[Update on physiology and pathophysiology of the inner ear: pathomechanisms of sensorineural hearing loss].

HNO
N StrenzkeT Moser

Abstract

Hearing impairment is the most common form of human sensory deficit. The most frequent form, sensorineural hearing loss (SNHL), which accounts for approximately 70% of cases, encompasses various pathologies in both the inner ear and the auditory nerve. The individual hearing impairment and its outcome following aiding with hearing devices critically depend on the underlying disorder. Here recent progress in our understanding of the cellular mechanisms of SNHL in genetically engineered mouse models is reviewed. First, insights gained from models for specific defects in cochlear sound amplification and ion homeostasis are discussed followed by a focus on disorders of the inner hair cell synapses (auditory synaptopathy) and the auditory nerve (auditory neuropathy). Both nosological entities have also attracted substantial clinical interest in recent years and share an impaired temporal processing of auditory stimuli. This results in poor speech recognition, often out of proportion to the pure tone threshold. Hearing loss can range from mild variants with exclusive deficits of temporal processing to complete deafness. At least initially, signs of normal outer hair cell function such as evoked otoacoustic emissions can be found. In ...Continue Reading

References

Nov 1, 1976·The Annals of Otology, Rhinology, and Laryngology·D J Lim
Jun 1, 1996·Brain : a Journal of Neurology·A StarrC I Berlin
Nov 3, 1998·Cell and Tissue Research·J LautermannE Winterhager
Feb 15, 2001·Nature Genetics·K P Steel, C J Kros
Mar 17, 2001·Nature Reviews. Neuroscience·T J Jentsch
Aug 31, 2001·Nature Genetics·N J ParkinsonB L Tempel
Oct 17, 2001·Laryngo- rhino- otologie·A Lesinski-SchiedatT Lenarz
Jan 22, 2002·Nature Neuroscience·Elisabeth Glowatzki, Paul A Fuchs
Oct 17, 2002·Journal of the Association for Research in Otolaryngology : JARO·Yong WangM Charles Liberman
Dec 20, 2002·Human Molecular Genetics·Barbara TeubnerKlaus Willecke
Apr 30, 2003·Human Molecular Genetics·Xue Zhong LiuZheng Yi Chen
Jun 14, 2003·Brain : a Journal of Neurology·Arnold StarrBronya Keats
Sep 3, 2003·Nature Reviews. Neuroscience·Ueli Suter, Steven S Scherer
Nov 25, 2003·Human Mutation·Montserrat Rodríguez-BallesterosIgnacio del Castillo
Apr 23, 2004·Trends in Neurosciences·Donata Oertel, Eric D Young
Sep 11, 2004·Journal of the Association for Research in Otolaryngology : JARO·Hongwei DouEbenezer N Yamoah
Sep 24, 2004·The Journal of Cell Biology·Sandra Lacas-GervaisMichele Solimena
Oct 8, 2004·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Konstantina StankovicGabriel Corfas
Nov 2, 2004·Nature Cell Biology·Thomas J JentschJens C Fuhrmann
Dec 14, 2004·Nature Cell Biology·Martina BeltramelloFabio Mammano
Dec 24, 2004·Journal of Neurophysiology·Fan-Gang ZengArnold Starr
Jan 28, 2005·Journal of the Association for Research in Otolaryngology : JARO·Arnold StarrMarci M Lesperance
Apr 23, 2005·The Journal of Physiology·Paul Albert Fuchs
May 10, 2005·Genes, Chromosomes & Cancer·Anders MolvenLars A Akslen
Jun 2, 2005·Nature Reviews. Molecular Cell Biology·Paul L McNeil, Tom Kirchhausen
Jul 5, 2005·Nature Neuroscience·P Kevin LeganGuy P Richardson
Aug 3, 2005·The Journal of Clinical Investigation·Thomas J JentschAnselm A Zdebik
Oct 18, 2005·International Journal of Pediatric Otorhinolaryngology·I RouillonN Loundon
Dec 24, 2005·Nature Reviews. Neuroscience·Robert Fettiplace, Carole M Hackney
May 24, 2006·Journal of the Association for Research in Otolaryngology : JARO·Rutger F PlantingaCor W R J Cremers
Jun 15, 2006·The Journal of Membrane Biology·R NouvianT Moser
Jun 15, 2006·The Journal of Membrane Biology·H-B ZhaoT W White
Aug 12, 2006·The Journal of Physiology·Tobias MoserDarina Khimich
Sep 1, 2006·Cell and Tissue Research·Tobias MoserAnna Lysakowski

❮ Previous
Next ❯

Citations

May 6, 2011·Expert Review of Medical Devices·Maura K Cosetti, Susan B Waltzman
Jun 25, 2008·Hearing Research·Jeffrey L HendricksDavid C Martin
May 28, 2014·Journal of Ophthalmology·Nandini Vijaya SinghamVisvaraja Subrayan
Dec 24, 2013·Acta Oto-laryngologica·Toru OhashiIzumi Koizuka
Sep 18, 2008·Current Opinion in Otolaryngology & Head and Neck Surgery

❮ Previous
Next ❯

Related Concepts

Related Feeds

Auditory Perception

Auditory perception is the ability to receive and interpret information attained by the ears. Here is the latest research on factors and underlying mechanisms that influence auditory perception.