Physiological and perceptual features of dysarthria in Moebius syndrome: directions for treatment

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.