Congenital Ear Anomalies

Congenital ear anomalies are birth defects that affect the shape and position of the ear and are treated by Riley plastic surgery. These conditions can involve both skin and cartilage of the ear and affect both the function and appearance of the ear. These anomalies can also cause significant social impairment.

Types of Congenital Ear Anomalies

There are several types of congenital ear anomalies. Each type has different symptoms. They include:

Microtia

Microtia means “small ear.” Most children with microtia have a normally formed inner ear but lack all or part of the structures that form the outer ear, including the canal (ear opening) in some cases.

A child with microtia does not have the normal structure of the ear, and because it does not look like most children’s ears, many children feel self-conscious. Some children do not have any parts of the outer ear at all, which is called “anotia.” This condition can occur on one or both sides of the head.

Without the external structures to funnel sound to the inner ear, children often have difficulty hearing and determining the direction of sound.

In some cases, a child with microtia has no hearing in the affected ear because of the absence of the canal. This is called aural atresia. Some children with aural atresia require a hearing aid. Children with aural atresia typically also have incomplete formation of the small middle ear bones and may lack an eardrum.

Microtia can also occur along with other facial differences such as hemifacial microsomia, Treacher Collins syndrome, and oculo auriculo vertebral spectrum disorders.

Prominent ear

This abnormality is primarily cosmetic, but it can have negative effects on self-esteem. Many children are teased at school for this. This is when the ears stick out farther from the head than most children’s ears.

Lop/cupped ears

Lop/cupped ear means that the rim of the ear is tightened or constricted. The condition can range from mild to severe. Children with the most severe cases have ears that are rolled almost into a tube.

Cryptotia

Cryptotia refers to an ear where the top of the ear looks like it is “stuck” to the scalp. This can prevent children from being able to wear glasses.

Stahl's Ear

This occurs when there is extra cartilage in the upper part of the ear, which makes the ear look pointed. Many children feel self-conscious about having this extra cartilage.

Diagnosis of Congenital Ear Anomalies

In general, congenital ear anomalies are diagnosed through a physical exam. Based on the physical exam, hearing and language screenings may be needed. There are several types of hearing tests available to evaluate the hearing of infants and children to determine the need for a hearing aid.

Treatments

Ear molding can be started for infants within the first several weeks of life for most congenital ear anomalies. This is non-invasive. It is highly effective and prevents the child from needing surgery later. It works best if initiated within the first 2-3 weeks of life. Most ears only need 2-4 weeks of treatment. Ear molding does not work for microtia.

Surgery is the typical treatment for congenital ear anomalies. Doctors with the Cleft & Craniofacial Anomalies Program at Riley at IU Health work closely with pediatric ear, nose and throat surgeons to address any hearing issues that your child may have. The surgeons work with your family to create a treatment plan that meets the specific needs of your child.

Surgical procedures are based on the type of anomaly being treated:

Microtia. Surgeries to treat microtia are usually performed around 10 years of age. The craniofacial surgeons at Riley prefer a two-stage approach, which requires a large amount of cartilage to rebuild your child’s ear. This is why the child needs to be at least 10 years old for this operation. The first operation is to build the ear using rib cartilage. The second operation is to elevate the ear off the scalp to give it some projection, which requires a skin graft. These operations are usually done 6 months apart. The surgeons at Riley do not perform Medpor reconstructions, as it is a foreign body and has a high risk of becoming infected or extruding after many years. Additional treatment to address hearing problems or the development of the middle ear may be required. Patients with bilateral aural atresia receive bone conduction hearing aids within the first few months of life. In addition, speech therapy will be recommended as soon as a child begins to talk. Older children may receive a bone-anchored hearing aid (BAHA), which works by directly stimulating the inner ear through the bone.
Prominent ear. The surgery to treat this condition is sometimes known as "pinning back the ears." Surgical correction of prominent ears does not affect the function of the ear or hearing. This procedure is typically performed when your child’s ears are close to their expected adult size, usually around age 5 or 6. It is an outpatient procedure. Furthermore, your child will have to wear a soft headband around the ears after surgery, which requires the patient to be able to follow instructions after surgery.
Lop/cupped ears. The surgical procedure used for lop/cupped ears depends on how severe the condition is. Surgery for a minor problem may involve shortening the cartilage for a more normal appearance. More severe cases can involve rebuilding the ear with rib cartilage, like in microtia.
Cryptotia. This requires elevation of the ear off the scalp with a skin graft. This is an outpatient procedure. A special dressing is placed on the skin graft and removed in the office 5-7 days later.
Stahl’s ear. Treatment of the extra cartilage can involve special stitching techniques or cartilage removal. Many children with this problem also have ears that protrude too far from the scalp, which is addressed at the same time. This is also an outpatient procedure.

The Cleft & Craniofacial Anomalies Program at Riley at IU Health strives to make treatment as convenient as possible for your family. Most of the specialists your child might need to see, such as plastic surgeons and audiologists, are available during one visit. This allows your family to make fewer trips to our facility. If necessary, your child may be referred to other pediatric experts within Riley at IU Health for additional treatment.

Key Points to Remember

Congenital ear anomalies can affect both the appearance and function of the ear.
Congenital ear anomalies include microtia (lack of all or part of the structures that form the outer ear), prominent ear, lop/cupped ear, cryptotia, and Stahl’s ear.
Surgery is the typical treatment for microtia and for congenital ear anomalies that were not treated with molding. It is based on the type of anomaly being treated.

Support Services & Resources

Visit these websites for more information on congenital ear anomalies.

Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.

Learn More About Riley Support Services

Children's Craniofacial Association

This nonprofit organization provides a downloadable guide about microtia in both English and Spanish.

American Academy of Otolaryngology—Head and Neck Surgery

Information on treatment for various types of ear anomalies is available at this professional medical society website.

FACES: The National Craniofacial Association

This nonprofit organization provides education and support to parents of children with microtia and other craniofacial conditions.

Locations

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