(See also Introduction to Birth Defects of the Face, Bones, Joints, and Muscles Introduction to Birth Defects of the Face, Bones, Joints, and Muscles Birth defects of the face and limbs are fairly common. They may involve only a specific body part, such as the mouth (cleft lip or cleft palate) or foot (clubfoot). Or they may be part of a... read more .)
Birth defects of the ear include the following:
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External auditory canal atresia Auditory canal atresia Developmental abnormalities are abnormalities that are present at birth or are identified soon after birth. Developmental abnormalities of the outer ear include microtia (also called small ear)... read more
, which is a partially or fully closed off ear canal -
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 Screening and Diagnosis Hearing loss in newborns most commonly results from cytomegalovirus infection or genetic defects and in older children results from ear infections or earwax. If children do not respond to sounds... read more 
to see whether hearing is affected and do imaging tests of the skull to look for problems with the bones.
