Laryngeal Ganglioneuromatosis in a Child With Multiple Endocrine Neoplasia Type 2B (MEN2B): Case Report and Review of Literature.

Multiple Endocrine Neoplasia type 2B (MEN2B) is an autosomal dominant cancer syndrome caused by a mutation in rearranged during transfection (RET) proto-oncogene and includes medullary thyroid carcinoma, pheochromocytoma, gastrointestinal neuromas, and mucosal ganglioneuromas. Medullary thyroid carcinoma is the major cause of mortality in MEN2B syndrome. Medullary thyroid carcinoma can often appear during the first years of life. While mucosal neuromas in MEN2B are common, laryngeal neuromas are extremely rare. We present a third case of a pediatric patient with a laryngeal neuroma localized to the left true vocal cord and conduct a literature review of vocal cord neuromas in MEN2B patients.

Flexible and Rigid Bronchoscopy for Critically Ill Children on Extracorporeal Membrane Oxygenation.

BACKGROUND: We aim to describe our experience with bronchoscopy to diagnose and relieve tracheobronchial obstruction in anticipation of decannulation in children on extracorporeal membrane oxygenation (ECMO) support. METHODS: A retrospective cohort study of children on ECMO between 1/2018 and 12/2022. RESULTS: A total of 107 children required ECMO support during the study period for cardiac (n = 48, 45%), pulmonary (n = 38, 36%), or cardiopulmonary dysfunction (n = 21, 20%). Thirty-seven (35%) patients underwent 99 bronchoscopies while on ECMO. Most (76%, n = 75) experienced no improvement or worsening of chest radiography 24 hours following bronchoscopy. Clinical improvement in tidal volumes 48 hours after the first bronchoscopy was noted in 13/25 patients with available data (p = 0.05). Adverse events were seen in 18 (49%) patients who underwent bronchoscopy, including pneumothorax (n = 8, 22%), pneumonia (n = 7, 19%), pulmonary hemorrhage (n = 6, 16%), and sepsis (n = 5, 14%). ECMO courses were longer (25.4 ± 37.2 vs 6.1 ± 8.8 days, p < 0.0001) and more likely to be complicated by pneumonia (p = 0.0004) and sepsis (p = 0.047) in patients who underwent bronchoscopy compared with those who did not. Adverse events following bronchoscopy were associated with the number of bronchoscopies (p = 0.0003) and the presence of obstructive materials but not with the type of bronchoscopy or indication for ECMO. Mortality rates were similar between patients who underwent bronchoscopy and those who did not. CONCLUSION: Children requiring bronchoscopy represent a subset of the sickest children on ECMO. Bronchoscopy may provide benefit in children with persistent cardiopulmonary failure who could not otherwise be decannulated. Adverse events are associated with the number of bronchoscopies and the presence of obstructive material. LEVEL OF EVIDENCE: 4 Laryngoscope, 2024.

Swallowing Function After Epiglottopexy in Children.

OBJECTIVE: Epiglottopexy has been an increasingly utilized intervention in children with epiglottic prolapse and airway obstruction. Given the role of the epiglottis in protecting the airway during swallowing and the potential effect of repositioning the epiglottis on the passage of the bolus, we aimed to compare swallowing outcomes before and after epiglottopexy in children. STUDY DESIGN: A retrospective cohort study. SETTING: Tertiary care children's hospital. METHODS: Data were extracted from charts of children who underwent epiglottopexy and had a subsequent instrumental swallowing evaluation between January 2018 and September 2022. RESULTS: A total of 93 patients underwent epiglottopexy. Of these, 38 patients met inclusion requirements. The mean age at surgery was 41 ± 47 months. Most patients (n = 37, 97.4%) had significant comorbidities such as secondary airway lesions (n = 33, 91.7%), a genetic or syndromic disorder (n = 25, 69.4%), and dysphagia (n = 29, 76.3%). All patients had a concurrent procedure at the time of epiglottopexy with supraglottoplasty (n = 24, 63.2%) and lingual tonsillectomy (n = 16, 42.1%) being the most common. No changes in initiation or patterns of swallowing were noted postoperatively. A total of 7 (18.4%) patients had worsening swallow function: 2 had new-onset dysphagia, and 5 had worsening pre-existing dysphagia. Liquid or food textures penetrated remained unchanged or improved in most cases. No risk factors for worsening dysphagia were identified in our cohort. CONCLUSION: Children with medical comorbidities undergoing epiglottopexy with additional airway interventions may experience new or worsening dysphagia. However, the procedure is generally safe without notable patterned changes in the swallowing mechanism.

