Recently, Smith et al. (2016) demonstrated that 5 of 70 patients (7%) presenting with a unilateral vestibular schwannoma and at least two nonintradermal, nonvestibular schwannomas have germline LZTR1 mutations; hence, these individuals have schwannomatosis rather than NF2 even, although they would fulfil current diagnostic criteria for NF2. This evidence concerns the gene LZTR1 and schwannoma.