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.
Cochlear gene therapy has undergone tremendous advances over the past decade. Beginning with some groundbreaking work in 2005 documenting hair cell regeneration using virally mediated delivery of the mouse atonal 1 gene, gene therapy is now being explored as a possible treatment for a variety of causes of hearing loss. Recent advances in cochlear gene therapy include improved methods of gene delivery with a better delineation of viral vectors that are suitable for this purpose, additional improvements in hair cell regeneration, and directed research towards: · Autoimmune hearing loss, · Ototoxicity, · Spiral ganglion survival, and · Genetic forms of hearing loss. If successful, cochlear gene therapy will dramatically alter our ability to treat a variety of forms of acquired and genetic hearing loss. An attempt has been made in this Booklet to highlight recent advances in cochlear gene therapy over the past several years. ….. Dr. H.K.Saboowala.M.B.(Bom) M.R.S.H.(London)
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 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.
The field of generic hearing impairment is one where rapid advances are taking place, and it can often be difficult for Audiologists to keep track of the broader picture. In this important new text the authors take a synoptic approach and summarize the causes and basis of hearing impairments, the impact on the individual and the therapies available
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 special issue represents the third of three issues containing papers arising from the 'Auditory Function and Dysfunction: Molecular and Physiological Mechanisms' symposium held in Auckland, New Zealand in August 2001. It reflects on the exciting advances that are being made in our understanding of the molecular basis of cochlear development, neurodegeneration and the attempts to provide otoprotective strategies. One group of papers deals with some of the genetic aspects of cochlear development and hearing loss. In a second group the advances being made in strategies to protect the inner ear from stress and ototoxic drugs that may ultimately form the basis of treatments for inner ear disorders are being discussed. The last group of papers considers the delivery of compounds to the inner ear for its genetic or pharmacological manipulation. This and the other two issues stemming from the symposium will be valuable contributions to the communication between the wide-ranging disciplines within the hearing sciences and the clinic.
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