Persistence of aerodigestive symptoms after vascular ring repair.

OBJECTIVE: Vascular rings are often associated with respiratory and swallowing difficulties due to tracheal or esophageal compression. While the results of a vascular ring repair are considered excellent, the long-term effect of tracheal and esophageal remodeling and the persistence of symptoms have scarcely been reported. Our study aims to evaluate the respiratory and swallowing outcomes of vascular ring repair. STUDY DESIGN AND METHODS: A retrospective cohort study of children who underwent vascular ring repair between 2010 and 2022 in a tertiary-care children's hospital. RESULTS: There were 108 patients enrolled: sixty-three patients (57.41 %) with a right aortic arch, 42 patients (38.89 %) with a double aortic arch, and 3 patients (2.78 %) with other vascular rings. Forty-three (39.81 %) patients were diagnosed prenatally. Of the 65 patients (60.19 %) diagnosed postnatally, 35/65 (53.85 %) had either respiratory or swallowing symptoms as the indication for diagnostic workup. Persistent respiratory and swallowing symptoms were noted in 34/108 (31.48 %) and 30/108 (27.78 %) patients, respectively, within a year of surgical repair. Fourteen patients underwent repeated laryngoscopy and bronchoscopy that demonstrated residual tracheomalacia; however, only 2/14 (1.9 %) patients required tracheostomy tube placement, and 6-out-of-7 patients were weaned off positive pressure airway support. Persistent respiratory symptoms were significantly more common in patients with a double aortic arch compared to a right aortic arch. No differences were noted in demographics, comorbidities, and preoperative aerodigestive symptoms between patients with residual symptoms and patients with no residual symptoms. CONCLUSIONS: Persistent respiratory and swallowing symptoms after vascular ring repair are not uncommon. Postoperative evaluation should be pursued by a dedicated team, and treatment considered as appropriate.

The Clinical Impact of Vagal Nerve Stimulator Implantation on Laryngopharyngeal Function in Children: A Single-Center Experience.

OBJECTIVE: A vagal nerve stimulator (VNS) has been established as the treatment of choice for children with refractory epilepsy. The outcomes of the procedure have been well documented in adults but are less clear in children. The goal of our study was to review laryngopharyngeal (LP) function following VNS implantation in children. STUDY DESIGN: Case series with chart review. SETTING: Tertiary-care children's hospital. METHODS: Voice, swallowing, and sleep apnea symptoms were extracted from the charts of children who underwent VNS implantation between 2013 and 2021. A questionnaire was sent to parents of implanted children to ascertain the degree of the social and functional impact of the implant. RESULTS: There were 69 patients, aged 2.3 to 21.4 years old, who met the inclusion criteria. LP symptoms were most common during the first year following implantation; 26 patients (37.6%) demonstrated at least 1 symptom (voice alteration, chronic cough, sleep-disordered breathing, or dysphagia), and 15 patients required adjustments to their implant settings. The incidence of symptoms and the need to adjust VNS settings significantly dropped during years 2 to 5 and 6 to 8 (22% vs 7% and 5%, respectively, p = .0002). The mean score of the Pediatric Voice Handicap Index differed greatly from a normal control group on each subscale and the total score. CONCLUSION: LP dysfunction in children following VNS implantation is comparable to adults, with the most burden noticed during the first year after implantation. The presence of voice alterations did not correlate with the presence of dysphagia and sleep-disordered breathing. Thorough evaluation, preferably by a multidisciplinary team, is required to assess LP dysfunction postoperatively.

Epiglottopexy in Infants Younger Than 6 Months Old: A Case Series.

