Remote Speech and Swallowing Assessment in ALS

Active, not recruiting

• Conditions: Amyotrophic Lateral Sclerosis
• Interventions: Device: Digital recording of speech and swallow sounds using the Beiwe Digital Health App on a personal smartphone; Behavioral: SIT Scoring; Radiation: Modified Barium Swallow Study (MBSS); Behavioral: Speech Intelligibility Test (SIT)

• Registration Number: NCT04889898
• Lead Sponsor: Milton S. Hershey Medical Center

Brief Summary

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.

Detailed Description

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 Timeline

• Study First Submitted: 2021-05-07
• Study First Submitted QC: 2021-05-12
• Study First Posted: 2021-05-17
• Study Start: 2022-01-01
• Primary Completion: 2024-08-31
• Status Verified: 2024-10
• Last Update Submitted: 2024-10-02
• Last Update Posted: 2024-10-04
• Study Completion: 2025-09

Recruitment & Eligibility

• Status: ACTIVE_NOT_RECRUITING
• Sex: All
• Target Recruitment: 170

Inclusion Criteria

Patients

1. At least 18 years of age
2. Possess a diagnosis of ALS (definite, probable, probable laboratory-supported, or possible by El Escorial criteria [35])
3. Have symptom onset within the last 3 years
4. Demonstrate a score of 2 or 3 in either the speech (item #1) or swallowing (item #3) components of the ALSFRS-R at time of enrollment
5. Demonstrate decline in bulbar function prior to enrollment, measured by a reduction of at least 1 point on either the speech or swallowing components of the ALSFRS-R in the 6 months prior to enrollment. If 6 months of data is not available, an estimate of the change in ALSFRS-R will be calculated from the time of symptom onset.
6. Have a life expectancy ≥ 6 months as determined by the attending neurologist
7. Be fluent in written and spoken English
8. Possess a smartphone capable of running the study application
9. Follow up in the Penn State Hershey multidisciplinary ALS clinic over next 6 months
10. Be able to visit the study site for in-person procedures at weeks 0 and 24

Listeners

1. Between 18 and 40 years of age
2. Native speakers of American English 2.3. Currently residing within the United States. 3.4. Hearing within normal limits, as defined by self report.

Exclusion Criteria

Patients

1. Possess a co-existing neurological or psychiatric illness that, in the opinion of the study team, exclude the subject from participation
2. Demonstrate clinically significant dementia, as determined by the ALS study neurologist
3. Possess abnormal speech or swallowing processes due to a condition independent of their ALS diagnosis

Listeners

1. Have an identified speech, language, learning, or neurological disorders per self-report
2. Experience communicating with people with motor speech disorders

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
ALS Patients	Speech Intelligibility Test (SIT)	All ALS patients will be enrolled in this arm, which involves a combination of clinical and home-based assessments of speech and swallowing.
ALS Patients	Modified Barium Swallow Study (MBSS)	All ALS patients will be enrolled in this arm, which involves a combination of clinical and home-based assessments of speech and swallowing.
ALS Patients	Digital recording of speech and swallow sounds using the Beiwe Digital Health App on a personal smartphone	All ALS patients will be enrolled in this arm, which involves a combination of clinical and home-based assessments of speech and swallowing.
Listeners	SIT Scoring	Healthy listeners will be recruited to listen to the audio recordings of patients in the study in order to judge the intelligibility of their speech.

Primary Outcome Measures

Name	Time	Method
Digital recording of speech and swallow	weekly for 24 weeks	Audio recordings of speech tasks and spontaneous swallowing task

Secondary Outcome Measures

Name	Time	Method
Speech Intelligibility Task (SIT) Intelligibility listener scoring	Weeks 0, 12, and 24	Listeners will score the intelligibility of patient SIT recordings
ALS Functional Rating Scale - Revised (ALSFRS-R)	Weeks 0, 12, and 24	ALS Specific Assessment of physical function ranging from 0-48, with 48 being normal function.
MBS Impairment Profile (MBSImp)	Weeks 0 and 24	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.
Center for Neurological Study - Bulbar Function Scale (CNS-BFS)	Weeks 0, 4, 8, 12, 16, 20, and 24	Self-administered rating scale for speech (7-49) and salivation (7-35) functions, with high scores indicating impaired function.
Mann Assessment of Swallowing Ability (MASA)	Weeks 0, 12, and 24	A standard clinical swallowing assessment performed by the speech-language pathologist. Scores range from 38-200, with lower scores indicating higher impairment.
EAT-10 Questionnaire	Weeks 0, 12, and 24	A self-reported assessment of eating and swallowing function. Scores range from 0-40, with higher scores indicating higher impairment.
Forced Vital Capacity (FVC)	Weeks 0, 12, and 24	A standard clinical respiratory assessment of vital capacity
Maximal Inspiratory Pressure (MIP)	Weeks 0, 12, and 24	A standard clinical respiratory assessment of inspiratory strength

Trial Locations

Locations (1)

Hershey Medical Center ALS Clinic; 🇺🇸 Hershey, Pennsylvania, United States