Neurofibromatosis type 2 is caused by mutation of neurofibromin 2 (NF2) gene mutation, a tumor suppressor gene with inhibitory roles in cell proliferation while the inherited loss of NF2 is not the cause of schwannomatosis (Kehrer-Sawatzki et al., 2017; MacCollin et al., 2003). This evidence concerns the gene NF2 and NF2-related schwannomatosis.