BACKGROUND: Severe laryngomalacia, characterized by apnea, hypoxia, and feeding difficulties, is an uncommon diagnosis that often requires surgical intervention with supraglottoplasty. Children who require surgery at a young age and those with additional comorbidities pose a special challenge and may require further surgical interventions. Posterior displacement of the epiglottis has been noted in some infants with congenital stridor and is commonly treated with epiglottopexy. The goal of our study is to review the outcomes of epiglottopexy combined with supraglottoplasty in our cohort of infants younger than 6 months old with severe laryngomalacia. METHODS: A retrospective chart review of infants younger than 6 months old who underwent epiglottopexy combined with supraglottoplasty for severe laryngomalacia from January 2018 to July 2021 at a tertiary care children's hospital. RESULTS: 13 patients (age 1.3 week-5.2 months) underwent supraglottoplasty and epiglottopexy for severe laryngomalacia and epiglottis retroflection. The patients were admitted to the intensive care unit and remained intubated for at least one night. All patients demonstrated subjective and objective improvement in upper airway respiratory signs and symptoms. Ten patients demonstrated aspiration immediately postoperatively, despite 4 of them having no concern for aspiration at preoperative evaluation. On follow-up, 1 patient required revision supraglottoplasty and epiglottopexy for persistent laryngomalacia, and 2 patients required tracheostomy tube placement due to cardiopulmonary comorbidities. CONCLUSION: Infants younger than 6 months old with medical comorbidities undergoing epiglottopexy with supraglottoplasty may demonstrate significant improvement in respiratory symptoms. Worsening dysphagia may complicate the postoperative period, particularly among children with medical comorbidities.

Epiglottopexy for refractory obstructive sleep apnea in children - A single-institution experience.

BACKGROUND: Refractory obstructive sleep apnea (OSA) is a common condition in children with medical comorbidities, leading to a significant impact on quality of life. Drug-induced sleep endoscopy (DISE) has become the standard of care in identifying the levels of obstruction in children with refractory OSA. Epiglottopexy has been shown to improve OSA symptoms in adults and healthy children with epiglottic prolapse in a few studies, with minimal long-term complications. The objective of our study was to evaluate the role of epiglottopexy in children with refractory OSA. METHODS: A retrospective chart review of children with refractory OSA who were found to have epiglottic prolapse on DISE, and underwent epiglottopexy between January 2018 and November 2021 at a pediatric tertiary care hospital. RESULTS: 42 patients (age 8.1 ± 5.1 years) met inclusion criteria. Thirty patients (71.4 %) suffered from neurodevelopmental disease or congenital syndrome, and 14 patients (33.3 %) were gastrostomy-tube dependent. All patients had at least one prior surgical procedure to address their OSA. Thirty-six patients (85.7 %) were diagnosed with refractory OSA by polysomnography prior to surgery, with an average apnea-hypopnea index (AHI) of 12.4 ± 9.7/h. Forty patients (95.2 %) required an additional procedure in conjunction with epiglottopexy including lingual tonsillectomy (n = 27, 64.3 %), supraglottoplasty (n = 14, 33.3 %), tonsillectomy with or without revision adenoidectomy (n = 9, 21.4 %) and tongue base suspension (n = 1, 2.4 %). Twenty-one patients had repeated polysomnography; 4 patients were found to have residual severe OSA post-operatively (average AHI 17.4 ± 11.4/h), while the remaining patients demonstrated clinical improvement and a significant reduction in OSA severity, with an average AHI of 1.5 ± 2.2/h. Regression analysis identified pre-operative oxygen nadir <75 % to be associated with residual OSA postoperatively. Following surgery, 7 patients were found to have new-onset or worsening dysphagia, 6 of whom were diagnosed with complex medical comorbidities. CONCLUSIONS: Epiglottopexy, as part of multi-level airway surgery, is associated with a significant improvement in the severity of refractory OSA. Dysphagia may complicate the post-operative course, particularly in children with medical comorbidities.

Salivary gland surgery and nonviral respiratory-related hospitalizations in children with neurodevelopmental impairment.

