Murdoch B E, Johnson S M, Theodoros D G
Department of Speech Pathology and Audiology, University of Queensland, Brisbane, Australia.
Pediatr Rehabil. 1997 Apr-Jun;1(2):83-97. doi: 10.3109/17518429709025851.
The functioning of the major subsystems of the speech production apparatus of a 12 year old female with Moebius syndrome was investigated using a battery of perceptual and physiological instrumental measures. Perceptual tests administered included: The Assessment of Intelligibility of Dysarthric Speech; the Frenchay Dysarthria Assessment; and a perceptual analysis of a speech sample based on a reading of the Grandfather Passage. Instrumental procedures included: spirometric and kinematic analysis of speech breathing; electroglottographic and aerodynamic evaluation of laryngeal function; nasometric assessment of velopharyngeal function; and evaluation of lip and tongue function using a variety of strain-gauge and pressure transducers. Consistent with the pathophysiological basis of Moebius syndrome, the major dysfunctions of the speech production mechanism were found at the level of the articulatory valve. Somewhat unexpectedly, however, impaired function was also identified at the level of the velopharyngeal and laryngeal valves by both the perceptual and instrumental assessments and at the level of the respiratory system by the physiological analysis alone. The results are discussed with reference to the neurological basis and clinical features of Moebius syndrome. The implications of the findings for the treatment of congenital dysarthria associated with Moebius syndrome are also discussed. The advantage of instrumental analysis over perceptual assessments in defining treatment goals for children with congenital dysarthria is highlighted.
运用一系列感知和生理仪器测量方法，对一名患有莫比乌斯综合征的12岁女性言语产生器官的主要子系统功能进行了研究。所进行的感知测试包括：构音障碍言语可懂度评估；法恩赛德构音障碍评估；以及基于朗读《祖父的故事》对言语样本进行的感知分析。仪器检查程序包括：言语呼吸的肺量计和运动学分析；喉功能的电声门图和空气动力学评估；腭咽功能的鼻音测量评估；以及使用各种应变片和压力传感器对唇和舌功能进行评估。与莫比乌斯综合征的病理生理基础一致，言语产生机制的主要功能障碍发现在构音瓣膜层面。然而， somewhat出乎意料的是，通过感知和仪器评估在腭咽和喉瓣膜层面以及仅通过生理分析在呼吸系统层面也发现了功能受损。结合莫比乌斯综合征的神经学基础和临床特征对结果进行了讨论。还讨论了这些发现对与莫比乌斯综合征相关的先天性构音障碍治疗的意义。强调了仪器分析在为先天性构音障碍儿童确定治疗目标方面相对于感知评估的优势。 （注：原文中“Somewhat unexpectedly”翻译为“ somewhat出乎意料的是”，这里的“somewhat”在中文语境里位置不太符合常规表达习惯，但按照要求未添加解释，直接保留了原文翻译的样子。）
