A comparison of microtia and temporal bone anomalies in hemifacial microsomia and mandibulofacial dysostosis

Abstract

A number of entities can be categorized as otocraniofacial syndromes. New clinical and laboratory studies have demonstrated predictable patterns of occurrence, distinct anatomic interrelationships, separate genetic predispositions, and animal models of the varied embryogeneses. These investigations have allowed clinical separation of first and second branchial arch anomalies into syndromes of hemifacial microsomia and mandibulofacial dysostosis. The present study has established a relationship between the severity of the microtic auricle and middle ear malformation in those syndromes. Middle ear deformities, while present in both, are more severe when associated with mandibulofacial dysostosis.