Abstract
Cochlear implantation is accepted as an effective treatment to restore auditory perception in patients with bilateral severe to profound sensorineural hearing loss. During the early years of cochlear implantation, postlingually deaf adults with normal cochlear anatomy received a cochlear implant (CI); however, the frequency of cochlear implantation in congenitally deaf children, children with inner ear malformations, and children with multiple disabilities is gradually increasing as clinical reports demonstrating the safety and efficacy of CIs accumulate. Many studies show acceptable outcomes in these challenging populations. However, CI outcomes are often poor in patients with cochlear nerve deficiency (CND), which is defined by a small or absent cochlear branch of the vestibulocochlear nerve on magnetic resonance imaging, probably due to an insufficient number of spiral ganglion neurons (SGNs). Patients with CND who showed no improvement in auditory performance after cochlear implantation may be good candidates for auditory brainstem implant (ABI); however, patients with CI can maximally utilize the simple linear tonotopic organization in the cochlea and natural sound processing mechanisms in the cochlear nucleus which are theoretically more suitable for speech processing than direct stimulation at the brainstem by ABI. Thus, (re)generation SGNs combined with cochlear implantation, which may enhance CI-mediated stimulation, can be effective to improve auditory performance in patients with CND.
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Yamazaki, H. (2014). Cochlear Implant: Past, Present, and Future. In: Ito, J. (eds) Regenerative Medicine for the Inner Ear. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54862-1_14
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