ArticleEuropean archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery2026
Cochlear duct length variability in prelingual sensorineural hearing loss: Emphasis on Waardenburg syndrome and implications for cochlear implantation.
Article in European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
purposeTo explore the association between cochlear duct length (CDL) and hearing loss etiologies, and evaluate its impact on cochlear implantation (CI) planning.
methodsWe conducted a retrospective study on 610 patients (825 ears) who underwent CI at a tertiary center, to identify the etiologies associated with reduced CDL and investigate their influence on electrode selection and surgical outcomes.
resultsPatients diagnosed with cochlear nerve deficiency, Waardenburg syndrome (WS) associated with SOX10 variants, enlarged vestibular aqueduct syndrome, and incomplete partition type III exhibited significantly reduced CDL. Of these, WS associated with SOX10 variants exhibited the highest decrease in CDL, often with vestibular malformations.
conclusionsShort CDL poses surgical challenges, necessitating tailored electrode selection for optimal CI outcomes. In SOX10-related WS cases, preoperative imaging and genetic testing can aid in the early detection of the disease, enabling appropriate CI planning. These findings indicate the importance of having a preliminary estimation of CDL based on etiology and the significance of this study.
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