Congenital Outer Ear Anomaly

A congenital outer ear anomaly occurs when a child is born with a malformation of the outer ear. The ear is misshaped or missing structures. Outer ear anomalies involve only the skin and soft tissue on the outside. The ear is called the auricle, or pinna, and it consists of three basic parts: tragus (a small piece of cartilage on the inner side of the outer ear), helix (the outer rim of the ear), and lobule (the ear lobe). An outer ear anomaly can affect any one or all three of these parts — and a child can have an anomaly on one ear or both. About one in 6,000 children are born with an anomaly.

There of the most common types of congenital anomalies of the outer ear are: 

• Microtia: When the ear is small or not completely formed. Most children with microtia ear experience only partial hearing loss because the inner ear is usually unaffected.
• Prominent/protruding ear: When an ear extends two centimeters more than three-quarters of an inch from the side of the head. Prominent ear does not normally affect hearing or balance.
• Constricted/lop/cup ear: When the rim of the ear folds over or is wrinkled. Constricted ear does not normally affect balance, but depending on the severity, it can affect hearing. Constricted/lop/cup ear can range in severity from mild to severe.

Outer ear anomalies can be caused by a variety of factors, such as:

• Genetics
• Fetal positioning during development or birth
• Prenatal exposure to certain medications, or alcohol
• Unknown cases — sometimes the case of a congenital outer ear anomaly is unknown

Congenital outer ear anomalies are apparent upon birth, and are usually diagnosed in the hospital when a baby is born. A physical exam will be performed to access the severity of the anomaly and determine which ear structures are involved. The inner ear might also be examined with an otoscope. If your child has microtia, an auditory brainstem response (ABR) evaluation is recommended.

Congenital outer ear anomalies have a variety of treatments depending on your child’s diagnosis and the severity of the anomaly. Surgical correction is one such treatment. By the time your child is 5 years old, their ears have developed and are ready for surgery. Surgical treatments can include otoplasty to correct protruding ears, or ear reconstruction to treat underdeveloped ear and microtia.

Yet some children don’t need treatment because as they grow, ear anomalies such as prominent ear and lop ear can sometimes correct themselves, if they aren’t severe. Also, those two conditions typically don’t affect a child’s hearing or balance. 

But severe anomalies should be treated in the first few weeks after birth, when the ear is still malleable. For example, before your baby is 3 months old, a plastic splint known as ear molding can be placed under the helix, or outer rim, of your child’s ear. Ear molding can help correct constricted, lop, or cup ears by reshaping the ear.

The Department of Plastic and Oral Surgery at Boston Children’s Hospital is ready to prepare the best treatment plan for your child. Our specialized, experienced team understands which treatments can address your child's individual needs, and we include you and your family in every step of treatment.