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]. The gene discussed is LZTR1; the disease is Vestibular schwannoma.