Synkinetic Brow Dysfunction: Diagnosis and Treatment Algorithm.

Importance: The diagnosis and management of brow dysfunction in patients with postfacial paralysis with synkinesis can be perplexing and challenging for the treating physician. Objective: To describe a novel diagnostic and treatment algorithm for brow dysfunction in patients with postfacial paralysis with synkinesis. Design: Surgical pearls-description of novel surgical technique. Setting: A private practice. Participants: Patients who underwent the operation.

End-to-Trunk Masseteric to Facial Nerve Transfer With Selective Neurectomy for Facial Reanimation.

OBJECTIVE: To examine functional outcomes following end-to-trunk masseteric to facial nerve transfer in patients with chronic flaccid facial paralysis. DESIGN: Retrospective chart review. SETTING: Tertiary-care private practice setting. PARTICIPANTS: Patients with complete unilateral facial paralysis of less than 24 months duration. INTERVENTIONS: Direct end-to-trunk masseteric to facial nerve anastomosis. OUTCOME MEASURES: Outcome measures included time to first movement, development of synkinesis, and an objective assessment of the resting tone and dynamic movement that was achieved. RESULTS: Patient age at the time of transfer ranged from 6 to 61. Follow-up ranged from 12 to 24 months. No patients had any perioperative complications. No patient experienced significant mass movement or synkinetic facial movement with chewing. No patient had worsened chewing or swallowing. Patients have not yet recovered significant resting tone. All patients achieved smile activity when biting down with a median (interquartile range) oral commissure excursion of 7.57 mm (5.19-9.94 mm), starting 3 to 5 months after transfer. CONCLUSIONS: End-to-trunk masseteric to facial nerve transfer is a safe and effective procedure. Patients had rapid reinnervation with good excursion and achieved a natural appearing smile. The rehabilitated smile appears better than that achieved with hypoglossal-facial nerve transfer. The procedure can be performed coincident with cross-facial nerve grafting, and in some cases may produce dynamic facial movement that obviates the need for free muscle transfer.

Epigenetics of obstructive sleep apnea syndrome: a systematic review.

STUDY OBJECTIVES: Obstructive sleep apnea (OSA) is a chronic and widely prevalent disease associated with multiple health disorders. Current diagnostic strategies for OSA are limited because of cost, time, and access. Epigenetic signatures offer insight into the relationships between disease and environment and could play a significant role in developing both diagnostic and therapeutic tools for OSA. In the current study, a systematic literature search was conducted to investigate the existing evidence of OSA-associated epigenetic modifications. METHODS: A systematic literature search was performed using electronic academic databases including PubMed, CINAHL, Scopus, Embase, EBM Reviews, and Web of Science. However, the current study focused on screening for original, English-language articles pertaining to OSA and associated epigenetic mechanisms. To produce unbiased results, screening was performed independently by authors. RESULTS: We identified 2,944 publications in our systematic search. Among them, 65 research articles were related to OS A-associated differential gene expression, genetic variation, and epigenetic modifications. Although these 65 articles were considered for full manuscript review, only 12 articles met the criteria of OSA-associated epigenetic modifications in human and animal models. Human patients with OSA had unique epigenetic changes compared to healthy control patients and, interestingly, epigenetic signatures were commonly identified in genes associated with metabolic and inflammatory pathways. CONCLUSIONS: Although the available studies are limited, this research provides novel insights for the development of epigenetic markers for the diagnosis and treatment of OSA. Thorough genome-wide investigations will be required to develop cost-effective, robust biomarkers for the identification of OSA among children and adults. Here, we offer a study design for such efforts. CITATION: Leader BA, Koritala BSC, Moore CA, Dean EG, Kottyan LC, Smith DF. Epigenetics of obstructive sleep apnea syndrome: a systematic review. J Clin Sleep Med. 2021;17(12):2533-2541.

Validation of an Objective Assessment Tool for Tonsillectomy in Otolaryngology Resident Training.