OBJECTIVES: Neurodevelopmentally impaired (NI) children with chronic sialorrhea are at elevated risk for aspiration and respiratory tract infections. Direct resection or ligation ("DROOL") of the submandibular glands (SMG) with parotid duct ligation are surgical interventions intended to decrease salivary output. The objective of this study is to determine the impact of DROOL surgery on the incidence of nonviral respiratory-related (NVR) post-procedure hospital encounters including emergency department visits and admissions. METHODS: Retrospective case series of NVR related outcomes after DROOL surgery in children performed at a single institution, tertiary referral center. RESULTS: A total of 35 gastrostomy tube-dependent patients (60% male, average age 8.2 [SD 6.0] years) with NI underwent DROOL surgery (86% SMG excision). Pre- and post-surgical follow-up time was 3.6 and 3.2 years, respectively. Presurgical and postsurgical NVR hospital encounters occurred in 28 (80%) and 14 (40%) patients, respectively (p < 0.01). Mean (SD) postoperative NVR hospital encounters occurred less frequently when compared to presurgical period (0.4 [0.6] vs. 1.0 [1.2] per year, p < 0.01) with average change of -0.7 encounters per year (SD 1.4, 95% CI -1.0 to -0.2). Patients with encounters within a year preceding DROOL (OR 4.9, p = 0.04, 95% CI 1.1-22.8), or those with at least 3 preoperative encounters (OR 8.0, p = 0.01, 95% CI 1.6-40.3) were significantly associated with a postsurgical NVR event. Fewer patients used anti-sialorrhea medication postoperatively compared to preoperatively (60% vs. 17%, p < 0.01). No patient developed surgical site complications requiring operative interventions. CONCLUSIONS: DROOL surgery for chronic sialorrhea in patients with NI was associated with decreased hospitalization and ED visits for NVR respiratory events post-procedurally. Sialorrhea may be an actionable source of recurrent respiratory illnesses requiring hospitalizations.

Management of broncho-esophageal fistula after button battery ingestion.

Button battery ingestion can cause serious injury or death in young children who cannot communicate symptoms. An 18-month-old male presented after his mother noted drooling, nonbilious emesis and a metallic smell to his breath. He underwent rigid esophagoscopy and a 3-V 20-mm button battery was removed. Subsequent bronchoscopy after a 1-week interval revealed progression to a large broncho-esophageal fistula on the posterior wall of the right mainstem bronchus past the carina. A fenestrated nasogastric tube for local control of secretion and a feeding jejunostomy was placed. Six weeks later, the patient underwent a right thoracotomy for division and repair of the fistula and intercostal muscle flap interposition. Utilizing a well-placed fenestrated nasogastric tube to manage secretions can help reduce fistula size and improve conservative management results. When surgical repair is required, an intercostal muscle flap can reinforce fistula closure while simultaneously buttressing the bronchus and esophagus.

Responsiveness and convergent validity of the chronic rhinosinusitis patient-reported outcome (CRS-PRO) measure in CRS patients undergoing endoscopic sinus surgery.

BACKGROUND: The chronic rhinosinusitis patient-reported outcome (CRS-PRO) measure is a 12-item measure with previously demonstrated validity in chronic rhinosinusitis (CRS) patients receiving medical therapy. This study establishes the factor structure, responsiveness, and convergent validity of the CRS-PRO following endoscopic sinus surgery (ESS). METHODS: Northwestern CRS Subject Registry patients had pre-ESS, 3-month (n = 111; CRS without nasal polyps [CRSsNP] = 60, CRS with nasal polyps [CRSwNP] = 51), and 6-month (n = 86; CRSsNP = 47, CRSwNP = 39) post-ESS assessments where patients completed the CRS-PRO, 22-item Sino-Nasal Outcome Test (SNOT-22), and four Patient-Reported Outcomes Measurement (PROM) Information System (PROMIS) short forms (general health measures). Patients had pre-ESS objective testing (endoscopic and radiographic assessment). Factor analysis was conducted using principal axis factoring with varimax rotation on the baseline CRS-PRO. The clinically important difference (CID) was estimated using both distribution-based and anchor-based methods. RESULTS: Factor analysis found the CRS-PRO comprised the "rhino-psychologic," "facial discomfort," and "cough" factors, which were responsive to ESS and correlated with the other PROMs. The changes observed in the CRS-PRO at 3 months had strong correlation with the corresponding changes in SNOT-22 (r = 0.792, p < 0.0001) and moderate correlations with changes in PROMIS fatigue and sleep domains. These changes had a very large effect size (Cohen's d 1.44) comparable to the longer SNOT-22 (Cohen's d 1.41) with slightly larger effect sizes observed in CRSwNP compared to CRSsNP patients. Similar convergent validity and responsiveness were observed in the 6-month data. The CRS-PRO CID was estimated to be between 5.0 and 7.5 (midpoint 6.0) using distribution-based and anchor-based methods. CONCLUSION: This study demonstrates the validity and responsiveness of the CRS-PRO in subjects receiving ESS.

