Listener Effort Quantifies Clinically Meaningful Progression of Dysarthria in People Living with Amyotrophic Lateral Sclerosis

Overview

Journal medRxiv

Date 2024 Jun 10

PMID 38853969

Authors

Indu Navar Bingham

Raquel Norel

Esteban G Roitberg

Julian Peller

Marcos A Trevisan

Carla Agurto

Diego E Shalom

Felipe Aguirre

Iair Embon

Alan Taitz

Donna Harris

Amy Wright

Katie Seaver

Stacey Sullivan

Jordan R Green

Lyle W Ostrow

Ernest Fraenkel

James D Berry

Affiliations

Soon will be listed here.

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative motor neuron disease that causes progressive muscle weakness. Progressive bulbar dysfunction causes dysarthria and thus social isolation, reducing quality of life. The Everything ALS Speech Study obtained longitudinal clinical information and speech recordings from 292 participants. In a subset of 120 participants, we measured speaking rate (SR) and listener effort (LE), a measure of dysarthria severity rated by speech pathologists from recordings. LE intra- and inter-rater reliability was very high (ICC 0.88 to 0.92). LE correlated with other measures of dysarthria at baseline. LE changed over time in participants with ALS (slope 0.77 pts/month; p<0.001) but not controls (slope 0.005 pts/month; p=0.807). The slope of LE progression was similar in all participants with ALS who had bulbar dysfunction at baseline, regardless of ALS site of onset. LE could be a remotely collected clinically meaningful clinical outcome assessment for ALS clinical trials.

References

Berry J, Paganoni S, Carlson K, Burke K, Weber H, Staples P . Design and results of a smartphone-based digital phenotyping study to quantify ALS progression. Ann Clin Transl Neurol. 2019; 6(5):873-881. PMC: 6529832. DOI: 10.1002/acn3.770. View

Paganoni S, Macklin E, Hendrix S, Berry J, Elliott M, Maiser S . Trial of Sodium Phenylbutyrate-Taurursodiol for Amyotrophic Lateral Sclerosis. N Engl J Med. 2020; 383(10):919-930. PMC: 9134321. DOI: 10.1056/NEJMoa1916945. View

Lirani-Silva C, Mourao L, Gobbi L . Dysarthria and Quality of Life in neurologically healthy elderly and patients with Parkinson's disease. Codas. 2015; 27(3):248-54. DOI: 10.1590/2317-1782/20152014083. View

Rowe H, Stipancic K, Campbell T, Yunusova Y, Green J . The association between longitudinal declines in speech sound accuracy and speech intelligibility in speakers with amyotrophic lateral sclerosis. Clin Linguist Phon. 2023; 38(3):227-248. PMC: 10613582. DOI: 10.1080/02699206.2023.2202297. View

Koo T, Li M . A Guideline of Selecting and Reporting Intraclass Correlation Coefficients for Reliability Research. J Chiropr Med. 2016; 15(2):155-63. PMC: 4913118. DOI: 10.1016/j.jcm.2016.02.012. View

Baxi E, Thompson T, Li J, Kaye J, Lim R, Wu J . Answer ALS, a large-scale resource for sporadic and familial ALS combining clinical and multi-omics data from induced pluripotent cell lines. Nat Neurosci. 2022; 25(2):226-237. PMC: 8825283. DOI: 10.1038/s41593-021-01006-0. View

Beukenhorst A, Burke K, Scheier Z, Miller T, Paganoni S, Keegan M . Using Smartphones to Reduce Research Burden in a Neurodegenerative Population and Assessing Participant Adherence: A Randomized Clinical Trial and Two Observational Studies. JMIR Mhealth Uhealth. 2022; 10(2):e31877. PMC: 8857693. DOI: 10.2196/31877. View

Desjardins J . The Effects of Hearing Aid Directional Microphone and Noise Reduction Processing on Listening Effort in Older Adults with Hearing Loss. J Am Acad Audiol. 2016; 27(1):29-41. DOI: 10.3766/jaaa.15030. View

Rowe H, Gochyyev P, Lammert A, Lowit A, Spencer K, Dickerson B . The efficacy of acoustic-based articulatory phenotyping for characterizing and classifying four divergent neurodegenerative diseases using sequential motion rates. J Neural Transm (Vienna). 2022; 129(12):1487-1511. PMC: 9859630. DOI: 10.1007/s00702-022-02550-0. View

10.

Norel R, Agurto C, Heisig S, Rice J, Zhang H, Ostrand R . Speech-based characterization of dopamine replacement therapy in people with Parkinson's disease. NPJ Parkinsons Dis. 2020; 6:12. PMC: 7293295. DOI: 10.1038/s41531-020-0113-5. View

11.

Stipancic K, Palmer K, Rowe H, Yunusova Y, Berry J, Green J . "You Say Severe, I Say Mild": Toward an Empirical Classification of Dysarthria Severity. J Speech Lang Hear Res. 2021; 64(12):4718-4735. PMC: 9150682. DOI: 10.1044/2021_JSLHR-21-00197. View

12.

Korner S, Sieniawski M, Siniawski M, Kollewe K, Rath K, Krampfl K . Speech therapy and communication device: impact on quality of life and mood in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2012; 14(1):20-5. DOI: 10.3109/17482968.2012.692382. View

13.

Rowe H, Shellikeri S, Yunusova Y, Chenausky K, Green J . Quantifying articulatory impairments in neurodegenerative motor diseases: A scoping review and meta-analysis of interpretable acoustic features. Int J Speech Lang Pathol. 2022; 25(4):486-499. PMC: 9950294. DOI: 10.1080/17549507.2022.2089234. View

14.

Beukelman D, Fager S, Nordness A . Communication Support for People with ALS. Neurol Res Int. 2011; 2011:714693. PMC: 3096454. DOI: 10.1155/2011/714693. View

15.

Vieira F, Venugopalan S, Premasiri A, McNally M, Jansen A, McCloskey K . A machine-learning based objective measure for ALS disease severity. NPJ Digit Med. 2022; 5(1):45. PMC: 8993812. DOI: 10.1038/s41746-022-00588-8. View

16.

Leite Neto L, Franca Junior M, Chun R . Speech intelligibility in people with Amyotrophic Lateral Sclerosis (ALS). Codas. 2021; 33(1):e20190214. DOI: 10.1590/2317-1782/20202019214. View

17.

Cedarbaum J, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B . The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci. 1999; 169(1-2):13-21. DOI: 10.1016/s0022-510x(99)00210-5. View

18.

Green J, Beukelman D, Ball L . Algorithmic Estimation of Pauses in Extended Speech Samples of Dysarthric and Typical Speech. J Med Speech Lang Pathol. 2010; 12(4):149-154. PMC: 2902000. View

19.

Eshghi M, Yunusova Y, Connaghan K, Perry B, Maffei M, Berry J . Rate of speech decline in individuals with amyotrophic lateral sclerosis. Sci Rep. 2022; 12(1):15713. PMC: 9489769. DOI: 10.1038/s41598-022-19651-1. View

20.

Rong P, Yunusova Y, Wang J, Green J . Predicting Early Bulbar Decline in Amyotrophic Lateral Sclerosis: A Speech Subsystem Approach. Behav Neurol. 2015; 2015:183027. PMC: 4468279. DOI: 10.1155/2015/183027. View