Congenital Ear Conditions We Treat at UPMC Children's Hospital of Pittsburgh

Parents whose children are born with ear abnormalities often face many unknowns.

For serious ear defects, parents worry about how it will affect their child's hearing and speech.

Since ear abnormalities can be part of various disorders, parents may have concerns about other health problems as well.

At the Congenital Ear Center (CEC), we give you answers quickly and offer advanced treatments and supports.

We often follow children from newborns through their teens and into and young adulthood.

Contact the Congenital Ear Center at UPMC Children's

To make your child's appointment, call the CEC at 412-692-5460.


This condition occurs when the ear doesn't form, either fully or at all, during the first trimester of pregnancy. It can affect both ears but mostly affects only one.

One in about 2,000 to 10,000 children is born each year in the U.S. with microtia.

Microtia can range from mild (a smaller ear but normal ear canal) to severe (a missing ear and ear canal). In most cases, the ear is small and misshapen and the child also is missing the ear canal.

Microtia is most often due to random gene changes. More rarely, certain medicinediets, and exposure to environmental toxins during pregnancy may lead to microtia.

It's often the only congenital health problem a child has.

But in 20 to 40% of children, microtia occurs with other issues. They're more likely to have facial abnormalities, kidney and heart problems, and other health problems.

At the CEC, our surgeons use advanced techniques to reconstruct ears based on each child's unique build.

We suggest surgery once the child is 7 or older.

Aural Atresia

In this condition, the ear canal (opening) is absent at birth. The eardrum and middle ear bones may also be missing or malformed.

More rarely, aural atresia starts in childhood due to a severe infection that causes the canal to close.

Children with aural atresia have moderate to severe hearing loss in the affected ear. Most also have microtia.

We treat hearing loss caused by aural atresia with a hearing aid that sends sound vibrations to the inner ear. This lets children hone their hearing skills in the years before they're old enough for surgery.

With surgery, we make an ear canal and reconstruct the middle ear, as needed.

Hearing aids and surgery give kids with aural atresia near-normal hearing.

External Auditory Canal Stenosis

Stenosis, or narrowing of the ear canal, is most often present at birth.

In some cases, the narrowing can happen later in life due to repeated infections and scarring.

The condition usually causes some degree of hearing loss, which is treatable with hearing aids and surgery.

Our skilled surgeons at UPMC Children's Hospital expand the opening of the ear canal and repair any damage to the middle ear.

Other Congenital Ear Problems

We assess and treat a wide range of congenital ear abnormalities, including:

  • Lop ear, where the top of the ear folds over.
  • Stahl's ear, a pointed ear.
  • Prominent ears, where the ear sticks out.
  • Earmolds can correct lop ear.

Plastic surgery is an option for other ear abnormalities. It's not necessary for hearing but can help with a child's self-esteem, especially as they get older.

Your child's surgeon will explain the surgery in detail, including when to plan for it and what the ear will look like post-op.