INI1 is a tumour suppressor gene located on chromosome 22 centromeric to NF2. It is involved in the development of malignant rhabdoid tumours and germline INI1 mutations are present in some cases of familial schwannomatosis, a condition characterized by the development of multiple spinal, peripheral and cranial-nerve schwannomas in the absence of vestibular schwannomas [19]. This evidence concerns the gene SMARCB1 and Vestibular schwannoma.