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Audiol Neurootol. 2011;16(6):398-413. doi: 10.1159/000323276. Epub 2011 Jan 22.

The mitochondrial connection in auditory neuropathy.

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1
Department of Communication Sciences and Disorders, Wayne State University, Detroit, Mich 48202, USA. cacacea @ wayne.edu

Abstract

'Auditory neuropathy' (AN), the term used to codify a primary degeneration of the auditory nerve, can be linked directly or indirectly to mitochondrial dysfunction. These observations are based on the expression of AN in known mitochondrial-based neurological diseases (Friedreich's ataxia, Mohr-Tranebjærg syndrome), in conditions where defects in axonal transport, protein trafficking, and fusion processes perturb and/or disrupt mitochondrial dynamics (Charcot-Marie-Tooth disease, autosomal dominant optic atrophy), in a common neonatal condition known to be toxic to mitochondria (hyperbilirubinemia), and where respiratory chain deficiencies produce reductions in oxidative phosphorylation that adversely affect peripheral auditory mechanisms. This body of evidence is solidified by data derived from temporal bone and genetic studies, biochemical, molecular biologic, behavioral, electroacoustic, and electrophysiological investigations.

PMID:
21266802
DOI:
10.1159/000323276
[Indexed for MEDLINE]
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