Vestibular schwannomas are known to occur within the context of tumour predisposition syndromes, neurofibromatosis type 2 (NF2)-related and non-NF2-related schwannomatosis.7–9 However, the majority of VSs occur sporadically in otherwise healthy individuals.2 No environmental factors have been robustly linked to risk of VS development, except for exposure to cranial radiotherapy,10 which occurs extremely infrequently in the general population. Here, NF2 is linked to schwannomatosis.