Progress Update: Development of a Novel Tool to Measure Orofacial Strength in FSDH

Posted by Friends of FSH Research on Apr 17, 2021

Report by Dr. Johnstone
See grant Development of a novel measurement tool of oral strength in FSHD

The purpose of this study was to assess the reliability and validity of a novel measurement tool (FaceFormer) to test orofacial strength in FSHD. This study design was a prospective test-retest design which included 2 groups; a control group of adults without FSHD and an age-matched group of those with FSHD. The analysis plan was to evaluate the relationships between same-day trials and then between day trials, then assess the relationship to the current gold standard (IOPI instrument). The original timeline for this phase of the project was expected to be 1 year with a start date of 10/01/2020.

Work Completed

Data collection began in October, 2020 and was discontinued in December 2020 due to COVID-19 research precautions and restrictions that evolved with the rising virus rates. The following was accomplished between October-December 2020

• Procedural troubleshooting was conducted
• Recruitment of 10 control subjects and 5 subjects with FSHD
• Baseline and follow-up testing of 5 subjects with FSDH and 10 control subjects
• Pilot data analysis which revealed the following
  • Poor test-retest correlations between same-visit trials for the FSHD group (r=.21)
  • Moderate test-retest correlations between same-visit trials for the control group (r=.62)
  • Poor correlational relationship between IOPI mid lip pressure findings and FaceFormer for both groups (r=.27)

Project Update

We have decided to discontinue this project for the following reasons: 1) the initial findings showing that this instrumentation is not showing adequate reliability in FSHD; 2) COVID-19 precautions/restrictions made trouble-shooting and recruitment difficult; 3) roles and responsibilities for the PI have changed and it is believed this difficult problem will require more time than initially anticipated.

Spending and Return of Funds

Our grants office started the funds return process on 3/3/2021 and a check will be mailed for ~$18,900 and should be expected within 2-3 weeks. We charged 1% for a total of 2 days work on a 1-year project (including fringe) which is ~$1100.00.