Autosomal dominant disorder with deafness and pigmentary disturbances probably as a result of defects in function of neural crest. Various forms of the syndrome are recognized. Waardenburg Syndrome 1 (WS1) and WS3 (also known as Klein-Waardenburg syndrome) are caused by mutation in Pax3 - an homologous defect to the mouse mutant Splotch that also has defective Pax -3. Waardenburg-Shah syndrome (WS4), in which Waardenburg&`s syndrome is associated with Hirschsprung&`s disease, is due to mutation in Sox10 and there is an homologous mutation in Dom mice (dominant megacolon), piebald-lethal and lethal spotting. WS2 is heterogeneous with mutation in the microphthalmia ( MITF ) gene.
Genetic defect found in white ferrets, blazes and pandas resulting in deafnessFound on http://baltoferretclub.com/glossary.html
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