Hereditary deafness and cochlear implantation

A large amount of information regarding genetic hearing loss has become available over the past 20 years along with the identification of genetic loci and causative genes. Although many forms of hearing loss are thought to have a genetic etiology, in reality, many subjects do not undergo genetic testing. Cochlear implantation has become one of the mainstream treatment options for severe to profound hearing loss. Information and experience regarding cochlear implantation with genetic deafness is a valuable tool in considering treatment in any patient who might be a candidate for cochlear implantation. In this chapter, our intention was to review the literature on incidences of cochlear implantation in genetic deafness families and to determine if there were any characteristic findings regarding performance outcome. We reviewed reports in the literature (from 1994 to 2012) on patients with definitive diagnoses of genetic hearing loss who underwent cochlear implantation (total of 104 reports). The reports were limited to a fraction of the known loci or causative gene mutations, in which most cases had favorable results after cochlear implantation.