%0 Journal Article
%T Cochlear Implantation in Neurofibromatosis Type 2-Related Schwannomatosis: Long-Term Hearing Outcomes.
%A Grenier B
%A Mosnier I
%A Ferrary E
%A Nguyen Y
%A Sterkers O
%A Kalamarides M
%A Lahlou G
%A Daoudi H
%J Otolaryngol Head Neck Surg
%V 171
%N 1
%D 2024 Jul 14
%M 38482961
%F 5.591
%R 10.1002/ohn.702
%X <B>OBJECTIVE: </B>To evaluate long-term hearing outcomes following cochlear implantation in patients with neurofibromatosis type 2 and ipsilateral vestibular schwannoma.<BR><B>METHODS: </B>Retrospective study.<BR><B>METHODS: </B>Tertiary general hospital.<BR><B>METHODS: </B>Twenty-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.<BR><B>RESULTS: </B>One 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.<BR><B>CONCLUSIONS: </B>Neurofibromatosis 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.