 Deprivation, 42, 75, 76, 125, 299, 315 Dermatitis, 251, 315, 317 Developed Countries, 163, 176, 315 Developing Countries, 24, 315 Diabetes Mellitus, 6, 109, 114, 115, 247, 255, 316, 324, 326 Diabetic Retinopathy, 7, 316 Diagnostic procedure, 147, 231, 316 Diagnostic Services, 65, 316 Diarrhea, 93, 255, 316 Diastolic, 316, 328 Diathermy, 316, 337 Diencephalon, 308, 316, 349, 361, 362 Diffusion, 49, 79, 304, 316, 332 Digestive system, 144, 316 Dihydrotestosterone, 316, 353 Dilatation, 316, 349 Diploid, 81, 312, 316, 347 Discrimination, 35, 49, 83, 192, 233, 302, 309, 316 Dislocation, 301, 316 Disorientation, 312, 316 Dissociation, 298, 316 Distal, 89, 254, 316, 318, 345, 349, 351 Dizziness, 206, 317, 366 Domesticated, 317, 325 Dominance, 206, 317, 333 Dorsal, 73, 75, 112, 310, 317, 348 Dorsum, 317, 323 Drug Interactions, 236, 317 Duct, 47, 253, 317, 318, 355 Dura mater, 317, 336, 344 Dyes, 50, 317

Index 373

Dynein, 74, 317 Dyslexia, 211, 317 Dysplasia, 248, 253, 255, 256, 317 Dystonia, 73, 85, 91, 95, 108, 317 Dystrophy, 7, 33, 248, 254, 255, 256, 317 E Ear Diseases, 172, 317 Eardrum, 162, 167, 168, 173, 262, 317 Ectopic, 62, 317 Eczema, 156, 317 Edema, 156, 316, 317, 331, 339, 340 Effector, 74, 297, 311, 317, 341, 346 Efficacy, 9, 12, 15, 30, 40, 72, 76, 82, 115, 205, 208, 317 Elastin, 311, 317 Elective, 318 Electrocardiogram, 171, 318 Electrode, 37, 318 Electrolyte, 318, 333, 348, 358 Electrons, 301, 303, 318, 332, 343, 352 Electrophysiological, 70, 80, 95, 318 Embolus, 318, 329, 331 Embryo, 36, 62, 307, 318, 329, 341, 348, 365 Embryology, 26, 318 Empirical, 71, 318 Emulsion, 303, 318, 322 Encephalitis, 156, 228, 318 Encephalitis, Viral, 318 Endemic, 84, 318, 359 Endocytosis, 63, 174, 318 Endolymph, 21, 62, 318 Endolymphatic Duct, 62, 318, 319, 366 Endolymphatic Sac, 318, 319 Endotoxins, 311, 319 End-stage renal, 65, 309, 319, 347 Enhancer, 44, 319 Environmental Exposure, 319, 342 Environmental Health, 240, 242, 319 Enzymatic, 306, 311, 319, 354 Epidemic, 156, 319, 359 Epidemiological, 96, 163, 176, 319 Epidermal, 97, 319, 336 Epidermis, 313, 319, 328 Epinephrine, 319, 364 Epithelial, 48, 59, 71, 109, 304, 319, 333 Epithelial Cells, 48, 71, 319, 333 Epithelium, 32, 36, 52, 59, 62, 64, 73, 303, 319 Equipment and Supplies, 275, 319 Erythrocytes, 299, 300, 305, 319 Esophagus, 302, 316, 319, 346, 359 Esotropia, 319, 359

Essential Tremor, 256, 320 Eukaryotic Cells, 74, 320, 329, 343 Eustachian tube, 173, 320 Evoke, 320, 359 Evoked Potentials, 101, 123, 320 Excitability, 60, 320 Excitation, 158, 320 Excitatory, 43, 124, 320, 324 Excrete, 301, 320, 332, 353 Exercise Test, 215, 320 Exogenous, 317, 320, 364 Exons, 103, 320 Exotropia, 320, 359 Extensor, 320, 352 External-beam radiation, 320, 332, 352, 367 Extracellular, 43, 46, 49, 53, 82, 302, 312, 318, 320, 321, 330, 343, 350, 358 Extracellular Matrix, 46, 82, 312, 320, 321, 330, 343 Extracellular Space, 53, 320, 321 Extraction, 301, 321 Extremity, 305, 321, 352 F Facial, 57, 131, 170, 247, 250, 253, 321, 336, 344 Facial Nerve, 321, 344 Family Planning, 241, 321 Family Relations, 195, 321 Family Therapy, 181, 321 Farsightedness, 208, 321, 327 Fasciculation, 321, 341 Fat, 301, 305, 306, 313, 318, 321, 334, 354 Fatigue, 7, 169, 170, 321 Fatty acids, 321, 349 Fetus, 321, 347, 348, 365 Fibrillation, 321 Fibroblasts, 74, 321 Fibrosis, 41, 256, 321, 355 Fistulas, 91, 321 Fixation, 53, 109, 250, 252, 321 Flatus, 322, 323 Flexor, 320, 322, 358 Fluorescence, 49, 59, 322 Focus Groups, 7, 243, 286, 322 Fold, 70, 322 Follow-Up Studies, 54, 322 Forearm, 304, 322 Fovea, 322 Friction, 163, 322 Frontal Lobe, 300, 308, 322

