Muscle weakness and speech in oculopharyngeal muscular dystrophy.

PURPOSE: We documented speech and voice characteristics associated with oculopharyngeal muscular dystrophy (OPMD). Although it is a rare disease, OPMD offers the opportunity to study the impact of myopathic weakness on speech production in the absence of neurologic deficits in a relatively homogeneous group of speakers. METHODS: Twelve individuals with OPMD and 12 healthy age-matched controls underwent comprehensive assessment of the speech mechanism including spirometry (respiratory support), nasometry (resonance balance), phonatory measures (pitch, loudness, and quality), articulatory measures (diadochokinetic rates, segment duration measures, spectral moments, and vowel space), tongue-to-palate strength measures during maximal isometric and speechlike tasks, quality-of-life questionnaire, and perceptual speech ratings by listeners. RESULTS: Individuals with OPMD had substantially reduced tongue strength compared to the controls. However, little impact on speech and voice measures or on speech intelligibility was observed except for slower diadochokinetic rates. CONCLUSIONS: Despite having less than half the maximal tongue strength of healthy controls, the individuals with OPMD exhibited minimal speech deficits. The threshold of weakness required for noticeable speech impairment may not have been reached by this group of adults with OPMD.

Swallow characteristics in patients with oculopharyngeal muscular dystrophy.

PURPOSE: This prospective investigation evaluates oral weakness and its impact on swallow function, weight, and quality of life in patients with oculopharyngeal muscular dystrophy (OPMD). METHOD: Intraoral pressure, swallow pressure, and endurance were measured using an Iowa Oral Performance Instrument in participants with OPMD and matched controls. Timed water swallow, weight, and quality of life were also assessed. RESULTS: Participants with OPMD were weaker than controls. Oral weakness impacted strength, swallow pressure, swallow capacity, swallow volume, swallow time, and quality of life. Tongue endurance was not affected by oral weakness. CONCLUSION: This investigation provides further insight into the swallow function of patients with myopathic disease. Muscle fiber loss leads to weakness, which results in reductions in swallow function and quality of life. Weight and endurance are not greatly altered.