The Waardenburg syndromes (WS) are rare genetic disorders described classically as neural crest syndromes and characterized by deafness and pigmentation abnormalities. WS type IV (WS4) is attributed to mutations in the SOX10 gene, which regulates pigment cell development. Here, Kirsten Dutton and colleagues study otic vesicle development using a zebrafish model of WS4, the colourless/sox10 mutant. WS4 patients and mutant fish display a similar spectrum of abnormalities in the ear, pigment cells and enteric neurons. Additionally, the researchers found that only a few neural crest cells contribute to the developing zebrafish ear, and therefore are unlikely to be wholly responsible for the resulting auditory deficits. Their work helps explain the origin of inner ear deficits that lead to hearing loss and vestibular problems in WS4 patients.
