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Auditory Temporal Processing and its Disorders$
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Jos J. Eggermont

Print publication date: 2015

Print ISBN-13: 9780198719090

Published to Oxford Scholarship Online: May 2015

DOI: 10.1093/acprof:oso/9780198719090.001.0001

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PRINTED FROM OXFORD SCHOLARSHIP ONLINE (oxford.universitypressscholarship.com). (c) Copyright Oxford University Press, 2021. All Rights Reserved. An individual user may print out a PDF of a single chapter of a monograph in OSO for personal use. date: 18 May 2021

Auditory neuropathy and multiple sclerosis

Auditory neuropathy and multiple sclerosis

(p.227) Chapter 13 Auditory neuropathy and multiple sclerosis
Auditory Temporal Processing and its Disorders

Jos Eggerrmont

Oxford University Press

Auditory neuropathy occurs frequently and is responsible for approximately 8% of newly diagnosed cases of hearing loss in children per year. Hyperbilirubinemia and hypoxia represent major risk factors, whereas generalized neuropathic disorders, or a genetic substrate involving the otoferlin (OTOF) gene, are responsible for the phenotype of auditory neuropathy in certain cases. Auditory nerve myelinopathy and/or desynchrony of neural discharges, and disordered function of the inner hair cell ribbon synapses are the most probable underlying pathophysiological mechanisms. There is no or very little hearing threshold loss. The disrupted neural activity did not affect intensity-related perception, however, significantly impaired timing-related perception, such as pitch discrimination at low frequencies, temporal integration, gap detection, temporal modulation detection, backward and forward masking, signal detection in noise, binaural beats, and sound localization using interaural time differences. There was a close association between gap detection thresholds measured psychoacoustically and electrophysiologically (N1 and P2 long-latency components) in auditory neuropathy subjects. Whereas auditory neuropathy is a purely peripheral disorder, multiple sclerosis (MS) is an inflammatory disease in which the fatty myelin sheaths around the axons of the central nervous system are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms. MS affects the ability of nerve cells in the brain and spinal cord to communicate with each other effectively. Audiograms are generally normal for age. Abnormal auditory brainstem responses showed prolonged inter-wave latencies reflecting conduction delays along the brainstem. Reduced mismatch negativity and white matter changes correlate with cognitive deficits.

Keywords:   electrocochleography, OTOF mutation, OPA1 mutation, otoacoustic emissions, psychoacoustics, human, temporal modulation transfer function, gap detection, abnormal loudness adaptation

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