2025 Volume 128 Issue 3 Pages 223-227
The patient was a 72-year-old man who received the diagnosis of Charcot-Marie-Tooth (CMT) disease at the age of 42 years. He developed right sensorineural hearing loss at the age of 55, which progressed gradually to bilateral severe hearing loss. At the age of 72, he underwent right cochlear implant surgery. The postoperative word recognition score (CI 2004) improved to 96%. Although there are conflicting reports about whether hearing loss in CMT disease arises from auditory neuropathy (AN), our patient could not be diagnosed as having AN due to the poor DPOAE response. There have only been seven reports of cochlear implantation performed in patients with CMT disease, including our case reported herein. The postoperative outcomes ranged from good to poor, but those with the characteristic finding of AN of a positive OAE, tended to have poorer outcomes.
