DESIPHER_Speech Degradation as an Indicator of Physiological Degeneration in ALS

DESIPHER Speech Degradation as an Indicator of Physiological Degeneration in ALS

A disease called Amyotrophic lateral sclerosis (or ALS), which leads to difficulty swallowing, breathing, and movement, has been found to be higher for those serving in the military than in the general population. There are approximately 4,200 Veterans with ALS and roughly 1,000 new cases each year. When doctors attempt to determine the degree to which an ALS patient is suffering from the disease, they apply tests that are "graded" by experts. However, this approach to testing patients may not be very accurate. Researchers aim to use a system called DESIPHER to "listen" to ALS patients and find speech mistakes related to their condition. Researchers believe that, by detecting different types of errors, DESIPHER serves as a new kind of indicator of medical problems such as difficulty breathing or swallowing, without human "grading". This may also lead to a better system for automatically understanding ALS patients' speech.

Study Overview

• Status: Completed
• Conditions: Amyotrophic Lateral Sclerosis

Detailed Description

In 2008, Amyotrophic lateral sclerosis (ALS) or Lou Gehrig's Disease became a presumptively compensable (service connected) disease as the Institute of Medicine (IOM) Committee stated an association between the development of ALS and military service. According to the IOM report, military service increases life risk of ALS by 1.5 fold. There are approximately 4,200 Veterans with ALS and roughly 1,000 new cases each year. At the Tampa VA, since 2007, there has been a consistent rise in the number of Veterans diagnosed and treated with ALS.

Most physiological assessments that are commonly used to determine the functional status of patients with ALS require trained clinical personnel to administer and interpret the results. The investigators propose to use automatic speech understanding and machine learning software (DESIPHER) to: identify speech pathologies and use them to predict other aspects of physiological degeneration associated with ALS (e.g., respiratory difficulty or inability to swallow), and ultimately improve speech recognition for those with speech impairments. The investigators expect this to improve the ability to appropriately identify and intervene when Veterans with ALS are at risk of serious adverse medical issues such as respiratory failure and aspiration. The investigators postulate that analyzing the overall divergence of (impaired) speech, from a "normal" baseline, will prove to be more robust and a better marker for involvement than others that have been proposed.

Specific research questions to be addressed by this study are: (1) Is it possible to train a speech recognition system to adapt to increasingly more frequent language/speech errors of particular types, to produce an accurate textual transcript that would be readable by an ALS patient's caregiver or physician? (2) Are specific changes in physiological functioning: Forced Vital Capacity, tongue strength, speech velocity, weight (loss), aspiration risk, or psychological distress, reflected in different types of language/speech errors associated with ALS?

By understanding how speech functioning correlates with the degree to which other biophysical functioning has degraded, it is possible to apply a new, non-invasive measure for assessing the functionality of an ALS patient. In addition, the features associated with speech degradation it is possible to adapt existing speech recognition software to a patient's speech as it evolves over time, so that the quality of life for patients may be improved through conversation with a computer.

Respiratory failure is the main cause of morbidity and mortality in ALS patients. The investigators expect that the method of analyzing speech will present an excellent biomarker for respiratory function, as there is an expected increase in pauses during speech due to the necessity of increased frequency of respirations, a decrease in loudness, and decreased overall velocity of speech. A second major cause of death is aspiration. As the articular muscles decline, the investigators expect to note a decrease in the clarity of speech. Speech involvement often precedes swallowing involvement in ALS; thus, the investigators expect that increasing "speech divergence" will indicate potential aspiration risk.

• Study Type: Observational
• Enrollment (Actual): 34

Contacts and Locations

Study Locations

• United States
  • Florida
    • Tampa, Florida, United States, 33612
      • James A. Haley Veterans' Hospital, Tampa, FL

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

A Cohort of Veteran diagnosed with ALS seen at the Tampa VA

Description

Inclusion Criteria:

• Veterans will be diagnosed with ALS
• Native speakers of U.S. English
• Will have bulbar involvement identified during initial ALS inpatient evaluation
• Forced vital capacity (FVC) of greater than 50% of the expected value for age
• An Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) Score of 40 or greater

Exclusion Criteria:

• A diagnosis of dementia
• FVC less than 50%
• Inability to speak
• Or inability to follow directions

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort
Veterans With ALS; This Cohort Study will admit all eligible, interested Veterans diagnosed with ALS who meet the inclusion criteria.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in Percent of Expected Lung Force Vital Capacity	Forced Vital Capacity (FVC) measures the ability of the lung to move air. The change in FVC was calculated from the initial time point to either the end of study (24 months) or to final visit due to either death or loss to followup.	Baseline, and every 3 months up to 24 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in Tongue Strength	A physiological measurement of tongue muscle strength, in multiple directions. A force measured with Iowa Oral Performance Instrument (IOPI)	Baseline, 3 Month intervals up to 24 months, calculated from the initial time point to either the end of study (24 months) or to final visit due to either death or loss to followup
Change in Speech - as Measured by Speech Divergence	A measure looking at changes in vocal characteristics away from standard language. Speech Divergence is a novel calculated measure based on identifying changes to 13 characteristics of speech. The score can range from 0-13. Zero would represent normal speech, while 13 would be distorted speech along all parameters.	Baseline, 3 Month intervals up to 24 months, calculated from the initial time point to either the end of study (24 months) or to final visit due to either death or loss to followup

Collaborators and Investigators

• Sponsor: VA Office of Research and Development
• Collaborators: Florida Institute for Human and Machine Cognition

Study record dates

Study Major Dates

• Study Start (Actual): January 1, 2016
• Primary Completion (Actual): December 31, 2017
• Study Completion (Actual): December 31, 2017

Study Registration Dates

• First Submitted: January 21, 2016
• First Submitted That Met QC Criteria: February 2, 2016
• First Posted (Estimate): February 5, 2016

Study Record Updates

• Last Update Posted (Actual): August 18, 2020
• Last Update Submitted That Met QC Criteria: August 7, 2020
• Last Verified: August 1, 2020

More Information

Terms related to this study

• Keywords: Amyotrophic Lateral Sclerosis; Voice Quality; Voice; Total Lung Capacity
• Additional Relevant MeSH Terms: Pathologic Processes; Metabolic Diseases; Central Nervous System Diseases; Nervous System Diseases; Neuromuscular Diseases; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Sclerosis; Motor Neuron Disease; Amyotrophic Lateral Sclerosis
• Other Study ID Numbers: N1902-P; I21RX001902 (U.S. NIH Grant/Contract)

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED

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