Microtia

Microtia (my-KRO-she-uh) is a rare, congenital condition that affects the shape and appearance of your baby’s outer ears. There may be a very small ear, or the outer ear may be missing some cartilage and skin. Rarely, babies with this condition are born without an outer ear. Microtia may cause conductive hearing loss. Microtia happens when a fetus’s ear doesn’t develop normally. Experts estimate this condition affects up to 4 in 10,000 babies globally.

There are different grades or types of microtia. Grades vary depending on how the condition affects your baby’s ear. Grades (types) include:

• Grade 1: Your baby’s outer ear is smaller than average. There may be minor issues with some parts of their ear.
• Grade 2: The condition affects the appearance of your baby’s upper ear. Their ear canal may be unusually narrow.
• Grade 3: This is the most common form. It affects all of your baby’s ear and ear canal.
• Grade 4: Their outer ear is missing.

Symptoms of microtia

Symptoms may include:

• Hearing loss in your baby’s affected ear
• Missing cartilage or tissue in their outer ear
• Missing or undeveloped ear canal
• Missing outer ear
• Smaller-than-expected ear size

Microtia causes

Several issues may cause this condition. But it often happens for no known reason. One known cause is genetic variations that happen during early pregnancy. Babies who have certain inherited disorders, like hemifacial microsomia, may also have microtia. And biological parents who have this condition may pass the condition on to their children.

Complications

The most common complication is hearing loss if the condition affects your child’s middle ear. Another complication may be less obvious.

Microtia can make your child’s ear look very different. Surgery can make the difference less noticeable. But most healthcare providers recommend waiting until a child’s ear is more developed before surgery. That leaves several years during which your child and their peers will become more aware of the difference in your child’s ear.

That new awareness may make your child feel self-conscious about their appearance. It may affect their self-esteem and lead to social isolation. If that’s your child’s situation, ask their healthcare provider for help and support

How doctors diagnose microtia

Healthcare providers usually diagnose it right after your baby is born. They may do a CT scan to get a detailed picture of the inside of your baby’s ear. This test helps them look for issues in your baby’s middle and inner ear.

An audiologist may do an auditory brainstem response test. This test measures the electrical activity or response to sound in your baby’s auditory nerve and brainstem.

What to ask your healthcare provider

Microtia is a rare condition. You may have lots of questions and concerns. Here are some questions you may want to ask your baby’s healthcare provider:

• Do you know what caused my baby’s condition?
• How severe is the condition?
• When will I know if my baby has hearing loss?
• Will my baby need treatment from specialists?

You may learn your baby has this condition before you and they go home from the hospital. Caring for a newborn can be exhausting at the best of times. In this case, you may feel overwhelmed. Try to take things a step at a time, starting with learning what the condition may mean for your baby and for you.

How is microtia treated?

The first step is to see whether the condition causes hearing loss. Microtia may affect your baby’s middle ear so that sound doesn’t make it to your baby’s inner ear. That’s the part of your ear that makes it possible for your baby to hear.

While all babies born in the U.S. have hearing screening, your baby should have follow-up tests as soon as possible. Early hearing loss diagnosis and treatment reduce the chance that your child will have communication and speech issues.

Microtia may make your child’s outer ear look different. Treatments to manage ear appearance include:

• Ear prosthetics: Usually made of silicone, an ear prosthetic attaches with adhesive or magnets. Ear prosthetics need maintenance over time. Your child’s healthcare provider will be glad to explain how to take care of an ear prosthetic. They’ll help you decide if this treatment is right for your child.
• Surgery: Older children may have an otoplasty if they have grades 3 or 4 microtia. This surgery reshapes or creates a new external ear. A healthcare provider may call this ear reconstruction.
• Bone-anchored hearing aid: These are surgically implanted hearing devices that may restore some hearing. Your child may have this surgery if microtia affects their ear canal.

Your child’s healthcare provider may recommend waiting on otoplasty until your child’s ear gets a bit bigger as they grow. Often, children are 7 or 8 years old before they have surgery. That waiting period could be challenging if your child feels self-conscious about their appearance. Your child’s provider may suggest they receive counseling or support from a child psychologist.

Is there a cure for microtia?

Treatment, including surgery, can fix the shape or appearance of your child’s outer ear. Surgery may also help improve hearing loss that happens when your child’s ear canal isn’t completely developed.

Are you deaf if you have microtia?

No. Microtia may cause some hearing loss, but it doesn’t cause you to lose your hearing.