In addition to inactivation of the neurofibromatosis type II (NF2) gene in schwannomas, other genes are involved in their pathogenesis, such as leucine zipper-like post-translational regulator 1 (LZTR1), the SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1), and coenzyme Q6 monooxygenase (COQ6). Here, SMARCB1 is linked to schwannoma.