374 Deafness

Functional magnetic resonance imaging, 42, 46, 75, 121, 322 Fundus, 213, 322, 342 Fungi, 322, 337, 368 G Gallbladder, 316, 322 Gamma Rays, 322, 339, 352 Ganglia, 36, 73, 297, 303, 322, 340, 345 Ganglion, 62, 77, 83, 322, 343, 359, 366 Gap Junctions, 32, 56, 78, 80, 312, 323, 361 Gas, 90, 299, 306, 316, 322, 323, 327, 339, 341, 365, 366 Gastrin, 323, 327 Gastrointestinal, 71, 319, 323, 360 Gelatin, 323, 324, 362 Gels, 85, 105, 323 Gene Expression, 32, 35, 47, 55, 59, 73, 82, 86, 257, 323 General practitioner, 23, 323 Genetic Code, 161, 323, 342 Genetic Counseling, 8, 10, 21, 34, 51, 211, 280, 323 Genetic Engineering, 79, 304, 310, 323 Genetic Markers, 59, 323 Genetic Techniques, 52, 323 Genetic testing, 10, 50, 90, 98, 99, 323 Genital, 54, 323, 365 Genitourinary, 323, 365 Genotype, 65, 323, 346 Germ Cells, 323, 336, 342 Gestation, 54, 324, 347 Gestational, 54, 324 Gestational Age, 54, 324 Gestures, 57, 77, 151, 152, 212, 324, 357 Gland, 314, 324, 335, 344, 350, 356, 359, 360, 362, 363 Glomerular, 65, 324, 332, 353 Glomerulus, 324, 340 Glucocorticoid, 324, 348 Glucose, 256, 308, 316, 324, 326, 330, 355 Glucose Intolerance, 316, 324 Glutamate, 324, 337, 355 Glutamic Acid, 324, 349 Glutathione Peroxidase, 72, 324 Glycine, 31, 43, 324, 356 Glycoprotein, 71, 324, 333, 362 Goiter, 47, 48, 324 Gonadal, 253, 324, 359 Gonads, 324, 328 Governing Board, 324, 348 Grade, 268, 278, 324, 325 Grading, 223, 324

Graft, 325, 327 Grafting, 325, 329 Gramicidin, 43, 325 Gram-positive, 325 Granulocytes, 325, 357, 367 Growth factors, 325, 341 Guinea Pigs, 29, 39, 49, 84, 325 H Habitual, 308, 325 Hammer, 163, 173, 325 Handicap, 26, 163, 176, 325 Haploid, 325, 347 Haptens, 298, 325 Health Promotion, 24, 325 Health Resources, iv, 28, 85, 325 Health Services, 27, 98, 209, 325 Health Status, 13, 19, 27, 326 Hearing Disorders, 17, 34, 58, 175, 201, 203, 260, 326 Hearing Impaired Persons, 242, 243, 244, 273, 326 Heart attack, 306, 326 Heartbeat, 326, 360, 366 Heme, 314, 326 Hemodialysis, 326, 332, 333 Hemoglobin, 300, 319, 326 Hemoglobinuria, 256, 326 Hemorrhage, 314, 326, 360, 367 Hemostasis, 326, 330 Heredity, 323, 326 Heterodimers, 326, 330 Heterogeneity, 41, 65, 91, 275, 298, 326 Heterogenic, 326 Heterogenous, 41, 326 Heterotropia, 326, 360 Heterozygotes, 8, 317, 326 Histology, 37, 38, 53, 54, 89, 326 Homeostasis, 21, 43, 62, 327 Homogeneous, 5, 313, 327 Homologous, 35, 43, 45, 51, 73, 81, 298, 312, 314, 326, 327, 356, 361, 364 Homozygote, 63, 327 Hormonal, 82, 302, 327 Hormone, 67, 82, 114, 314, 319, 323, 327, 330, 331, 336, 349, 354, 357, 362 Host, 69, 172, 303, 307, 327 Housekeeping, 38, 327 Human Development, 39, 42, 240, 268, 327 Hybrid, 65, 66, 175, 310, 327 Hybridization, 31, 58, 327 Hydrogen, 297, 303, 306, 324, 327, 334, 338, 341, 342, 343

Index 375

Hydrogen Peroxide, 324, 327, 334 Hydrolysis, 309, 327, 332, 346, 347, 350 Hydrophilic, 79, 327 Hydroxylysine, 311, 327 Hydroxyproline, 311, 327 Hyperbaric, 121, 122, 228, 327 Hyperbaric oxygen, 121, 122, 228, 327 Hyperlipidemia, 13, 327 Hyperopia, 208, 321, 327, 353 Hypersensitivity, 328, 354 Hypertension, 60, 156, 306, 328, 331 Hypertrophy, 328 Hypnotic, 179, 328 Hypoglycemia, 60, 328 Hypogonadism, 113, 252, 255, 328 Hypoplasia, 51, 328 Hypothyroidism, 67, 328 Hypoxia, 55, 305, 308, 328, 362 I Iatrogenic, 189, 328 Ichthyosis, 93, 97, 100, 104, 253, 328 Id, 116, 126, 260, 282, 292, 294, 328 Idiopathic, 18, 113, 114, 164, 173, 208, 328 Illusion, 328, 366 Immune function, 328, 329 Immune response, 84, 300, 314, 325, 328, 360, 367 Immune system, 328, 329, 335, 365, 367 Immunity, 84, 328 Immunochemistry, 66, 328 Immunodeficiency, 132, 222, 243, 244, 256, 270, 328 Immunodeficiency syndrome, 244, 328 Immunoglobulin, 300, 320, 329, 338 Immunohistochemistry, 38, 329 Immunologic, 309, 324, 329, 345, 352 Immunology, 298, 329 Immunosuppressive, 8, 30, 314, 324, 329 Immunosuppressive Agents, 8, 329 Implant radiation, 329, 331, 332, 352, 368 Implantation, 12, 14, 29, 46, 49, 71, 76, 92, 162, 178, 216, 217, 228, 329 In situ, 31, 32, 34, 53, 64, 66, 68, 209, 329 In Situ Hybridization, 31, 32, 34, 53, 64, 66, 68, 329 In vitro, 37, 63, 68, 74, 78, 82, 85, 109, 163, 175, 304, 329, 363 In vivo, 43, 49, 63, 66, 68, 77, 82, 114, 329 Incus, 329, 359 Indicative, 53, 152, 197, 329, 344, 365 Indomethacin, 14, 329 Induction, 8, 40, 62, 84, 316, 329

