DySMA - an Instrument to Monitor Swallowing Function in Children with Spinal Muscular Atrophy ages 0 to 24 Months: Development, Consensus, and Pilot Testing.

Background: The manifestation of bulbar symptoms, especially swallowing, is important for evaluating disease-modifying therapies for spinal muscular atrophy (SMA). Due to the lack of instruments, the topic is still underrepresented in research. Objective: This study aimed to develop a tool to monitor swallowing development in children aged 0 to 24 months with SMA. Methods: The method was guided by the COSMIN guidelines and followed a multi-stage Delphi process. The first step was a rapid review of swallowing outcomes in children with SMA younger than 24 months. In the second step, online group interviews with experts (n = 7) on dysphagia in infants were conducted, followed by an anonymous online survey among experts in infants with SMA (n = 19). A predefined consensus threshold for nominal scaled voting was set at≥75 % and for 5-point Likert scale voting at 1.25 of the interquartile range. The third step was the pilot test of the instrument, performed with three groups (healthy controls n = 8; pre-symptomatic n = 6, symptomatic n = 6). Results: Based on the multi-level interprofessional consensus, the DySMA comprises two parts (history and examination), ten categories, with 36 items. Implementation and scoring are clearly articulated and easy to implement. The pilot test showed that swallowing development could be recorded in all groups. Conclusion: The DySMA is well suited for monitoring swallowing development in pre-symptomatic and symptomatic treated infants with SMA. It can be performed in a time-efficient and interprofessional manner. The resulting score is comparable to results from other instruments measuring other domains, e.g., motor function.

Narrow Band Imaging in Flexible Endoscopic Evaluation of Swallowing-How Does It Work?

PURPOSE: The purposes of this study were to compare the visibility of various color groups in different dilutions using narrow band imaging (NBI) and white light (WL) and to determine an optimal color combination using NBI and WL for multicolor flexible endoscopic evaluation of swallowing (FEES), for example, to test different consistencies. METHOD: Preliminary examinations were performed in the oral cavity of two healthy volunteers. Various dyes were tested using NBI and WL to evaluate their visibilities. In the case of a clearly discernible color change, the visibility differences in the dilution series under WL and NBI were recorded and compared. Subsequently, an abbreviated dilution series with NBI and WL was performed in a volunteer as part of a swallow endoscopy to determine whether the results from the oral cavity could be transferred to the hypopharynx. RESULTS: The enhancement of visibility using NBI compared with WL can be proven. When NBI was used, yellow and red food dyes and their mixtures showed distinct color changes. The reacting dyes were still visible under NBI, even at a 10-times-higher dilution, requiring a lower dye concentration for FEES. For increased visibility, the dyes used for FEES with NBI must contain colors from a small range in the yellow and red spectra, which are close to the filter maxima of NBI. Both colors are well visible under WL when combining red and green (secondary color of yellow). CONCLUSIONS: Certain food colorings are visible 10 times more diluted under NBI than under WL. In a multicolored approach, optimal visibility under NBI and WL can be achieved by combining green and red. This new high-sensitivity FEES should be highlighted to allow an instant distinction from WL-FEES; we suggest "FEES+." SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.23280290.

Flexible endoscopic evaluation of swallowing in children with type 1 spinal muscular atrophy.

PURPOSE: This study aimed to report on implementing flexible endoscopic evaluation of swallowing (FEES) in infants and toddlers with type 1 spinal muscular atrophy (SMA). In addition, a comparison of FEES results and clinical scores was carried out. METHODS: A prospective pilot study was conducted including ten symptomatic children with SMA type 1 (two SMN2 copies). They started treatment with one of the three currently approved therapies for SMA at a median age of 3.8 months (range 0.7-8.9). FEES was performed according to a standard protocol using Penetration-Aspiration Scale (PAS) and Murray Secretion Scale as a primary outcome. The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) for motor function, Neuromuscular Disease Swallowing Status Scale (NdSSS), Oral and Swallowing Abilities Tool (OrSAT), and single clinical swallowing-related parameters were also assessed. RESULTS: Distinct swallowing disorders were already evident in eight children at inclusion. The most common findings from FEES were pharyngeal secretion pooling, penetration, and aspiration of saliva and food as well as delayed initiation of swallowing. Despite an average increase in motor function, no comparable improvement was found in swallowing function. None of the surveyed clinical scores showed a significant dependence on PAS in a mixed linear model. CONCLUSIONS: Valuable information regarding the status of dysphagia can be gathered endoscopically, particularly concerning secretion management and when oral intake is limited. Currently available clinical tools for children with type 1 may represent a change in nutritional status but are not yet mature enough to conclude swallowing ability. Further development is still required.

