Congenital absence of the ear creates one of our most challenging reconstructive problems. A loss due to avulsion, laceration, or burn leaves at least the external auditory meatus and usually some or all of the concha. Congenital absence of the ear in its full-blown form presents a complete lack of the external structure, except for a small, formless tab, in addition to an absent ear canal usually associated with hypoplasia of the underlying middle ear. All of these abnormalities stem from maldevelopment of the first branchial pouch and the first ectodermal groove. The inner ear, having a separate point of origin dorsal to the second branchial cleft, is rarely affected; hence bone conduction remains unimpaired. When one ear alone is involved, the hearing problem is relatively unimportant and a reconstruction may be done without any attempt at restoring air conduction. Occasionally, however, one encounters atresia of both external auditory canals.
TANZER RC. Total Reconstruction of the External Auricle. Arch Otolaryngol. 1961;73(1):64–68. doi:10.1001/archotol.1961.00740020068008
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