What is congenital atresia of external auditory canal?

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Congenital atresia of the external auditory canal is a kind of common malformation of the ear, which is manifested as hypoplasia of the external auditory canal, often accompanied by malformations of the auricle and middle ear, leading to conductive deafness. Some cases are accompanied by malformations of the internal ear and sensorineural deafness.

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Congenital atresia of the external auditory meatus with auricle deformity is more common than that of the simple atresia of the external auditory meatus. One ear is about four times more likely to develop the disease than both ears.

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Congenital atresia of the external auditory canal is a malformation caused by the abnormal development of the first and second branchial arches in the fetus. The cause of the disease has not been fully elucidated, genetic and environmental factors may play a role.

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Hefter and Ganz (1969) reported for the first time cases of familial atresia of the external auditory canal and conductive deafness, which showed autosomal dominant inheritance. At present, the pathogenic gene of the disease has not been located, which needs further study.

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Atresia of external auditory canal can be divided into four types

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Type I features: the external auditory canal is arcuate, the junction of cartilage and bone is narrow, often accompanied by auricle position abnormality or small ear disease; except for the inner 1 / 3, the other external auditory canal is absent, only bone plate, hammer and anvil deformity can be found in tympanic membrane, but the anvil often still has joint and stapes head.

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Type II showed complete absence of external auditory canal and fusion of malleus and incus. Fibrosis of long process of incus. In this case, the auricle is often accompanied with deformity and the tragus is often absent.

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Type III is obviously deformed, often accompanied by hypoplasia of mandible.

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Type Ⅳ is characterized by excessive curvature of the external auditory canal, malleus head deformity, fusion with the wall of the upper tympanum, obvious incus deformity, absence or deformity of stapes.

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The main clinical manifestation is hearing loss, which is mainly treated by operation. There is no fixed operation. We should follow the principle of tympanoplasty according to the anatomical variation, and take the opportunity during the operation.

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The general purpose is to reconstruct the transmission structure so as to improve the listening ability. Or after the otoplasty, install the hearing aid to enhance the hearing. The deformity of auricle can be treated by plastic surgery.

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The results of the whole operation are related to the degree of deformity of the ear, the way of operation, the experience of the operator and the complications after operation.

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