Two Cases of Goldenhar's Syndrome Associated with the Narrowing of Internal Auditory Canal

Abstract

Goldenhar's syndrome is an association of symptoms such as epibulbar dermoids, preauricular appendages or pretragal fistulas, and mandibulo-facial dystosis. The disting uishing feature, therefore, is the underdevelopment of one half of the face. Sensorinerual hearing loss and conductive hearing loss is associated with this syndrome. Facial paralysis has been reported to occur in patients with Goldenhar's syndrome. It was suggested that a pathologically narrow internal auditory canal may indicate a failure of facial nerve development and that hypoplasia of facial nerve may result in facial paralysis. Furthermore, hypoplasia of acoustic nerve may result in sensorinerual hearing loss. In this report, we present two patients with Goldenhar's syndrome, and suggest that the defects in acoustic and facial nerve function might be closely related to the narrowing of the internal auditory canal.