Patient Two was heterozygous for a LZTR1 missense variant (c.1210G>A; p. Gly404Arg) that may create or strengthen a splice site; this variant has previously been reported in two patients with schwannomatosis from the United States, including a 70-year old male who presented with a right vestibular schwannoma at age 34, two thoracic spinal schwannomas removed at age 43, and multiple cranial nerve schwannomas since age 55 [21]; he fulfilled Manchester criteria for clinical diagnosis of NF2 but was found instead to carry an LZTR1 variant. This evidence concerns the gene NF2 and schwannomatosis.