Hearing and Aural Atresia
Fortunately, nine out of ten children born with microtia have one normal ear with normal hearing and do quite well even without treatment for hearing on the affected side.
An infant’s hearing may be objectively tested shortly following birth by auditory response testing (ABR) to determine whether the cochlea and middle ear reacts normally to stimulation. This testing is worthwhile for most microtia patients, as the internal structures of the ear can sometimes be malformed, even when the external structures appear to be normal.
Hearing Loss Treatment for Patients with Microtia/Aural Atresia
Non-Surgical Hearing Aids
Because most microtia patients have a normal inner ear on their affected side, they may, as noted above, have normal hearing on that side. Newborns with unilateral microtia often get bone conduction hearing aids before leaving the hospital. Several months later, these may be replaced by bone-anchored hearing aids (BAHAs). A BAHA is a tiny box attached to a small screw imbedded in the skull. This can be placed within the hair so it is not noticeable and tends to provide better amplification than other hearing aids. All hearing aid patients should be followed by an audiologist, and should develop normal speech, although some may require the assistance of a speech therapist.
Basic Treatment Plan
- Audiogram within the first week of life, preferably before the baby is discharged from the hospital.
- Subsequent testing should continue annually. If a patient develops frequent infections in their normal ear, more audiograms may be recommended.
- Speech development should be normal.
- Audiogram within the first week of life, preferably before the baby is discharged from the hospital. Subsequent testing should continue until reliable results are obtained.
- Bone conduction aids within a few months.
- Bone conduction aids may be converted to BAHA aids depending on the treatment plan.
- A speech therapist as soon as the child begins speaking.
Surgical Correction – Aural Atresia Repair
Congenital aural atresia, or the absence of the external ear canal, is a birth defect that is almost always accompanied by abnormalities of both the middle ear bones and the external ear.
As noted above, most children with unilateral microtia function well without surgical intervention for hearing loss. In some patients, however, middle ear surgery is considered to improve hearing by creating an ear canal and rebuilding the eardrum.