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Progressive Decline in Voice and Voice-Related Quality of Life in X-Linked Dystonia Parkinsonism

  • Sungjin A. Song
    Correspondence
    Address correspondence and reprint requests to Sungjin A. Song, Department of Otolaryngology, Massachusetts Eye and Ear, 243 Charles Street, Boston, MA 02114.
    Affiliations
    Department of Otolaryngology, Massachusetts Eye and Ear, Boston, Massachusetts

    Department of Otolaryngology, Harvard Medical School, Boston, Massachusetts
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  • Criscely L. Go
    Affiliations
    Department of Neurology, Jose Reyes Memorial Medical Center, Manila, Philippines

    The Collaborative Center for X-linked Dystonia Parkinsonism, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts
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  • Patrick B. Acuna
    Affiliations
    The Collaborative Center for X-linked Dystonia Parkinsonism, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts
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  • Jan Kristopher Palentinos De Guzman
    Affiliations
    Department of Neurology, Jose Reyes Memorial Medical Center, Manila, Philippines

    The Collaborative Center for X-linked Dystonia Parkinsonism, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts
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  • Nutan Sharma
    Affiliations
    The Collaborative Center for X-linked Dystonia Parkinsonism, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts
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  • Phillip C. Song
    Affiliations
    Department of Otolaryngology, Massachusetts Eye and Ear, Boston, Massachusetts

    Department of Otolaryngology, Harvard Medical School, Boston, Massachusetts
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Published:December 14, 2020DOI:https://doi.org/10.1016/j.jvoice.2020.11.014

      Summary

      Objective

      To characterize the evolution of swallowing and voice in patients with X-linked dystonia parkinsonism (XDP).

      Study Design

      Retrospective case series.

      Methods

      Retrospective review of 59 patients with XDP from January 2016 to January 2018. All patients underwent complete examinations and quality of life (QOL) surveys (Swallowing Quality of Life questionnaire [SWAL-QOL], Voice-Related Quality of Life [V-RQOL], and Voice Handicap Index [VHI]), and functional endoscopic examination of swallowing. We excluded patients with incomplete records or patients lost to follow-up. Univariate analysis was used to compare 2016 to 2018 Penetration-Aspiration Scale (PAS), SWAL-QOL, V-RQOL, and VHI scores.

      Results

      Ten patients met the inclusion criteria. Nine patients had oromandibular dystonia. Voice-related measures significantly worsened with an increase in mean VHI from 81 to 109.9 (P = 0.026) and decrease in mean V-RQOL from 58 to 28 (P = 0.013). Vocal strain also significantly worsened 0.4 to 1.4 (P = 0.001). Mean PAS scores increased from 4.2 to 5.1 (P = 0.068) and mean SWAL-QOL decreased from 50.4 to 43.5 (P = 0.157). In the SWAL-QOL, the mean Eating Duration score worsened from 0.9 to 0.4 (P = 0.052) and Mental Health score declined from 10.1 to 6.1 (P = 0.077).

      Conclusions

      Both vocal strain and voice-related QOL measures considerably worsened over the 2-year interval in our limited group of XDP patients with no significant change in PAS scores or swallowing QOL. The findings demonstrated that the pace of disease affecting voice symptoms was different from swallowing symptoms in our study group and that changes in communication ability may be a more sensitive marker for disease progression than swallowing dysfunction.

      Key words

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