Implementation of Pediatric Flexible-Endoscopic Evaluation of Swallowing: A Systematic Review and Recommendations for Future Research.

BACKGROUND: Although pediatric flexible-endoscopic evaluation of swallowing (FEES) has developed into a standard in dysphagia diagnostics, there are no valid protocols and procedures for children available to date. OBJECTIVE: This systematic PROSPERO-registered review aimed to identify implementation protocols for pediatric FEES described in research studies, and to analyze them in detail concerning procedural steps, equipment, and reported outcome. METHODS: Included were all studies reporting a pediatric FEES protocol for children aged 0-18 years, if they described at least two criteria defined in advance. The databases MEDLINE and CINHAL were searched systematically from January 2000 to February 2021. Risk of bias for included studies was assessed using the National Institutes of Health (NIH) quality assessment tool for observational cohort and cross-sectional studies. A narrative synthesis of the FEES protocols was conducted and the results compared in tabular form. RESULTS: In total 22 studies were included, reporting on FEES in 1547 infants, children, and adolescents with a wide range of diagnoses. It was possible to identify protocols related to all age groups in general as well as to particular groups such as breastfed or bottle-fed infants. None of the included studies demonstrated a good methodological quality; all studies had missing data. Uniform implementation for sub-groups could not be determined. The reported outcome of FEES examinations could not be compared. DISCUSSION: None of the included studies showed good methodological quality and a significant amount of data were missing; the review still offers a systematic basis for future research to close the serious gap in the area of pediatric FEES. A proposal is made for a minimum requirement for pediatric FEES protocols in scientific studies.

Prognostic factors in non-organic hearing loss in children.

OBJECTIVE: Exploring factors that predict a favourable clinical outcome in non-organic hearing loss (NOHL) in children. NOHL is defined as a subjective hearing loss without correlation in objective measures. DESIGN: Retrospective analysis of all childrens' data with the diagnosis NOHL seen between 2005 and 2017 at a tertiary referral centre. STUDY SAMPLE: Sixty-seven children (44 female, 23 male; mean age 11.26 years) were included. Statistical analyses included descriptive statistics, logistic regression, correlations, and Mann-Whitney U tests. RESULTS: A quarter of the children (17/67) had been provided previously with hearing aids without objective sensorineural hearing loss. Having been provided with hearing aids before the first visit to our clinic lowered the chance of normal hearing at the final visit by 76%. The prescription of hearing aids was significantly more likely in girls. Children provided with hearing aids were significantly older than those without and the time span between the first occurrence of the NOHL and first presentation at our department was longer than in children not provided with hearing aids. CONCLUSIONS: In cases of NOHL, hearing aid prescription is not indicated and significantly lowers a child's chance of full recovery and, thus, should not be prescribed unless NOHL is ruled out.

Pediatric Flexible Endoscopic Evaluation of Swallowing: Critical Analysis of Implementation and Future Perspectives.

This study aimed to critically review pediatric swallowing assessment data to determine the future need for standardized procedures. A retrospective analysis of 152 swallowing examinations in 128 children aged 21 days to 18 years was performed. The children were presented at a university dysphagia center between January 2015 and June 2020 for flexible-endoscopic evaluation of swallowing (FEES). Descriptive analysis was conducted for the sample, swallowing pathologies, diagnosis, and missing values. Using binary logistic regression, the relationship between dysphagia and underlying diseases was investigated. The largest group with a common diagnosis in the cohort were children with genetic syndromes (n = 43). Sixty-nine children were diagnosed with dysphagia and 59 without dysphagia. The non-dysphagic group included 15 patients with a behavioral feeding disorder. The presence of an underlying disease significantly increased the chance of a swallowing problem (OR 13.08, 95% CI 3.66 to 46.65, p = .00). In particular, the categories genetic syndrome (OR 2.60, 95% CI 1.15 to 5.88) and neurologic disorder (OR 4.23, 95% CI 1.31 to 13.69) were associated with higher odds for dysphagia. All pediatric FEES were performed without complications, with a completion rate of 96.7%, and with a broad variability of implementation. Several charts lacked information concerning swallowing pathologies, though. Generally, a more standardized protocol and documentation for pediatric FEES is needed to enable better comparability of studies on epidemiology, assessment, and treatment outcomes in future.

