Genetics of Deafness offers a journey through areas crucial for understanding the causes and effects of hearing loss. It covers such topics as the latest approaches in diagnostics and deafness research and the current status and future promise of gene therapy for hearing restoration. The book begins by bringing attention to how hearing loss affects the individual and society. Methods of hearing loss detection and management throughout the lifespan are highlighted as is a particularly new development in newborn hearing screening. The challenges of hearing loss, an extremely heterogeneous impairment, are addressed. Additional topics include current research interests, ranging from novel gene identification to their functional validation in the mouse and zebrafish. The book ends with a chapter on the state of the art of gene therapy—an area that is certain to gain increasing attention as molecular mechanisms of deafness are better understood. Genetics of Deafness, written by leading authors in the field, is a must read for clinicians, researchers, and students. It provides much needed insight into the diagnosis and research of hereditary hearing loss.
This volume reviews current concepts and recent research findings of the application of gene therapy to the inner ear. Various forms of gene therapy, disorders that are potential targets for gene therapy, techniques for gene delivery, and mechanisms of gene targeting, and also obstacles that have yet to be overcome are discussed.
This is the third edition of the foremost medical reference on hereditary hearing loss. Chapters on epidemiology, embryology, non-syndromic hearing loss, and syndromic forms of hearing loss have all been updated with particular attention to the vast amount of new information on molecular mechanisms, and chapters on clinical and molecular diagnosis and on genetic susceptibility to ototoxic factors have been added. As in previous editions, the syndromes are grouped by system (visual, metabolic, cardiologic, neurologic, musculoskeletal, endocrine, etc.), with each chapter written by a recognized expert in the field. Written for practicing clinicians, this volume is an excellent reference for physicians, audiologists, and other professionals working with individuals with hearing loss and their families, and can also serve as a text for clinical training programs and for researchers in the hearing sciences.
Heredity, either alone or in combination with environmental factors, is the most prominent underlying cause of hearing impairment. Thanks in large part to positional cloning techniques, scientists have identified nearly 100 gene loci implicated in hearing loss since 1995-an extraordinarily rapid rate of gene identification. Genetic Hearing Loss branches into syndromic and nonsyndromic categorical directions in its coverage of the genetics behind hearing loss. Authored by 60 internationally recognized researchers, the book describes the normal development of the ear, updates the classification and epidemiology of hearing loss, and surveys the usage of audiometric tests and diagnostic medical examinations.
This book systematically discusses the pathogenesis, prevention, and the current and potential clinical treatment of hearing loss, as well as the latest advances in hearing research. Hearing loss is a prevalent sensory disorder, which according to a 2015 World Health Organization (WHO) report affected 9% of the global population in 2015. As populations continue to age, more and more people are suffering from the condition, with 60% of those aged between 65 and 75 affected. Hearing loss seriously affects patients’ ability to work ability and quality of life, and as such deafness has become an increasingly urgent social problem around the globe. Sensorineural hearing loss is mainly caused by damage to the hair cells (HCs), and the subsequent loss of spiral ganglion neurons (SGNs). Damage to the HCs in the inner ear can result from exposure to loud noises and environmental and chemical toxins as well as genetic disorders, aging, and certain medications. This book provides ENT specialists and researchers, as well as individuals affected a comprehensive introduction to the field of hearing loss.
For clinical researchers in audiology and otolaryngology, this fifth book in the Kresge- Mirmelstein Award series features the proceedings of the 1998 symposium. The book includes contributions from leading researchers on genetic causes of hearing loss and includes a CD-ROM containing audio and video footage from a Balinese village with a large genetically deaf population that have adopted a sign language indigenous to their culture. The CD-ROM also features samples of American Sign Language and Cued Speech. In the tradition of Berlin's previous work this book presents stunning new and evocative information for both researchers and clinicians.
