Behavioral Management of Respiratory/Phonatory Dysfunction for Dysarthria Associated With Neurodegenerative Disease: A Systematic Review.

PURPOSE: This systematic review represents an update to previous reviews of the literature addressing behavioral management of respiratory/phonatory dysfunction in individuals with dysarthria due to neurodegenerative disease. METHOD: Multiple electronic database searches and hand searches of prominent speech-language pathology journals were conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards. RESULTS: The search yielded 1,525 articles, from which 88 met inclusion criteria and were reviewed by two blinded co-investigators. A large range of therapeutic approaches have been added to the evidence base since the last review, including expiratory muscle strength training, singing, and computer- and device-driven programs, as well as a variety of treatment modalities, including teletherapy. Evidence for treatment in several different population groups-including cerebellar ataxia, myotonic dystrophy, autosomal recessive spastic ataxia of Charlevoix-Saguenay, Huntington's disease, multiple system atrophy, and Lewy body dementia-were added to the current review. Synthesis of evidence quality provided strong evidence in support of only one behavioral intervention: Lee Silverman Voice Treatment Program (LSVT LOUD) in people with Parkinson's disease. No other treatment approach or population included in this review demonstrated more than limited evidence, reflecting that these approaches/populations require urgent further examination. CONCLUSION: Suggestions about where future research efforts could be significantly strengthened and how clinicians can apply research findings to their practice are provided. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.24964473.

Sensorimotor Cough Dysfunction in Cerebellar Ataxias.

Cerebellar ataxias are neurological conditions with a high prevalence of aspiration pneumonia and dysphagia. Recent research shows that sensorimotor cough dysfunction is associated with airway invasion and dysphagia in other neurological conditions and may increase the risk of pneumonia. Therefore, this study aimed to characterize sensorimotor cough function and its relationship with ataxia severity. Thirty-seven participants with cerebellar ataxia completed voluntary and/or reflex cough testing. Ataxia severity was assessed using the Scale for the Assessment and Rating of Ataxia (SARA). Linear multilevel models revealed voluntary cough peak expiratory flow rate (PEFR) estimates of 2.61 L/s and cough expired volume (CEV) estimates of 0.52 L. Reflex PEFR (1.82 L/s) and CEV (0.34 L) estimates were lower than voluntary PEFR and CEV estimates. Variability was higher for reflex PEFR (15.74% coefficient of variation [CoV]) than voluntary PEFR (12.13% CoV). 46% of participants generated at least two, two-cough responses following presentations of reflex cough stimuli. There was a small inverse relationship between ataxia severity and voluntary PEFR (ß = -0.05, 95% CI: -0.09 - -0.01 L) and ataxia severity and voluntary CEV (ß = -0.01, 95% CI: -0.02 - -0.004 L/s). Relationships between reflex cough motor outcomes (PEFR ß = 0.03, 95% CI: -0.007-0.07 L/s; CEV ß = 0.007, 95% CI: -0.004-0.02 L) and ataxia severity were not statistically robust. Results indicate that voluntary and reflex cough sensorimotor dysfunction is present in cerebellar ataxias and that increased severity of ataxia symptoms may impact voluntary cough function.

Quantifying Impairments in Swallowing Safety and Efficiency in Progressive Supranuclear Palsy and Parkinson's Disease.

Dysphagia is a largely inevitable symptom in both progressive supranuclear palsy (PSP) and Parkinson's disease (PD). To date, comparative studies in these diseases have failed to detect differences in the severity of impairments in swallowing safety or efficiency, potentially due to small sample sizes and outcome measures with low sensitivity. Therefore, this study sought to address these limitations by using novel measurement methodology to comprehensively compare swallowing safety and efficiency impairments between these populations in order to better understand whether differences may exist and guide clinical management. Twenty-four participants with PSP and 24 with PD were matched for disease duration and completed flexible endoscopic evaluations of swallowing. A visual analog scale and penetration-aspiration scale quantified swallowing safety and efficiency. Bayesian multilevel models compared the frequency, severity, and variability of swallowing impairments. Individuals with PSP demonstrated greater impairments in swallowing safety, including deeper and more variable airway invasion and more frequent vocal fold and subglottic residue. Swallowing efficiency was also more impaired among individuals with PSP, including more frequent hypopharyngeal residue (with solids) and more severe residue in the oropharynx (with thin liquids and solids) and hypopharynx (with thin liquids). When airway or pharyngeal residue was present, similar within-subject variability of the amount of residue was appreciated across anatomic landmarks. This is the first study comparing the frequency, severity, and variability of swallowing impairments between PSP and PD populations. Our findings demonstrate more pronounced impairments in swallowing safety and efficiency for PSP compared to PD. These findings provide a clinically relevant characterization of swallowing measures using novel methodological and statistical approaches attempting to resolve some limitations of prior studies.

