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Anotia

What Is Anotia?

Anotia when a baby is born with the outer part of their ear missing.

Children with anotia (ann-OH-shee-uh) have hearing loss. In many cases, surgery can rebuild the ear and help with hearing loss.

What Are the Signs & Symptoms of Anotia?

A baby with anotia is born without an ear. It usually happens only on one side. 

Anotia affects hearing. Sound can’t get through to the inside part of the ear, and some parts of the inner ear may not have formed normally.

What Causes Anotia?

Doctors don’t know the exact cause of anotia in most cases. Some types of gene mutations (changes) are associated with with anotia.

Anotia happens more often in babies whose moms:

  • have diabetes
  • took certain medicines (including isotretinoin, brand name Accutane®) or drank alcohol during pregnancy
  • had a diet low in folic acid and carbohydrates during pregnancy

Some babies with anotia also may have a genetic condition (such as Treacher Collins syndrome) or problems with the way their face, heart, kidneys, or limbs are formed.

How Is Anotia Diagnosed?

Doctors will see anotia as soon as the baby is born. Sometimes they find the condition before birth on a prenatal ultrasound. After the baby is born, the doctor will do an exam to see if there are other problems, and will do hearing tests and genetic tests.

They also might order imaging studies, such as a CT scan, and other tests as needed.

How Is Anotia Treated?

Babies with anotia need a care team to help them get the best treatment. The team includes:

  • a pediatric plastic surgeon to rebuild the ear
  • an otolaryngologist (ear, nose, and throat doctor) to treat hearing loss
  • a speech therapist to help with speech development
  • a geneticist to help families understand how anotia can run in families

Plastic surgeons can do two types of procedures for anotia. They can:

  • Use cartilage from the child’s ribs as a framework to form the ear. They put tissue from under the child’s scalp and/or a skin graft over the cartilage to rebuild the ear. This type of surgery is usually done when a child is 6–10 years old. The repair is done in stages, so several surgeries are needed.

or

  • Use a plastic implant as a framework to form the ear. They put tissue from under the child’s scalp and/or a skin graft over the implant to rebuild the ear. This type of surgery can be done when a child is around 3 years old. The repair sometimes can be done in one surgery, but two might be needed.

If the inside of a child's ear did not develop normally, an otolaryngologist might do more surgery.

What Else Should I Know?

If your child has anotia, treatments can help. Talk to the doctor about which are best for your child. If your child has other medical problems, follow the doctor’s instructions for care.

As kids get older, they might worry about their condition or the way their ear looks. Talking to a counselor can help.

You also can find more information and resources online at:

Reviewed by: Amy W. Anzilotti, MD
Date Reviewed: Oct 1, 2022

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