Remote Speech and Swallowing Assessment in ALS

Home Telemonitoring of Bulbar Function by Acoustic Measurement of Swallowing and Speech Sounds in ALS

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.

Study Overview

• Status: Active, not recruiting
• Conditions: Amyotrophic Lateral Sclerosis
• Intervention / Treatment: Device: Digital recording of speech and swallow sounds using the Beiwe Digital Health App on a personal smartphone; Radiation: Modified Barium Swallow Study (MBSS); Behavioral: Speech Intelligibility Test (SIT); Behavioral: SIT Scoring

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 Type: Observational
• Enrollment (Estimated): 170

Contacts and Locations

Study Locations

• United States
  • Pennsylvania
    • Hershey, Pennsylvania, United States, 17033
      • Hershey Medical Center ALS Clinic

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes

• Sampling Method: Non-Probability Sample

Study Population

Population 1: Patients with ALS Population 2: Healthy Listeners

Description

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 Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
ALS Patients; All ALS patients will be enrolled in this arm, which involves a combination of clinical and home-based assessments of speech and swallowing.	Device: Digital recording of speech and swallow sounds using the Beiwe Digital Health App on a personal smartphone; [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.; Radiation: Modified Barium Swallow Study (MBSS); [Patients only] MBSS is an instrumented evaluation of oropharyngeal swallowing function that is performed using videofluoroscopy.; Other Names: Videofluoroscopic Swallowing Study (VFSS); Behavioral: Speech Intelligibility Test (SIT); [Patients only] The SIT is a standardized test for measuring speech intelligibility.
Listeners; 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.	Behavioral: SIT Scoring; [Listeners only] Listeners will judge the intelligibility of speech recordings made by patients in the study.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Digital recording of speech and swallow	Audio recordings of speech tasks and spontaneous swallowing task	weekly for 24 weeks

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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.	Weeks 0 and 24
Speech Intelligibility Task (SIT) Intelligibility listener scoring	Listeners will score the intelligibility of patient SIT recordings	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.	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.	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.	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.	Weeks 0, 12, and 24
Forced Vital Capacity (FVC)	A standard clinical respiratory assessment of vital capacity	Weeks 0, 12, and 24
Maximal Inspiratory Pressure (MIP)	A standard clinical respiratory assessment of inspiratory strength	Weeks 0, 12, and 24

Collaborators and Investigators

• Sponsor: Milton S. Hershey Medical Center
• Collaborators: Penn State University; Congressionally Directed Medical Research Programs
• Investigators: Principal Investigator: Andrew Geronimo, PhD, Penn State College of Medicine

Study record dates

Study Major Dates

• Study Start (Actual): January 1, 2022
• Primary Completion (Actual): August 31, 2024
• Study Completion (Estimated): September 1, 2026

Study Registration Dates

• First Submitted: May 7, 2021
• First Submitted That Met QC Criteria: May 12, 2021
• First Posted (Actual): May 17, 2021

Study Record Updates

• Last Update Posted (Estimated): October 8, 2025
• Last Update Submitted That Met QC Criteria: October 3, 2025
• Last Verified: October 1, 2025

More Information

Terms related to this study

• Keywords: mobile health; digital health; telemonitoring; dysphagia; dysarthria
• Additional Relevant MeSH Terms: Neurologic Manifestations; Central Nervous System Diseases; Nervous System Diseases; Neuromuscular Diseases; Metabolic Diseases; Digestive System Diseases; Gastrointestinal Diseases; Neurobehavioral Manifestations; Neurodegenerative Diseases; Esophageal Diseases; Otorhinolaryngologic Diseases; Pharyngeal Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Motor Neuron Disease; Communication Disorders; Language Disorders; Speech Disorders; Articulation Disorders; Pathological Conditions, Signs and Symptoms; Nutritional and Metabolic Diseases; Signs and Symptoms; Deglutition Disorders; Amyotrophic Lateral Sclerosis; Dysarthria
• Other Study ID Numbers: STUDY00016872

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No