Esophageal Dysphagia in Adults: When It Sticks.

Esophageal dysphagia is a common yet difficult to diagnose condition. This article underscores the role of detailed patient history and physical examinations, including prompt endoscopic evaluation, for accurate differentiation between esophageal and oropharyngeal dysphagia. The authors discuss the heightened importance of early intervention in certain patient groups, such as elderly individuals and patients with head and neck cancer, to mitigate the risk of malnutrition and infection. The authors delve into etiologic factors highlighting the complexity of clinical presentations and the significance of tailored management strategies.

Increasing Prevalence of Voice Disorders in the USA: Updates in the COVID Era.

OBJECTIVE: To estimate the current prevalence of voice disorders among adults in the United States; to determine the association of individual factors with voice disorders. METHODS: The 2022 National Health Interview Survey (NHIS) was analyzed to identify adults reporting voice problems in the past 12 months. Demographics were assessed, as well as the duration, severity, and resolution of the voice problem. The relationship between voice problems, gender, lost workdays, and long COVID was investigated. A comparison to the 2012 NHIS was made to determine changes in voice disorder prevalence. RESULTS: 29.9 million Americans (95%CI[28.3-31.5]) annually report a voice problem, representing 12.2% of the population (95%CI[11.7-12.8%]). Overall, 26.8% and 13.2% reported the severity of their voice problem as moderate or severe, respectively. Only 5.1% (95%CI[4.3-6.0%]) of respondents sought treatment. Most voice problems were resolved within 1 week (53.0%,95%CI[50.9-55.1%]). Females were more likely than males to report a voice problem (14.4% vs. 10.0%,95%CI[13.7-15.1] and [9.3-10.7], respectively). The 17.6 million Americans with long COVID symptoms were more likely to have voice complaints than those without (21.1% vs. 11.6%,95%CI[18.9-23.5%] and [11.1-12.1%], respectively). Lost workdays were not significantly higher for those with voice disorders compared to those without (17.1 vs. 12.9 days,95%CI[12.0-22.1] and [11.0-14.8], respectively). CONCLUSIONS: Voice problems affect approximately 1 in 8 adults in the U.S. annually, demonstrating an alarming increased prevalence since 2012 using the same survey methodology. Relatively few individuals seek care for their voice problem, despite significant self-reported impact. Further study is required regarding the impact of COVID and changes in voice use patterns on voice disorders. LEVEL OF EVIDENCE: 3 Laryngoscope, 2024.

Identifying bias in models that detect vocal fold paralysis from audio recordings using explainable machine learning and clinician ratings.

Introduction: Detecting voice disorders from voice recordings could allow for frequent, remote, and low-cost screening before costly clinical visits and a more invasive laryngoscopy examination. Our goals were to detect unilateral vocal fold paralysis (UVFP) from voice recordings using machine learning, to identify which acoustic variables were important for prediction to increase trust, and to determine model performance relative to clinician performance. Methods: Patients with confirmed UVFP through endoscopic examination (N=77) and controls with normal voices matched for age and sex (N=77) were included. Voice samples were elicited by reading the Rainbow Passage and sustaining phonation of the vowel "a". Four machine learning models of differing complexity were used. SHapley Additive explanations (SHAP) was used to identify important features. Results: The highest median bootstrapped ROC AUC score was 0.87 and beat clinician's performance (range: 0.74 - 0.81) based on the recordings. Recording durations were different between UVFP recordings and controls due to how that data was originally processed when storing, which we can show can classify both groups. And counterintuitively, many UVFP recordings had higher intensity than controls, when UVFP patients tend to have weaker voices, revealing a dataset-specific bias which we mitigate in an additional analysis. Conclusion: We demonstrate that recording biases in audio duration and intensity created dataset-specific differences between patients and controls, which models used to improve classification. Furthermore, clinician's ratings provide further evidence that patients were over-projecting their voices and being recorded at a higher amplitude signal than controls. Interestingly, after matching audio duration and removing variables associated with intensity in order to mitigate the biases, the models were able to achieve a similar high performance. We provide a set of recommendations to avoid bias when building and evaluating machine learning models for screening in laryngology.

Reproducibility of laryngeal force sensor measurements.

