Refsum's Disease and Cochlear Implantation

Objectives: The objective was to describe a case of bilateral cochlear implantation in a 59-year-old man with hearing and visual impairment due to Refsum's disease. Method: A retrospective case review was performed. Results: After cochlear implantation, the patient demonstrated much improved audiometric performance and reported improved sound localization. Conclusions: Bilateral cochlear implantation is an important strategy in the improvement of hearing and quality of life in individuals with Refsum's disease.