For example, two PTPN11 variants of c.922A>G and c.836A>G from three probands were identified by EVIDENCE, none of whom (SH 271–631, SH 250–590, and SB308–611) showed abnormal facial features or skeletal malformations associated with Noonan syndrome, but demonstrated only severe SNHL. Here, PTPN11 is linked to Noonan syndrome.