Congenital Atresia, the nonexistence of the external ear canal, is a birth defect which is almost always accompanied by irregularity of both the middle ear bones in a variety of degrees, as well as the external ear. When it happen in both the newborn’s ears, the pediatrician must readily refer the child to both a facial plastic surgeon and an ear surgeon.Narrowing of the ear canal is called aural atresia. It can range from a slight narrowing to whole absence of the external ear canal. When this happen at birth, we use the term congenital atresia. There may also be changes in the shape of outer ear or pinna (termed microtia) as well as malformations of the middle ear structures, including problems with the three bones of hearing. Rarely, there may also be abnormalities of the inner ear structures. Aural atresia occurs in 1 in 10,000 to 20,000 live births, with atresia of one side being three times more common than atresia of both sides.
Atresia of the ear canal needed evaluation by a multidisciplinary team including an otolaryngologist, audiologist, and radiologist. An absent ear canal leads to a conductive hearing loss even if the inner ear and hearing nerve are normal. In addition to hearing loss, a narrow canal can predispose to infections, or in more severe cases, to cysts or entrapment of skin of the canal and middle ear called cholesteatomas.
In some situations, the ear canal can be surgically reconstructed to improve hearing. Alternatively, bone conduction hearing aids maybe an option. Surgery to reconstruct the ear canal (termed atresiaplasty) is typically recommended at 5-6 years of age for patients with atresia of one or both sides. However, in some situations, surgery may be performed prior to this age. The decision to repair the external part of the ear (microtia repair) and the type of the repair used (rib graft technique versus medpor implant) impacts the timing of atresiaplasty. In order to maintain initial surgical results and to avoid late complications following atresiaplasty, such as narrowing of the ear canal, the patient needs diligent postoperative office care.
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