Van Lierde Kristiane M, Van Cauwenberge Paul, Stevens Ines, Dhooge Ingeborg
Department of Speech and Language Pathology, Head and Neck Surgery, Ghent University Hospital, Ghent, Belgium.
Folia Phoniatr Logop. 2004 May-Jun;56(3):131-43. doi: 10.1159/000076935.
The purpose of this study was to describe the language, articulation, voice and resonance characteristics in children with Goldenhar syndrome. The 4 Dutch-speaking subjects were 2 boys (age 4.5 and 10.2 years) and 2 girls (aged 5.0 and 5.4 years) with normal cognitive functioning. Language testing showed a delay in the development of morphosyntactic abilities. Speech analysis showed that these four children were capable of producing all the sounds of their mother tongue. One type of distortion error, namely dentalization, was found in 2 children. Phonological process analysis revealed the persistence of processes such as final consonant deletion, unstressed syllable deletion and cluster reduction, which are normal in young children but are suppressed with maturation. Also the substitution process of devoicing was observed in these 4 children. It is not clear how the speech and language problems demonstrated in these 4 children are to be explained. It must be taken into consideration that the Goldenhar syndrome has a variable expression. It is possible that the presence of a mandibular defect, hearing impairment, the type and severity of palatal abnormality and decreased linguistic processing skills influence articulation in an individual child. The voices of 2 children were characterized by the presence of slight roughness, corresponding with a higher jitter percentage. How the occurrence of the hoarse voices is to be explained is not quite clear. Laryngoscopic evaluation of the vocal folds showed no organic or functional voice disorder. Regarding resonance, only the child with a cleft palate showed hypernasality and nasal emission. The ENT specialist and the speech-language pathologist must be aware of these communication disorders in order to start preventive and early 'tailor-made' speech and language intervention as soon as possible.
本研究的目的是描述患有Goldenhar综合征儿童的语言、发音、嗓音和共鸣特征。4名说荷兰语的受试者为2名男孩（年龄分别为4.5岁和10.2岁）和2名女孩（年龄分别为5.0岁和5.4岁），认知功能正常。语言测试显示其形态句法能力发育延迟。语音分析表明，这4名儿童能够发出母语的所有音素。在2名儿童中发现了一种失真错误，即齿音化。音系过程分析显示，诸如词尾辅音省略、非重读音节省略和音丛简化等过程持续存在，这些在幼儿中是正常的，但会随着成熟而受到抑制。在这4名儿童中还观察到了清化的替代过程。尚不清楚如何解释这4名儿童所表现出的言语和语言问题。必须考虑到Goldenhar综合征具有可变的表现形式。下颌骨缺损、听力障碍、腭裂异常的类型和严重程度以及语言处理技能下降可能会影响个别儿童的发音。2名儿童的嗓音特点是有轻微粗糙感，对应较高的抖动百分比。尚不清楚如何解释嗓音嘶哑的发生情况。喉镜对声带的评估未发现器质性或功能性嗓音障碍。关于共鸣，只有腭裂患儿表现出高鼻音和鼻漏气。耳鼻喉科专家和言语治疗师必须了解这些沟通障碍，以便尽快开始预防性和早期的“量身定制”言语和语言干预。