Infancy, 9, 120, 283, 329 Infarction, 107, 308, 313, 329, 337, 339 Inflammation, 54, 300, 305, 309, 315, 318, 321, 330, 332, 333, 336, 340, 341, 343, 344, 354, 365 Infuse, 78, 330 Inhalation, 55, 330, 347 Initiation, 168, 330, 363 Inlay, 330, 354 Innervation, 55, 305, 321, 330, 351, 352 Inoculum, 330, 360 Inorganic, 174, 309, 330, 338 Inositol, 79, 330, 337 Inositol Phosphates, 79, 330 Insight, 21, 35, 44, 52, 53, 65, 67, 95, 185, 330 Insomnia, 89, 330 Insulin, 6, 60, 330, 364 Insulin-dependent diabetes mellitus, 330 Integrins, 69, 330 Intelligence Tests, 204, 330 Interferon, 164, 331 Interferon-alpha, 331 Interleukins, 329, 331 Internal Medicine, 6, 48, 115, 331 Internal radiation, 331, 332, 352, 368 Interpersonal Relations, 153, 184, 331 Interstitial, 305, 321, 331, 332, 340, 353, 368 Intestinal, 306, 331, 335 Intestines, 302, 323, 331 Intoxication, 331, 367 Intracellular, 43, 45, 66, 69, 78, 308, 323, 329, 330, 331, 336, 337, 348, 350, 357 Intracellular Membranes, 331, 336 Intracranial Embolism, 308, 331 Intracranial Embolism and Thrombosis, 308, 331 Intracranial Hypertension, 331, 363 Intrinsic, 28, 298, 303, 331 Invasive, 328, 331, 335 Involuntary, 303, 320, 321, 331, 339, 353, 358 Iodine, 331, 337 Ion Channels, 45, 60, 302, 331, 341, 361 Ion Transport, 48, 332 Ionophores, 332, 365 Ions, 49, 56, 79, 303, 306, 309, 316, 318, 327, 332, 338, 346, 348 Ipsilateral, 12, 103, 332, 353 Irradiation, 205, 332, 368 Ischemia, 302, 305, 332 Isomorphism, 23, 332

376 Deafness

J Job Satisfaction, 176, 332 K Kb, 90, 240, 332 Keratitis, 93, 97, 100, 104, 253, 332 Kidney Disease, 4, 7, 14, 89, 144, 240, 256, 332 Kidney Failure, 14, 319, 332, 333 Kidney Failure, Acute, 332 Kidney Failure, Chronic, 332, 333 Kinetic, 47, 333 Kink, 80, 333 L Labile, 311, 333 Labyrinth, 41, 51, 310, 318, 319, 330, 333, 343, 345, 356, 366 Labyrinthine, 41, 164, 333 Labyrinthitis, 40, 228, 333 Lacrimal, 253, 321, 333, 343 Lag, 277, 333 Laminin, 46, 303, 333 Language Development, 9, 24, 71, 76, 187, 194, 196, 201, 211, 214, 333 Language Disorders, 198, 333, 358 Large Intestine, 316, 331, 333, 353, 357 Larva, 44, 333, 337 Larynx, 99, 114, 115, 121, 148, 333, 363 Latent, 333, 348 Laterality, 131, 333 Lectin, 333, 336 Legal blindness, 213, 333 Lens, 213, 301, 307, 334, 367 Lesion, 7, 31, 122, 334 Lethal, 303, 334, 339 Lethargy, 328, 334 Leukemia, 256, 309, 334 Leukocytes, 305, 309, 325, 329, 331, 334, 345 Library Services, 292, 334 Life cycle, 322, 333, 334, 342, 351 Life Expectancy, 156, 157, 334 Ligament, 41, 334, 350 Ligands, 307, 330, 334 Linkage, 14, 34, 35, 40, 53, 58, 61, 65, 67, 90, 323, 334 Linkage Disequilibrium, 34, 58, 334 Lip, 95, 151, 159, 160, 283, 334 Lipid, 72, 330, 332, 334, 336, 344 Lipid Peroxidation, 72, 334, 344 Lipreading, 7, 148, 160, 278, 334 Liver, 299, 301, 303, 314, 316, 318, 322, 325, 334, 348, 355, 365

