Birth defects of the ear include the following:
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Microtia, which is a small and deformed external ear (pinna)
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External auditory canal atresia, which is a partially or fully closed off ear canal
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Low-set ears, which are located below where ears are typically positioned on the head
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Ear pits and ear tags, which are minor defects typically located in front of the ear
Microtia and external auditory canal atresia often occur together and are often identified at or soon after birth.
Low-set ears may occur with a number of genetic syndromes, and children often have developmental delays. Ears are considered low set if the top of the ear is below the outer corners of the eyes.
Ear pits and ear tags may be signs that children have other problems, so doctors often test them for hearing loss and for other birth defects.
Doctors can identify many ear defects during a physical examination. Doctors also do hearing tests to see whether hearing is affected and do imaging tests of the skull to look for problems with the bones.