Dysphagia Screening in Parkinson's Disease. A diagnostic accuracy cross-sectional study investigating the applicability of the Gugging Swallowing Screen (GUSS).

BACKGROUND: Simple water-swallowing screening tools are not predictive of aspiration and dysphagia in patients with Parkinson's Disease (PD). We investigated the diagnostic accuracy of a multi-texture screening tool, the Gugging Swallowing Screen (GUSS) to identify aspiration and dysphagia/penetration in PD patients compared to flexible endoscopic evaluation of swallowing (FEES). METHODS: Swallowing function was evaluated in 51 PD participants in clinical 'on-medication' state with the GUSS and a FEES examination according to standardized protocols. Inter-rater reliability and convergent validity were determined and GUSS- and FEES-based diet recommendations were compared. KEY RESULTS: Inter-rater reliability of GUSS ratings was high (rs  = 0.8; p < 0.001). Aspiration was identified by the GUSS with a sensitivity of 50%, and specificity of 51.35% (PPV 28%, NPV 73%, LR+ 1.03, LR- 0.97), dysphagia/penetration was identified with 72.97% sensitivity and 35.71% specificity (PPV 75%, NPV 33.33%, LR+ 1.14, LR- 0.76). Agreement between GUSS- and FEES-based diet recommendations was low (rs  = 0.12, p = 0.42) with consistent NPO (Nil per Os) allocation by GUSS and FEES in only one participant. CONCLUSIONS AND INFERENCES: The multi-texture screening tool GUSS in its current form, although applicable with good inter-rater reliability, does not detect aspiration in PD patients with acceptable accuracy. Modifications of the GUSS parameters "coughing," "voice change" and "delayed swallowing" might enhance validity. The GUSS' diet recommendations overestimate the need for oral intake restriction in PD patients and should be verified by instrumental swallowing examination.

Impact of simultaneous subthalamic and nigral stimulation on dysphagia in Parkinson's disease.

OBJECTIVES: Dysphagia is a frequent and highly relevant symptom in Parkinson's disease (PD) due to high associated morbidity and mortality. To compare the effect of simultaneous stimulation of the subthalamic nucleus (STN) and substantia nigra (SNr) with conventional STN-stimulation on swallowing function in Parkinson's disease. METHODS: In this controlled, randomized, double-blind, cross-over clinical trial, 15 PD patients were assessed with DBS switched off (STIM OFF), STN-DBS, STN + SNr-DBS. Patients and 32 age-matched healthy controls were examined clinically and by flexible-endoscopic evaluation of swallowing (FEES) to evaluate the swallowing function. The primary endpoint was the assessment of residues, secondary endpoints were penetration/aspiration, leakage, retained pharyngeal secretions, drooling, and assessments of the patient's self-perception of swallowing on a visual analog scale. RESULTS: Compared with healthy controls PD patients showed significantly more pharyngeal residues in STIM OFF and both DBS modes. Residues or aspiration events were found in 80% of the patients under STN-stimulation. Simultaneous STN + SNr-stimulation had no additional positive effect on objective dysphagia and self-reported swallowing function compared to STN-DBS. INTERPRETATION: Simultaneous STN + SNr-stimulation seems to have no additional beneficial effects on dysphagia when compared with conventional STN-stimulation, but did not deteriorate the swallowing function. If STN + SNr-stimulation is planned to be applied for the improvement of axial symptoms and gait disorders in PD patients, it can be considered safe in terms of dysphagia.

Swallowing speed is no adequate predictor of aspiration in Parkinson's disease.

BACKGROUND: There is still a lack of a clinical test to reliably identify patients with Parkinson's disease (PD) being at risk for aspiration. METHODS: In this prospective, controlled, cross-sectional study, we assessed if swallowing speed for water is a useful clinical test to predict aspiration proven by flexible endoscopic evaluation of swallowing (FEES). Due to this, we measured the swallowing speed for 90 mL water in 115 consecutive and unselected PD outpatients of all clinical stages and 32 healthy controls. KEY RESULTS: Average swallowing speed was lower in patients compared with controls (6.5 ± 3.9 mL/s vs 8.5 ± 3.2 mL/s; P < .01). The disease-independent widely used threshold of <10 mL/s showed insufficient sensitivity of 88% and specificity of 19% with high false-positive rates of 63% for patients and 69% for controls. Receiver operating characteristic (ROC) analysis was carried out to define a suitable cutoff value for detection of aspiration of water (area under the curve 0.72, P < .001) in PD patients. The optimized cutoff value was 5.5 mL/s with a sensitivity of 69% and a specificity of 64%. CONCLUSION AND INFERENCES: Measuring swallowing speed is prone to methodological errors and not suitable as a screening instrument to predict aspiration in PD patients.

