Van Lierde Kristiane M, Luyten Anke, Mortier Geert, Tijskens Anouk, Bettens Kim, Vermeersch Hubert
Department of Otorhinolaryngology and Speech Language Pathology, University Gent, Belgium.
Int J Pediatr Otorhinolaryngol. 2011 Feb;75(2):270-6. doi: 10.1016/j.ijporl.2010.11.017. Epub 2010 Dec 8.
The purpose of this study was to provide a description of the language and speech (intelligibility, voice, resonance, articulation) in a 7-year-old Dutch speaking boy with Nager syndrome. To reveal these features comparison was made with an age and gender related child with a similar palatal or hearing problem.
Language was tested with an age appropriate language test namely the Dutch version of the Clinical Evaluation of Language Fundamentals. Regarding articulation a phonetic inventory, phonetic analysis and phonological process analysis was performed. A nominal scale with four categories was used to judge the overall speech intelligibility. A voice and resonance assessment included a videolaryngostroboscopy, a perceptual evaluation, acoustic analysis and nasometry.
The most striking communication problems in this child were expressive and receptive language delay, moderately impaired speech intelligibility, the presence of phonetic and phonological disorders, resonance disorders and a high-pitched voice. The explanation for this pattern of communication is not completely straightforward. The language and the phonological impairment, only present in the child with the Nager syndrome, are not part of a more general developmental delay. The resonance disorders can be related to the cleft palate, but were not present in the child with the isolated cleft palate. One might assume that the cul-de-sac resonance and the much decreased mandibular movement and the restricted tongue lifting are caused by the restricted jaw mobility and micrognathia. To what extent the suggested mandibular distraction osteogenesis in early childhood allows increased mandibular movement and better speech outcome with increased oral resonance is subject for further research.
According to the results of this study the speech and language management must be focused on receptive and expressive language skills and linguistic conceptualization, correct phonetic placement and the modification of hypernasality and nasal emission.
本研究旨在描述一名患有纳格尔综合征的7岁荷兰语男孩的语言和言语（可懂度、嗓音、共鸣、发音）情况。为揭示这些特征，将其与一名年龄和性别相仿、存在类似腭裂或听力问题的儿童进行了比较。
使用适合其年龄的语言测试工具，即荷兰语版的《语言基本能力临床评估》对语言进行测试。在发音方面，进行了语音清单分析、语音分析和音系过程分析。使用一个包含四个类别的标称量表来判断整体言语可懂度。嗓音和共鸣评估包括视频喉镜频闪检查、感知评估、声学分析和鼻音测量。
该儿童最突出的沟通问题是表达性和接受性语言延迟、言语可懂度中度受损、存在语音和音系障碍、共鸣障碍以及高音调嗓音。这种沟通模式的原因并非完全一目了然。仅在患有纳格尔综合征的儿童中出现的语言和音系损伤，并非更普遍发育迟缓的一部分。共鸣障碍可能与腭裂有关，但在单纯腭裂儿童中并未出现。有人可能会认为盲端共鸣以及下颌运动大幅减少和舌抬起受限是由下颌活动受限和小颌畸形导致的。儿童早期建议的下颌牵张成骨术在多大程度上能增加下颌运动并通过增强口腔共鸣改善言语效果，有待进一步研究。
根据本研究结果，言语和语言管理必须侧重于接受性和表达性语言技能以及语言概念化、正确的语音定位以及鼻音过重和鼻漏气的矫正。
