This contrasts with a 50% likelihood of inheriting a disease associated variant in offspring of people with schwannomatosis and a pathogenic variant in LZTR1. Nonetheless, several cases of incomplete penetrance have also been observed for this gene even within families with confirmed cases [17, 18, 20, 24, 29–31], although the penetrance is not yet determined it may be in the region of 40–50%. Here, LZTR1 is linked to schwannomatosis.