Neurofibromatosis 2: Primary Modality of Hearing Rehabilitation with Cochlear Implant

Auditory Brainstem Implants have been recommended as the gold standard in hearing rehabilitation of Neurofibromatosis Type 2 patients who lose hearing completely in both the ears and the cochlear nerves are not stimulable. Some patients have undergone cochlear implantation, in whom the cochlear nerve was spared during surgery or have undergone stereotactic radiotherapy preserving the function of the cochlear nerve. Here we report a case in whom we chose cochlear implantation prior to any definitive treatment for the tumour itself during the ‘wait and watch’ period. The reasons in favour of this approach have been discussed in this article. Post switch-on the implant is benefitting the patient satisfactorily and she is on regular follow up for monitoring the bilateral tumours.