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Multidimensional Measurements of Dysarthria in Myotonic Dystrophy Type 1

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Title: Multidimensional Measurements of Dysarthria in Myotonic Dystrophy Type 1
Language: English
Authors: Sanne van Hellemond, Nicole Voet (ORCID 0000-0001-7381-2817), Rosemarie Kroon (ORCID 0000-0002-6609-0649), Yelda Sahin, Ilse Karnebeek, Marthè Nijkamp, Simone Knuijt (ORCID 0000-0002-9277-2643)
Source: International Journal of Language & Communication Disorders. 2026 61(3).
Availability: Wiley. Available from: John Wiley & Sons, Inc. 111 River Street, Hoboken, NJ 07030. Tel: 800-835-6770; e-mail: cs-journals@wiley.com; Web site: https://www.wiley.com/en-us
Peer Reviewed: Y
Page Count: 13
Publication Date: 2026
Document Type: Journal Articles
Reports - Research
Descriptors: Human Body, Physical Disabilities, Speech Impairments, Neurological Impairments, Genetic Disorders, Heredity, Fatigue (Biology), Intelligibility, Adults, Self Evaluation (Individuals), Articulation (Speech), Severity (of Disability), Attitudes toward Disabilities, Foreign Countries
Geographic Terms: Netherlands
DOI: 10.1111/1460-6984.70239
ISSN: 1368-2822
1460-6984
Abstract: Background: Myotonic dystrophy type 1 (DM1) is a heterogeneous neuromuscular disorder characterized by progressive muscle weakness and myotonia. Dysarthria is a known symptom of DM1, but literature is lacking about the patient's own perception in relationship to dysarthria characteristics and severity. Aims: The aim of the study was to describe the acoustic speech characteristics of dysarthria in patients with DM1, examine the perceptually determined dysarthria severity through speech and language therapy assessment, gather subjective evaluations of speech and intelligibility from patients and relatives and examine the relationship between these outcomes. Methods and Procedures: The speech of 22 adult patients with DM1 (nine females) was acoustically assessed during spontaneous speech, reading, and maximum performance tasks and analysed using the Praat-software. Dysarthria severity was rated on a severity scale from 0 (no dysarthria)-5 (very severe dysarthria/anarthria). Patients and relatives rated the speech with a short questionnaire and a visual analogue scale (VAS). Outcomes and Results: Acoustic analysis showed a deviant speech rate (SR), articulation rate (AR), maximum phonation volume (MPV), and fundamental frequency range compared to normative values. Perceptually, the dysarthria severity scores varied between 1 (minimal dysarthria) and 4 (severe dysarthria). In more severe dysarthria, SR, AR, and MPV decreased. Patients were sufficiently satisfied about their speech, with no relationship to dysarthria severity. However, the scores of relatives decreased when perceptual dysarthria severity increased. Conclusion and Implications: As dysarthria severity increased, speech quality and intelligibility declined, particularly when assessed by speech therapists and relatives. Patients with DM1 generally reported minimal conversational restrictions due to dysarthria. Multidimensional measurements may improve the understanding of speech impairment in DM1. Self-awareness should be a topic in speech therapy interventions.
Abstractor: As Provided
Entry Date: 2026
Accession Number: EJ1506868
Database: ERIC
FullText Text:
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  Data: Multidimensional Measurements of Dysarthria in Myotonic Dystrophy Type 1
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  Data: <searchLink fieldCode="AR" term="%22Sanne+van+Hellemond%22">Sanne van Hellemond</searchLink><br /><searchLink fieldCode="AR" term="%22Nicole+Voet%22">Nicole Voet</searchLink> (ORCID <externalLink term="https://orcid.org/0000-0001-7381-2817">0000-0001-7381-2817</externalLink>)<br /><searchLink fieldCode="AR" term="%22Rosemarie+Kroon%22">Rosemarie Kroon</searchLink> (ORCID <externalLink term="https://orcid.org/0000-0002-6609-0649">0000-0002-6609-0649</externalLink>)<br /><searchLink fieldCode="AR" term="%22Yelda+Sahin%22">Yelda Sahin</searchLink><br /><searchLink fieldCode="AR" term="%22Ilse+Karnebeek%22">Ilse Karnebeek</searchLink><br /><searchLink fieldCode="AR" term="%22Marthè+Nijkamp%22">Marthè Nijkamp</searchLink><br /><searchLink fieldCode="AR" term="%22Simone+Knuijt%22">Simone Knuijt</searchLink> (ORCID <externalLink term="https://orcid.org/0000-0002-9277-2643">0000-0002-9277-2643</externalLink>)
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  Data: <searchLink fieldCode="SO" term="%22International+Journal+of+Language+%26+Communication+Disorders%22"><i>International Journal of Language & Communication Disorders</i></searchLink>. 2026 61(3).
