Cochlear Implantation in Charcot-Marie-Tooth Disease: Case Report and Review of the Literature

Case Reports in Medicine
C Lane AnzaloneMatthew L Carlson

Abstract

Charcot-Marie-Tooth (CMT) disease is a peripheral hereditary neuropathy associated with motor and sensory impairment and can result in profound sensorineural hearing loss (SNHL). Currently, the role of cochlear implantation in the setting of CMT and other progressive peripheral neurodegenerative disorders is not well established. Case report and review of the English literature. A 70-year-old male with CMT was referred for evaluation of progressive asymmetric SNHL and reported a 15-year duration of deafness involving the left ear. Audiometric testing confirmed profound SNHL in the left ear, while the right ear exhibited moderate-to-severe SNHL. Left-sided cochlear implantation was performed using a conventional length lateral wall electrode. Intraoperative device testing found normal impedance levels throughout the array; however, electrically evoked auditory potentials were absent on all electrodes. Upon initial activation 3 weeks after surgery, the patient reported excellent access to sound in the cochlear implant-only condition. He has made good progress at each subsequent visit; speech perception testing after seven months showed improvement from 0% to 32% on AzBio sentence and 53% on CNC phoneme testing in the cochlear imp...Continue Reading

References

Dec 22, 1999·Neuroreport·F G ZengA Starr
Feb 13, 2003·Neurology·Nyamkhishig SambuughinKumaraswamy Sivakumar
Feb 16, 2012·Cochlear Implants International·Jay GoswamyMartin P O'Driscoll
Jun 6, 2012·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Matthew L CarlsonCharles W Beatty
Jun 13, 2015·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Mara C ModestColin L Driscoll

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Citations

Aug 9, 2019·Molecular Genetics & Genomic Medicine·Justine LeratAnne-Sophie Lia
Jul 25, 2019·International Journal of Molecular Sciences·Jonathan MorenaJ Chad Hoyle
Apr 9, 2020·Auris, Nasus, Larynx·Masumi KobayashiMichihiko Sone
Feb 25, 2021·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Shinsaku Matsuda, Kimitaka Kaga

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cochlear implants

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