Congenital Atresia of the Ear

Congenital atresia of the ear occurs when the ear canal develops abnormally or does not develop at all. The condition can occur by itself or in combination with other anomalies involving the head or neck. Congenital atresia of the ear most often affects the right ear; however, it can occur bilaterally or only in the left ear.

 

How Congenital Atresia of the Ear is Diagnosed

Congenital atresia of the ear is typically diagnosed at birth since the condition often causes a visible malformation of the outer ear. An audiologist can evaluate the functioning of the cochlea using a specialized hearing test called an ABR. The cochlea is the hearing organ that is situated in the inner ear. Children with the condition should be assessed and monitored at an early age for hearing loss. If not addressed, the hearing loss can impact speech development as well as school performance. An estimated 30 percent of children with one-sided hearing loss resulting from congenital atresia of the ear are held back in school at least once before graduating.

 

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Treatments for Congenital Atresia of the Ear

Several options are available to improve both the function and the appearance of the malformed ear. A prosthesis can be constructed that mirrors the patient’s normal ear. The prosthetic ear is then anchored into place using titanium screws that are implanted in the head. An alternative procedure involves reconstructing the ear using rib cartilage that is harvested from the patient. This is a complex technique that typically involves multiple procedures performed over a period of months to achieve the final result.

Children with congenital atresia of the ear typically benefit from various non-surgical and surgical rehabilitation options to maximize their hearing function.

A bone conduction hearing aid is a non-surgical method of improving hearing. A device that resembles a headband holds the hearing aid in place behind the affected ear. The hearing aid vibrates the bones behind the ear, which sends the sound to the inner ear.

If a patient with congenital atresia of the ear cannot benefit from conventional hearing aids, the doctor may recommend a bone anchored hearing aid, sometimes referred to as a BAHA. A bone anchored hearing aid is an implantable device that works by directly stimulating the inner ear. The device is implanted during an outpatient procedure that usually takes less than an hour to perform. A BAHA typically offers a greater degree of hearing improvement than conventional hearing aids. The device is also more discreet than traditional hearing aids and can be easily covered by the hair.

A surgical procedure to reconstruct and open the ear canal may allow some patients with congenital atresia of the ear to hear without hearing aids. Patients considering this type of surgery will need to undergo a CT scan to determine if surgery is feasible based on the anatomy of their ear. It is important to note that the hearing in the affected ear will likely never be as good as the unaffected ear because of the various anomalies involving the ear structure that are common with the condition.

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