Mutations within the INI1 gene that are linked to schwannomatosis, in contrast, are missense mutations and in-frame deletions affecting residues in the N-terminal portion of the protein (Bacci et al., 2010; Christiaans et al., 2011; Hadfield et al., 2008; Smith et al., 2012b, 2014). Here, SMARCB1 is linked to schwannomatosis.