Temporal Bone Pathology in Digeorge's Syndrome

Abstract

DiGeorge's syndrome is characterized by partial or complete absence of the parathyroid and thymus glands and is often associated with other developmental anomalies, particularly of the structures arising from the third and fourth pharyngeal pouches. The temporal bone findings in three cases of DiGeorge's syndrome are presented. Patients with this condition have a high incidence of Mondini dysplasia in both ears, sometimes with other anomalies of the external or middle ears. Hearing may range from normal to profound deafness and may manifest sensorineural, conductive, or mixed losses of varying degrees.