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Ear Defects

By

Simeon A. Boyadjiev Boyd

, MD, University of California, Davis

Last full review/revision May 2020| Content last modified May 2020

Ears can be missing, deformed, or incompletely developed at birth.

(See also Introduction to Birth Defects of the Face, Bones, Joints, and Muscles.)

Birth defects of the ear include the following:

  • Microtia, which is a small and deformed external ear (pinna)
  • External auditory canal atresia, 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
  • 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.

Examples of Birth Defects of the Ear

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.

Treatment

  • 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.

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