Cochlear Implantation in Patients With Neurofibromatosis Type 2

OBJECTIVE: To describe cochlear implantation (CI) outcomes in patients with neurofibromatosis type 2 (NF2). STUDY DESIGN: Retrospective case series. SETTING: A multidisciplinary NF2 clinic at a university hospital. PATIENTS/INTERVENTIONS: Patients with NF2 who underwent CI. MAIN OUTCOME MEASURES: Pre- and postimplantation audiometric data, including pure-tone average (PTA) and AzBio Sentence scores. RESULTS: Eight patients with NF2 underwent CI. The mean age at implantation was 28.6 years (range: 17-63 yr) and six were female. The average length of deafness before CI was 3.5 years (range: 0.3-10 yr). Two patients underwent previous microsurgical resection via middle fossa craniotomy and one patient was treated with stereotactic radiotherapy before CI. Two tumors were growing at the time of CI, five tumors were not growing for an average period of 3.8 years (range: 1-6 yr), and one tumor had undergone previous gross total resection. Mean preoperative unaided PTA was 103.1 dB (range: 81.3-115 dB) and all preoperative AzBio scores were 0% in the ear to be implanted. These improved to a mean postoperative PTA of 30.9 dB (range: 12.5-43.8 dB) and mean postoperative AzBio score of 20% (range: 0-82%) with an average follow-up length of 16.6 months (range: 1.4-27.6 mo). Data logging demonstrated that six patients were daily CI users and two were nonusers, one of whom had normal hearing in the contralateral ear. CONCLUSIONS: CI is an effective option for rehabilitating hearing loss in patients with NF2; however, patients with normal contralateral hearing or poor follow-up do not perform as well.