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Function and Dysfunction of the Cochlea: From Mechanisms to Potential Therapies

Book Series:  A Cold Spring Harbor Perspectives in Medicine Collection
Subject Area(s):  Cell BiologyHuman Biology and Disease

Edited by Guy P. Richardson, Sussex Neuroscience, University of Sussex; Christine Petit, Collège de France, Institut Pasteur

Download a Free Excerpt from Function and Dysfunction of the Cochlea: From Mechanisms to Potential Therapies:

Molecular Structure of the Hair Cell Mechanoelectrical Transduction Complex

© 2019 • 348 pages, illustrated (75 color and 7 B&W), index
Hardcover •
ISBN  978-1-621822-79-0

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  •     Description    
  •     Contents    


The cochlea, the mammalian auditory organ, is a spiral-shaped structure in the inner ear that is responsible for hearing. It converts sound vibrations into electrical signals and sends them to the brain with a remarkable temporal precision. Defects in the cellular or molecular components of the cochlea can lead to deafness and other hearing impairments.

Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Medicine provides a comprehensive review of the complex molecular mechanisms that are responsible for cochlear function and were, for the most part, revealed by the discovery of genes responsible for hearing impairment. It describes major advances in our understanding of the pathogenic processes underlying various forms of hearing loss and the emergence of treatments for deafness. The contributors focus on the highly specialized cells which mediate auditory transduction—the hair cells—and their unique architectural, mechanical, and molecular characteristics. They also consider the properties of other cochlear components, including the tectorial membrane, the synaptic connections with auditory neurons, and the resident macrophages, as well as the development and evolution of the cochlea as a whole. Topics such as hair cell regeneration, otoacoustic emissions, and ototoxicity are also discussed.

In addition, the authors examine the epidemiology of hearing loss and emerging, innovative, therapeutic strategies (e.g., gene therapy, optical cochlear implants). This volume is therefore a valuable reference for cell biologists and neuroscientists interested in the biology of hearing, as well as physician–scientists seeking new approaches to alleviate hearing impairments.


A Functional Perspective on the Evolution of the Cochlea
Christine Köppl and Geoffrey A. Manley
Cochlear Frequency Tuning and Otoacoustic Emissions
Christopher A. Shera and Karolina K. Charaziak
Active Biomechanics of Sensory Hair Bundles
Dolores Bozovic
Development and Patterning of the Cochlea: From Convergent Extension to Planar Polarity
Mireille Montcouquiol and Matthew W. Kelley
Hair-Bundle Links: Genetics as the Gateway to Function
Guy P. Richardson and Christine Petit
Function and Dysfunction of TMC Channels in Inner Ear Hair Cells
David P. Corey, Nurunisa Akyuz, and Jeffrey R. Holt
Molecular Structure of the Hair Cell Mechanoelectrical Transduction Complex
Christopher L. Cunningham and Ulrich Müller
Molecular Composition of Vestibular Hair Bundles
Jocelyn F. Krey and Peter G. Barr-Gillespie
The Tectorial Membrane: Mechanical Properties and Functions
Jonathan B. Sellon, Roozbeh Ghaffari, and Dennis M. Freeman
Hair Cell Afferent Synapses: Function and Dysfunction
Stuart L. Johnson, Saaid Safieddine, Mirna Mustapha, and Walter Marcotti
Outer Hair Cells and Electromotility
Jonathan Ashmore
Efferent Inhibition of the Cochlea
Paul Albert Fuchs and Amanda M. Lauer
Inner Ear Connexin Channels: Roles in Development and Maintenance of Cochlear Function
Fabio Mammano
The Epidemiology of Deafness
Abraham M. Sheffield and Richard J.H. Smith
Hidden Hearing Loss: A Disorder with Multiple Etiologies and Mechanisms
David Kohrman, Guoqiang Wan, Luis Cassinotti, and Gabriel Corfas
Aminoglycoside- and Cisplatin-Induced Ototoxicity: Mechanisms and Otoprotective Strategies
Corné J. Kros and Peter S. Steyger
Age-Related Hearing Loss
Michael R. Bowl and Sally J. Dawson
Interactions between Macrophages and the Sensory Cells of the Inner Ear
Mark E. Warchol
Stem Cells and the Bird Cochlea—Where Is Everybody?
Amanda S. Janesick and Stefan Heller
Cochlear Gene Therapies
Lawrence Lustig and Omar Akil
Toward the Optical Cochlear Implant
Tobias Dombrowski, Vladan Rankovic, and Tobias Moser