Pill swallowing in Parkinson's disease: A prospective study based on flexible endoscopic evaluation of swallowing.

BACKGROUND: This study evaluates the prevalence, characteristics, and predictors of the difficulty of swallowing medication in Parkinson's disease (PD). METHODS: In this prospective controlled, cross-sectional cohort study, the ability to swallow four different placebos was assessed using flexible endoscopic evaluation of swallowing (FEES) in 118 PD patients and 32 controls. The association between a patient's swallowing ability for each pill and water, patient characteristics and dopaminergic response was examined. The value of two swallowing screening questions was also evaluated. RESULTS: Substantially impaired ability to swallow pills was found in 28% (n = 33/118) of patients and 16% (n = 5/32) of controls (p = 0.18). Higher disease severity was associated with more problems with swallowing pills (p = 0.03), but PD patients with short disease duration (<2 years), low H&Y stage (1-2), and younger age (<70 years) were also affected (each at least in 20%). Capsules were the easiest to swallow while oval tablets were the most difficult (p < 0.01, r = 0.21). Most patients (73%, n = 24/33) presented with swallowing problems only for a single formulation. Aspiration of water was found in 48% of patients, suggesting a possible increased risk of aspiration when taking dissolved tablets. Standardized questionnaires showed insufficient sensitivity (52% both) but fairly good specificity (69-74%) for dysphagia of pills. Dysphagia for medication was not associated with a lack of dopaminergic response. CONCLUSIONS: Dysphagia of medication occurs preferentially in advanced disease stages. An assessment of pill swallowing using FEES is suggested at least in patients reporting swallowing problems. Capsules might be preferentially used when dysphagia is suspected.

Pin-up Glottoplasty: Feasibility Study of a Novel Approach Medializing or Lateralizing Immobile Vocal Folds.

Principles in medializing and lateralizing vocal folds have not changed a lot within the last decades (Isshiki et al, 1974; Bruenings, 1911). We present a feasibility study for a completely new approach to perform medialization and lateralization of immobile vocal folds. The method was tested on 20 human larynges by inserting a 21G needle into the vocal fold, medializing (or, in other cases, lateralizing) the vocal cord and fixing the needle at the cricoid cartilage posteriorly. The anterior and posterior fixation points of the needle allow for a stable positioning of the needle, which we consider necessary in both cases of medialization or lateralization. The needle position was examined visually as well as three-dimensionally via cone beam computed tomography.

Is the Munich dysphagia Test-Parkinson's disease (MDT-PD) a valid screening tool for patients at risk for aspiration?

BACKGROUND: Dysphagia is common in Parkinson's disease (PD) and leads to pneumonia, malnutrition, and reduced quality of life. For detecting dysphagia-exposed PD patients, the Munich dysphagia test-Parkinson's disease (MDT-PD) is a patient self-reported tool that has been developed specifically for PD patients. The MDT-PD is available in both German and English. This study aimed to ascertain whether the MDT-PD can detect aspiration in PD patients and, therefore, serve as a suitable screening tool. METHODS: In a controlled, cross-sectional, observational study, a total of 119 PD outpatients were examined clinically and were evaluated by the MDT-PD and the one swallowing question (NMS-3) from the nonmotor symptom questionnaire for Parkinson's disease (NMSQuest). The results of the MDT-PD and the NMS-3 were compared to the penetration-aspiration scale (PAS) rating defined by flexible endoscopic evaluation of swallowing (FEES). KEY RESULTS: Half of the patients with aspiration as determined using FEES were not detected by the MDT-PD and NMS-3 self-reported tools. The proportion of false positive patients was high with both tools. The sensitivity of the MDT-PD to detect patients who are at risk for aspiration is insufficient (0.37) and not superior to applying the dysphagia screening question from the NMSQuest (0.5). CONCLUSION: This study reveals that the MDT-PD is not suitable for detecting aspiration in PD patients and, therefore, cannot be considered as a screening tool for aspiration. However, at present, there is no alternative validated screening tool that can reliably detect aspiration in PD patients. A readjustment of the MDT-PD is urgently needed.

Predictive clinical factors for penetration and aspiration in Parkinson's disease.

