The investigators propose a longitudinal home study of ALS patients to measure the severity of speech and swallowing (bulbar) impairment via a smartphone-based, remote speech and swallow assessment (rSSA). The study is designed to assess the feasibility and validity of such a monitoring intervention. Furthermore, it is proposed that regular monitoring of these two bulbar processes may shed light on their co-evolution over the course of ALS.
Most individuals with ALS experience bulbar function deterioration over the course of the disease which impacts their quality of life significantly. Furthermore, in some patients, bulbar symptoms are an initial presenting symptom of ALS. Identifying these changes, which may be rapid in some individuals, is complicated by the recent acceleration of virtual care delivery. The investigators propose a longitudinal home study of ALS patients to assess bulbar progression via a smartphone-based, self-administered remote speech and swallow assessment (rSSA). The overall hypothesis is that this monitoring protocol can be used in a way that, 1) is satisfactory to the patient, 2) performs at least as well as standard clinical measures of dysarthria and dysphagia, and 3) resolves the development of emergence of speech and swallowing pathologies in ALS.
Study Type
OBSERVATIONAL
Enrollment
170
\[Patients only\] Weekly completion of audio recordings of speech and swallowing performed at home by the subject using the study smartphone application on their personal device.
\[Patients only\] MBSS is an instrumented evaluation of oropharyngeal swallowing function that is performed using videofluoroscopy.
\[Patients only\] The SIT is a standardized test for measuring speech intelligibility.
Hershey Medical Center ALS Clinic
Hershey, Pennsylvania, United States
Digital recording of speech and swallow
Audio recordings of speech tasks and spontaneous swallowing task
Time frame: weekly for 24 weeks
MBS Impairment Profile (MBSImp)
Standardized scoring for the MBSS in 17 domains. Domains are aggregated in to scores for Oral Impairment (0-22), Pharyngeal Impairment (0-29), and Esophageal Impairment (0-4), with higher scores indicating impaired function.
Time frame: Weeks 0 and 24
Speech Intelligibility Task (SIT) Intelligibility listener scoring
Listeners will score the intelligibility of patient SIT recordings
Time frame: Weeks 0, 12, and 24
ALS Functional Rating Scale - Revised (ALSFRS-R)
ALS Specific Assessment of physical function ranging from 0-48, with 48 being normal function.
Time frame: Weeks 0, 12, and 24
Center for Neurological Study - Bulbar Function Scale (CNS-BFS)
Self-administered rating scale for speech (7-49) and salivation (7-35) functions, with high scores indicating impaired function.
Time frame: Weeks 0, 4, 8, 12, 16, 20, and 24
Mann Assessment of Swallowing Ability (MASA)
A standard clinical swallowing assessment performed by the speech-language pathologist. Scores range from 38-200, with lower scores indicating higher impairment.
Time frame: Weeks 0, 12, and 24
EAT-10 Questionnaire
A self-reported assessment of eating and swallowing function. Scores range from 0-40, with higher scores indicating higher impairment.
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.
\[Listeners only\] Listeners will judge the intelligibility of speech recordings made by patients in the study.
Time frame: Weeks 0, 12, and 24
Forced Vital Capacity (FVC)
A standard clinical respiratory assessment of vital capacity
Time frame: Weeks 0, 12, and 24
Maximal Inspiratory Pressure (MIP)
A standard clinical respiratory assessment of inspiratory strength
Time frame: Weeks 0, 12, and 24