Congenital Atresia of the Ear

In this condition, the ear canal either does not develop properly, or it does not develop at all. Also referred to as congenital aural atresia, this medical issue can occur alone or in connection with other conditions that affect the development of the head and neck. The condition can occur on one side or on both.

• When congenital atresia occurs on one side, it is more commonly seen on the right side than on the left.
• This condition is seen more frequently in boys than in girls.

Congenital atresia occurs when the development of the auditory system is somehow disrupted during the regular growth of the fetus. The exact cause is not known. It can occur alone or with other syndromes that results in abnormalities in the development of the head and neck. Some of these concurrent conditions include:

• Treacher-Collins syndrome
• Branchial-oto-renal syndrome
• Goldenhar syndrome

At birth, the physician may notice a physical anomaly of the ear canal. The outer ear may also show some abnormalities. Because the child’s ear canal, ear drum, and bones that relegate hearing will not be developed properly or may be missing, hearing will be affected. This makes it extremely important for the affected child to have the appropriate hearing tests completed to determine the severity of the condition. A CT scan may also be performed to better aid the physician in making the appropriate recommendations for treatment.

In most cases, surgical intervention will be necessary to correct any physical abnormalities, as well as to improve the child’s chances of hearing. Surgeries that correct the appearance of an ear that is malformed may include reconstruction of the outer ear with cartilage obtained from the patient’s rib or through the use of a prosthetic ear. If only one ear is affected by atresia and hearing is normal in the unaffected ear, external reconstruction may be all that is necessary.

The ear canal may also require reconstruction. Surgery can open or create a new ear canal, which is then lined with skin taken from the patient’s leg. While canal reconstruction can be useful for appearance, it may not improve hearing much if the inner ear is too malformed.

Special types of hearing aids can be useful to increase the hearing ability of a child who is affected by this condition. One of these devices that does not require surgery is called a bone conduction hearing aid. Held in place in back of the ear with an appliance that resembles a headband, the hearing aid vibrates the bone behind the ear to allow sounds to reach the middle and inner ear areas. Another type of hearing device can be surgically implanted to stimulate the inner ear. Known as a bone anchored hearing aid, or BAHA, this mechanism is attached with a titanium screw.