BACKGROUND: Do the current swallow-specific subquestions of neurological diagnostic tools reflect the objective swallowing function in Parkinson's disease (PD) patients or are clinical factors superior to predict critical aspiration? METHODS: In a cross-sectional, observational study a total of 119 Parkinson outpatients were examined clinically and by flexible-endoscopic evaluation of swallowing (FEES). Self-reported dysphagia by subquestions of the MDS-UPDRS and NMS questionnaire and history of subjective aspiration signs were collected. KEY RESULTS: Nearly, all PD patients showed deglutition abnormalities in FEES (113/119) while only 12%-27% of them reported swallowing problems in the swallow-specific subquestions of neurological standard diagnostic tools (MDS-UPDRS and NMS-Quest), and the answers were heterogeneous and poorly reproducible. With a sensitivity of up to a maximum of 50%, self-reported dysphagia is therefore no reliable tool for identifying dysphagia in PD. While most clinical parameters were linked to dysphagia to some extent, logistic regression analysis revealed high age (Odds Ratio (OR) 1.1 in years, 95% CI 1.03-1.18, P < 0.01), gender (OR 0.3 for females, 95% CI 0.08-0.97, P = 0.04), and affirmed subjective aspiration signs (OR 8.6, 95% CI 3.05-26.52, P < 0.001) as the most significant predictors for critical dysphagia. CONCLUSIONS AND INFERENCES: Self-perception of swallowing is no reliable tool for identifying dysphagia and questionnaires are insufficient for detecting previous aspiration. Consequent and specific asking for previous subjective aspiration signs is the single most important measure for identifying PD patients at risk for critical aspiration.

Critical Dysphagia is Common in Parkinson Disease and Occurs Even in Early Stages: A Prospective Cohort Study.

To assess the prevalence of dysphagia and its typical findings in unselected "real-world" Parkinson patients using an objective gold-standard method. This was a prospective, controlled, cross-sectional study conducted in 119 consecutive Parkinson patients of all stages independent of subjective dysphagia. Patients and 32 controls were clinically and endoscopically examined by flexible endoscopic evaluation of swallowing (FEES) to evaluate the deglutition with regard to three consistencies (water, biscuit, and bread). Typical findings of dysphagia like penetration and aspiration, residues, and leakage were assessed. Dysphagia was common in Parkinson patients and occurred in all, even early, disease stages. Only 5% (6/119) of patients showed a completely unremarkable deglutition. Aspiration was seen in 25% (30/119) of patients and always related to water. Residues occurred in 93% (111/119), most commonly for bread. Leakage was much less frequent and was found in only 3-18%, depending on consistency. In a significant fraction of patients, objective dysphagia was not subjectively perceived. A total of 16% of asymptomatic patients suffered from critical aspiration. Significant swallowing deficiencies already occurred in early disease. Aspiration was found in 4 of 20 (20%) patients with disease duration of less than 2 years. Seven of 57 patients (12%) with Hoehn and Yahr stage 2 suffered from severe aspiration. Given the high frequency of critical aspiration in Parkinson disease, these patients should be evaluated early for dysphagia to avoid complications and recommend an adequate therapy. FEES is a simple, cost efficient, minimally invasive method that is ideally suited for this purpose.

Narrow Band Imaging Enhances the Detection Rate of Penetration and Aspiration in FEES.

Narrow band imaging (NBI) is widely used in gastrointestinal, laryngeal, and urological endoscopy. Its original purpose was to visualize vessels and epithelial irregularities. Based on our observation that adding NBI to common white light (WL) improves the contrast of the test bolus in fiberoptic endoscopic evaluation of swallowing (FEES), we now investigated the potential value of NBI in swallowing disorders. 148 FEES images were analyzed from 74 consecutive patients with swallowing disorders, including 74 with and 74 without NBI. All images were evaluated by four dysphagia specialists. Findings were classified according to Rosenbek's penetration-aspiration scale modified for evaluating these FEES images. Intra- and inter-rater reliability was determined as well as observer confidence. A better visualization of the bolus is the main advantage of NBI in FEES. This generally leads to sharper optical contrasts and better detection of small bolus quantities. Accordingly, NBI enhances the detection rate of penetration and aspiration. On average, identification of laryngeal penetration increased from 40 to 73% and of aspiration from 13 to 24% (each p < 0.01) of patients. In contrast to WL alone, the use of NBI also markedly increased the inter- and intra-rater reliability (p < 0.01) and the rating confidence of all experts (p < 0.05). NBI is an easy and cost-effective tool simplifying dysphagia evaluation and shortening FEES evaluation time. It leads to a markedly higher detection rate of pathological findings. The significantly better intra- and inter-rater reliability argues further for a better overall reproducibly of FEES interpretation.