Wen and co-workers (2021) have recently reported the establishment of hiPSC lines from WS patients who carry mutations in genes that have been associated with the disease; thus, hiPSC lines are now available that carry mutations within the Paired Box Gene 3 (PAX3) gene [104,105] and the SRY-Box Transcription Factor 10 (SOX10) gene [69] that play key roles during the development and differentiation of NC cells and the formation of the stria vascularis in the cochlea. The gene discussed is SOX10; the disease is Werner syndrome.