Liver scan, 334, 355 Lobe, 308, 334 Localization, 32, 43, 45, 52, 58, 61, 63, 66, 67, 69, 75, 77, 175, 232, 329, 334 Localized, 35, 62, 73, 150, 299, 305, 322, 329, 333, 334, 347, 366 Locomotion, 334, 347 Longitudinal study, 54, 334 Loop, 165, 335 Low vision, 213, 335 Lucida, 333, 335 Lumen, 40, 47, 335 Lymph, 308, 335, 355, 360 Lymph node, 308, 335, 355 Lymphatic, 330, 335, 355, 359 Lymphatic system, 335, 355, 359 Lymphocyte, 300, 335 Lymphoid, 300, 314, 335 Lymphoma, 256, 335 M Macrophage, 69, 335 Macula, 53, 322, 335 Magnetic Resonance Imaging, 335, 355 Malabsorption, 256, 335 Malformation, 4, 65, 107, 250, 254, 335 Malignant, 256, 301, 335, 340, 352, 361 Malnutrition, 302, 335, 339 Malondialdehyde, 72, 336 Manifest, 336, 360 Manual Communication, 132, 184, 206, 336 Meatus, 305, 317, 336, 343, 364 Medial, 43, 45, 167, 168, 310, 320, 336, 343, 366 Mediate, 42, 46, 60, 61, 70, 72, 73, 78, 83, 307, 310, 336 MEDLINE, 241, 246, 256, 336 Megaloblastic, 114, 336 Meiosis, 336, 361 Melanin, 336, 346, 364 Melanocytes, 336 Melanoma, 256, 336 Membrane Fluidity, 49, 336 Membrane Glycoproteins, 336 Membrane Proteins, 78, 79, 336 Memory, 12, 17, 315, 336 Meningeal, 7, 336 Meninges, 308, 314, 317, 325, 336, 365 Meningitis, 18, 41, 85, 112, 262, 264, 336 Mental Disorders, 145, 336, 351 Mental Health Services, iv, 28, 209, 245, 337

Index 377

Mental Processes, 316, 337, 351 Mental Retardation, 54, 71, 73, 89, 108, 210, 247, 248, 252, 253, 254, 255, 257, 337 Mentors, 15, 337 Metabotropic, 45, 337 Metamorphosis, 86, 337, 342 Metastasis, 307, 337 Metastatic, 92, 337 Methimazole, 68, 337 MI, 114, 176, 210, 211, 295, 337 Microbiological, 54, 337 Microbiology, 297, 302, 337 Microorganism, 311, 337, 367 Microphthalmos, 219, 337 Microscopy, 32, 37, 38, 41, 55, 64, 78, 303, 337 Microwaves, 160, 337, 352 Migration, 74, 337 Mineralization, 174, 337 Minority Groups, 20, 27, 209, 278, 337 Mitochondria, 55, 72, 78, 161, 337, 343 Mitosis, 301, 338 Mobility, 135, 219, 265, 338 Modeling, 24, 62, 80, 338 Modems, 160, 338 Modification, 79, 85, 87, 112, 213, 323, 338, 351 Modulator, 160, 169, 338 Molecular Structure, 46, 338 Monitor, 88, 120, 152, 277, 338, 342 Monoclonal, 332, 338, 352, 368 Monogenic, 163, 176, 338 Morphogenesis, 46, 73, 81, 338 Morphological, 33, 64, 68, 81, 318, 336, 338 Morphology, 51, 56, 69, 307, 338 Motility, 63, 69, 329, 338 Motion Perception, 57, 338 Motion Sickness, 338, 339 Mucinous, 323, 338 Mucins, 71, 338, 355 Mucus, 338 Muscle Fibers, 338, 339, 357 Muscle Hypertonia, 338, 341 Muscular Atrophy, 256, 338 Muscular Dystrophies, 46, 317, 339 Mustard Gas, 339 Mutagen, 44, 339 Mutagenesis, 63, 71, 79, 81, 82, 339 Myelin, 79, 172, 339 Myocardial Ischemia, 300, 339 Myocardium, 300, 337, 339 Myopathy, 7, 93, 254, 255, 339

Myopia, 208, 250, 253, 255, 339, 353 Myosin, 25, 63, 68, 69, 83, 339 Myotonic Dystrophy, 256, 339 N Natural selection, 304, 339 Nausea, 7, 339, 365 NCI, 1, 144, 239, 309, 339 Nearsightedness, 208, 339 Necrosis, 301, 305, 308, 329, 337, 339 Needs Assessment, 286, 340 Neomycin, 81, 340 Neonatal, 26, 54, 108, 124, 340 Neonatal Screening, 26, 340 Neoplasia, 256, 340 Neoplasm, 340, 364 Neoplastic, 335, 340 Neostriatum, 31, 307, 313, 340, 351 Nephritis, 65, 340 Nephropathy, 65, 90, 247, 254, 255, 332, 340 Nephrosis, 254, 340 Nephrotic, 87, 340 Nephrotic Syndrome, 87, 340 Nerve Endings, 157, 340 Nerve Fibers, 45, 57, 150, 305, 310, 340 Nerve Growth Factor, 340, 341 Networks, 59, 73, 133, 160, 181, 340 Neural, 29, 32, 33, 37, 39, 42, 49, 57, 64, 73, 75, 86, 87, 124, 251, 252, 340, 341, 349 Neurites, 62, 341 Neuroeffector Junction, 340, 341 Neuroma, 101, 260, 341 Neuromuscular, 33, 161, 297, 341 Neuromuscular Diseases, 33, 161, 341 Neuromuscular Junction, 297, 341 Neuronal, 30, 31, 45, 54, 55, 60, 73, 79, 341 Neurons, 29, 31, 43, 45, 49, 60, 70, 77, 149, 150, 310, 315, 320, 322, 340, 341, 361, 366 Neuropathy, 125, 251, 254, 261, 341, 345 Neurophysiology, 315, 341 Neuroretinitis, 341, 354 Neurotransmitters, 341, 349 Neurotrophins, 114, 341 Neutrons, 332, 341, 352 Night Blindness, 341, 354 Nitrogen, 78, 298, 314, 322, 332, 341, 364 Nonverbal Communication, 186, 336, 341, 351 Normotensive, 14, 341 Notochord, 73, 341 Nuclear, 61, 81, 93, 100, 105, 114, 120, 123, 161, 303, 318, 320, 321, 322, 340, 342, 362

