Aural Atresia and Microtia

What is aural atresia and microtia?

Severe deformity of the external ear (microtia) with absence of an ear canal and tympanic membrane (aural atresia) occurs in 1 in 10,000 to 20,000 births. Aural atresia/microtia is usually an isolated condition, but can be associated with a number of other craniofacial anomalies such as Treacher Collins syndrome or hemifacial microsomia. Although auditory nerve function is typically normal, there is a severe loss of hearing secondary to the absence of a sound-conduction pathway to the cochlea. In addition to the hearing loss, the psychological implications of a severe cosmetic ear deformity in a young child can be significant.

Pediatric patients with microtia and/or atresia are typically seen by otolaryngologists within the first few weeks of life. Proper use of hearing aids in a timely fashion is especially important when bilateral atresia or underlying sensorineural hearing loss is present. Surgical treatment of microtia/atresia is typically begun between five and seven years of age.