Radiographic features of the ear-related developmental anomalies in patients with mandibulofacial dysostosis.

Among the conditions that arise from disturbances in development of the first two branchial arches, the symmetric syndrome of mandibulofacial dysostosis and the asymmetric anomalies of the hemicraniofacial microsomia represent a characteristic pattern of craniofacial malformation distinct from other first and second arch syndromes. Since the usual embryonic aberration in both of these anomalies involves the first and second branchial arch derivatives, malformations of the external and middle ear are common. The external auditory canal is absent in the vast majority of the cases. Incudomallear deformities are usually present. In this paper, radiographic analysis of the ear-related developmental anomalies of 17 patients with mandibulofacial dysostosis is described.