Sensorineural Hearing Loss
A major objective of the Ear and Hearing Center at Cincinnati Children's Hospital Medical Center 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 Ear and Hearing Center 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 Ear and Hearing Center 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 Ear and Hearing Center 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 benefitting from amplification with hearing aides, the Ear and Hearing Center offers cochlear implantation as an outstanding option for families choosing an auditory / verbal communication mode for their child. Cincinnati Children's Hospital Medical Center and the Ear and Hearing Center provide comprehensive care for children undergoing cochlear implantation -- from preoperative evaluation to surgical care to post-implant therapy and rehabilitation.
