Auditory and vestibular findings in Waardenburg's type II syndrome.

A group of 33 patients with the autosomal dominant form of Waardenburg Type II syndrome underwent hearing and vestibular examination. A bilateral, symmetrical, sensorineural hearing loss was found in 51 per cent of the tested subjects. The severity of hearing loss varied from mild to severe. Strong evidence indicated the possible progressive nature of the hearing loss. Concerning the frequency of vestibular findings, the study highlights the frequent divergence between cochlear and vestibular involvement.