![]() ![]() 2ĬIs allow children with deafness to attain speech outcomes on par with their normal‐hearing peers. In addition to including adults and children with severe to profound sensorineural hearing loss (SNHL) not improved with hearing aids, CI candidacy criteria has also changed considerably and now includes children as young as 12 months old (and younger under certain conditions) and adults with retained low frequency hearing via the hybrid CI which uses a combination of electrical and acoustic stimulation. Today, the goal is to improve quality of life through excellent speech recognition, functional hearing in noise, and the ability to appreciate nuanced sound with precisely coded temporal elements like music. Initially, the goal was perception of sound, then speech recognition, and serviceable hearing. In the intervening six decades, the goals for CI recipients' sound perception have advanced and continue to be refined. The first surgery for implantation of a single channel cochlear implant (CI) occurred in 1961, by House and Doyle, and was followed by the first multichannel CI in 1978. Improvements in our understanding of genetic site of lesions and their effects on CI function should lead to better CI outcomes, not just for individuals with auditory neuropathy, but all individuals with hearing loss.Ĭochlear implantation has revolutionized the care for children and adults with deafness. neuropathy) as this has implications for expected CI outcomes. We propose that description of ANSD patients should be based on the molecular site of lesion typically derived from genetic evaluation (synaptopathy vs. ![]() We put forth a nuanced molecular classification of ANSD that has implications for preoperative counseling for patients with this disorder prior to cochlear implantation. Individuals with lesions that affect the auditory nerve show poor performance with CIs, likely because neural transmission of the electrical signal from the CI is affected. Reviewed data show that individuals with lesions that primarily affect the cochlear sensory system and the synapse, which are bypassed by the CI, have optimal CI outcomes. In this review, we examine the precise mechanisms of genetic lesions causing ANSD and the effect of these lesions on CI outcomes. Auditory neuropathy spectrum disorder (ANSD) is characterized by dysfunctional transmission of sound from the cochlea to the brain due to defective synaptic function or neural conduction. There is growing evidence that qualitative as well as quantitative deficits in the auditory nerve may affect cochlear implant (CI) outcomes. Cochlear implantation has become the standard‐of‐care for adults and children with severe to profound hearing loss. ![]()
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