Telehealth Management of Dysphagia in Adults: A Survey of Speech Language Pathologists' Experiences and Perceptions.

The goal of this study was to explore telehealth use for dysphagia management in response to COVID-19 to understand variables associated with clinician confidence and perceived effectiveness of this service delivery model and determine clinician-perceived benefits and challenges of managing dysphagia via telehealth. Speech-language pathologists (SLPs, n = 235) completed a web-based survey, providing information on demographics, telehealth use during the pandemic, and perspectives on current and future tele-management of dysphagia. Analyses included descriptive statistics to examine usage patterns; logistic regression to determine which variables were associated with telehealth use, clinician confidence, and perceived-effectiveness; and conventional content analysis to analyze responses to open-ended questions. Results revealed a sharp increase in the tele-management of dysphagia during the pandemic. Years of experience with dysphagia management (p = .031) and pre-pandemic use of telehealth (p < .001) were significantly associated with current use patterns. Working in the outpatient setting was associated with greater clinician confidence (p = .003) and perceived effectiveness (p = .007), and use of guidelines (p = .042) was also associated with greater clinician confidence. Key challenges identified included inadequate technological infrastructure, inadequate patient digital literacy, and reimbursement restrictions. Key benefits were treatment continuity, improving access to care, and time savings. The majority (67%) of respondents reported that they would use telehealth in the future. These findings demonstrate SLPs' abilities and desire to expand their practice patterns to include telehealth for dysphagia management. Therefore, clinician training and more research on best practices for assessment and treatment of dysphagia via telehealth is warranted to refine models of care for dysphagia tele-management.

Rehabilitating Cough Dysfunction in Parkinson's Disease: A Randomized Controlled Trial.

BACKGROUND: Disorders of airway protection (cough and swallowing) are pervasive in Parkinson's disease (PD) resulting in a high incidence of aspiration pneumonia and death. However, there are no randomized controlled trials comparing strength and skill-based approaches to improve airway protection in PD. OBJECTIVES: The aim of this study was to compare expiratory muscle strength training (EMST) and sensorimotor training for airway protection (smTAP) to improve cough-related outcomes in people with PD. METHODS: Participants with PD and dysphagia were recruited for this prospective phase II randomized-blinded controlled clinical trial. Participants completed baseline assessment, 5 weeks of EMST or smTAP, and a post-training assessment. Primary outcome measures included maximum expiratory pressure (MEP) and voluntary cough peak expiratory flow rate (PEFR). Mixed effects models were used to assess the effects of EMST and smTAP on outcomes. RESULTS: A total of 65 participants received either EMST (n = 34) or smTAP (n = 31). MEP improved from pre- to post-treatment for smTAP (P < 0.001, d = 0.19) and EMST (P < 0.001, d = 0.53). Voluntary PEFR increased from pre- to post-treatment for smTAP (P < 0.001, d = 0.19) and EMST (P < 0.001, d = 0.06). Moreover, reflex cough PEFR (P < 0.001, d = 0.64), reflex cough expired volume (P < 0.001, d = 0.74), and urge to cough (P = 0.018, OR = 2.70) improved for the smTAP group but not for the EMST group. CONCLUSIONS: This clinical trial confirmed the efficacy of smTAP to improve reflex and voluntary cough function, above and beyond EMST, the current gold standard. © 2022 International Parkinson and Movement Disorder Society.

