Congenital atresia of the external auditory canal.

The accompanying otoscopic view is that of stenosis of the external auditory canal on the left. The opening is one-tenth the normal size, and it ends in a blunted, skin-covered, bony obstruction 5 mm from the lateral surface. There is no tympanic membrane, and the bony external auditory canal is absent. Anomalies of this type can be unilateral or bilateral. They can be associated with microtia of varying degrees; in other cases, the pinna can otherwise be completely normal.

If confronted with such a case, the otologist must consider three factors. First, the otologist must exclude squamous epithelium lining a portion of the external auditory canal medial to the obstruction. When such a pouch is present, it is identified by thin-section polytomography. Timely surgery is mandatory. Second, hearing must be provided promptly by surgery or amplification, so that the individual can learn speech and become educated. Third, the cosmetic appearance should be improved by constructing a pinna by plastic surgery or by use of a prosthesis.

Because constructing a new external auditory canal and tympanic membrane requires special skill, this surgery is usually performed by only a small number of otologic surgeons who have a special interest in this condition. Surgery to reconstruct the ear canal and facilitate hearing is recommended for both bilateral and unilateral cases. Reasonable success for the attainment of near-normal hearing can be expected. Patients and their families are usually given the choice of having an auricle made by plastic surgical techniques from rib cartilage or having an appliance anchored to the side of the skull, to which a prosthetic ear is attached.