378 Deafness

Nuclear Family, 321, 342 Nuclei, 300, 310, 318, 320, 323, 335, 338, 341, 342, 343, 355, 359, 366 Nucleic acid, 303, 323, 327, 329, 341, 342, 351 Nucleic Acid Hybridization, 327, 342 Nursing Care, 342, 344 Nymph, 337, 342 Nystagmus, 219, 333, 342 O Occipital Lobe, 342, 366, 367 Ocular, 65, 195, 199, 207, 212, 213, 218, 219, 247, 319, 320, 342 Oliguria, 332, 342 Omnidirectional, 154, 342 Oncogene, 256, 342 Oncogenic, 330, 342 On-line, 57, 295, 342 Oocytes, 47, 70, 342 Opacity, 307, 315, 342 Ophthalmic, 87, 342 Ophthalmologic, 6, 342 Ophthalmology, 322, 342 Opsin, 342, 354 Optic Disk, 316, 342 Optic Nerve, 299, 341, 342, 343, 344, 354 Orbicularis, 96, 343 Organelles, 72, 315, 336, 343, 347 Osmosis, 343 Osmotic, 66, 343 Ossicle, 167, 343 Ossification, 41, 343 Osteoblasts, 41, 343 Osteolysis, 99, 343 Otitis, 24, 343 Otitis Media, 24, 343 Otosclerosis, 172, 262, 264, 343 Ototoxic, 22, 70, 161, 216, 343 Outer ear, 173, 343 Oval Window, 150, 343 Ovum, 324, 334, 343, 349, 368 Oxidation, 297, 301, 314, 324, 334, 337, 343, 344, 365 Oxidative Phosphorylation, 161, 343 Oxidative Stress, 72, 344 Oxygen Consumption, 320, 344, 353 Oxygenation, 42, 344 P Pachymeningitis, 336, 344 Palliative, 344, 362 Palsy, 54, 247, 250, 344 Pancreas, 316, 330, 344

Pancreatic, 256, 344 Pancreatic cancer, 256, 344 Paralysis, 304, 319, 344, 351 Parietal, 42, 300, 344 Parietal Lobe, 300, 344 Parotid, 7, 344 Paroxysmal, 256, 300, 344 Particle, 78, 81, 344, 358, 364 Partnership Practice, 344, 349 Patch, 43, 344 Pathogenesis, 26, 33, 40, 51, 61, 66, 68, 100, 105, 114, 344 Pathologic, 7, 40, 195, 207, 213, 219, 297, 301, 304, 313, 328, 344 Pathologic Processes, 301, 344 Pathologies, 5, 62, 219, 344 Pathophysiology, 215, 344 Patient Care Management, 211, 344 Patient Education, 264, 280, 290, 292, 295, 345 Pediatrics, 14, 33, 60, 61, 64, 97, 108, 112, 113, 115, 122, 124, 345 Pedigree, 161, 345 Peer Group, 195, 345 Pelvic, 345, 350 Pentoxifylline, 113, 122, 345 Peptide, 61, 91, 108, 345, 347, 350, 362 Perception, 11, 12, 18, 31, 35, 57, 91, 141, 150, 155, 194, 217, 229, 312, 326, 345, 355 Percutaneous, 29, 345 Perforation, 262, 345 Perfusion, 328, 345, 363 Perilymph, 44, 345, 355 Peripheral Nervous System, 341, 344, 345, 349, 360 Peripheral Nervous System Diseases, 341, 345 Peripheral Neuropathy, 79, 252, 345 Peripheral vision, 345, 367 Periventricular Leukomalacia, 54, 345 Pernicious, 336, 345 Peroxidase, 334, 337, 345 Phagocytosis, 63, 69, 345 Phallic, 322, 346 Pharmacologic, 72, 172, 300, 302, 346, 363 Pharynx, 173, 346 Phenotype, 33, 34, 41, 43, 50, 51, 53, 61, 62, 65, 81, 89, 91, 106, 312, 346 Phenylalanine, 346, 364 Phosphodiesterase, 345, 346 Phospholipases, 346, 357 Phospholipids, 321, 330, 336, 346

Index 379

Phosphorus, 306, 346 Phosphorylate, 77, 346 Phosphorylated, 64, 310, 346 Phosphorylating, 77, 346 Phosphorylation, 45, 64, 77, 161, 346, 365 Photoreceptor, 46, 346 Physical Examination, 211, 324, 346 Physiologic, 48, 173, 216, 304, 346, 349, 352 Phytic Acid, 330, 346 Pigment, 298, 336, 346 Pilot Projects, 56, 346 Pilot study, 52, 114, 346 Pitch, 26, 143, 208, 346 Placenta, 347, 349, 365 Plants, 156, 306, 324, 333, 338, 347, 355, 363 Plaque, 49, 347 Plasma, 78, 79, 300, 304, 307, 308, 323, 324, 326, 332, 347, 356, 363 Plasma cells, 300, 347 Plasticity, 30, 33, 43, 46, 57, 60, 85, 93, 120, 122, 123, 347 Plastids, 343, 347 Platelet Activation, 347, 357 Platelet Aggregation, 299, 345, 347 Platinum, 309, 335, 347 Point Mutation, 6, 78, 101, 112, 347 Poisoning, 331, 339, 347, 356 Polycystic, 256, 347 Polyhydramnios, 14, 347 Polymorphic, 58, 347 Polymorphism, 61, 347 Polypeptide, 163, 175, 299, 311, 325, 327, 347, 364, 368 Polysaccharide, 298, 300, 308, 348, 350 Pons, 305, 348 Posterior, 97, 299, 302, 308, 309, 317, 342, 343, 344, 348, 352, 366 Postnatal, 68, 81, 348, 359 Postoperative, 216, 348 Postsynaptic, 43, 45, 341, 348, 357, 361 Post-synaptic, 31, 348 Post-translational, 106, 348 Potassium, 14, 45, 60, 61, 157, 348 Potassium Channels, 45, 60, 348 Potentiates, 55, 348 Potentiation, 348, 357 Practice Guidelines, 245, 348 Precursor, 59, 72, 85, 86, 301, 314, 317, 319, 346, 348, 364 Predisposition, 17, 161, 348 Prednisolone, 348

