Cochlear Implantation in Patients With Neurofibromatosis Type 2

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
Anthony M TolisanoJoe Walter Kutz

Abstract

To describe cochlear implantation (CI) outcomes in patients with neurofibromatosis type 2 (NF2). Retrospective case series. A multidisciplinary NF2 clinic at a university hospital. Patients with NF2 who underwent CI. Pre- and postimplantation audiometric data, including pure-tone average (PTA) and AzBio Sentence scores. 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...Continue Reading

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