The Relationship Between Lingual Strength and Functional Swallowing Outcomes in Parkinson's Disease.

The purpose of this retrospective study was to determine whether reduced lingual strength was associated with functional swallowing outcomes in individuals with Parkinson's disease (PD). Participants (N = 42) completed evaluations of maximal lingual isometric pressure (MIP) and mean lingual swallowing pressure (MSP), and flexible endoscopic evaluations of swallowing. Regression models were used to determine the association between lingual strength and functional swallowing outcomes of airway invasion, the presence of post-swallow pharyngeal residue, and the amount of pharyngeal residue (when present). Results revealed that higher MIP (p = 0.002, OR 0.93) and higher MSP (p = 0.001 OR 0.88) were associated with less airway invasion of thin liquids. Both MIP and MSP were able to differentiate between those with and without dysphagia (MIP: AUC 0.7935, p = 0.001; MSP: AUC 0.75, p = 0.026). Neither MIP nor MSP was related to the presence of residue. However, when thin liquid oropharyngeal residue was present, both MIP (p < 0.001, OR 0.99) and MSP (p < 0.001; OR 0.98) were significantly associated with the amount of residue observed. Similarly, when thin liquid hypopharyngeal residue was present, MIP (p < 0.001, OR 0.99) and MSP (p < 0.001, OR 0.98) were associated with the amount of residue observed. These findings suggest a relationship between reduced lingual strength and worse thin liquid swallowing safety and efficiency; however, the magnitude of these effects was small. This indicates that lingual strength is one important contributing factor to functional swallowing impairments in PD and may identify those with unsafe swallowing. These findings have important clinical implications for including lingual strength in the screening, assessment, and management of dysphagia in PD.

Rehabilitation of Airway Protection in Individuals With Movement Disorders: A Telehealth Feasibility Study.

PURPOSE: Airway protective deficits (swallowing and cough) greatly reduce health and quality of life and are a pervasive consequence of neurodegenerative movement disorders. Expiratory muscle strength training (EMST) and cough skill training (CST) are two treatment approaches to improve airway protection; however, many patients are unable to access these treatments. Telehealth may improve access to care, but it remains unknown whether these treatments are feasible and efficacious via telehealth. This study aimed to determine the practical feasibility and preliminary treatment effect of EMST and CST via telehealth. METHOD: Twenty participants with movement disorders completed 4 weeks of EMST and 2 weeks of CST, including two clinician-directed treatment sessions via telehealth and 3 days of home practice per week. Feasibility was calculated for each treatment. Practical feasibility was defined as completing treatment (EMST or CST) and obtaining the relevant outcome measures-a proxy of maximum expiratory pressure (pMEP) for EMST and peak expiratory flow rate (PEFR) for CST-within a 30-min session/period. Session factors that may have influenced feasibility were examined. Preliminary treatment effect was defined as changes in pMEP and PEFR. RESULTS: Time taken to obtain pMEP and complete EMST was 17.48 min, and time taken to obtain PEFR and complete CST was 17.69 min. pMEP, single voluntary cough PEFR, and sequential voluntary cough PEFR increased from pre- to posttreatment. CONCLUSIONS: Findings suggest that the delivery of EMST and CST is feasible via telehealth and yield improvements to pMEP and PEFR. This has important implications for expanding service delivery of airway protective interventions and reducing health care disparities in people with neurodegenerative movement disorders. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.21357669.

Immediate Effects of Sensorimotor Training in Airway Protection (smTAP) on Cough Outcomes in Progressive Supranuclear Palsy: A Feasibility Study.

