Cochlear Implantation in Siblings With Refsum's Disease.

DISCUSSION: A new mutation causing Refsum's disease is presented. Cochlear implantation in case of severe hearing loss leads to an improvement in speech perception and should be recommended for patients with Refsum's disease, especially when the hearing loss is combined with a severe loss of vision. Decrease of speech perception in the long-term follow-up could indicate an additional retrocochlear degeneration.
PMID: 28681609 [PubMed - as supplied by publisher]

Source: The Annals of Otology, Rhinology, and Laryngology - July 1, 2017 Category: ENT & OMF Authors: Stähr K, Kuechler A, Gencik M, Arnolds J, Dendy M, Lang S, Arweiler-Harbeck D Tags: Ann Otol Rhinol Laryngol Source Type: research