Audiologic manifestations of Noonan syndrome.

A comprehensive audiologic study of a family with Noonan syndrome is reported together with a review of 20 cases of this syndrome with regard to hearing sensitivity and middle ear status. An incidence of progressive sensorineural hearing loss at the high frequencies is found for 50% of the ears. It is emphasized that early audiologic management may improve the quality of life for patients with Noonan syndrome.