Progressive supranuclear palsy (PSP) is a neurodegenerative disease characterized by a high prevalence of dysphagia, cough dysfunction, and resultant aspiration pneumonia. Sensorimotor cough function is important for airway clearance in people with dysphagia. Upregulation of cough has been demonstrated in healthy adults and Parkinson's disease; however, the feasibility of cough rehabilitation in PSP is unknown. We sought to assess feasibility by examining the immediate effects of a novel sensorimotor training in airway protection (smTAP) on upregulation of cough function in PSP. Fifteen individuals with PSP enrolled in this study. Baseline voluntary and reflex cough testing were completed. During smTAP, participants were presented with subthreshold capsaicin and instructed to cough with sufficient intensity to hit a target line (set 25% above baseline reflex peak cough flow) via cough airflow visual biofeedback. Twenty-five repetitions were targeted within a single session. Wilcoxon signed-rank tests compared cough airflow measures between baseline voluntary cough testing, the initial five trials of smTAP, and final five trials. Mean peak expiratory flow rate (PEFR) significantly increased from initial to final smTAP trials (p < 0.001). Fourteen participants increased PEFR, with gains of more than 10% in 11 participants. Variability of PEFR (p = 0.01) and cough expired volume (p = 0.01) significantly decreased across smTAP trials. This study is the first to demonstrate the ability of people with PSP to immediately upregulate cough function, providing preliminary support for the feasibility of cough rehabilitation in this population with this novel treatment approach. Future research examining the effects of multiple sessions of smTAP on cough outcomes is warranted.

Sensorimotor Cough Dysfunction Is Prevalent and Pervasive in Progressive Supranuclear Palsy.

BACKGROUND: Pneumonia, a leading cause of death in progressive supranuclear palsy (PSP), results from progressive and pervasive deficits of airway protection, including both cough and swallowing dysfunction. Cough protects the airway by expelling aspirate and may be an important therapeutic target to protect against pneumonia in the presence of dysphagia. However, cough has not been objectively characterized in PSP or compared to other common forms of parkinsonism, such as Parkinson's disease (PD). OBJECTIVE: The purpose of this study was to examine voluntary and reflex cough function in PSP, as compared to patients with PD matched for disease duration. METHODS: Twenty-six patients with PSP and 26 with PD completed voluntary and reflex cough testing via spirometry. Linear mixed effects models examined comparisons between groups and within cough types across cough sensory and motor outcomes. RESULTS: Patients with PSP demonstrated significantly reduced cough motor function compared to PD, specifically reduced peak expiratory flow rate (P < 0.001), cough expiratory volume (P < 0.001), and cough inspiratory volume (P = 0.008). Both groups showed similar reflex cough thresholds (P = 0.694), but PSP demonstrated an increased perception of cough stimuli (P = 0.041). CONCLUSIONS: These findings suggest that sensorimotor cough dysfunction is prevalent in PSP, and cough motor deficits, in particular, are worse in PSP than in PD. These deficits likely contribute to the pathogenesis of pneumonia in PSP. Therefore, cough should be integrated into assessments of airway protection and considered as a therapeutic target to potentially reduce adverse health events and improve quality of life in this population. © 2021 International Parkinson and Movement Disorder Society.

Obtaining Objective Clinical Measures During Telehealth Evaluations of Dysarthria.

Purpose COVID-19 has shifted models of health care delivery, requiring the rapid adoption of telehealth, despite limited evidence and few resources to guide speech-language pathologists. Management of dysarthria presents specific challenges in the telehealth modality. Evaluations of dysarthria typically rely heavily on perceptual judgments, which are difficult to obtain via telehealth given a variety of technological factors such as inconsistencies in mouth-to-microphone distance, changes to acoustic properties based on device settings, and possible interruptions in connection that may cause video freezing. These factors limit the validity, reliability, and clinicians' certainty of perceptual speech ratings via telehealth. Thus, objective measures to supplement the assessment of dysarthria are essential. Method This tutorial outlines how to obtain objective measures in real time and from recordings of motor speech evaluations to support traditional perceptual ratings in telehealth evaluations of dysarthria. Objective measures include pause patterns, utterance length, speech rate, diadochokinetic rates, and overall speech severity. We demonstrate, through clinical case vignettes, how these measures were completed following three clinical telehealth evaluations of dysarthria conducted via Zoom during the COVID-19 pandemic. This tutorial describes how each of these objective measures were utilized, in combination with subjective perceptual analysis, to determine deviant speech characteristics and their etiology, develop a patient-specific treatment plan, and track change over time. Conclusion Utilizing objective measures as an adjunct to perceptual ratings for telehealth dysarthria evaluations is feasible under real-world pandemic conditions and can be used to enhance the quality and utility of these evaluations.

