(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:
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 canalLow-set ears, which are located below where ears are typically positioned on the head
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.
This photo shows a small and deformed external ear (microtia).
© Springer Science+Business Media
This photo shows a child who has low-set ears and other facial features of a disorder called r(18) syndrome.
© Springer Science+Business Media
This photo shows a child with two ear pits, which are birth defects. The black arrow points to an ear pit that appears in a spot where ear pits more commonly occur. The white arrow points to an ear pit that appears in a spot where ear pits less commonly occur.
© Springer Science+Business Media
This photo shows an ear tag (arrow), which is a birth defect.
© Springer Science+Business Media
Doctors can identify many ear defects during a physical examination. Doctors also do hearing tests Screening and Diagnosis Hearing impairment refers to any degree of hearing loss, mild to severe, and can occur when there is a problem with a part of the ear, including the inner, middle, and outer ears, or the... read more 
to see whether hearing is affected and do imaging tests of the skull to look for problems with the bones.
Treatment of Ear Defects
Surgery
Hearing aid
Treatment of ear defects may involve reconstructive surgery to create a normal-looking external ear and to create an external ear canal.
Sometimes, a hearing aid is necessary.
