NF2 mutations may be tumor specific, as in unilateral, sporadic VS, or rare germline mutations leading to bilateral VS and NF2-related schwannomatosis, formerly known as neurofibromatosis type 2 (NF2) (U.S. incidence 1:10,000 versus 1:33,000, respectively) (2–4). The gene discussed is NF2; the disease is schwannomatosis.