Author(s)
Sungjin A. Song MD
Phillip C. Song MD
Affiliation(s)
Massachusetts Eye and Ear Infirmary/Harvard Medical School
Abstract:
Educational Objective: At the conclusion of this presentation, the participants should be able to demonstrate understanding of the swallowing and voice quality of life measurement tools as well as the changes seen in X-linked dystonia parkinsonism. Objectives: The primary objective was to determine the progression of voice and swallowing quality of life in patients with X-linked dystonia parkinsonism (XDP) over a two year interval. Study Design: Retrospective matched cohort study. Methods: Patients diagnosed with XDP were surveyed from 2016 to 2018. They underwent a battery of tests aimed to assess swallowing status, aspiration risk with the penetration aspiration scale (PAS), self-reported psychosocial measure, and voice quality of life with the Voice Handicap Index (VHI) and Voice Related Quality of Life Survey (VRQL). Results: Ten patients were included. Mean followup was 2 years. Mean initial age of patient was 49.2 +/- 8.6 years. Mean PAS score increased from 4.2 +/- 1.87 to 5.1 +/- 1.66 (p=0.27), eating duration increased from 0.9 +/- 1.29 to 2.89 +/- 0.93 (p=0.001), symptom frequency increased from 13.4 +/- 7.0 to 24.2 +/- 9.08 (p=0.01). Mental health or social measures did not significantly change. VHI increased from 81.0 +/- 28.5 to 109.7 +/- 31.8 (p=0.048) and VRQL increased from 26.7 +/- 8.8 to 38.8 +/- 10.8. Out of the three domains of voice perception, only emotional effect of the voice on daily life showed a statistically significant increase from 25.3 +/- 10.4 to 36 +/- 11.9 (p=0.046). Conclusions: There is a negative impact on swallowing and voice quality of life in XDP with progressive worsening in the eating duration, symptom frequency and worsening VRQL and VHI, especially with regard to the emotional component of voice perception.