Hearing Loss

Normal hearing depends upon transmission of sound through the ear canal, eardrum and the three bones (ossicles) located in the middle ear space: the malleus, incus and stapes. Due to problems such as chronic ear infections, cholesteatoma, congenital defects or even trauma, these ossicles can be damaged, scarred or absent. This results in a "conductive" or "mechanical" hearing loss.

Treatment Options

At the Center for Hearing and Deafness Research, surgical therapies have evolved which allow correction of some of these hearing losses by implantation of prosthetic--or artificial--ossicles, which are called partial or total ossicular reconstruction prostheses.

In addition, the Center for Hearing and Deafness Research offers state-of-the-art management for otosclerosis, a sometimes hereditary and sometimes congenital problem in which the stapes bone becomes abnormally fixed and fails to transmit sound to the inner ear. Using a surgical laser, the fixed stapes bone is removed and replaced by a stainless steel piston. Hearing is restored to normal or near normal levels in more than 90 percent of patients.

Sensorineural Hearing Loss

A major objective of the Center for Hearing and Deafness Research is to facilitate identification of hearing loss in children as early as possible. This is because of the significant detriments to communication skill development, learning and social maturation caused by hearing loss. In line with this objective, the Center for Hearing and Deafness Research is involved in statewide efforts (including Ohio Department of Health committees) to implement universal newborn hearing screening. If implemented, this universal screening would require the testing of every baby born in Ohio for congenital hearing loss. Legislation for such a process has already passed at a federal level.

With the anticipated increase in the number of children identified with hearing loss, the Center for Hearing and Deafness Research has optimized protocols for evaluating children with hearing loss. In this case, hearing loss refers to sensorineural hearing loss (SNHL) -- or "nerve deafness" -- in particular. In large part, a radical advance in workup of children with sensorineural hearing loss has come about through human molecular genetics.

A standard step in our protocol now involves obtaining a routine blood sample for DNA analysis. Several common gene mutations have been identified as causing sensorineural hearing loss in children. Defects of the connexin 26 gene, for example, have been shown to be one of the most common causes of hearing loss (accounting for as much as 40 percent of all congenital, hereditary and sporadic deafness). The Center for Hearing and Deafness Research is one of the relatively few centers in the United States routinely offering this genetic testing as a standard part of the hearing loss workup.

Additional diagnostic tests for children identified with hearing loss include comprehensive audiologic (hearing) testing, blood and urine lab testing, as well as diagnostic imaging studies (CT and MRI scans).

Treatment Options

For those children with sensorineural hearing loss, management involves an interdisciplinary team approach due to the multiple aspects of a child's life impacted by hearing loss. Children with useful residual hearing are managed with hearing aides or FM systems and also receive auditory and speech-language rehabilitation therapies where indicated.

For those children not benefiting from amplification with hearing aides, the Center for Hearing and Deafness Research offers cochlear implantation as an outstanding option for families choosing an auditory/verbal communication mode for their child. The Center for Hearing and Deafness Research provides comprehensive care for children undergoing cochlear implantation -- from preoperative evaluation to surgical care to post-implant therapy and rehabilitation.