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

  • J T F Postelmans (a1) and R J Stokroos (a1)

Abstract

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.

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Corresponding author

Address for correspondence: Dr Job T F Postelmans, Department of ENT/Head and Neck Surgery, P Debyelaan 25, PO Box 5800, 6202 AZ Maastricht, The Netherlands. Fax: (0031) 43 387 55 80 E-mail: J.Postelmans@student.unimaas.nl

Keywords

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

  • J T F Postelmans (a1) and R J Stokroos (a1)

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