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| Name | Class |
|---|---|
| Penn State University | OTHER |
| Congressionally Directed Medical Research Programs | FED |
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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| 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. |
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| 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. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Digital recording of speech and swallow sounds using the Beiwe Digital Health App on a personal smartphone | Device | [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. |
| Measure | Description | Time Frame |
|---|---|---|
| Digital recording of speech and swallow | Audio recordings of speech tasks and spontaneous swallowing task | weekly for 24 weeks |
| 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 |
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Inclusion Criteria:
Patients
Listeners
Exclusion Criteria:
Patients
Listeners
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Population 1: Patients with ALS Population 2: Healthy Listeners
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| Name | Affiliation | Role |
|---|---|---|
| Andrew Geronimo, PhD | Penn State College of Medicine | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hershey Medical Center ALS Clinic | Hershey | Pennsylvania | 17033 | United States |
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| ID | Term |
|---|---|
| D000690 | Amyotrophic Lateral Sclerosis |
| D003680 | Deglutition Disorders |
| D004401 | Dysarthria |
| ID | Term |
|---|---|
| D013118 | Spinal Cord Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D016472 | Motor Neuron Disease |
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| Modified Barium Swallow Study (MBSS) | Radiation | [Patients only] MBSS is an instrumented evaluation of oropharyngeal swallowing function that is performed using videofluoroscopy. |
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| Speech Intelligibility Test (SIT) | Behavioral | [Patients only] The SIT is a standardized test for measuring speech intelligibility. |
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| SIT Scoring | Behavioral | [Listeners only] Listeners will judge the intelligibility of speech recordings made by patients in the study. |
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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 |
| D019636 | Neurodegenerative Diseases |
| D057177 | TDP-43 Proteinopathies |
| D009468 | Neuromuscular Diseases |
| D057165 | Proteostasis Deficiencies |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D004935 | Esophageal Diseases |
| D005767 | Gastrointestinal Diseases |
| D004066 | Digestive System Diseases |
| D010608 | Pharyngeal Diseases |
| D010038 | Otorhinolaryngologic Diseases |
| D001184 | Articulation Disorders |
| D013064 | Speech Disorders |
| D007806 | Language Disorders |
| D003147 | Communication Disorders |
| D019954 | Neurobehavioral Manifestations |
| D009461 | Neurologic Manifestations |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |