Patient Evaluation

The diagnosis of aural atresia is most often made secondary to a deformed auricle (microtia). Microtia does not always accompany atresia, however, and unilateral cases without microtia may not be diagnosed until the child fails a school hearing test. The history should search for problems in the prenatal, including exposures (tobacco and alcohol included), infections, and overall maternal health; birth; and postnatal periods.

Speech and motor development and family history not just of ear problems, but other associated craniofacial disorders should be assessed as well. Physical examination should include otoscopy with attention to grading the caliber of the ear canal and searching for debris/canal cholesteatoma. Facial musculature and nerve function of all branches is critical to test and document. Tuning fork testing should be performed if the child is able to cooperate. An overall otolaryngologic examination should be performed to look for associated craniofacial or branchial arch anomalies, including cleft lip/palate, hemifacial microsomia, and mandibular hypoplasia.

We cannot overemphasize the importance of complete audiologic evaluation even in the child with unilateral atresia with or without microtia. The infant with bilateral or unilateral atresia/microtia should undergo brain stem auditory response testing of both ears early in infancy to evaluate sensorineural function. Bone conducting hearing aids should be placed on the child with bilateral atresia as early as possible. In addition to inner ear anomalies that are occasionally seen with atresia cases, there is a reported incidence of conductive hearing loss in the apparently normal contralateral ear of up to 27%, with a sensorineural loss in as many as 18%.4

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