Objective and Subjective Clinical Swallowing Outcomes via Telehealth: Reliability in Outpatient Clinical Practice.

Purpose The COVID-19 pandemic has drastically increased the use of telehealth. Prior studies of telehealth clinical swallowing evaluations provide positive evidence for telemanagement of swallowing. However, the reliability of these measures in clinical practice, as opposed to well-controlled research conditions, remains unknown. This study aimed to investigate the reliability of outcome measures derived from clinical swallowing tele-evaluations in real-world clinical practice (e.g., variability in devices and Internet connectivity, lack of in-person clinician assistance, or remote patient/caregiver training). Method Seven raters asynchronously judged clinical swallowing tele-evaluations of 12 movement disorders patients. Outcomes included the Timed Water Swallow Test (TWST), Test of Masticating and Swallowing Solids (TOMASS), and common observations of oral intake. Statistical analyses were performed to examine inter- and intrarater reliability, as well as qualitative analyses exploring patient and clinician-specific factors impacting reliability. Results Forty-four trials were included for reliability analyses. All rater dyads demonstrated "good" to "excellent" interrater reliability for measures of the TWST (intraclass correlation coefficients [ICCs] ≥ .93) and observations of oral intake (≥ 77% agreement). The majority of TOMASS outcomes demonstrated "good" to "excellent" interrater reliability (ICCs ≥ .84), with the exception of the number of bites (ICCs = .43-.99) and swallows (ICCs = .21-.85). Immediate and delayed intrarater reliability were "excellent" for most raters across all tasks, ranging between ICCs of .63 and 1.00. Exploratory factors potentially impacting reliability included infrequent instances of suboptimal video quality, reduced camera stability, camera distance, and obstruction of the patient's mouth during tasks. Conclusions Subjective observations of oral intake and objective measures taken from the TWST and the TOMASS can be reliably measured via telehealth in clinical practice. Our results provide support for the feasibility and reliability of telehealth for outpatient clinical swallowing evaluations during COVID-19 and beyond. Supplemental Material https://doi.org/10.23641/asha.13661378.

The Role of Vocal Fold Bowing on Cough and Swallowing Dysfunction in Progressive Supranuclear Palsy.

OBJECTIVES: Progressive supranuclear palsy (PSP) is a neurodegenerative disease which results in cough and swallowing dysfunction and aspiration pneumonia. Relationships among vocal fold atrophy, cough, and swallowing have been identified in related diseases, but remain unknown in PSP. This study examined: 1) the prevalence of vocal fold bowing in PSP, and 2) the influence of vocal fold bowing on cough and swallowing in PSP. STUDY DESIGN: Prospective Cohort Study. METHODS: Twenty-three participants with PSP completed instrumental assessments of cough and swallowing. Vocal fold bowing (BI) and swallowing safety (PAS) was assessed using flexible laryngoscopy. Measures of cough effectiveness were obtained using spirometry. Statistical analyses were used to determine the frequency of mild-moderate (BI > 0) and severe (BI > 12.2) bowing, and to assess the influence of BI on PAS and cough effectiveness in PSP. RESULTS: Fifty-two percent (n = 12) of participants exhibited severe bowing while 48% (n = 11) exhibited mild-to-moderate bowing. Voluntary cough peak expiratory flow rate (P = .01), as well as reflex (P = .02) and voluntary (P = .005) cough volume acceleration were lower for participants with severe BI when compared to mild-to-moderate BI. However, BI did not influence PAS (P > .05). CONCLUSIONS: Findings from this study suggest that vocal fold bowing is highly prevalent in PSP and associated with reduced reflex and voluntary cough effectiveness. These findings provide insight into the pathophysiology of compromised airway protection in this patient population. Future studies should examine vocal fold atrophy as a treatment target for behavioral and medical intervention in PSP. LEVEL OF EVIDENCE: 3 (Prospective Observational Study) Laryngoscope, 131:1217-1222, 2021.