(mentions CMT) The Mitochondrial Connection in Auditory Neuropathy

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Audiol Neurootol. 2011 Jan 22;16(6):398-413. [Epub ahead of print]

The Mitochondrial Connection in Auditory Neuropathy.

Cacace AT, Pinheiro JM.

Department of Communication Sciences and Disorders, Wayne State University,

Detroit, Mich., USA.

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.