Abstract：
OBJECTIVE: To examine the clinical characteristics and auditory performance of patients with CHARGE syndrome following cochlear implantation (CI), as well as the prognostic factors affecting auditory outcomes.
METHODS: Retrospective cohort.
METHODS: Tertiary academic center.
METHODS: A retrospective chart review was performed in patients with CHARGE syndrome who underwent CI from 2007 to 2022. The category of auditory performance (CAP) score was used to assess the CI outcomes, and factors that may affect the speech outcomes were also evaluated.
RESULTS: In 14 children with CHARGE syndrome, 22 CIs were performed, 6 unilaterally and 8 bilaterally. The mean age at CI was 25.9 months (range: 10-62). All patients had ear abnormalities and developmental delays, and cochlear nerve deficiency (CND) was present in all ears. At the last follow-up (mean: 49.6 months), the mean CAP score improved significantly compared to the preoperative measure (from 0.36 ± 0.81 to 3.21 ± 1.70, P = .001), with 6 patients (42.9%) achieving a CAP score of 4 points or higher. However, between the unilateral and bilateral CI groups, the final CAP score or change in CAP score was similar. Factors including age, coloboma, and CND did not significantly affect speech outcomes (all P > .05).
CONCLUSIONS: Even though CHARGE syndrome features challenging anomalies, CI can be conducted safely and can offer effective contribution to significant speech improvement. Patients with CHARGE syndrome should be given the opportunity to undergo CI to maximize their audiological progress.
METHODS: Retrospective cohort.
METHODS: Tertiary academic center.
METHODS: A retrospective chart review was performed in patients with CHARGE syndrome who underwent CI from 2007 to 2022. The category of auditory performance (CAP) score was used to assess the CI outcomes, and factors that may affect the speech outcomes were also evaluated.
RESULTS: In 14 children with CHARGE syndrome, 22 CIs were performed, 6 unilaterally and 8 bilaterally. The mean age at CI was 25.9 months (range: 10-62). All patients had ear abnormalities and developmental delays, and cochlear nerve deficiency (CND) was present in all ears. At the last follow-up (mean: 49.6 months), the mean CAP score improved significantly compared to the preoperative measure (from 0.36 ± 0.81 to 3.21 ± 1.70, P = .001), with 6 patients (42.9%) achieving a CAP score of 4 points or higher. However, between the unilateral and bilateral CI groups, the final CAP score or change in CAP score was similar. Factors including age, coloboma, and CND did not significantly affect speech outcomes (all P > .05).
CONCLUSIONS: Even though CHARGE syndrome features challenging anomalies, CI can be conducted safely and can offer effective contribution to significant speech improvement. Patients with CHARGE syndrome should be given the opportunity to undergo CI to maximize their audiological progress.
摘要：
目的：观察CHARGE综合征患者在人工耳蜗植入术后的临床特点和听觉表现。以及影响听觉结果的预后因素。
方法：回顾性队列。
方法：高等教育中心。
方法：对2007-2022年CHARGE综合征患者进行回顾性分析。听觉表现（CAP）评分类别用于评估CI结果，并对可能影响言语结果的因素进行了评估.
结果：在14名CHARGE综合征儿童中，进行了22CI，6单边和8双边。CI的平均年龄为25.9个月（范围：10-62）。所有患者都有耳朵异常和发育迟缓,全耳存在耳蜗神经缺陷（CND）。在最后一次随访（平均：49.6个月），与术前测量相比，平均CAP评分显着改善（从0.36±0.81到3.21±1.70，P=.001），有6例患者（42.9％）达到4分或更高的CAP评分。然而,在单边和双边CI组之间，最终CAP评分或CAP评分变化相似.因素包括年龄,结肠瘤,CND对言语结果无显著影响(P均>.05)。
结论：尽管CHARGE综合征具有挑战性异常，CI可以安全地进行，并且可以为显着的语音改善提供有效的贡献。CHARGE综合征患者应给予undergoCI的机会，以最大限度地提高其听力学进展。
方法：回顾性队列。
方法：高等教育中心。
方法：对2007-2022年CHARGE综合征患者进行回顾性分析。听觉表现（CAP）评分类别用于评估CI结果，并对可能影响言语结果的因素进行了评估.
结果：在14名CHARGE综合征儿童中，进行了22CI，6单边和8双边。CI的平均年龄为25.9个月（范围：10-62）。所有患者都有耳朵异常和发育迟缓,全耳存在耳蜗神经缺陷（CND）。在最后一次随访（平均：49.6个月），与术前测量相比，平均CAP评分显着改善（从0.36±0.81到3.21±1.70，P=.001），有6例患者（42.9％）达到4分或更高的CAP评分。然而,在单边和双边CI组之间，最终CAP评分或CAP评分变化相似.因素包括年龄,结肠瘤,CND对言语结果无显著影响(P均>.05)。
结论：尽管CHARGE综合征具有挑战性异常，CI可以安全地进行，并且可以为显着的语音改善提供有效的贡献。CHARGE综合征患者应给予undergoCI的机会，以最大限度地提高其听力学进展。
