What is Congenital Aural Atresia?

In rare cases, children are born without a well-formed external ear (microtia) and/or ear canal (aural atresia). These children have very poor hearing due to lack of the ear canal, ear drum, and malformation of the hearing bones. Their inner ear and hearing nerve are usually normal. In cases of unilateral (single sided) atresia, with normal hearing on the other side, no specific early treatment is needed. If there is bilateral atresia with bilateral hearing loss, the affected child will be at significant risk for delay in speech and language development. Such children should be fitted with a bone conduction hearing aid as soon as possible.

Congenital aural atresia repair

Congenital aural atresia repair is an outpatient surgery that takes 3 to 4 hours.  It is performed under general anesthesia.  The surgery is performed through an incision behind the ear.

How the Surgery Works

A new opening is created for the ear canal.  This new canal is lined with a skin graft taken from the inner portion of the upper arm.  A new eardrum is created using the patient’s own tissue, called fascia.  Although the hearing bones (ossicles) are usually malformed, they are typically functional.  In rare cases, the hearing bones are not functional and need to be reconstructed at the same time.

After Surgery Care

Antibiotic eardrops are used in the ear after surgery.  Regular activities are started the next day.  Patients are typically seen 2 weeks after surgery and then periodically over the next 2 to 3 months to ensure proper healing.  A hearing test is obtained 2 to 3 months after surgery.  These reconstructed ear canals require cleaning in the office about 1 to 2 times per year after the healing is complete.

By Joseph Chang, MD