Congenital Atresia of the Ear

Sometimes occurring on its own and sometimes linked to other head or neck irregularities, congenital atresia of the ear is the non-development of the ear canal. It may affect one or both ears.

• When a single ear is affected, it’s usually the right one.
• The most common issue associated with abnormal ear canal development is problems with hearing, which may contribute to learning difficulties. The inner ear structures are generally normal.

Diagnosis usually occurs at birth with the observation of a deformity of one or both ears. Children with the deformity often have a malformed or under-developed outer ear. After congenital atresia of the ear is detected, a special hearing test known as an auditory brainstem response (ABR) test is usually done to determine how well the hearing organ in the inner ear (the cochlea) is working.

Performed with electrodes attached to the patient’s head, an ABR test is especially beneficial with young children who aren’t able to verbalize what’s going on with their hearing like what’s normally done with a traditional hearing test. Early diagnosis and treatment is critical since approximately 30 percent of children with non-development of the ear canal affecting a single ear end up being held back in school.

The appearance of a child’s ear may be improved with an artificial ear (prosthesis). The artificial ear is usually modeled after the other normally shaped ear. The prosthesis can be attached to the head via glue (less commonly used) or is secured to prevent movement with titanium screws implanted surgically into the head and held on with magnets.

Another option is reconstruction of the affected ear with cartilage from one of the patient’s ribs. It usually takes multiple procedures performed over several months to complete reconstruction and produce an ear with a normal appearance. Most commonly this takes 3 procedures spaced apart from each other by 6 months.

If a child has ear canal development problems because of congenital atresia of the ear, some type of surgical or non-surgical rehabilitation will likely be recommended. The purpose of such efforts is to improve hearing capabilities as much as possible.

This non-surgical rehabilitation option involves the use of a specially designed hearing aid. It works by transmitting sound by vibrating the bone behind the ear that’s affected. Sound waves then travel to the inner parts of the ear, resulting in improved hearing.

For patients not able to benefit from a traditional hearing aid or a bone conduction hearing aid, a bone anchored hearing aid may be recommended. A BAHA is a hearing assistance device that’s implanted and anchored to the affected side of the head with titanium screw. Newer options for bone anchored hearing devices include the Sophono, Baha Attract, Baha SoundArc, AdHEAR, Ponto and Bone Bridge.

A bone anchored hearing aid is put into place during an outpatient procedure. The procedure typically takes about an hour to complete. Once attached and secured in place, the device stimulates the inner ear to help with the interpretation of sound. The device is appealing because it’s more discrete than a conventional hearing aid, meaning it can easily be concealed by the wearer.

Note: Bone anchored hearing aids have been approved by the FDA for use on children five and older.

Congenital atresia repair is a surgical procedure that’s sometimes performed to open and reconstruct the ear canal. For patients who respond well to the surgery, it may result in the ability to hear without the need for a hearing aid. During the procedure, the affected ear canal is reopened and lined with skin, which is usually taken from the patient’s leg.

A CT scan of the temporal bone that surrounds the ear will need to be done when this type of surgery is being considered. It’s done to view the patient’s ear structure in greater detail to determine if this type of surgery will be appropriate.

Sound conduction won’t be the same as what occurs with a normal ear. Even so, patients may enjoy the ability to hear well enough to improve daily functioning and comprehension. If an infection in the affected ear occurs, there is always the possibility that the ear canal could close again.

Individuals with congenital atresia of the ear will not have a tympanic membrane (eardrum) and the smaller bones located behind the affected ear may not be normal either, further impeding hearing. Because of these abnormalities, hearing isn’t likely to be fully restored to “normal” even with early intervention during childhood. Ultimately, the degree of hearing impairment will depend on how the inner ear mechanisms are affected. Appropriate treatments and adaptions may, however, help restore or maintain some hearing capabilities.