External auditory canal atresia (EACA) can occur bilaterally as well as unilaterally. Usually there is an accompanying auricular deformity. Surgical intervention in bilateral cases is mandatory to promote normal speech and language development. Unilateral EACA cases often do not receive surgical intervention until the patient is 18 years of age. Preoperative evaluation of the EACA patient requires behavioral audiometry, auditory brain stem evoked response, and a computerized tomographic scan.