OBJECTIVE: Create and validate an objective structured assessment of technical skills (OSATS) for otolaryngology residents learning how to perform a tonsillectomy. STUDY DESIGN: Multicenter prospective longitudinal validation study. METHODS: A multi-institutional study at six tertiary academic otolaryngology residency programs from July 2009 to May 2012. Using the modified Delphi technique, a panel of pediatric otolaryngologists created a tonsillectomy task-based checklist (TBC) for a tonsil OSATS using a 5-point Likert-type scale. Residents were assessed by pediatric otolaryngology staff at the time of surgery with the TBC and a global rating scale. Procedure time, patient age, number of previously performed tonsillectomies, and surgical technique were also collected. RESULTS: One hundred sixty-seven tonsil OSATS were completed for 38 residents, and competency was recorded for 99 (59.2%). Residents scored as competent had performed significantly more previous tonsillectomies than those deemed noncompetent, 44.4 ± 35.6 and 13.5 ± 11.6, respectively (P < .001). The mean overall score on the tonsil TBC was 4.0 ± 0.8 and 2.6 ± 1.0 for competent and noncompetent, respectively (P < .001). Higher number of tonsillectomies performed and mean tonsil TBC score significantly increased the likelihood of competency (P < .001). Each additional tonsillectomy performed increased the likelihood of achieving competency by 6.3% (P = .006, 95% confidence interval (CI): 1.330-1.110), and each 1.0 point increase in mean tonsil TBC score increased the likelihood of competency by a factor of 2.71 (P = .006, 95% CI:1.330-5.513). There is a 95% likelihood of competency at 48 tonsillectomies or a tonsil TBC score of 4.91. CONCLUSION: The tonsil OSATS is a valid and feasible instrument to assess resident competency with tonsillectomy and provides timely objective feedback. LEVEL OF EVIDENCE: 4. Laryngoscope, 131:E359-E366, 2021.

Synkinetic Unilateral Lower Lip Palsy: Diagnosis and Technical Considerations for Facial Reanimation.

Importance: There are no universally adopted surgical techniques to treat depressor labii inferioris (DLI) dysfunction in patients with postfacial paralysis synkinesis. We describe a novel description of this disorder and technical surgical considerations for reanimation. Objective: To describe a new classification for DLI dysfunction and a surgical option to restore a natural appearing full dentition smile. Design: Surgical pearls-description of novel surgical technique. Setting: A private practice. Participants: Patients who underwent the operation.

The impact of resident involvement on tonsillectomy outcomes and surgical time.

OBJECTIVE: Posttonsillectomy hemorrhage can be life-threatening, so we investigated whether patients are at increased risk with an inexperienced surgeon. There is scant information on how surgical experience affects outcomes in pediatric tonsillectomy. We hypothesized that supervised residents would have longer operative times but no difference in complication rates compared to attending surgeons. STUDY DESIGN: Retrospective case series of children who underwent tonsillectomy from July 2014 to April 2017 at a tertiary pediatric medical center. METHODS: We assessed outcomes and operative times, based on the primary surgeon's level of training, for children (14 months to 22 years) who underwent tonsillectomy. RESULTS: A total of 7,606 children were included (mean age 7.0 ± 4.1 years, 51% female) with a mean body mass index (BMI) of 18.6 ± 5.48 kg/m2 ; 76% were white; and 13% were black. Residents assisted with tonsillectomy in 43% of cases. The readmission rate (5%-6%) was not different (P = 0.48) by level of experience. Similarly, return to the operating room for control of hemorrhage (3.3%-3.5%) did not differ by level of experience (P = 0.95). The median procedure time for adenotonsillectomy was shortest for attendings (9 minutes), followed by fellows (13 minutes), and residents (14 minutes, P < 0.0001). Among residents, time for adenotonsillectomy decreased significantly with each increasing year of training (P < 0.0001) from postgraduate year (PGY) 1 (17 minutes), to PGY2 (15 minutes), to PGY3 (14 minutes), and to PGY4 (12.5 minutes). CONCLUSION: Attending surgeons completed tonsillectomy more quickly, and operative times decreased with increasing experience level. However, there was no difference in readmission or postoperative hemorrhage rates between residents and attending surgeons. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:2481-2486, 2020.