OBJECTIVE: The laryngeal force sensor (LFS) measures force during suspension microlaryngoscopy (SML) procedures, and has been previously shown to predict postoperative complications. Reproducibility of its measurements has not been described. STUDY DESIGN: Prospective cohort study. SETTING: Academic medical center. METHODS: 291 adult patients had force data collected from 2017 to 2021 during various SML procedures. 94 patients had passive LFS monitoring (surgeon blinded to intraoperative recordings) and 197 had active LFS monitoring (surgeon able to see LFS recordings). 27 of these patients had repeat procedures, with unique LFS metrics for each procedure. The 27 patients were divided into three groups. Group 1 had passive use for both procedures, group 2 had passive use for the first procedure and active use for the second, and group 3 had active use for both procedures. Force metrics from the two procedures were compared with a paired samples t-test. RESULTS: For airway dilation procedures and cancer resection procedures, average force variances were significantly lower with active versus passive use of the LFS. Group 1-no significant changes in maximum force (procedure 1 = 163.8 N, procedure 2 = 133.8 N, p = 0.324) or average force (procedure 1 = 93.6 N, procedure 2 = 78.3 N, p = 0.617). Group 2-maximum force dropped by 35 % between procedures 1 (219.2 N) and 2 (142.5 N), p = 0.013. Average force dropped by 42.5 % between procedures 1 (147.2 N) and 2 (84.6 N), p = 0.007. Group 3-no significant changes in maximum force (procedure 1 = 158.6 N, procedure 2 = 158.2 N, p = 0.986) or average force (procedure 1 = 94.2, procedure 2 = 81.8, p = 0.419). CONCLUSIONS: LFS measurements were reproducible for similar procedures in the same patient when the type of LFS monitoring was not a confounder.

Progressive Decline in Voice and Voice-Related Quality of Life in X-Linked Dystonia Parkinsonism.

OBJECTIVE: To characterize the evolution of swallowing and voice in patients with X-linked dystonia parkinsonism (XDP). STUDY DESIGN: Retrospective case series. METHODS: Retrospective review of 59 patients with XDP from January 2016 to January 2018. All patients underwent complete examinations and quality of life (QOL) surveys (Swallowing Quality of Life questionnaire [SWAL-QOL], Voice-Related Quality of Life [V-RQOL], and Voice Handicap Index [VHI]), and functional endoscopic examination of swallowing. We excluded patients with incomplete records or patients lost to follow-up. Univariate analysis was used to compare 2016 to 2018 Penetration-Aspiration Scale (PAS), SWAL-QOL, V-RQOL, and VHI scores. RESULTS: Ten patients met the inclusion criteria. Nine patients had oromandibular dystonia. Voice-related measures significantly worsened with an increase in mean VHI from 81 to 109.9 (P = 0.026) and decrease in mean V-RQOL from 58 to 28 (P = 0.013). Vocal strain also significantly worsened 0.4 to 1.4 (P = 0.001). Mean PAS scores increased from 4.2 to 5.1 (P = 0.068) and mean SWAL-QOL decreased from 50.4 to 43.5 (P = 0.157). In the SWAL-QOL, the mean Eating Duration score worsened from 0.9 to 0.4 (P = 0.052) and Mental Health score declined from 10.1 to 6.1 (P = 0.077). CONCLUSIONS: Both vocal strain and voice-related QOL measures considerably worsened over the 2-year interval in our limited group of XDP patients with no significant change in PAS scores or swallowing QOL. The findings demonstrated that the pace of disease affecting voice symptoms was different from swallowing symptoms in our study group and that changes in communication ability may be a more sensitive marker for disease progression than swallowing dysfunction.

Design, development, and face validation of an intubation simulation device using real-time force data feedback.

