Three genes centromeric to NF2 on 22q have now been found shown to account for ~ 70% of familial and ~ 30% of sporadic patients with multiple non-vestibular schwannomas: SMARCB1 [53], LZTR1 [22], and much less commonly DGCR8 [54]. This evidence concerns the gene SMARCB1 and Vestibular schwannoma.