Endoscopic Removal of a Cervical Esophageal Duplication Cyst.

A 6-month-old female presented for 2 months of noisy breathing. Flexible laryngoscopy showed limited bilateral vocal fold abduction. Computed tomography revealed a non-enhancing 3.6 × 2.3 × 3.5 cystic prevertebral mass spanning C2-T. Using an endoscopic approach, the overlying mucosa was incised, and the cyst was freed and fully excised from the surrounding mucosa with blunt microlaryngeal instruments without complication. Three months postoperatively she had no respiratory issues and was eating well. Flexible laryngoscopy revealed bilateral vocal fold mobility. We propose that endoscopic removal of a cervical esophageal duplication cyst in selected cases is an alternative to open excision. Laryngoscope, 130:2053-2055, 2020.

The impact of persistent pediatric obstructive sleep apnea on the Quality of Life of Patients' families.

OBJECTIVES: To (1) quantify the quality of life (QOL) for families of children affected by persistent obstructive sleep apnea (OSA), and (2) assess factors contributing to the negative impact of OSA on families. METHODS: Prospective case series in a multidisciplinary upper airway center at a tertiary pediatric institution. Our study included patients with persistent OSA referred to our clinic from 2014 to 2016. Both patients and their families completed validated questionnaires assessing QOL and OSA symptoms, including the Family Impact Questionnaire (FIQ), Pediatric Sleep Questionnaire, the Pediatric Quality of Life Inventory, the OSA-18, and the Epworth Sleepiness Scale. RESULTS: Families of 67 patients were included. The mean patient age was 12.5 (95%CI 11.9-13.1); 23 (34.3%) were female, and the mean BMI percentile was 80.2 (95%CI 73.6-86.8). The single most common comorbidity was Down syndrome (45.6%). The mean obstructive apnea-hypopnea index was 9.7 ±â€¯10.3 events/hour. A significant association exists between OSA severity and 18 FIQ negative subscore (P < 0.001). Financial impact was the primary negative concern for parents of patients with OSA compared to those without OSA (P = 0.03). There were no other significant differences between those with and without OSA. CONCLUSIONS: There was a significant correlation between persistent OSA severity and the FIQ negative impact of disease score on patients' families. Concern regarding financial burden was more common for families of children with OSA than for those without OSA. This suggests that targeted interventions, particularly regarding the financial burden of persistent OSA diagnosis and management, may provide some relief to families.

CO2 laser supraglottic resurfacing for non-granulomatous chronic supraglottitis in two children.

Chronic epiglottitis and supraglottitis are clinical entities that present with respiratory distress and are primarily associated with autoimmune disorders, gastroesophageal reflux disease, or angioedema. First described in adults with sarcoidosis in 2010, CO2 laser epiglottis resurfacing has been effective in reducing epiglottic edema. We present two cases of adolescent males with non-granulomatous chronic supraglottitis who were successfully treated with CO2 laser supraglottic resurfacing.

Relative Preservation of Superior Semicircular Canal Architecture in CHARGE Syndrome.

