Cochlear Implantation in Neurofibromatosis Type 2-Related Schwannomatosis: Long-Term Hearing Outcomes

ObjectiveTo evaluate long-term hearing outcomes following cochlear implantation in patients with neurofibromatosis type 2 and ipsilateral vestibular schwannoma. Study DesignRetrospective study. SettingTertiary general hospital. MethodsTwenty-two patients undergoing cochlear implantation between 2004 and 2018 with at least 1 year of follow-up were included. Patients were categorized as "users" or "nonusers" of their cochlear implant (CI). For users, speech perception (disyllabic words) without lip-reading was assessed in quiet conditions 1-year postimplantation, and annually thereafter. CI users were classified into 2 groups on the basis of speech intelligibility (>= 40% or <40%). Demographic data, treatment options, and tumor size were also recorded. ResultsOne year after implantation, 16 (73%) patients used their CI daily. Twelve of these patients had a speech intelligibility >= 40% (mean: 74 +/- 21.9%). Three had a Koos stage IV tumor. At the last visit (mean duration of follow-up: 6 +/- 5 years), 12 of these 16 patients were still using their implant daily, and 6 had a speech intelligibility >= 40%. No predictive factors for good performance at 1 year or performance stability were identified. ConclusionNeurofibromatosis type 2 is a complex disease profoundly affecting patient quality of life, and cochlear implantation should always be considered on a case-by-case basis. In some individuals, cochlear implantation can provide good speech intelligibility for extended periods, even posttreatment or in cases of large tumors.