Hearing Dysfunction in a Large Family Affected by Dominant Optic Atrophy (OPA8-Related DOA): A Human Model of Hidden Auditory Neuropathy

Hearing Dysfunction in a Large Family Affected by Dominant Optic Atrophy (OPA8-Related DOA): A Human Model of Hidden Auditory Neuropathy

Hidden auditory neuropathy is characterized by reduced performances in challenging auditory tasks with the preservation of hearing thresholds, resulting from diffuse loss of cochlear inner hair cell (IHC) synapses following primary degeneration of unmyelinated terminals of auditory fibers. We report...

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Bibliographic Details
Main Authors: Rosamaria Santarelli, Chiara La Morgia, Maria Lucia Valentino, Piero Barboni, Anna Monteleone, Pietro Scimemi, Valerio Carelli
Format: Article
Language:English
Published: Frontiers Media S.A. 2019-05-01
Series:Frontiers in Neuroscience
Subjects:
hearing impairment
electrocochleography
speech perception
optic atrophy
acoustic reflexes
Online Access:https://www.frontiersin.org/article/10.3389/fnins.2019.00501/full

Internet

https://www.frontiersin.org/article/10.3389/fnins.2019.00501/full

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