GOLDENHARS SYNDROME ASSOCIATED WITH ANOMALOUS INTERNAL AUDITORY MEATUS

In 1952 Goldenhar first described an infant who had an epibulbar dermoid, auricular appendages, and an auricular fistula. He also mentioned three other cases with epibulbar dermoid and auricular appendages with or without the aural fistula. A number of anomalies including auricular deformities, atresia of the external auditory meatus, and inner ear defects have been associated with this syndrome. Gorlin and Sugar emphasized the frequent association of cervical spine abnormalities and they proposed the synonymous name of ocula-auriculo-vertebral dysplasia in place of Goldenhar's syndrome. Despite the fact that Goldenhar's syndrome is associated with these anomalies of the ear, the otoneurological details of this syndrome have not hitherto been reported. In this report, we present the unusual association of an abnormality of the internal auditory meatus in a patient with Goldenhar's syndrome.