Objectives: To develop a novel laryngoscope device capable of dynamically measuring force and torque measurements in real-time during intubation and to explore the efficacy of such a device through a face validation simulation. Methods: The torque sensor laryngoscope is designed for use during intubation and is modeled after a standard, single-use plastic laryngoscope. After device calibration, a face validation study was performed with intubation experts in the field. Quantitative data (intubation force metrics) and qualitative data (expert feedback on the device) were collected from three intubations using a Mac blade and three intubations with the Miller blade. Results: Three experts (two anesthesiologists and one otolaryngologist) participated in the study. The mean maximum force exerted with the Mac blade was 24.5 N (95% confidence interval [CI], 22.3-26.8). The average force exerted was 13.6 N (95% CI, 11.7-15.5). The average total suspension time was 13.1 s (95% CI, 10.4-15.8). The average total impulse was 164.6 N·s (95% CI, 147.9-181.4). The mean maximum force exerted with the Miller blade was 31.6 N (95% CI, 26.4-36.8). The average force exerted was 15.8 N (95% CI, 13.8-17.9). The average total suspension time was 11.3 s (95% CI, 9.9-12.6). The average total impulse was 216.2 N·s (95% CI, 186.5-245.9). The mean maximum force (p = .0265) and total impulse (p = .009) were significantly higher in the Miller blade trials than in the Mac blade trials. Survey results found that this device, while bulky, intubated similarly to standard-use models and has potential as an intubation teaching tool. Conclusion: The torque sensor laryngoscope can measure and display real-time intubation force metrics for multiple laryngoscope blades. Initial validation studies showed a significantly lower maximum force and total impulse when intubating with the Mac blade than with the Miller blade. Face validation survey results were positive and suggested the potential for this device as a teaching tool. Level of Evidence: Level 5.

Clinical, Radiologic, and Endolaryngeal Findings in Laryngeal Fractures: A 15-Year Case Series.

OBJECTIVE: Laryngeal fractures are rare injuries; recent data describing these injuries and associated examination findings are limited. This study aims to describe injury etiology and outcomes associated with laryngeal fractures. STUDY DESIGN: Retrospective case series. SETTING: Academic tertiary center. METHODS: Patients with laryngeal fractures from 2005 to 2020 were identified in a retrospective chart review. Patient demographics, injury mechanisms, management, and voice outcomes were examined. Fracture type, radiologic, and endolaryngeal examination findings were analyzed for associations between fracture etiology and examination characteristics. RESULTS: Laryngeal fractures most commonly occurred at the thyroid cartilage. Fractures were most commonly due to sport-related injuries. Mechanism of injury was not associated with specific radiologic or endolaryngeal findings. Mechanism of injury was additionally not significantly associated with the need for intubation, surgical intervention, or tracheotomy. Fracture location was significantly associated with intubation requirement (P = .015), with 40% of patients with concomitant thyroid and cricoid fractures requiring intubation. Mechanism of injury significantly correlated with dysphonia at follow-up (P = .033). Mechanism of injury, fracture location, and surgical management were not associated with increased vocal fold injury or dysphonia. CONCLUSION: There are no significant correlations between injury mechanism and fracture location, characteristics, radiologic findings, or endolaryngeal findings. These features emphasize the importance of a thorough and comprehensive laryngeal examination.

Laryngeal Subsite Analysis of Granulomatosis With Polyangiitis (Wegener's).

OBJECTIVE: To analyze specific intralaryngeal findings associated with granulomatosis with polyangiitis (GPA). STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. METHODS: A retrospective chart review was performed on all patients diagnosed with GPA who were evaluated at the laryngology division of Massachusetts Eye and Ear Infirmary between January 2006 and September 2019. RESULTS: Forty-four patients (14 male, 30 female) were evaluated for laryngeal pathology. The mean age at onset was 48 years. Nine patients (21%) were identified with only vocal fold disease, 11 (25%) with subglottic disease, and 8 (18%) with disease at the glottis and subglottis (transglottic). The remaining 16 patients (36%) had a normal airway upon examination although they presented with laryngeal symptoms. Patients with glottic disease had statistically significantly lower voice-related quality of life scores than patients with isolated subglottic stenosis. CONCLUSIONS: Although laryngeal manifestations of GPA is often described as a subglottic disease presenting with respiratory symptoms, subsite analysis show that only 25% of patients had subglottic disease alone, with similar rates of glottic disease alone. Laryngeal subsites have different epithelial mucosa, function, and physiology, and understanding the specific sites of involvement will determine symptoms and enable better analysis of the underlying mechanisms of disease. Glottic disease is associated with a reduction in vocal fold motion and voice changes. Subglottic involvement presents more frequently with airway symptoms. Further research is necessary to better define the specific regions of laryngeal involvement in patients diagnosed with GPA.

