Since the Myosin 15A (MYO15A) gene, located in the 17p11.2 SMS critical region, was implicated as a candidate gene for the hearing abnormalities of SMS [41], it is of interest to explore MYO15A expression in SMS patients as well as the role of RAI1 in MYO15A expression. Here, RAI1 is linked to Smith-Magenis syndrome.