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Purpose <p>To explore the association between cochlear duct length (CDL) and hearing loss etiologies, and evaluate its impact on cochlear implantation (CI) planning.</p> Methods <p>We 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.</p> Results <p>Patients diagnosed with cochlear nerve deficiency, Waardenburg syndrome (WS) associated with <i>SOX10</i> variants, enlarged vestibular aqueduct syndrome, and incomplete partition type III exhibited significantly reduced CDL. Of these, WS associated with <i>SOX10</i> variants exhibited the highest decrease in CDL, often with vestibular malformations.</p> Conclusions <p>Short CDL poses surgical challenges, necessitating tailored electrode selection for optimal CI outcomes. In <i>SOX10</i>-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.</p>

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Cochlear duct length variability in prelingual sensorineural hearing loss: Emphasis on Waardenburg syndrome and implications for cochlear implantation

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月5日

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  • Kyu Ha Shin,
  • Chang-Hee Kim,
  • Yehree Kim,
  • Jung Kyu Lee,
  • Hye-Rim Park,
  • Jin Hee Han,
  • Jiyeon Yang,
  • Bong Jik Kim,
  • Byung Yoon Choi

摘要

Purpose

To explore the association between cochlear duct length (CDL) and hearing loss etiologies, and evaluate its impact on cochlear implantation (CI) planning.

Methods

We 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.

Results

Patients 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.

Conclusions

Short 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.