Abstract
DiGeorge syndrome is characterized by partial or complete absence of the thymus gland which 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 syndrome are presented. Patients with this syndrome have a high incidence of Mondini dysplasia in both ears, sometimes with other anomalies of the external or middle ears. The hearing may range from normal to profound deafness and may manifest sensorineural, conductive or mixed losses of varying degree.