Prednisone, 30, 263, 264, 348 Pregnancy Tests, 324, 348 Prejudice, 7, 348 Prenatal, 10, 14, 318, 348 Prenatal Diagnosis, 10, 14, 348 Presbycusis, 17, 23, 51, 189, 349 Presynaptic, 31, 45, 340, 341, 349, 361 Presynaptic Terminals, 45, 340, 349, 361 Prevalence, 7, 8, 13, 24, 104, 106, 141, 176, 206, 207, 210, 349 Private Practice, 189, 349 Probe, 28, 70, 349 Problem Solving, 273, 349 Progeny, 67, 81, 349 Progesterone, 349, 359 Progression, 40, 50, 65, 81, 233, 300, 349 Progressive Bulbar Palsy, 100, 349 Projection, 151, 315, 343, 349, 353 Proline, 80, 311, 327, 349 Promoter, 48, 349 Prophase, 174, 342, 349, 361 Prophylaxis, 172, 349 Prosencephalon, 316, 349, 361 Prospective study, 334, 349 Prostaglandin, 113, 349 Prostaglandins A, 329, 350 Prostate, 256, 350 Prosthesis, 38, 49, 149, 150, 162, 167, 168, 350 Protease, 109, 350 Protein C, 21, 60, 73, 79, 264, 299, 303, 310, 350, 365 Protein S, 72, 175, 204, 256, 257, 304, 323, 340, 350, 354 Protein Transport, 63, 350 Proteinuria, 340, 350 Proteoglycans, 303, 350 Proteolytic, 65, 105, 311, 350 Protocol, 24, 27, 39, 166, 169, 350 Protozoa, 337, 351 Proximal, 316, 349, 351 Pruritic, 317, 351 Psychiatry, 107, 321, 351, 366 Psychic, 351, 356 Psychology, 15, 42, 76, 91, 100, 138, 184, 188, 199, 205, 206, 209, 210, 243, 316, 351 Psychotherapy, 181, 198, 311, 321, 351 Ptosis, 219, 250, 351 Public Health, 19, 24, 30, 39, 232, 245, 351 Public Policy, 4, 241, 351 Publishing, 5, 12, 13, 84, 185, 188, 193, 194, 205, 208, 211, 214, 216, 268, 281, 351

380 Deafness

Pulmonary, 54, 55, 95, 112, 304, 305, 313, 320, 332, 351, 366 Pulmonary Artery, 304, 351, 366 Pulmonary Edema, 95, 112, 332, 351 Pulse, 35, 39, 158, 338, 351 Pupa, 337, 351 Purines, 303, 351, 356 Putamen, 300, 303, 313, 340, 351 Q Quality of Life, 11, 15, 22, 23, 40, 51, 68, 89, 176, 265, 266, 351 Quiescent, 32, 59, 351 R Race, 309, 337, 351 Radial Nerve, 157, 352 Radiation, 300, 303, 319, 320, 322, 327, 331, 332, 352, 355, 367 Radiation therapy, 320, 327, 331, 332, 352, 368 Radio Waves, 316, 337, 352 Radioactive, 303, 305, 327, 329, 331, 332, 334, 342, 352, 355, 368 Radiography, 324, 352 Radiolabeled, 332, 352, 368 Radiological, 216, 345, 352 Radiology, 280, 352 Radiotherapy, 305, 332, 352, 368 Random Allocation, 352 Randomization, 39, 352 Randomized, 113, 122, 317, 352 Receptor, 30, 43, 45, 66, 70, 82, 167, 297, 300, 312, 320, 337, 346, 352, 357 Recombinant, 70, 352, 365 Recombination, 43, 45, 323, 353 Rectum, 301, 305, 316, 322, 323, 333, 350, 353 Recurrence, 107, 353 Red Nucleus, 302, 353 Reductase, 72, 353 Refer, 1, 311, 317, 322, 334, 335, 341, 353, 366 Reflective, 151, 353 Reflex, 12, 353 Refraction, 151, 339, 353, 358 Refractive Errors, 207, 299, 353 Refractive Power, 339, 353 Regeneration, 31, 32, 53, 55, 81, 353 Regimen, 8, 317, 353 Rehabilitative, 4, 27, 215, 216, 353 Reliability, 37, 216, 353 Remission, 353 Renal failure, 4, 353

