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

Abstract

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.