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首页> 外文期刊>The Laryngoscope: A Medical Journal for Clinical and Research Contributions in Otolaryngology, Head and Neck Medicine and Surgery, Facial Plastic and Reconstructive Surgery .. >Cochlear implantation in Children with CHARGE syndrome: therapeutic decisions and outcomes.
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Cochlear implantation in Children with CHARGE syndrome: therapeutic decisions and outcomes.

机译：CHARGE综合征患儿的人工耳蜗植入：治疗决策和结果。

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OBJECTIVES: Ear anomalies and deafness are associated with CHARGE syndrome, which also presents with a cluster of features including coloboma of the eye, heart defects, atresia of the choanae, developmental retardation, and genitourinary abnormalities. The aim of this study is to explore the viability of cochlear implantation in children with CHARGE syndrome and to assess the outcome. STUDY DESIGN: Retrospective chart review. METHODS: Eleven children presenting with severe to profound sensorineural hearing loss associated with CHARGE syndrome were the subjects of this study. Routine audiometric measurements and the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) were performed pre- and postoperatively. In addition, the degree of the subjects' cochlear deformity were measured and correlated to outcome. RESULTS: All patients had varying degrees of ear anomalies, seven patients suffered from coloboma of the eyes, two had heart defects, five exhibited choanal atresia, eleven showed developmental retardation, and six had genitourinary abnormalities. Ten of the children underwent cochlear implantation with complete insertion of the electrode array without complication and were followed over a 3-month to a 7-year period. The eleventh child was not implanted because of severe retardation. All of the implanted children showed varying, but limited degrees, of auditory benefit as measured by routine audiometry and the IT-MAIS. CONCLUSIONS: Careful treatment planning for children with sensorineural hearing loss and CHARGE syndrome can lead to varying, but limited degrees, of auditory benefit with no increase in surgical complications. Although the implant enhanced the children's 'connectivity' to the environment, it did not promote the development of oral language skills in this population.
机译：目的：耳畸形和耳聋与CHARGE综合症有关，CHARGE综合症还具有一系列特征，包括眼球瘤，心脏缺陷，胸膜闭锁，发育迟缓和泌尿生殖系统异常。这项研究的目的是探讨CHARGE综合征患儿人工耳蜗的可行性，并评估其结局。研究设计：回顾性图表审查。方法：本研究的对象为11例与CHARGE综合征相关的严重至严重的感音神经性听力丧失的儿童。术前和术后进行常规听力测定和婴儿有意义的听觉综合量表（IT-MAIS）。此外，测量受试者的耳蜗畸形程度并将其与预后相关。结果：所有患者均出现不同程度的耳异常，其中7例患有眼球盲，2例患有心脏缺陷，5例表现为闭锁性闭锁，11例表现为发育迟缓，6例为泌尿生殖系统异常。十名儿童接受了耳蜗植入，电极阵列完全插入而没有并发症，并进行了3个月至7年的随访。由于严重的发育迟缓，第十一胎没有被植入。通过常规测听和IT-MAIS测量，所有植入的儿童均表现出不同程度但有限的听觉益处。结论：对患有感音神经性听力减退和CHARGE综合征的儿童进行仔细的治疗计划可能会导致听觉益处的变化，但程度有限，但不会增加手术并发症。尽管植入物增强了儿童与环境的“连接性”，但并未促进该人群口语技能的发展。

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