Decision aid and preference assessment of topical anesthesia for otolaryngology procedures.

OBJECTIVES: To determine preference patterns for topical anesthesia in patients undergoing endoscopy pre-coronavirus (2019 coronavirus disease [COVID-19]) pandemic and analyze outcomes based on preference, using a decision aid format. METHODS: A decision aid was developed with expert and patient input. New patients presenting to subspecialty clinics over a 2-month pre-COVID-19 period completed a pre-procedure survey about their priorities, then were asked to choose between topical oxymetazoline/lidocaine spray or none. A post-procedure outcome survey followed. RESULTS: Of 151 patients, 90.1% patients elected to have topical anesthesia. Top patient priorities were "I want the scope to be easy for the doctor" and "I want to be as comfortable as possible." Patients who strongly wanted to avoid medication (P = .002) and bad taste (P = .003) were more likely to select no spray, whereas those who wanted to avoid pain received anesthetic (P = .011). According to the post-procedure assessment, 95.4% of patients were satisfied or strongly satisfied their choice, and this did not correlate with anesthetic vs none. CONCLUSIONS: Patient preferences are easily elicited and correlate with treatment choices. Most patients chose to have topical anesthetic and were willing to tolerate side effects; however, both patients with and without topical anesthetic were satisfied with their choices. This decision aid can be used to optimize shared decision making in the otolaryngology clinic. Given the aerosolizing potential of both spray and no spray conditions, this insight may be consequential when devising office protocols for post-COVID-19 practice. LEVEL OF EVIDENCE: II.

How Patients Choose a Laryngologist: A Pilot Stated Preference Study.

OBJECTIVE: Patients consider many factors when deciding how to receive medical care. This study used best-worst scaling (BWS), a technique novel to otolaryngology, to quantitatively examine preferences among patients choosing a laryngologist. Our objective was to quantify in a pilot cohort the relative importance patients place on a variety of attributes when seeking a laryngologist. STUDY DESIGN: BWS survey. SETTING: Academic voice clinic. METHODS: New patients were recruited to take a computerized BWS survey developed using attributes derived from patient input, expert opinion, and literature review. Attributes were grouped into 4 categories: physician reputation, physician qualifications, hospital-related factors, and other nonclinical factors. Responses were analyzed using multinomial logit regression to determine importance scores and associations with other variables. RESULTS: Eighty-seven of 93 patients recruited participated (93.5% response rate). Physician qualifications were the most important attributes to patients, with specialty laryngology training receiving the highest importance score (20.8; 95% CI, 20.2 to 21.5; P < .0001). Recommendations from referring physicians (15.6; 95% CI, 14.3 to 16.9) and use of cutting-edge technology (11.9; 95% CI, 10.7 to 13.1) were the second and third most important, respectively. Least important were nonclinical factors, including wait time to get an appointment (4.3; 95% CI, 2.8 to 5.8) and convenience of office location (1.5; 95% CI, 0.9 to 2.1). Just over half of patients (51.2%) reported willingness to wait 4 weeks for an appointment with a laryngologist. Older patients were less concerned with convenience-related factors. CONCLUSION: Nonclinical factors were less important to patients than clinical factors, and laryngology-specific training was paramount. Stated preference methodologies can elucidate underlying preferences and help providers make care more patient centered.

Application of a Computer Vision Tool for Automated Glottic Tracking to Vocal Fold Paralysis Patients.

