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Bilateral sudden sensorineural hearing loss caused by Charcot-Marie-Tooth disease

Published online by Cambridge University Press:  08 March 2006

Chariton E. Papadakis ,
Jiannis K. Hajiioannou ,
Dionysios E. Kyrmizakis  and
John G. Bizakis
Chariton E. Papadakis
Affiliation:
Department of Otolaryngology, University of Crete School of Medicine, Heraklion, Crete, Greece.
Jiannis K. Hajiioannou
Affiliation:
Department of Otolaryngology, University of Crete School of Medicine, Heraklion, Crete, Greece.
Dionysios E. Kyrmizakis
Affiliation:
Department of Otolaryngology, University of Crete School of Medicine, Heraklion, Crete, Greece.
John G. Bizakis
Affiliation:
Department of Otolaryngology, University of Crete School of Medicine, Heraklion, Crete, Greece.
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Abstract

Charcot-Marie-Tooth (CMT) disease or hereditary motor and sensory neuropathy (HMSN) is a relatively common neurological syndrome, which has seldom been associated with hearing dysfunction, particularly sudden sensorineural hearing loss (SNHL). Families with autosomal dominant, autosomal recessive and X-linked forms of inheritance have been described.

Sudden sensorineural hearing loss is a frustrating and frightening condition, especially if the hearing loss is bilateral. Regarding the site of the lesion, the evidence from the literature on HMSN suggests that either the VIIIth nerve or central auditory pathways are primarily involved in patients with hearing loss.

We report the first case in the English literature of a patient with Charcot-Marie-Tooth type II disease presenting bilateral SNHL in the course of his disease. The patient was hospitalized for 15 days, and undergoing treatment without any audiological improvement. Detailed clinical, audiological and laboratory examination was performed.

The aetiology and prognostic indicators of bilateral SNHL are discussed, as well as, the incidence of hearing loss in CMT patients.

Keywords

Charcot-Marie-Tooth diseaseHearing Loss Sensorineural
Type
Research Article
Information
The Journal of Laryngology & Otology , Volume 117 , Issue 5 , May 2003 , pp. 399 - 401
Copyright
© Royal Society of Medicine Press Limited 2003

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