Renal tubular, 89, 94, 115, 353 Renal tubular acidosis, 89, 94, 115, 353 Research Support, 268, 353 Respiration, 301, 306, 338, 353 Respiratory distress syndrome, 306, 353 Restoration, 93, 120, 159, 176, 354, 367 Reticular, 45, 354 Retina, 46, 63, 207, 301, 309, 316, 334, 339, 341, 343, 354, 355, 367 Retinae, 335, 354 Retinal, 7, 46, 63, 207, 248, 312, 316, 343, 354, 367 Retinitis, 46, 69, 219, 248, 255, 354 Retinitis Pigmentosa, 46, 69, 219, 248, 255, 354 Retinoblastoma, 256, 354 Retinol, 354 Retinopathy, 88, 316, 354 Retrospective, 14, 115, 202, 354 Rheology, 345, 354 Rheumatism, 89, 354 Rheumatoid, 107, 354 Rheumatoid arthritis, 107, 354 Ribosome, 354, 364 Rigidity, 347, 354 Riluzole, 179, 355 Risk factor, 23, 54, 115, 233, 277, 349, 355 Rod, 46, 255, 303, 310, 341, 346, 355 Rubella, 22, 126, 219, 355 S Saccule, 73, 167, 168, 355, 366 Saliva, 355 Salivary, 7, 316, 321, 344, 355, 360 Salivary glands, 7, 316, 321, 355 Saponins, 355, 359 Satellite, 77, 193, 222, 223, 355 Scala Tympani, 310, 355 Scans, 50, 355 Schizoid, 355, 367 Schizophrenia, 179, 355, 367 Schizotypal Personality Disorder, 355, 367 Sclerosis, 179, 256, 355 Screening, 6, 8, 9, 11, 22, 26, 65, 66, 81, 96, 98, 99, 175, 212, 263, 310, 340, 356 Secretion, 106, 328, 330, 331, 338, 356, 365 Secretory, 93, 255, 341, 356, 361 Segregation, 106, 353, 356 Seizures, 45, 171, 254, 344, 356 Semen, 350, 356 Semicircular canal, 62, 73, 330, 356 Senile, 115, 156, 349, 356 Sensor, 80, 148, 152, 168, 356

Index 381

Sensory Deprivation, 122, 205, 233, 356 Septicemia, 97, 356 Sequencing, 70, 95, 356 Serine, 109, 356 Serum, 299, 311, 332, 356 Sex Determination, 256, 356 Sex Education, 6, 356 Sexually Transmitted Diseases, 232, 356 Sharpness, 356, 367 Shock, 173, 356, 364 Side effect, 235, 298, 314, 356, 363 Signal Transduction, 175, 330, 357 Signs and Symptoms, 277, 353, 357 Silicon, 28, 357 Silicon Dioxide, 357 Single Person, 40, 357 Sinoatrial Node, 120, 357 Skeletal, 61, 90, 250, 310, 338, 339, 357, 358 Skeleton, 297, 350, 357 Skull, 158, 305, 314, 357, 362 Sleep apnea, 87, 357 Small intestine, 327, 331, 357 Social Environment, 14, 351, 357 Social Sciences, 59, 189, 357 Social Work, 135, 224, 273, 357 Socialization, 11, 132, 194, 195, 203, 215, 358 Sodium, 109, 358 Software Design, 211, 358 Solvent, 343, 358 Somatic, 297, 336, 338, 345, 358, 361 Songbirds, 31, 32, 358 Sound wave, 150, 157, 163, 173, 312, 316, 353, 358 Spasm, 341, 358 Spatial disorientation, 317, 358 Specialist, 213, 222, 273, 286, 358 Species, 32, 156, 317, 319, 325, 326, 327, 336, 337, 338, 345, 351, 358, 360, 364, 367 Specificity, 8, 42, 48, 175, 298, 306, 358, 363 Spectrum, 42, 98, 102, 191, 337, 352, 358 Speech Intelligibility, 76, 215, 216, 358 Speech Perception, 12, 18, 71, 76, 125, 160, 183, 194, 358 Speech-Language Pathology, 5, 358 Sperm, 161, 309, 358 Sphincter, 333, 359 Spinal cord, 301, 305, 308, 309, 317, 322, 336, 340, 341, 344, 345, 352, 353, 359 Spiral Ganglion, 41, 83, 112, 310, 359, 366 Spiral Lamina, 303, 359, 361, 364 Spleen, 299, 335, 359

Sporadic, 61, 93, 95, 230, 354, 359 Stabilization, 46, 74, 359 Staff Development, 186, 359 Staging, 355, 359 Stapes, 150, 167, 250, 252, 329, 343, 359 Statistically significant, 15, 359 Steel, 95, 108, 310, 359 Stem Cells, 359, 365 Sterility, 314, 359 Steroid, 87, 114, 314, 355, 359 Stimulus, 25, 35, 37, 39, 43, 83, 149, 150, 299, 302, 317, 318, 320, 330, 331, 333, 353, 359, 362 Stomach, 316, 319, 322, 323, 327, 331, 339, 346, 357, 359 Strabismus, 219, 299, 359 Stress, 5, 15, 60, 66, 137, 182, 188, 209, 210, 218, 339, 344, 348, 354, 360 Stria, 41, 53, 57, 66, 360 Stria Vascularis, 41, 53, 66, 360 Striatum, 340, 360 Stroke, 107, 145, 169, 240, 306, 360 Subacute, 330, 360 Subarachnoid, 41, 360 Subclinical, 12, 329, 356, 360 Subculture, 232, 360 Subcutaneous, 317, 360 Submandibular, 360 Submaxillary, 71, 360 Subspecies, 358, 360 Substance P, 356, 360 Substrate, 48, 83, 360 Substrate Specificity, 48, 360 Sudden death, 61, 156, 157, 171, 360 Superior vena cava, 357, 360 Support group, 16, 190, 220, 269, 270, 272, 284, 360 Suppurative, 41, 360 Symphysis, 350, 360 Symptomatic, 173, 360 Synapses, 43, 46, 124, 341, 360, 361 Synapsis, 361 Synaptic, 43, 45, 60, 70, 120, 124, 357, 361 Synaptic Transmission, 120, 361 Synaptic Vesicles, 361 Syncope, 171, 361, 366 Syrinx, 358, 361 Systemic, 56, 173, 181, 236, 299, 304, 305, 319, 328, 330, 331, 332, 348, 352, 356, 361, 368 Systemic disease, 328, 356, 361 Systolic, 328, 361

