Outcomes of cochlear implantation for the patients with specific genetic etiologies: a systematic literature review

Acta Oto-laryngologica
Shin-Ya Nishio, S Usami

Abstract

Most of the cases with gene mutations of intra-cochlear etiology showed relatively good CI outcomes. To progress toward more solid evidence-based CI intervention, a greater number of reports including CI outcomes for specific gene mutations are desired. Cochlear implantation (CI) is the most important and effective treatment for patients with profound sensorineural hearing loss. However, the outcomes of CI vary among patients. One of the reasons of this heterogeneous outcome for cochlear implantation is thought to be the heterogeneous nature of hearing loss. Indeed, genetic factors, the most common etiology in severe-to-profound hearing loss, might be one of the key determinants of outcomes for CI and electric acoustic stimulation (EAS). Patients with genetic causes involving an 'intra-cochlear' etiology show good CI/EAS outcomes. This review article aimed to summarize the reports on CI/EAS outcomes in patients with special genetic causes as well as to assist in future clinical decision-making. Most of the cases were suspected of an intra-cochlear etiology, such as those with GJB2, SLC26A4, and OTOF mutations, which showed relatively good CI outcomes. However, there have only been a limited number of reports on patients with ot...Continue Reading

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Citations

Jan 19, 2020·Journal of Clinical Medicine·Dominika OziębłoMonika Ołdak
Feb 7, 2020·The Anatomical Record : Advances in Integrative Anatomy and Evolutionary Biology·Shin-Ichi UsamiHidekane Yoshimura
Nov 13, 2020·Clinical and Experimental Otorhinolaryngology·Doo-Yi Oh, Byung Yoon Choi
Apr 23, 2021·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Eric NisenbaumXue Zhong Liu
Jul 22, 2019·Brazilian Journal of Otorhinolaryngology·Tatiana Mendes de MeloNatália Barreto Frederigue Lopes
Mar 13, 2021·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Ying ChenHao Wu

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