Cochlear Implantation in Patients With Neurofibromatosis Type 2

Abstract

Cochlear implantation is an attractive alternative to auditory brainstem implantation for hearing rehabilitation in patients with NF2. Approximately 70% of patients achieve open-set speech discrimination, many scoring at the ceiling of audiometric testing. Given a favorable risk profile and superior audiometric outcomes, CI should be strongly considered in patients with nonserviceable hearing who have an anatomically intact cochlear nerve, whereas auditory brainstem implantation should be reserved for patients with evidence of cochlear nerve loss. Akin to conventional cochlear implant recipients, prolonged hearing loss, unfavorable electrophysiological testing, and cochlear ossification may predict poor subject performance. Finally, useful hearing in the contralateral ear may present a barrier to daily device use.