What is Aural Atresia?

Aural atresia is a birth defect in which there is an absence or incomplete formation of an external ear canal. Both the external and middle ear may be malformed, but the inner ear and auditory nerve are often normal. It can occur in one or both ears.

Symptoms

A malformed outer ear is the most common indicator of aural atresia. All atretic ears have abnormal middle-ear anatomy to a greater or lesser degree.

Diagnosis

It is important that infants with atresia undergo a complete audiologic evaluation, including an auditory brainstem evoked-response test. A moderate conductive hearing loss is often present in an atretic ear and can affect a child’s understanding of spoken language at normal conversational levels. A hearing loss may also be present in the child’s normal-appearing ear. A CT scan is used to determine if surgery would result in improved hearing.

Treatment

Surgical reconstruction at our Orange County treatment center can be very effective for improving hearing in the affected ear.

Bone-anchored hearing aids (BAHA) are surgically implantable device for the treatment of hearing loss that works through direct bone conduction. The system works by utilizing natural bone transmission as a pathway for sound to travel to the inner ear, bypassing the external auditory canal and middle ear. BAHA can be an effective way to restore hearing in children born with aural atresia or chronic ear conditions. Also, it can be used in patients with single sided deafness in order to take sound from the deaf ear and transmit it to the good ear. It is implanted in a single surgery in adults and in two separate procedures in children.

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Jennifer & son Miles

Our son Miles was born with aural atresia, leaving him with no ear canal on his left and only a partial ear canal on his right. We were told that nothing could be done for him until he was about nine. We had come to the point where we accepted that Miles would never speak much. But when we enrolled him in a special school for children who are deaf or hard of hearing, we learned about Dr. Jack Shohet and how progressive he is. Dr. Shohet did surgery on Miles when he was five, and he actually built a new ear canal for Miles. We’ve gone from no hearing on his left side at all, to full hearing with the assistance of a hearing aid to detect very quiet sounds. In eight months, Miles became a completely different child. He went from not speaking much, to developing language skills where he could speak complete sentences. During one visit, Miles got off the exam table and told Dr. Shohet, ‘Thank you for helping me.’ In a few weeks, Miles will have surgery to correct his right ear canal, and we are confident that he will be in the best of hands. Dr. Shohet gave us our son back!

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