Congenital Ear Anomalies

Congenital ear anomalies are birth defects that affect the shape and position of the ear. These conditions can involve the soft cartilage around the ear along with other structures that affect both the function and appearance of the ear. These anomalies can also cause significant social impairment.

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 opening in some cases. 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 external ear 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 anomalies such as oculo auriculo vertebral spectrum disorders.
Prominent ears. This abnormality is primarily cosmetic, but it can have negative effects on self-esteem. Practical concerns, such as proper fitting of eyeglasses, are also factors in treating prominent ears.
Lop/cupped ears. Lop/cupped ears 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, with inner ear deformities that can affect hearing.

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

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 inner ear 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.

Surgery is the typical treatment for congenital ear anomalies. Surgical procedures are based on the type of anomaly being treated:

Microtia. Surgeries to treat microtia can be performed as early as 4 years of age. Depending on the surgeon’s preferred technique, one stage or multiple stages of surgery may be needed. Ear reconstruction can be based on a Medpore framework (one stage) or a cartilaginous framework (multiple stages). Further treatment to address hearing problems or the development of the middle or inner 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 ears. 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 after your child’s ears have reached their full size, usually around age 5 or 6.
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 removal or grafting of cartilage to rebuild the ear structure or repositioning the entire ear.

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 ears and lop/cupped ears.
Surgery is the typical treatment for congenital ear anomalies and 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 Facial Plastic and Reconstructive Surgery

This specialty medical association contains detailed information on treatment for protruding ears.

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

In addition to our primary hospital location at the Academic Health Center in Indianapolis, IN, we have convenient locations to better serve our communities throughout the state.