382 Deafness

T Tape Recording, 7, 361 Tectorial Membrane, 25, 68, 229, 361 Telangiectasia, 256, 361 Telecommunications, 153, 159, 161, 162, 164, 165, 170, 183, 219, 274, 276, 285, 312, 361 Telencephalon, 31, 303, 349, 361 Telomerase, 79, 361 Temporal, 29, 35, 41, 42, 46, 49, 50, 64, 66, 73, 92, 211, 302, 326, 336, 361, 362, 366 Temporal Lobe, 302, 362, 366 Terminalis, 361, 362 Terminator, 310, 362 Testosterone, 353, 362 Thalamic, 302, 362 Thalamic Diseases, 302, 362 Thalamus, 313, 316, 362, 366 Therapeutics, 34, 236, 362 Thiamine, 114, 115, 362 Thoracic, 305, 352, 362, 367 Threonine, 356, 362 Threshold, 12, 18, 49, 55, 57, 191, 217, 320, 328, 362 Thrombin, 347, 350, 362 Thrombomodulin, 350, 362 Thrombosis, 330, 331, 350, 360, 362 Thrombus, 313, 329, 339, 347, 362 Thyroid, 47, 48, 67, 82, 324, 328, 331, 337, 362, 364 Thyroid Gland, 324, 362 Thyroid Hormones, 47, 337, 362, 364 Thyrotropin, 328, 362 Tin, 345, 347, 363 Tinnitus, 23, 45, 62, 173, 178, 179, 189, 206, 208, 269, 275, 278, 343, 363, 366 Tissue Culture, 341, 363 Tissue Distribution, 306, 363 Tomography, 123, 363 Tonicity, 317, 363 Tooth Preparation, 297, 363 Torsion, 329, 363 Toxic, iv, 30, 299, 315, 319, 325, 328, 341, 363, 364 Toxicity, 317, 363 Toxicology, 242, 363 Toxins, 299, 300, 318, 319, 329, 356, 363 Trace element, 357, 363 Trachea, 305, 333, 346, 362, 363 Traction, 310, 363 Transcriptase, 361, 363 Transcription Factors, 82, 363

Transduction, 45, 62, 66, 68, 357, 364 Transfection, 64, 304, 364 Transgenes, 59, 69, 364 Translating, 153, 157, 170, 264, 364 Translation, 59, 161, 175, 267, 340, 364 Translational, 364 Translocate, 73, 364 Translocation, 40, 72, 350, 364 Transmitter, 31, 45, 60, 70, 148, 149, 152, 154, 159, 265, 297, 302, 331, 361, 364 Transplantation, 59, 309, 333, 364 Transposons, 44, 364 Trauma, 22, 189, 339, 364 Triad, 244, 364 Trophic, 77, 364 Tryptophan, 311, 364 Tuberous Sclerosis, 256, 364 Tumour, 92, 322, 364 Tympani, 163, 364 Tympanic membrane, 148, 150, 173, 364 Type 2 diabetes, 6, 364 Tyrosine, 77, 364 Tyrothricin, 325, 364 U Ultrasonography, 324, 365 Umbilical Arteries, 365 Umbilical Cord, 54, 365 Umbilical cord blood, 54, 365 Unconscious, 315, 328, 365 Uncoupling Agents, 56, 332, 365 Underachievement, 278, 365 Urea, 14, 332, 365 Uremia, 332, 353, 365 Urethra, 350, 365 Urinary, 254, 323, 342, 365 Urine, 301, 304, 326, 332, 342, 350, 365 Urogenital, 71, 323, 365 Uterus, 308, 313, 322, 349, 365 V Vaccine, 84, 172, 350, 365 Vacuoles, 318, 343, 365 Vascular, 109, 125, 173, 308, 309, 329, 330, 347, 360, 362, 365 Vasculitis, 308, 365 VE, 183, 273, 274, 365 Vector, 77, 364, 365 Vein, 301, 342, 344, 355, 360, 365 Venous, 301, 305, 308, 331, 350, 365, 366 Venous blood, 305, 308, 366 Ventilation, 306, 366 Ventral, 73, 75, 310, 348, 366 Ventricle, 302, 307, 351, 357, 361, 362, 366

Index 383

Ventricular, 157, 172, 366 Ventricular fibrillation, 172, 366 Vertebral, 342, 366 Vertebrobasilar Insufficiency, 107, 366 Vertigo, 7, 83, 107, 343, 366 Vesicular, 350, 366 Vestibular Aqueduct, 48, 211, 366 Vestibule, 163, 167, 310, 330, 343, 355, 356, 366 Vestibulocochlear Nerve, 302, 310, 363, 366 Vestibulocochlear Nerve Diseases, 363, 366 Veterinary Medicine, 241, 366 Viral, 8, 63, 79, 84, 164, 318, 342, 364, 367 Virus, 34, 84, 222, 243, 244, 270, 303, 319, 323, 331, 347, 355, 364, 367 Viscosity, 304, 354, 367 Visual Acuity, 150, 151, 313, 333, 367 Visual Cortex, 299, 367 Visual field, 42, 213, 334, 338, 354, 367 Visual Perception, 75, 367 Vitamin A, 330, 354, 367

Vitreous Body, 309, 354, 367 Vitreous Hemorrhage, 316, 367 Vitro, 63, 74, 78, 367 Vivo, 64, 66, 78, 83, 367 Vocational Guidance, 184, 367 Voltage-gated, 43, 61, 367 W War, 132, 202, 269, 339, 367 White blood cell, 300, 304, 309, 334, 335, 338, 347, 367 Windpipe, 346, 362, 367 Withdrawal, 243, 367 Wound Healing, 74, 307, 330, 367 X Xenograft, 300, 367 X-ray, 79, 312, 322, 332, 339, 342, 352, 355, 367 X-ray therapy, 332, 367 Y Yeasts, 322, 346, 368 Z Zygote, 161, 368 Zymogen, 350, 368

384 Deafness

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