OBJECTIVES: (1) Describe common patterns of semicircular canal (SCC) anomalies in CHARGE syndrome (CS) and (2) recognize that in CS, the architecture of the superior SCC may be relatively preserved. STUDY DESIGN: This is a retrospective review of temporal bone imaging studies. SETTING: Quaternary care center. SUBJECTS AND METHODS: A sample of 37 patients with CS. All subjects met clinical diagnostic criteria for CS. The presence/absence of anomalies of the middle ear, mastoid, temporal bone venous anatomy, inner ear, and internal auditory canal was recorded. Anomalies of each SCC were considered separately and by severity (normal, dysplasia, aplasia). RESULTS: Thirty-seven subjects (74 temporal bones) were reviewed. Thirty-four (92.0%) patients demonstrated bilateral SCC anomalies. Three (8.0%) had normal SCCs. In patients with SCC anomalies, all canals demonstrated bilateral abnormalities. Thirty-two (86.5%) patients had bilateral horizontal SCC aplasia. These 32 patients also demonstrated posterior SCC aplasia in at least 1 ear. Of 74 temporal bones, 37 (50.0%) had superior SCC dysplasia. All dysplastic superior SCCs showed preservation of the anterior limb. Complete superior SCC aplasia was found in 28 (37.8%) temporal bones. CONCLUSION: SCC anomalies occur with high frequency in CS. Complete absence of the horizontal and posterior canals is typical and usually bilateral. By contrast, the superior SCC often demonstrates relative preservation of the anterior limb.

Safety of radiofrequency ablation for adenotonsillectomy after cochlear implantation.

OBJECTIVE: While a cadaveric animal study has suggested that radiofrequency ablation can be safely used in patients with cochlear implants, no in vivo studies have been published to confirm that radiofrequency ablation does not alter the integrity of the cochlear implant device. METHODS: Cochlear implant impedance and functional performance were studied through a prospective case series in five children with seven functioning multichannel implants before and after radiofrequency ablation adenotonsillectomy. RESULTS: There were 4 females and 1 male patient, aged 6-10 years (mean 8.5 ±â€¯1.95 years) with 7 functioning implants. Pre- and post-surgical impedance testing revealed all electrodes were within normal operating limits. There was no statistically significant difference between the mean pre and post-operative impedances in 5 of the 7 tested implants (P = 0.2-0.8). The other two implants showed statistically significant improvement in impedance values which were not clinically significant (P = 0.02 and P < 0.001). Speech perception was unchanged as was functional performance for all 7 tested implants. CONCLUSIONS: We found that radiofrequency ablation used in the oropharynx during adenotonsillectomy did not alter the integrity of the cochlear implant devices when assessed using electrode impedance testing, audiometry and speech perception evaluation. These results confirm those reported in previous in vitro studies and confirm the safety of radiofrequency ablation adenotonsillectomy for children who have undergone previous cochlear implant placement.

Tonsillar Small Cell Carcinoma: Potential Contribution of Human Papillomavirus.

BACKGROUND Extrapulmonary small cell carcinoma (SmCC) is a relatively rare clinical entity constituting only 2.5-5% of SmCCs. Recently, evidence has emerged that high-risk types of human papillomavirus (HPV) might play an etiologic role in oropharyngeal SmCC, similar to squamous cell carcinoma. CASE REPORT Here, we present a case of tonsillar SmCC that presented as combined SmCC-squamous cell carcinoma in a cervical lymph node, raising the possibility that the SmCC-component represents disease progression. CONCLUSIONS This case lends further support to the importance of HPV in the development of oropharyngeal SmCC and suggests a mechanism of disease progression.

Granulomatosis with polyangiitis presenting as facial nerve palsy in a teenager.

Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e.g. fever, malaise), or when other organs are involved (kidney, lungs, etc.). We present an interesting case of a 14-year-old female with eight-weeks of bilateral otalgia, unilateral facial nerve palsy, decreased appetite, and fatigue refractory to steroid, anti-viral, and antibiotic treatment ultimately diagnosed with GPA.

Perceived compliance and barriers to care in sublingual immunotherapy.

