Polanski José Fernando, Kochen Ana Paula, de Oliveira Carolina Arcoverde
Universidade Federal do Paraná - UFPR - Curitiba (PR), Brasil.
Faculdade Evangélica Mackenzie do Paraná - Curitiba (PR), Brasil.
Codas. 2020 Dec 9;32(6):e20180295. doi: 10.1590/2317-1782/20202018295. eCollection 2020.
Waardenburg syndrome (WS) is a rare autosomal-dominant syndrome that can be presented with sensorineural hearing loss. In this report, we describe the outcomes of three children with WS at zero, three, nine, twelve and sixty months after cochlear implant (CI) fitting. The outcomes were assessed using IT-MAIS (Infant-Toddler Meaningful Auditory Integration Scale - younger than 5 year), MAIS (Meaningful Auditory Integration Scale - older than 5 year), MUSS (Meaningful Use of Speech Scale), and categories of auditory performance and speech intelligibility. The results showed an improvement in auditory and language performance over time, two patients who used CI for 5 years achieved 100% in IT-MAIS and MUSS tests. In addition, both were able to understand sentences in open set and achieve fluent speech. Moreover, both reached fluency on auditory and language performance scale. The third patient with 50 months of follow-up and in the 48 months evaluation, is in category 5 of auditory performance and 3 of speech intelligibility. We concluded that all children who had low levels of hearing and language before cochlear implant have improved hearing and language skills after implantation and rehabilitation.
瓦登伯革氏综合征（WS）是一种罕见的常染色体显性综合征，可伴有感音神经性听力损失。在本报告中，我们描述了三名患有WS的儿童在接受人工耳蜗（CI）植入后0、3、9、12和60个月的情况。使用婴幼儿有意义听觉整合量表（IT-MAIS，适用于5岁以下儿童）、有意义听觉整合量表（MAIS，适用于5岁以上儿童）、言语有意义使用量表（MUSS）以及听觉表现和言语可懂度类别对结果进行评估。结果显示，随着时间推移，听觉和语言表现有所改善，两名使用CI达5年的患者在IT-MAIS和MUSS测试中取得了100%的成绩。此外，两人都能够理解开放式语句并实现流利言语。而且，两人在听觉和语言表现量表上都达到了流利程度。第三名患者随访50个月，在48个月评估时，听觉表现为5级，言语可懂度为3级。我们得出结论，所有在人工耳蜗植入前听力和语言水平较低的儿童在植入和康复后听力和语言技能均有提高。
