2024-03-29T05:15:45Z
https://nagoya.repo.nii.ac.jp/oai
oai:nagoya.repo.nii.ac.jp:02001913
2023-01-16T04:42:13Z
499:500:501
Cochlear implantation in patient with Charcot-Marie-Tooth disease
Kobayashi, Masumi
Yoshida, Tadao
Sugimoto, Satofumi
Teranishi, Masaaki
Hara, Daisuke
Kimata, Yukari
Sone, Michihiko
open access
© 2021. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/
Charcot-Marie-Tooth disease
Auditory neuropathy
Cochlear implants
Two patients with auditory neuropathy spectrum disorder (ANSD) considered to be associated with Charcot-Marie-Tooth (CMT) are reported. In case 1, a 23-year-old man presented with progressive bilateral sensorineural hearing loss (SNHL) from 10 years of age and was diagnosed with ANSD. He was later diagnosed with CMT by neurological testing. In case 2, a 16-year-old girl, the younger sister of the patient in case 1, presented with progressive SNHL with similar auditory findings since 6 years of age as those of her brother. Both cases underwent bilateral cochlear implantation. In case 1, the maximum discrimination score improved to 45% 24 months after the first side of cochlear implantation from 5% before the surgery. In case 2, the score was 5% 10 months after cochlear implant (CI) surgery from 0% before the surgery. CI treatment for CMT patients has been considered difficult because of both failure in synchronization of nerve conduction due to demyelination and axonal failure of the auditory nerve. Though slower progress compared to the average subset of patients receiving CI was seen, significant improvement was gradually observed in the present patients after bilateral cochlear implantations. CI is thus a viable option for rehabilitation of SNHL in CMT patients.
Elsevier
2022-04-05
2021-04-05
eng
journal article
AM
http://hdl.handle.net/2237/0002001913
https://nagoya.repo.nii.ac.jp/records/2001913
https://doi.org/10.1016/j.anl.2020.03.003
0385-8146
Auris Nasus Larynx
48
2
327
330
https://nagoya.repo.nii.ac.jp/record/2001913/files/ANL-D-20-00071_R2.pdf
application/pdf
338 KB
2022-04-05