Remote Speech and Swallowing Assessment in ALS

Study Overview
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Brief Summary
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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
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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.

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

Conditions
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Amyotrophic Lateral Sclerosis

Intervention / Treatment
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* 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


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients At least 18 years of age Possess a diagnosis of ALS (definite, probable, probable laboratory-supported, or possible by El Escorial criteria [35]) Have symptom onset within the last 3 years 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 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. Have a life expectancy ≥ 6 months as determined by the attending neurologist Be fluent in written and spoken English Possess a smartphone capable of running the study application Follow up in the Penn State Hershey multidisciplinary ALS clinic over next 6 months Be able to visit the study site for in-person procedures at weeks 0 and 24 Listeners Between 18 and 40 years of age 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 Possess a co-existing neurological or psychiatric illness that, in the opinion of the study team, exclude the subject from participation Demonstrate clinically significant dementia, as determined by the ALS study neurologist Possess abnormal speech or swallowing processes due to a condition independent of their ALS diagnosis Listeners Have an identified speech, language, learning, or neurological disorders per self-report Experience communicating with people with motor speech disorders

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| ALS Patients<br>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<br>* [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.<br>Radiation: Modified Barium Swallow Study (MBSS)<br>* [Patients only] MBSS is an instrumented evaluation of oropharyngeal swallowing function that is performed using videofluoroscopy.<br>* Other names: Videofluoroscopic Swallowing Study (VFSS);Behavioral: Speech Intelligibility Test (SIT)<br>* [Patients only] The SIT is a standardized test for measuring speech intelligibility.<br>|
| Listeners<br>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<br>* [Listeners only] Listeners will judge the intelligibility of speech recordings made by patients in the study.<br>|

What is the study measuring?
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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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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telemonitoring, digital health, mobile health, dysphagia, dysarthria