Auditory Implantation in Neurofibromatosis Type 2: A Cambridge Experience

Objective: To report the objective benefit of cochlear implantation and auditory brainstem implantation in NF2 patients.

Study Design: Prospective case cohort.

Patients: Between 2000 and 2011, 23 patients underwent auditory implantation.

Results: In 20 cases, auditory brainstem implantation was performed after translabyrinthine resection of vestibular schwannoma. Nine of the patients are active users of the implant, four have sleepers in place, three are non-users, and one patient died of NF2 disease progression. In three patients, implant insertion was not possible due to anatomical considerations secondary to tumor load.

Of the nine users, six patients used their ABI as an aid to lipreading with an average improvement of 38% in audiovisual BKB when compared with lipreading alone. Five of these patients have audiovisual BKB scores of greater than 70%, averaging 85%. Of this subgroup, four are able to use the telephone with familiar voices. There are currently no data for three newly activated patients; however, they are active users with good auditory percept.

Three patients had cochlear implantation as a separate procedure, and all are active users, two with open set speech and 1 as an aid to lipreading.

Conclusion: ABI and CI implantation can provide profoundly deaf NF2 patients with good auditory rehabilitation.