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  Data: Wiley. Available from: John Wiley & Sons, Inc. 111 River Street, Hoboken, NJ 07030. Tel: 800-835-6770; e-mail: cs-journals@wiley.com; Web site: https://www.wiley.com/en-us
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  Data: 13
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  Data: <searchLink fieldCode="DE" term="%22Human+Body%22">Human Body</searchLink><br /><searchLink fieldCode="DE" term="%22Physical+Disabilities%22">Physical Disabilities</searchLink><br /><searchLink fieldCode="DE" term="%22Speech+Impairments%22">Speech Impairments</searchLink><br /><searchLink fieldCode="DE" term="%22Neurological+Impairments%22">Neurological Impairments</searchLink><br /><searchLink fieldCode="DE" term="%22Genetic+Disorders%22">Genetic Disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Heredity%22">Heredity</searchLink><br /><searchLink fieldCode="DE" term="%22Fatigue+%28Biology%29%22">Fatigue (Biology)</searchLink><br /><searchLink fieldCode="DE" term="%22Intelligibility%22">Intelligibility</searchLink><br /><searchLink fieldCode="DE" term="%22Adults%22">Adults</searchLink><br /><searchLink fieldCode="DE" term="%22Self+Evaluation+%28Individuals%29%22">Self Evaluation (Individuals)</searchLink><br /><searchLink fieldCode="DE" term="%22Articulation+%28Speech%29%22">Articulation (Speech)</searchLink><br /><searchLink fieldCode="DE" term="%22Severity+%28of+Disability%29%22">Severity (of Disability)</searchLink><br /><searchLink fieldCode="DE" term="%22Attitudes+toward+Disabilities%22">Attitudes toward Disabilities</searchLink><br /><searchLink fieldCode="DE" term="%22Foreign+Countries%22">Foreign Countries</searchLink>
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  Data: <searchLink fieldCode="DE" term="%22Netherlands%22">Netherlands</searchLink>
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  Data: 10.1111/1460-6984.70239
– Name: ISSN
  Label: ISSN
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  Data: 1368-2822<br />1460-6984
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Background: Myotonic dystrophy type 1 (DM1) is a heterogeneous neuromuscular disorder characterized by progressive muscle weakness and myotonia. Dysarthria is a known symptom of DM1, but literature is lacking about the patient's own perception in relationship to dysarthria characteristics and severity. Aims: The aim of the study was to describe the acoustic speech characteristics of dysarthria in patients with DM1, examine the perceptually determined dysarthria severity through speech and language therapy assessment, gather subjective evaluations of speech and intelligibility from patients and relatives and examine the relationship between these outcomes. Methods and Procedures: The speech of 22 adult patients with DM1 (nine females) was acoustically assessed during spontaneous speech, reading, and maximum performance tasks and analysed using the Praat-software. Dysarthria severity was rated on a severity scale from 0 (no dysarthria)-5 (very severe dysarthria/anarthria). Patients and relatives rated the speech with a short questionnaire and a visual analogue scale (VAS). Outcomes and Results: Acoustic analysis showed a deviant speech rate (SR), articulation rate (AR), maximum phonation volume (MPV), and fundamental frequency range compared to normative values. Perceptually, the dysarthria severity scores varied between 1 (minimal dysarthria) and 4 (severe dysarthria). In more severe dysarthria, SR, AR, and MPV decreased. Patients were sufficiently satisfied about their speech, with no relationship to dysarthria severity. However, the scores of relatives decreased when perceptual dysarthria severity increased. Conclusion and Implications: As dysarthria severity increased, speech quality and intelligibility declined, particularly when assessed by speech therapists and relatives. Patients with DM1 generally reported minimal conversational restrictions due to dysarthria. Multidimensional measurements may improve the understanding of speech impairment in DM1. Self-awareness should be a topic in speech therapy interventions.
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