CMT/HSMN 1A cochlear implantation in a patient with deafness

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J Laryngol Otol. 2006 Jun;120(6):508-10.

Cochlear implantation in a patient with deafness induced by Charcot-

Marie-Tooth disease (hereditary motor and sensory neuropathies).

Postelmans JT, Stokroos RJ.

Department of ENT/Head and Neck Surgery, Academic Hospital

Maastricht, Maastricht, The Netherlands.

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.