Waardenburg-Shah syndrome (WSS) is a neurocristopathy characterized by the association of Waardenburg syndrome (sensorineural hearing loss and pigmentary abnormalities) and Hirschsprung disease. The disease is Monarch Disease Ontology identifier MONDO_0019518 (Waardenburg-Shah syndrome). Also known as: Shah-Waardenburg syndrome, WS4, Waardenburg syndrome type 4, Waardenburg syndrome type IV, Waardenburg-Hirschsprung syndrome.