OBJECTIVES: (1) Demonstrate true vocal fold (TVF) tracking software (AGATI [Automated Glottic Action Tracking by artificial Intelligence]) as a quantitative assessment of unilateral vocal fold paralysis (UVFP) in a large patient cohort. (2) Correlate patient-reported metrics with AGATI measurements of TVF anterior glottic angles, before and after procedural intervention. STUDY DESIGN: Retrospective cohort study. SETTING: Academic medical center. METHODS: AGATI was used to analyze videolaryngoscopy from healthy adults (n = 72) and patients with UVFP (n = 70). Minimum, 3rd percentile, 97th percentile, and maximum anterior glottic angles (AGAs) were computed for each patient. In patients with UVFP, patient-reported outcomes (Voice Handicap Index 10, Dyspnea Index, and Eating Assessment Tool 10) were assessed, before and after procedural intervention (injection or medialization laryngoplasty). A receiver operating characteristic curve for the logistic fit of paralysis vs control group was used to determine AGA cutoff values for defining UVFP. RESULTS: Mean (SD) 3rd percentile AGA (in degrees) was 2.67 (3.21) in control and 5.64 (5.42) in patients with UVFP (P < .001); mean (SD) 97th percentile AGA was 57.08 (11.14) in control and 42.59 (12.37) in patients with UVFP (P < .001). For patients with UVFP who underwent procedural intervention, the mean 97th percentile AGA decreased by 5 degrees from pre- to postprocedure (P = .026). The difference between the 97th and 3rd percentile AGA predicted UVFP with 77% sensitivity and 92% specificity (P < .0001). There was no correlation between AGA measurements and patient-reported outcome scores. CONCLUSIONS: AGATI demonstrated a difference in AGA measurements between paralysis and control patients. AGATI can predict UVFP with 77% sensitivity and 92% specificity.

Laryngeal Force Sensor for Suspension Microlaryngoscopy: A Prospective Controlled Trial.

OBJECTIVES: The laryngeal force sensor (LFS) provides real-time force data for suspension microlaryngoscopy. This study investigates whether active use of the LFS can prevent the development of complications. STUDY DESIGN: Prospective controlled trial. SETTING: Academic tertiary center. METHODS: The LFS and custom software were developed to track intraoperative force metrics. A consecutive series of 100 patients had force data collected with operating surgeons blinded to intraoperative readings. The subsequent 100 patients had surgeons actively use the LFS monitoring system. Patients were prospectively enrolled, completing pre- and postoperative surveys to assess the development of tongue pain, paresthesia, paresis, dysgeusia, or dysphagia. RESULTS: On univariate analysis, the active monitoring group had lower total impulse (P < .001) and fewer extralaryngeal complications (P < .01). On multiple logistic regression, maximum force (odds ratio [OR], 1.08; 95% CI, 1.01-1.16; P = .02) was a significant predictive variable for the development of postoperative complications. Similarly, active LFS monitoring showed a 29.1% (95% CI, 15.7%-42.4%; P < .001) decrease in the likelihood of developing postoperative complications. These effects persisted at the first postoperative visit for maximum force (P = .04) and active LFS monitoring (P = .01). Maximum force (OR, 1.11; 95% CI, 1.04-1.18; P < .01) and active LFS monitoring (16.6%; 95% CI, 2.7%-30.5%; P = .02) were also predictive for the development of an abnormal 10-item Eating Assessment Tool score. These effects also persisted at the first postoperative visit for maximum force (P = .01) and active LFS monitoring (P = .01). CONCLUSION: Maximum force is predictive of the development postoperative complications. Active monitoring with the LFS is able to mitigate these forces and prevent postoperative complications. LEVEL OF EVIDENCE: 2.

Force Metrics and Suspension Times for Microlaryngoscopy Procedures.

OBJECTIVE: To determine the difference in force metrics measured by the laryngeal force sensor for various suspension microlaryngoscopy (SML) procedures and their perioperative narcotic requirements. STUDY DESIGN: Prospective observational study. SETTING: Academic tertiary center. METHODS: The laryngeal force sensoris a force sensor designed for SML procedures. Prospectively enrolled patients had dynamic recordings of maximum force, average force, suspension time, and total impulse. Procedures were grouped into excision of striking zone lesions, nonstriking zone lesions, endoscopic cancer surgery with margin control, and airway dilation. Narcotic administration in the intraoperative period and postanesthesia care unit was also recorded and converted into IV morphine equivalents. Surgeons were blinded to the force recordings during surgery to prevent operator bias. RESULTS: In total, 110 patients completed the study. There was no significant difference in average force across different procedures, however, a significant difference was seen for maximum force (P = 0.025), suspension time (P < 0.001), and total impulse (P = 0.002). The highest values were seen for endoscopic cancer surgeries with margin control with a mean maximum force of 49.4 lbf (95%CI, 37.1-61.7), mean suspension time of 60.2 minutes (95%CI, 40.5-79.9), and mean total impulse of 31.3 ton*s (95%CI, 15.2-47.3). A significant difference (P < 0.01) in perioperative narcotic requirements was also seen, with endoscopic cancer surgery cases having the highest requirements at 27.6 mg of ME (95%CI, 16.1-39.2 mg). CONCLUSION: Significant differences in force metrics exist between various SML procedures. Endoscopic cancer surgery is associated with higher force metrics and perioperative narcotic requirements.

