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Cochlear implantation in a patient with deafness induced by Charcot–Marie–Tooth disease (hereditary motor and sensory neuropathies)

Published online by Cambridge University Press:  15 March 2006

J T F Postelmans  and
R J Stokroos
J T F Postelmans
Affiliation:
Department of ENT/Head and Neck Surgery, Academic Hospital Maastricht, Maastricht, The Netherlands
R J Stokroos
Affiliation:
Department of ENT/Head and Neck Surgery, Academic Hospital Maastricht, Maastricht, The Netherlands
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Abstract

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Charcot–Marie–Tooth disease (CMT), also named hereditary motor and sensory neuropathies (HMSN), comprises a clinically and genetically heterogeneous group of disorders affecting the peripheral nervous system. Deafness induced by CMT is clinically distinct among the genetically heterogeneous group of CMT disorders. Deafness in CMT patients is associated with point mutations or deletions in the transmembrane domain in the peripheral myelin gene (PMP) 22, which are in close proximity to the extracellular component of this gene. We present a patient with deafness induced by CMT type 1A, undergoing cochlear implantation. Prior investigations showed good results due to replacing a synchronous impulse by means of cochlear implantation in patients with auditory neuropathy.

Keywords

Hereditary Motor and Sensory NeuropathiesCochlear NerveCochlear Implants
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 120 , Issue 6 , June 2006 , pp. 508 - 510
Copyright
© 2006 JLO (1984) Limited