Abstract
Intravestibular schwannomas (IVS) are uncommon tumors in Neurofibromatosis type 2 (NF2) and are mainly associated with multiple internal auditory meatus (IAM) and cerebellopontine angle (CPA) tumors. They usually induce profound hearing loss which can be rehabilitated by cochlear implantation (CI). The aim of this study was to analyze the long-term outcomes of CI during the unpredictable evolution of NF2 disease. Retrospective case review. Tertiary referral center. Three adults with neurofibromatosis type 2 and intravestibular schwannomas, and who were cochlear implant recipients. Periodic radiologic follow-up, tumor resection, and hearing rehabilitation. Audiological evolution, tumor evolution, surgical outcome, cochlear implant outcome. Three NF2 patients (mean age at diagnosis, 26.3 ± 3.2 yr) were identified with IVS in the period between 2000 and 2017. IVS were first observed by serial MRI and profound hearing loss occurred in this ear after 4 ± 1.5 years of follow-up. IVS were removed via a translabyrinthine approach, and ipsilateral cochlear implantations were simultaneously performed. In two patients, large contralateral CPA tumors had previously been removed without hearing preservation, whereas in the third patient, a small, growing contralateral VS was excised via a retrosigmoid approach 6 months after IVS removal/cochlear implantation with serviceable hearing preservation. In all cases, CI provided good hearing outcomes. In two cases, hearing outcomes were even better for more than 5 years when ipsilateral intracanalicular vestibular schwannomas were removed in either the same or subsequent procedures. Rehabilitation of hearing with CI provides a favorable long-term outcome in patients with NF2-related IVS which could be altered by the occurrence of other intracanalicular and/or CPA NF2-related tumors.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.