What is Microtia?

Microtia is a birth defect of a baby’s ear:

• Occurs when the outer ear or ears don’t fully develop during pregnancy.
• The outer ear may be smaller than normal, a partial ear, or no ear present.
• Affects only one ear in most cases; rarely are both affected.
• Occurs most often in boys.
• More common in people of Asian and Hispanic descent.
• Rarely occurs with other abnormalities, such as cleft lip or palate, or jaw and facial abnormalities.
• Sometimes seen in children with Treacher Collins syndrome.

Microtia involves two main issues:

1. The Outer Ear Deformity
• In most cases, one ear is visibly smaller than the other.
• No outer ear at all, called anotia, is very rare.
• Children may become self-conscious about their ear as they grow, even if it’s a mild deformity.
2. Hearing Loss
• Child almost always has some hearing loss.
• Degree of hearing loss can range from mild to severe.
• Child’s hearing can be affected even if parts of the middle ear appear normal.

Causes of Microtia

• The parts of the outer ear that are affected form during the first trimester of pregnancy.
• Ear malformations may be due to altered development during this time.
• In nearly all cases, the mother could not have done anything to prevent microtia.
• In most cases, the cause of microtia is not known.

Scientists have found some specific causes of microtia. These include:

• Rubella infection during pregnancy.
• Abnormal vitamin A levels during pregnancy.
• Use of the acne medication Accutane® (isotretinoin) during pregnancy.
• Some hereditary conditions.

Microtia Signs and Symptoms

• A smaller-than-normal ear, partial ear, or no ear.
• Seen after birth during a physical exam.
• Not usually found during a prenatal ultrasound.

Microtia Diagnosis

• Occurs during a physical exam after a baby is born.
• A doctor will look for other birth defects that may also occur.
• If diagnosed with microtia, the doctor will order a CT scan. This will help the doctor see the middle and inner parts of the ear, and is usually done when the child is a little older.
• Babies will also have a hearing test called an Auditory Brainstem Response (ABR) Evaluation.

This boy was treated for microtia at Cincinnati Children’s. Photos show before (left) and after ear reconstruction surgery.

Microtia Treatment

• Depends on the severity of the microtia.
• Typically involves addressing any hearing loss and reconstructing the ear (called reconstruction surgery).

At Cincinnati Children’s, microtia is treated with a team approach. Your child will see doctors in Plastic Surgery for their ear reconstruction and Otolaryngology (ENT) and Audiology if there is any hearing loss. These doctors work together when planning treatment for microtia. Your child may also see a specialist in Speech-Language Pathology.

Hearing Loss

It is important to address any hearing loss as soon as it is detected. Even slight hearing loss in one ear can affect your child’s speech and learning abilities.

If your child has hearing loss, you will work with our Audiology and Speech-Language Pathology teams on a plan for both before and after your child’s ear reconstruction.

Treatment options for hearing loss can include:

• Conventional hearing aids.
• Bone conduction hearing devices, also called bone-anchored hearing aids (BAHA). These can be on a head band for younger children or surgically placed in older children.
• Cochlear implants (for inner ear deafness).
• Middle ear reconstruction surgery, usually performed after the outer ear reconstruction. Not all children are good candidates for this.

Ear Reconstruction

More than one type of ear reconstruction surgery is available. At Cincinnati Children’s, we most often do a Nagata type ear reconstruction. You may also hear this called autologous ear reconstruction using rib cartilage or rib cartilage graft surgical reconstruction

During your visit, our plastic surgeon reviews all options with your family, and explains our approach.

Nagata Ear Reconstruction

• Uses a child’s own rib cartilage to create a new ear.
• Is a two-stage reconstruction:
1. First surgery: Cartilage is removed from the ribs in a way that allows it to grow back. The plastic surgeon carves this cartilage to match your child’s other ear as closely as possible. This is placed under the skin where the ear should be. Your child will now have what looks like a normal ear, but it sits flat against the side of the head. This will need to heal for about six months.
2. Second surgery: This procedure is done at least six months after the first surgery. It involves lifting the ear away from the side of the head, so the ear appears more like a normal ear. Once this surgery is complete and healed, a child will be able to wear glasses. It is often possible to pierce the ear as well.

It is a complex surgery, and the child needs to be mentally prepared for it. Children need to be around age 7 or 8 before having this surgery. It’s best to wait until this age, to match the ears in size as much as possible.

A child also needs to be emotionally mature enough and understand the reasons to have this surgery.

After Surgery

• The new ear will need to be protected while it heals.
• Your child will be out of sports for a couple of months.
• Once healed, the ear is part of their body and they can engage in the same activities as other kids.

Long-Term Outlook

The majority of children who have ear reconstruction are very satisfied afterward. Kids with microtia typically go on to live normal, healthy lives. Their ears are usually not exactly symmetrical, but the slight differences are generally not noticeable to others. Kids often have more self-confidence (feel better about themselves) after surgery.

Hearing loss treatment often helps improve or prevent speech, language and cognitive delays. Children who have hearing loss will continue to be seen by Audiology for regular check-ups as they grow.

Children who are treated for other disorders, in addition to microtia, may need long-term care into adulthood.