Unilateral and asymmetric hearing loss in a Waardenburg Syndrome Type 2 (WS2) pedigree was reported to be caused by mutation in KIT or KITLG. KIT-KITLG signaling pathway and MITF were suggested to mutually interact in the migration process of melanocyte from the neural crest to stria vascularis (SV). This evidence concerns the gene KIT and Waardenburg syndrome type 2.