Millions of Americans experience some degree of hearing loss. The Social Security Administration (SSA) operates programs that provide cash disability benefits to people with permanent impairments like hearing loss, if they can show that their impairments meet stringent SSA criteria and their earnings are below an SSA threshold. The National Research Council convened an expert committee at the request of the SSA to study the issues related to disability determination for people with hearing loss. This volume is the product of that study. Hearing Loss: Determining Eligibility for Social Security Benefits reviews current knowledge about hearing loss and its measurement and treatment, and provides an evaluation of the strengths and weaknesses of the current processes and criteria. It recommends changes to strengthen the disability determination process and ensure its reliability and fairness. The book addresses criteria for selection of pure tone and speech tests, guidelines for test administration, testing of hearing in noise, special issues related to testing children, and the difficulty of predicting work capacity from clinical hearing test results. It should be useful to audiologists, otolaryngologists, disability advocates, and others who are concerned with people who have hearing loss.
Weaving together lyrical history and personal memoir, Virdi powerfully examines society’s—and her own—perception of life as a deaf person in America. At the age of four, Jaipreet Virdi’s world went silent. A severe case of meningitis left her alive but deaf, suddenly treated differently by everyone. Her deafness downplayed by society and doctors, she struggled to “pass” as hearing for most of her life. Countless cures, treatments, and technologies led to dead ends. Never quite deaf enough for the Deaf community or quite hearing enough for the “normal” majority, Virdi was stuck in aural limbo for years. It wasn’t until her thirties, exasperated by problems with new digital hearing aids, that she began to actively assert her deafness and reexamine society’s—and her own—perception of life as a deaf person in America. Through lyrical history and personal memoir, Hearing Happiness raises pivotal questions about deafness in American society and the endless quest for a cure. Taking us from the 1860s up to the present, Virdi combs archives and museums to understand the long history of curious cures: ear trumpets, violet ray apparatuses, vibrating massagers, electrotherapy machines, airplane diving, bloodletting, skull hammering, and many more. Hundreds of procedures and products have promised grand miracles but always failed to deliver a universal cure—a harmful legacy that is still present in contemporary biomedicine. Blending Virdi’s own experiences together with her exploration into the fascinating history of deafness cures, Hearing Happiness is a powerful story that America needs to hear. Praise for Hearing Happiness “In part a critical memoir of her own life, this archival tour de force centers on d/Deafness, and, specifically, the obsessive search for a “cure”. . . . This survey of cure and its politics, framed by disability studies, allows readers—either for the first time or as a stunning example in the field—to think about how notions of remediation are leveraged against the most vulnerable.” —Public Books “Engaging. . . . A sweeping chronology of human deafness fortified with the author’s personal struggles and triumphs.” —Kirkus Reviews “Part memoir, part historical monograph, Virdi’s Hearing Happiness breaks the mold for academic press publications.” —Publishers Weekly “In her insightful book, Virdi probes how society perceives deafness and challenges the idea that a disability is a deficit. . . . [She] powerfully demonstrates how cures for deafness pressure individuals to change, to “be better.” —Washington Post
Since the first edition was published in 1998, considerable advances have been made in the fields of pitch perception and speech perception. In addition, there have been major changes in the way that hearing aids work, and the features they offer. This book will provide an understanding of the changes in perception that take place when a person has cochlear hearing loss so the reader understands not only what does happen, but why it happens. It interrelates physiological and perceptual data and presents both this and basic concepts in an integrated manner. The goal is to convey an understanding of the perceptual changes associated with cochlear hearing loss, of the difficulties faced by the hearing-impaired person, and the limitations of current hearing aids.
Otolaryngology Head and Neck Surgery is the medical and surgical specialty addressing disorders of the head and neck in both adult and pediatric populations. The goal of the encyclopedia is to serve as a single and comprehensive source of all the information that is essential for students and practitioners of the specialty. The vast amount of information included in the encyclopedia is divided into 5 volumes in line with the subspecialties of general otolaryngology, head and neck surgery, pediatric otolaryngology, otology–neurotology, and facial plastics. All volume editors are internationally recognized otolaryngologists with experience in publishing. Each section editor recruited experienced authors from all over the world to contribute on structured topics, and all entries are supported by published references. Thus, all information included in the encyclopedia is from credible sources and has been carefully screened for accuracy. The strength of the encyclopedia is its online availability and quick search features, which allow rapid retrieval of definitions and more in-depth information. Key words are hyperlinked to provide a gateway to numerous referenced manuscripts, journals, and books.
