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 ... Research Article
Research Article  |   February 01, 2015
Muscle Weakness and Speech in Oculopharyngeal Muscular Dystrophy
 
Author Affiliations & Notes
  • Amy T. Neel
    University of New Mexico, Albuquerque
  • Phyllis M. Palmer
    University of New Mexico, Albuquerque
  • Gwyneth Sprouls
    University of New Mexico, Albuquerque
  • Leslie Morrison
    University of New Mexico, Albuquerque
  • Disclosure: The authors have declared that no competing interests existed at the time of publication.
    Disclosure: The authors have declared that no competing interests existed at the time of publication.×
  • Correspondence to Amy T. Neel: atneel@unm.edu
  • Editor: Jody Kreiman
    Editor: Jody Kreiman×
  • Associate Editor: Julie Liss
    Associate Editor: Julie Liss×
Article Information
Speech, Voice & Prosodic Disorders / Special Populations / Genetic & Congenital Disorders / Speech / Research Articles
Research Article   |   February 01, 2015
Muscle Weakness and Speech in Oculopharyngeal Muscular Dystrophy
Journal of Speech, Language, and Hearing Research, February 2015, Vol. 58, 1-12. doi:10.1044/2014_JSLHR-S-13-0172
History: Received July 2, 2013 , Revised April 14, 2014 , Accepted September 15, 2014
 
Journal of Speech, Language, and Hearing Research, February 2015, Vol. 58, 1-12. doi:10.1044/2014_JSLHR-S-13-0172
History: Received July 2, 2013; Revised April 14, 2014; Accepted September 15, 2014

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.

Acknowledgments
We are very grateful to the individuals with OPMD and to the healthy controls who participated in this study. We also acknowledge the contributions of Aaron Padilla and Diondra Maestas in collecting and analyzing data.
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