BACKGROUND: For allergy immunotherapy to be effective, patient compliance is critical. However, aqueous sublingual immunotherapy (SLIT) may be considered demanding for patients, requiring strict daily dosing and refilling of medication at regular intervals. In this study we sought to determine how patients perceive their own compliance with SLIT and identify barriers that may hinder compliance. METHODS: Using a retrospective review, 46 patients currently undergoing aqueous SLIT were identified to have less-than-excellent compliance based on frequency of clinic visits for SLIT refills. Twenty-eight of these patients completed a phone survey regarding symptom improvement, compliance, and barriers to care. In addition, 56 patients who withdrew from therapy before completion were polled about barriers to adherence. RESULTS: Of the 28 patients participating in the initial phone survey, 24 (86%) reported subjective improvement in symptoms on SLIT, despite less-than-excellent compliance. Nineteen (68%) of these patients perceived their own compliance to be excellent. Eighteen patients (65%) reported the inconvenience of scheduling and attending clinic appointments to be the main reason for noncompliance. The most common reasons for withdrawal from therapy were cost (27%), lack of effectiveness (25%), and difficulty with compliance (14%). CONCLUSION: The majority of aqueous SLIT patients perceived their compliance to be excellent, although, based on a previous study, these patients did not reach excellent compliance benchmarks. Inconvenience of clinic visits and cost of therapy were found to be the most common barriers to care. Despite what providers perceived as less-than-excellent compliance, 82% of patients reported symptom improvement with SLIT.

Immunotherapy compliance: comparison of subcutaneous versus sublingual immunotherapy.

BACKGROUND: Patient compliance is critical for successful allergen immunotherapy (AIT). Previous studies suggest that AIT compliance is worse outside of controlled clinical trials, with reported subcutaneous immunotherapy (SCIT) and sublingual immunotherapy (SLIT) noncompliance at 11% to 50% and 3% to 25%, respectively. METHODS: A retrospective review of 384 AIT patients at a single, tertiary care otolaryngic allergy practice evaluated SCIT and SLIT compliance, based on treatment stage. SCIT compliance was defined as the number of 2-week breaks per year or in compliance with their defined schedule: excellent = 2 or fewer; good = 3 to 4; fair = 5 to 6; and poor = 7 or more. Compliance with SLIT was defined as the number of days vials were refilled within the defined expiration date: excellent = 10 days or fewer; good = 11 to 15 days, fair = 16 to 20 days; and poor = 25 or more days. Fisher exact and chi square tests were used for statistical analysis. RESULTS: Seventy-four SCIT and 200 SLIT patients had data appropriate for analysis. Compliance rates were excellent (62%) or good (22%) in 62 SCIT patients and excellent (31%) or good (35%) in 131 SLIT patients. Comparing excellent compliance rates, SCIT patients had a higher rate of excellent compliance at all stages of treatment compared to SLIT patients (p < 0.05). For SCIT patients there was no significant difference in excellent compliance rates between escalation, first year of maintenance, and greater than 1 year of maintenance (p > 0.05). CONCLUSION: The results of this study showed higher rates of patient adherence to treatment protocols among SCIT patients. There was no decrease in SCIT compliance rates across treatment stages.

Pruritus epidemiology and quality of life.

The burden of chronic pruritus is increasingly recognized as significant worldwide. As wet-laboratory researchers investigate the pathophysiology of chronic pruritus, epidemiologists and health services researchers are quantifying the impact of pruritus by incidence, prevalence, and quality of life measures. Outcomes researchers are also investigating factors that may predict chronic pruritus incidence and severity. Such efforts will direct resources for research, public health intervention, and clinical care.

Functional characterization of a SUMO deconjugating protease of Plasmodium falciparum using newly identified small molecule inhibitors.

Small ubiquitin-related modifier (SUMO) is implicated in the regulation of numerous biological processes including transcription, protein localization, and cell cycle control. Protein modification by SUMO is found in Plasmodium falciparum; however, its role in the regulation of the parasite life cycle is poorly understood. Here we describe functional studies of a SUMO-specific protease (SENP) of P. falciparum, PfSENP1 (PFL1635w). Expression of the catalytic domain of PfSENP1 and biochemical profiling using a positional scanning substrate library demonstrated that this protease has unique cleavage sequence preference relative to the human SENPs. In addition, we describe a class of small molecule inhibitors of this protease. The most potent lead compound inhibited both recombinant PfSENP1 activity and P. falciparum replication in infected human blood. These studies provide valuable new tools for the study of SUMOylation in P. falciparum.