Speech motor impairment in ALS is associated with multiregional cortical thinning beyond primary motor cortex.

Introduction: Cortical thinning is well-documented in individuals with amyotrophic lateral sclerosis (ALS), yet its association with speech deterioration remains understudied. This study characterizes anatomical changes in the brain within the context of speech impairment patterns in individuals with ALS, providing insight into the disease's multiregional spread and biology.

Methods: To evaluate patterns of cortical thickness in speakers with ALS with and without functional speech changes compared to healthy controls (HCs) using whole-brain and region of interest (ROI) analyses.

Automatic Speech Recognition of Conversational Speech in Individuals With Disordered Speech.

Purpose: This study examines the effectiveness of automatic speech recognition (ASR) for individuals with speech disorders, addressing the gap in performance between read and conversational ASR. We analyze the factors influencing this disparity and the effect of speech mode-specific training on ASR accuracy.

Method: Recordings of read and conversational speech from 27 individuals with various speech disorders were analyzed using both (a) one speaker-independent ASR system trained and optimized for typical speech and (b) multiple ASR models that were personalized to the speech of the participants with disordered speech.

Listener effort quantifies clinically meaningful progression of dysarthria in people living with amyotrophic lateral sclerosis.

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.

The relationship of rate and pause features to the communicative participation of people living with ALS.

Introduction/aims: Many people living with amyotrophic lateral sclerosis (PALS) report restrictions in their day-to-day communication (communicative participation). However, little is known about which speech features contribute to these restrictions. This study evaluated the effects of common speech symptoms in PALS (reduced overall speaking rate, slowed articulation rate, and increased pausing) on communicative participation restrictions.

Exploring Motor Speech Disorders in Low and Minimally Verbal Autistic Individuals: An Auditory-Perceptual Analysis.

Purpose: Motor deficits are widely documented among autistic individuals, and speech characteristics consistent with a motor speech disorder have been reported in prior literature. We conducted an auditory-perceptual analysis of speech production skills in low and minimally verbal autistic individuals as a step toward clarifying the nature of speech production impairments in this population and the potential link between oromotor functioning and language development.

Method: Fifty-four low or minimally verbal autistic individuals aged 4-18 years were video-recorded performing nonspeech oromotor tasks and producing phonemes, syllables, and words in imitation.

Individualized white matter connectivity of the articulatory pathway: An ultra-high field study.

In current sensorimotor theories pertaining to speech perception, there is a notable emphasis on the involvement of the articulatory-motor system in the processing of speech sounds. Using ultra-high field diffusion-weighted imaging at 7 Tesla, we visualized the white matter tracts connected to areas activated during a simple speech-sound production task in 18 healthy right-handed adults. Regions of interest for white matter tractography were individually determined through 7T functional MRI (fMRI) analyses, based on activations during silent vocalization tasks.

Vowel distinctiveness as a concurrent predictor of expressive language function in autistic children.

Speech ability may limit spoken language development in some minimally verbal autistic children. In this study, we aimed to determine whether an acoustic measure of speech production, vowel distinctiveness, is concurrently related to expressive language (EL) for autistic children. Syllables containing the vowels [i] and [a] were recorded remotely from 27 autistic children (4;1-7;11) with a range of spoken language abilities.

Oral diadochokinetic markers of X-linked dystonia-parkinsonism.

Introduction: X-linked dystonia-parkinsonism (XDP) is a neurodegenerative disorder that may result in severe speech impairment. The literature suggests that there are differences in the speech of individuals with XDP and healthy controls. This study aims to examine the motor speech characteristics of the mixed dystonia-parkinsonism phase of XDP.

O-GlcNAcylation regulates neurofilament-light assembly and function and is perturbed by Charcot-Marie-Tooth disease mutations.

The neurofilament (NF) cytoskeleton is critical for neuronal morphology and function. In particular, the neurofilament-light (NF-L) subunit is required for NF assembly in vivo and is mutated in subtypes of Charcot-Marie-Tooth (CMT) disease. NFs are highly dynamic, and the regulation of NF assembly state is incompletely understood.

Feedback From Automatic Speech Recognition to Elicit Clear Speech in Healthy Speakers.

Purpose: This study assessed the effectiveness of feedback generated by automatic speech recognition (ASR) for eliciting clear speech from young, healthy individuals. As a preliminary step toward exploring a novel method for eliciting clear speech in patients with dysarthria, we investigated the effects of ASR feedback in healthy controls. If successful, ASR feedback has the potential to facilitate independent, at-home clear speech practice.

Remote Assessment for ALS using Multimodal Dialog Agents: Data Quality, Feasibility and Task Compliance.

We investigate the feasibility, task compliance and audiovisual data quality of a multimodal dialog-based solution for remote assessment of Amyotrophic Lateral Sclerosis (ALS). 53 people with ALS and 52 healthy controls interacted with Tina, a cloud-based conversational agent, in performing speech tasks designed to probe various aspects of motor speech function while their audio and video was recorded. We rated a total of 250 recordings for audio/video quality and participant task compliance, along with the relative frequency of different issues observed.

Amyotrophic Lateral Sclerosis-Bulbar Dysfunction Index-Remote: Test-Retest and Interrater Reliability of Candidate Items.

Purpose: The primary aim of this study was to establish the reliability of candidate items as a step in the development of the Amyotrophic Lateral Sclerosis-Bulbar Dysfunction Index-Remote (ALS-BDI-Remote), a novel tool being developed for the detection and monitoring of bulbar signs and symptoms in remote settings.

