Aural atresia is a congenital malformation in which the ear canal and the middle ear are underdeveloped or even absent. It can occur in one or both ears. It results in a conductive hearing loss. It is frequently associated with incomplete development or absence of the outer ear (auricle), a condition known as microtia.
Aural atresia (absence of ear canal, middle ear) and microtia (malformation of auricle) are part of a congenital syndrome involving variably incomplete development of the side of the face, called hemifacial microsomia. Aural atresia and microtia can be the only manifestation of hemifacial microsomia or present along with other parts of this syndrome, such as incomplete jaw development. Frequently, these patients are best evaluated by an experienced multi-discipline craniofacial team, which includes a plastic surgeon, otologist (ear surgeon), orthodontist, and nurse coordinator. Dr Hammerschlag is co-director of the Congenital Ear Development Anomalies Center at the Institute of Plastic and Reconstructive Surgery, New York University Medical Center, where aural atresia and microtia (www.microtia.com) are treated.
Auditory Testing is necessary to determine the extent of the condition’s impact on hearing for initial management for hearing loss, especially in the infant. Frequently amplification with hearing aids or bone conduction devices (BAHA), are utilized before aural atresia surgery is performed.
Once it is established that the hearing in the inner ear is intact by audiometric testing, and high resolution CT scan determines that the middle ear configuration is amenable to successful surgical reconstruction of the external ear canal and middle ear, surgical repair for aural atresia may be an option to restore hearing.
Auricular (microtia) reconstruction surgery can improve the visual appearance of the outer ear and surgical repair of the atresia or BAHA bone-anchored hearing aid restores hearing. While surgery can be performed earlier it is usually recommended that patients wait until between ages 8-10, when sufficient rib cartilage can be obtained to create an ear that is closer to its adult size. The aural atresia surgery follows the microtia repair to allow for a best cosmetic reconstruction. Please go to Aural Atresia Post Operative Information for more details.