Congenital aural atresia

Abstract

Congenital aural atresia is viewed by many physicians as a poorly characterized, arcane, clinical entity associated with a variety of hearing deficits. In fact, congenital aural atresia represents a disease spectrum predicated on the reliable embryological development of the first and second branchial apparatus. All degrees of microtia, canal atresia, and middle ear structure malformation are identified by this disease process. Many classification schemata have been proffered since the turn of the century to assist the otologist with a better means of assessing the patient's suitability as a surgical candidate. Clinical, audiological, and radiographic evaluation of congenital aural atresia is essential in selecting the appropriate candidates for surgery or hearing amplification. The high resolution CT scan has advanced the understanding and preoperative assessment of this condition. Surgical repair of the external ear and middle ear malformations is effective in properly selected patients. Essential background information, relevant embryology, patient evaluation, treatment, and current controversies related to congenital aural atresia are discussed.