Laryngeal fractures in professional and semiprofessional ice hockey players.

OBJECTIVE: Injuries in professional ice hockey players are common, however significant laryngeal trauma is rare. Here, we present a case series of professional and semiprofessional ice hockey players to demonstrate the mechanism and nature of laryngeal injuries they sustain during play, and to recommend best practices for treatment, prevention, and return to the ice. METHODS: A retrospective case review was done of hockey-related laryngeal injuries between 2016 and 2019 at a tertiary laryngology practice. Only semiprofessional and professional hockey players were included. RESULTS: In total, four cases were included. All cases involved trauma from a hockey puck to the neck. No cases were the result of punching, fighting, high sticks or routine checking. Notably, 1 of 4 presented with severe airway compromise, requiring urgent intubation, whereas most presented with pain or a significant voice complaint. Two patients required operative intervention with open reduction and internal fixation of significantly displaced fractures. One patient experienced significant mucosal disruption with cartilaginous exposure at the posterior vocal complex requiring microflap. The average return to ice was 6 weeks for those who required operative intervention and 4 weeks for those who were managed conservatively. One patient had persistent mild dysphonia and all others had a return to baseline phonation. None were wearing neck guards or other protective equipment at the time of injury. CONCLUSION: Though voice and airway injuries are rarely sustained by ice hockey players, they may require urgent intervention. We recommend that protective equipment be worn and improved to prevent laryngeal trauma. LEVEL OF EVIDENCE: 4.

Laryngeal complications of COVID-19.

OBJECTIVE: To describe and visually depict laryngeal complications in patients recovering from coronavirus disease 2019 (COVID-19) infection along with associated patient characteristics. STUDY DESIGN: Prospective patient series. SETTING: Tertiary laryngology care centers. SUBJECTS AND METHODS: Twenty consecutive patients aged 18 years or older presenting with laryngological complaints following recent COVID-19 infection were included. Patient demographics, comorbid medical conditions, COVID-19 diagnosis dates, symptoms, intubation, and tracheostomy status, along with subsequent laryngological symptoms related to voice, airway, and swallowing were collected. Findings on laryngoscopy and stroboscopy were included, if performed. RESULTS: Of the 20 patients enrolled, 65% had been intubated for an average duration of 21.8 days and 69.2% requiring prone-position mechanical ventilation. Voice-related complaints were the most common presenting symptom, followed by those related to swallowing and breathing. All patients who underwent flexible laryngoscopy demonstrated laryngeal abnormalities, most frequently in the glottis (93.8%), and those who underwent stroboscopy had abnormalities in mucosal wave (87.5%), periodicity (75%), closure (50%), and symmetry (50%). Unilateral vocal fold immobility was the most common diagnosis (40%), along with posterior glottic (15%) and subglottic (10%) stenoses. 45% of patients underwent further procedural intervention in the operating room or office. Many findings were suggestive of intubation-related injury. CONCLUSION: Prolonged intubation with prone-positioning commonly employed in COVID-19 respiratory failure can lead to significant laryngeal complications with associated difficulties in voice, airway, and swallowing. The high percentage of glottic injuries underscores the importance of stroboscopic examination. Otolaryngologists must be prepared to manage these complications in patients recovering from COVID-19. LEVEL OF EVIDENCE: IV.

Aerosolization During Common Ventilation Scenarios.

