Bilateral vestibular schwannoma (BVS) is the hallmark of neurofibromatosis type 2 (NF2), both of them being present at diagnosis. We report four cases of metachronous BVS, a contralateral intracanalicular vestibular schwannomas (VS) being visible 2 to 13 years after resection of a unilateral VS. NF2 workup was negative except in one case where two NF2 gene mutations were found in tumor analysis. These cases raise the questions of whether the contralateral VS occurred by chance and how to manage it on the only hearing ear. Otologists should be aware of this rare eventuality for decision making of the first unilateral VS. Laryngoscope, 131:E250-E254, 2021.
Keywords: Bilateral vestibular schwannoma; metachronous benign tumors; neurofibromatosis type 2; sporadic vestibular schwannoma.
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