Efficacy of Cochlear Implantation in Neurofibromatosis Type 2 Related Hearing Loss

Abstract

Objective:

To investigate the results of cochlear implantation in subjects with neurofibromatosis type 2 (NF2) and bilateral vestibular schwannomas (VS).

Study Design:

Retrospective case series.

Setting:

University-based tertiary referral center.

Subjects:

Five subjects with NF2 and severe-to-profound sensorineural hearing loss.

Intervention:

Cochlear implantation.

Main Outcome Measure:

Surgical outcomes and audiometric performance after cochlear implantation.

Results:

Five subjects (3 female, 2 male) were included in the study. The mean age at the time of implantation was 54 years old (range 35-78 years). Follow-up after cochlear implantation averaged 38 months (range 21-106 months). In the 5 implanted ears, 2 had no prior treatment, 1 had undergone prior radiation therapy, 1 underwent prior microsurgical removal, and 1 underwent prior microsurgical removal with adjuvant radiation therapy. The mean ipsilateral VS dimensions at time of implantation were 14 mm × 7.2 mm × 6.1 mm (mediolateral × anteroposterior × craniocaudal). Following cochlear implant activation, all 5 subjects achieved sound awareness, open set speech recognition, and 4 continue to be daily users of the devices.

Conclusion:

Cochlear implantation is a viable hearing rehabilitation option for subjects with NF2 and severe-to-profound sensorineural hearing loss. All subjects reported benefit with their cochlear implant, including open set speech recognition, enhanced lip-reading skills and environmental awareness of sound. Four subjects continued to demonstrate improved open-set speech recognition at the time of their last evaluations.