Otolaryngologists are at increased risk for exposure to suspected aerosol-generating procedures during the ongoing coronavirus disease 2019 (COVID-19) pandemic. In the present study, we sought to quantify differences in aerosol generation during common ventilation scenarios. We performed a series of 30-second ventilation experiments on porcine larynx-trachea-lung specimens. We used an optical particle sizer to quantify the number of 1- to 10-µm particles observed per 30-second period (PP30). No significant aerosols were observed with ventilation of intubated specimens (10.8 ± 2.4 PP30 vs background 9.5 ± 2.1, P = 1.0000). Simulated coughing through a tracheostomy produced 53.5 ± 25.2 PP30, significantly more than background (P = .0121) and ventilation of an intubated specimen (P = .0401). These data suggest that undisturbed ventilation and thus intubation without stimulation or coughing may be safer than believed. Coughing increases aerosol production, particularly via tracheostomy. Otolaryngologists who frequently manage patient airways and perform tracheostomy are at increased risk for aerosol exposure and require appropriate personal protective equipment, especially during the ongoing COVID-19 pandemic.

Functional Dystonia: Differentiation From Primary Dystonia and Multidisciplinary Treatments.

Dystonia is a common movement disorder, involving sustained muscle contractions, often resulting in twisting and repetitive movements and abnormal postures. Dystonia may be primary, as the sole feature (isolated) or in combination with other movement disorders (combined dystonia), or as one feature of another neurological process (secondary dystonia). The current hypothesis is that dystonia is a disorder of distributed brain networks, including the basal ganglia, cerebellum, thalamus and the cortex resulting in abnormal neural motor programs. In comparison, functional dystonia (FD) may resemble other forms of dystonia (OD) but has a different pathophysiology, as a subtype of functional movement disorders (FMD). FD is the second most common FMD and amongst the most diagnostically challenging FMD subtypes. Therefore, distinguishing between FD and OD is important, as the management of these disorders is distinct. There are also different pathophysiological underpinnings in FD, with for example evidence of involvement of the right temporoparietal junction in functional movement disorders that is believed to serve as a general comparator of internal predictions/motor intentions with actual motor events resulting in disturbances in self-agency. In this article, we present a comprehensive review across the spectrum of FD, including oromandibular and vocal forms and discuss the history, clinical clues, evidence for adjunctive "laboratory-based" testing, pathophysiological research and prognosis data. We also provide the approach used at the Massachusetts General Hospital Dystonia Center toward the diagnosis, management and treatment of FD. A multidisciplinary approach, including neurology, psychiatry, physical, occupational therapy and speech therapy, and cognitive behavioral psychotherapy approaches are frequently required; pharmacological approaches, including possible targeted use of botulinum toxin injections and inpatient programs are considerations in some patients. Early diagnosis and treatment may help prevent unnecessary investigations and procedures, while facilitating the appropriate management of these highly complex patients, which may help to mitigate frequently poor clinical outcomes.

Voice and swallowing dysfunction in X-linked dystonia parkinsonism.

OBJECTIVES: To systematically characterize and describe voice and swallowing manifestations in patients with X-linked dystonia parkinsonism (XDP) and correlate with quality-of-life (QOL) measures. METHODS: Thirty-four patients with XDP with communication and swallowing difficulties underwent neurological examination, head and neck examination, nasopharyngoscopy, QOL surveys (Swallowing Quality of Life questionnaire [SWAL-QOL] and Voice Handicap Index [VHI]), and functional endoscopic evaluation of swallowing (FEES) to assess the extent of dysfunction. RESULTS: All patients showed high rates of lingual, oromandibular, and laryngeal dysfunction, as well as severe QOL changes in swallowing and communication ability. The most common head and neck manifestations of dystonic symptoms were difficulty coordinating the mouth and tongue (79%), uncontrollable tongue thrusting (53%), and jaw opening (35%). Laryngeal symptoms including vocal strain (adductor voice breaks) or stridor (32%), as well as velopharyngeal insufficiency (20%), were also identified. Of the patients with laryngeal symptoms, 18% had respiratory dystonia. Swallowing assessments showed significant abnormalities in oral bolus control and oropharyngeal dysphagia. FEES examinations showed that 87.5% of the study group had penetration or aspiration. QOL scores showed an average VHI of 94.4 (severe dysfunction), and SWAL-QOL showed an average of 37.7 (severe dysfunction). CONCLUSION: Swallowing and voice impairment in XDP is not well characterized and presents a more distinctive phenomenology than other neurological disorders, with a unique set of challenges for treatment. This is the first study to systematically evaluate laryngeal and pharyngeal dysfunction in XDP patients and correlate with QOL measures. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:171-177, 2020.