Aural Atresia

What You Should Know

Causes

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.

Treatment

Surgical reconstruction can be very effective for improving hearing in the affected ear. A CT scan is used to determine if surgery would result in improved hearing.

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.

The Baha(www.entific.com) is a 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.