Cochlear Implantation Outcomes in Patients With OTOF Mutations.

ABSTRACT: Auditory neuropathy is a special type of hearing loss caused by dysfunction of the synapse of the inner hair cells, the auditory nerve, and/or the auditory nerve itself. For patients with auditory neuropathy who have severe to profound hearing loss or failed auditory skills development with hearing-aids, cochlear implantation (CI) serves as the only possible effective treatment. It is accepted that the exact sites of lesion causing auditory neuropathy determine the CI performance. Mutations in the OTOF gene were the first identified and the most common cause of congenital auditory neuropathy. The site of lesion in patients with auditory neuropathy caused by biallelic OTOF mutations (OTOF-related auditory neuropathy) is presumed to be presynaptic, leaving auditory nerve

SUBMITTER: Zheng D 

PROVIDER: S-EPMC7253664 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature