Ipsilateral cochlear implantation after cochlear nerve preserving vestibular schwannoma surgery in patients with neurofibromatosis type 2

OBJECTIVE: To investigate the outcomes from ipsilateral simultaneous or sequential cochlear implantation in patients with neurofibromatosis type 2 (NF2) after vestibular schwannoma removal with cochlear nerve preservation. STUDY DESIGN: Retrospective case series. SETTING: Single tertiary referral NF2 center. PATIENTS: Six patients with NF2. INTERVENTION: Removal of vestibular schwannoma (VS) with preservation of the cochlear nerve and cochlear implantation. Four patients had their surgery via a translabyrinthine approach. Two patients had a retrosigmoid approach. A cochlear implant was inserted at the same time as tumor removal in 4 cases and sequentially in 2 cases. MAIN OUTCOME MEASURES: Surgical and audiometric outcomes using Bamford-Kowal-Bench (BKB) and City of New York University (CUNY) sentence scores. RESULTS: The average age at implantation was 24 years (range, 15-36 yr). Follow-up ranged from 5 to 93 months, with an average of 38 months. All patients had useful hearing in the contralateral ear before surgery. One patient gained no benefit from cochlear implantation and proceeded to have an auditory brainstem implant. Of those that had functional cochlear nerves, the average BKB score in quiet was 64%, BKB score in noise was 42%, and CUNY score with lipreading was 97%. Results varied within the group, but all patients gained significant benefit and continue to use their CI at least intermittantly. CONCLUSION: The present series demonstrates that in selected cases, cochlear implantation can be successful after a translabyrinthine approach for VS removal and for restoring hearing after failed retrosigmoid hearing preservation surgery. All patients found the cochlear implant offered useful hearing even in the presence of contralateral hearing.