Case of Charcot-Marie-Tooth Disease and Audiometric Decline

Cochlear Implantation in Charcot-Marie-Tooth Disease

Patient with CMT-associated deafness successfully treated with cochlear implant.

This article describes the case of a 70-year-old male patient with Charcot-Marie-Tooth disease. She was referred to the hospital for asymmetric sensorineural hearing loss. And deafness in the left year with a history of 15 years. Initially the patient was using a bilateral hearing aid, however, because of progressive audiometric decline, he stopped using the hearing aid. Doctors performed an audiometric testing which confirmed the presence of moderate-to-severe sensorineural hearing loss in the left ear.

Charcot-Marie-Tooth Disease (CMT)

CMT is a group of disorders, clinically and genetically heterogenous that affect the peripheral nervous system. The disease presents with profound sensorineural hearing loss because of a disruption of synchronous activity of cochlear nerve.

Doctors advised a left-sided cochlear implant using conventional length lateral wall electrode. The electrodes were fully inserted and introduced without any resistance. According to an intra-operative device testing, the impedance level was normal throughout the array. However, auditory potentials evoked electrically were absent on all electrodes. Doctors activated the cochlear implant 3 weeks after surgery and the patient reported having excellent access to sound. The patient showed good progress at each follow-up visit. In addition, his speech perception showed improvement from 0% to 32% after seven months. Similarly, the patient reported subjective benefit and consistent usage of the device at his 18-month follow-up.


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

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Dr. Aiman Shahab is a dentist with a bachelor’s degree from Dow University of Health Sciences. She is an experienced freelance writer with a demonstrated history of working in the health industry. Skilled in general dentistry, she is currently working as an associate dentist at a private dental clinic in Karachi, freelance content writer and as a part time science instructor with Little Medical School. She has also been an ambassador for PDC in the past from the year 2016 – 2018, and her responsibilities included acting as a representative and volunteer for PDC with an intention to make the dental community of Pakistan more connected and to work for benefiting the underprivileged. When she’s not working, you’ll either find her reading or aimlessly walking around for the sake of exploring. Her future plans include getting a master’s degree in maxillofacial and oral surgery, settled in a metropolitan city of North America.


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