Method: The set of candidate items included 40 items covering three domains: cranial nerve examination, auditory-perceptual evaluation, and functional assessment. Forty-eight participants diagnosed with ALS and exhibiting a range of bulbar disease severity were included.

Establishing a natural history of X-linked dystonia parkinsonism.

X-linked dystonia parkinsonism is a neurodegenerative movement disorder that affects men whose mothers originate from the island of Panay, Philippines. Current evidence indicates that the most likely cause is an expansion in the gene that may be amenable to treatment. To prepare for clinical trials of therapeutic candidates for X-linked dystonia parkinsonism, we focused on the identification of quantitative phenotypic measures that are most strongly associated with disease progression.

The association between longitudinal declines in speech sound accuracy and speech intelligibility in speakers with amyotrophic lateral sclerosis.

The purpose of this study was to examine how neurodegeneration secondary to amyotrophic lateral sclerosis (ALS) impacts speech sound accuracy over time and how speech sound accuracy, in turn, is related to speech intelligibility. Twenty-one participants with ALS read the Bamboo Passage over multiple data collection sessions across several months. Phonemic and orthographic transcriptions were completed for all speech samples.

Oromotor skills in autism spectrum disorder: A scoping review.

Oromotor functioning plays a foundational role in spoken communication and feeding, two areas of significant difficulty for many autistic individuals. However, despite years of research and established differences in gross and fine motor skills in this population, there is currently no clear consensus regarding the presence or nature of oral motor control deficits in autistic individuals. In this scoping review, we summarize research published between 1994 and 2022 to answer the following research questions: (1) What methods have been used to investigate oromotor functioning in autistic individuals? (2) Which oromotor behaviors have been investigated in this population? and (3) What conclusions can be drawn regarding oromotor skills in this population? Seven online databases were searched resulting in 107 studies meeting our inclusion criteria.

Acoustic Measures of Dysphonia in Amyotrophic Lateral Sclerosis.

Purpose: Identifying efficacious measures to characterize dysphonia in complex neurodegenerative diseases is key to optimal assessment and intervention. This study evaluates the validity and sensitivity of acoustic features of phonatory disruption in amyotrophic lateral sclerosis (ALS).

Method: Forty-nine individuals with ALS (40-79 years old) were audio-recorded while producing a sustained vowel and continuous speech.

Comorbidity and Severity in Childhood Apraxia of Speech: A Retrospective Chart Review.

Purpose: The purpose of this study was to investigate comorbidity prevalence and patterns in childhood apraxia of speech (CAS) and their relationship to severity.

Method: In this retroactive cross-sectional study, medical records for 375 children with CAS ( = 4;9 [years;months], = 2;9) were examined for comorbid conditions. The total number of comorbid conditions and the number of communication-related comorbidities were regressed on CAS severity as rated by speech-language pathologists during diagnosis.

Measuring Articulatory Patterns in Amyotrophic Lateral Sclerosis Using a Data-Driven Articulatory Consonant Distinctiveness Space Approach.

Purpose: The aim of this study was to leverage data-driven approaches, including a novel articulatory consonant distinctiveness space (ACDS) approach, to better understand speech motor control in amyotrophic lateral sclerosis (ALS).

Method: Electromagnetic articulography was used to record tongue and lip movement data during the production of 10 consonants from healthy controls ( = 15) and individuals with ALS ( = 47). To assess phoneme distinctness, speech data were analyzed using two classification algorithms, Procrustes matching (PM) and support vector machine (SVM), and the area/volume of the ACDS.

Face and content validation of the amyotrophic lateral sclerosis-Bulbar dysfunction index (ALS-BDI).

Purpose: Early detection and tracking of bulbar dysfunction in amyotrophic lateral sclerosis (ALS) are critical for directing management of the disease. Existing physiological assessments of bulbar dysfunction are often inaccessible and cost-prohibitive for clinical application. Existing clinical assessments are limited.

Review of methods for conducting speech research with minimally verbal individuals with autism spectrum disorder.

The purpose of this paper was to review best-practice methods of collecting and analyzing speech production data from minimally verbal autistic speakers. Data on speech production data in minimally verbal individuals are valuable for a variety of purposes, including phenotyping, clinical assessment, and treatment monitoring. Both perceptual ("by ear") and acoustic analyses of speech can reveal subtle improvements as a result of therapy that may not be apparent when correct/incorrect judgments are used.

Acoustic and Kinematic Assessment of Motor Speech Impairment in Patients With Suspected Four-Repeat Tauopathies.

Purpose: The aim of this study was to use acoustic and kinematic speech measures to characterize type of motor speech impairment-apraxia of speech (AOS) versus dysarthria-in individuals with four-repeat tauopathy (4RT)-associated syndromes, including nonfluent variant primary progressive aphasia (nfvPPA), primary progressive AOS (PPAOS), corticobasal syndrome (CBS), and progressive supranuclear palsy syndrome (PSPs).

Method: Twenty patient participants were recruited and stratified into two groups: (a) a motor-speech-impaired group of individuals with nfvPPA, PPAOS, CBS, or PSPs and suspected 4RT pathology ("MSI+") and (b) a non-motor-speech-impaired group of individuals with logopenic variant primary progressive aphasia ("MSI-"). Ten healthy, age-matched controls also participated in the study.