About Facial Deformities

Hearing Restoration

When is hearing restoration necessary?

Children born with ear deformities often suffer hearing loss due to the absence of their ear canals and/or middle ear structures. The impact of hearing loss on the child's speech and language development has been well documented. Children born with both ears deformed need immediate attention and hearing rehabilitation in order to avoid the devastating effects of early deafness on their growth and development. They are immediately fitted with a bone-conduction hearing aid until they are of age to undergo more-definitive therapy. Children born with one normal ear and one deformed ear are rehabilitated from age five and on. The need of both ears in sound localization and better speech understanding in noise is well proven. While children with one good ear may do well with preferential seating in school, restoring and recovering hearing from both ears is far superior.

Hearing restoration is achieved in one of two ways:

1) The ear canal may be recreated using soft tissue and bone-remodeling techniques until the middle ear is reached. The ossicles (three bones in the tympanic cavity of the ear) are then tested and repaired or replaced as necessary, and the eardrum is recreated using grafts. If feasible, such surgical reconstruction offers the child natural hearing without use of hearing aids. These procedures, however, may not be feasible in cases of severe middle-ear deformities, particularly when accompanied by an abnormal course of the facial nerve. Risks of these procedures include facial nerve injury, failure to correct the hearing loss, recurrent ear infections, and reclosure of the newly created canal.

2) A less invasive hearing rehabilitation technique consists of the implementation of a titanium fixture on the surface of the mastoid bone behind the ear, which, following a few months of integration with the bone, is connected to a cochlear stimulator, also known as the BAHA. This technique is far less risky, and more predictable, than the reconstruction described earlier. The titanium implantation is further combined with the auricular reconstruction procedure, thus cutting down significantly on the number of procedures these children require. Both ears can be implanted in cases of bilateral deformities, thereby ensuring binaural hearing and better sound localization.

Combining the external ear reconstruction with functional restoration of hearing provides these children with a significant cosmetic improvement and recovery of useful hearing, while enabling them to mingle with their peers, participate in activities, learn and compete in the classroom, and eventually in the workplace.