The nerve of origin is most commonly sensory, with vestibular schwannoma, or acoustic neuroma, the most common tumour site.1 Schwannoma is associated with neurofibromatosis type 2 (NF2), an autosomal dominant syndrome caused by mutation in the NF2 suppressor gene located at chromosome 22q11.2, in 2–18% of cases.2, –4 The NF2 gene encodes merlin protein, regulating cell growth, particularly in Schwann cells, and cell-cell adhesion.5 This evidence concerns the gene NF2 and Down syndrome.