Sudden hearing loss in Waardenburg syndrome

Sudden profound hearing loss in a young man with Waardenburg syndrome type I is reported. The classical features of white forelock and dystopia canthorum were present. Audiometry and auditory brainstem responses confirmed the nature of the deafness. This is the first case of this rare syndrome with the unusual presentation of sudden hearing loss reported from this part of the Kingdom of Saudi Arabia. The medical literature about this syndrome with different patterns of hearing loss is reviewed