Socioeconomic and clinical factors influencing treatment selection in microtia and aural atresia.

OBJECTIVES: Patients with microtia and aural atresia have multiple options for treatment of conductive hearing loss (CHL) and auricle reconstruction; however, little is known about the factors influencing treatment selection. This study aims to review the socioeconomic and clinical data of microtia/atresia patients to evaluate congruency with national data and whether these factors affect treatment decisions. METHODS: Retrospective review of patients evaluated in the microtia and atresia multidisciplinary clinic (MDC) at a tertiary academic children's hospital between 2008 and 2018. Outcomes included demographic, socioeconomic and clinical factors associated with hearing surgery and framework surgery. RESULTS: 373 patients were seen in the Microtia MDC: 193 (51.7%) were male, 187 (50.1%) identified as Hispanic and 23 (6.2%) identified as Asian. 267 (75.6%) patients received a nonsurgical bone conduction hearing device (BCHD); fitting at a younger age was associated with better nonsurgical BCHD compliance. Multivariate analysis was performed on the patients that were eligible for surgery based on age and appropriate follow-up. 70 (18.8%) patients had placement of an osseointegrated BCHD; inconsistent compliance with nonsurgical BCHD decreased the odds of proceeding with osseointegrated BCHD placement. 60 (16.1%) patients underwent framework surgery for external reconstruction. Placement of osseointegrated BCHD was the only factor that was associated with proceeding with framework surgery. Other assessed demographic and socioeconomic factors were statistically not associated with selection of surgical intervention. CONCLUSION: Fitting a nonsurgical BCHD at a younger age is associated with higher likelihood of nonsurgical BCHD compliance, that is in turn associated with patients and families proceeding with osseointegrated BCHD and framework surgery.

Minimally invasive transoral image-guided drainage of a retropharyngeal abscess with mediastinal extension.

Retropharyngeal abscess (RPA) in children is a serious deep neck space infection that rarely is complicated by extension into the mediastinum. RPA with mediastinal abscess requires prompt surgical management, generally via external or transoral approach. We present the case of a 3-year-old boy with RPA with mediastinal extension who was managed with a unique multidisciplinary surgical approach with otolaryngology and interventional radiology. A transoral approach was utilized to pass a transnasal drain with image guidance into the mediastinal fluid collection. This report reviews the presentation and surgical management of RPA with mediastinal extension and describes a unique minimally invasive approach to drainage.

Responsiveness and Convergent Validity of a New Patient-Reported Outcome Measure for Chronic Rhinosinusitis (CRS-PRO).

BACKGROUND: The CRS-PRO is a new patient-reported outcome measure (PROM) for chronic rhinosinusitis (CRS) that was developed using extensive patient input per Food and Drug Administration guidance on PROMs acceptable for use as end points in clinical trials. OBJECTIVE: To assess the responsiveness and convergent validity of the CRS-PRO following standard-of-care medical therapy. METHODS: This was a prospective study of 51 patients (21 with nasal polyps and 30 without) with newly diagnosed CRS or having an acute CRS exacerbation who were initiated on appropriate medical therapy. At the baseline visit each patient completed the CRS-PRO questionnaire, the 22-item Sino-Nasal Outcome Test, the EuroQol 5-dimensional questionnaire, and 4 Patient-Reported Outcome Measure Information System short forms along with objective testing including endoscopic and radiographic scores, smell discrimination, and nasal inspiratory flow testing. This same battery of questionnaires and testing was administered at a follow-up visit 4 to 8 weeks later. RESULTS: We verified that shortening the 21-item CRS-PRO to 12 items as previously described maintains its psychometric properties. The 12-item CRS-PRO was responsive with a large effect size (Cohen's d, 0.94) comparable to the longer 22-item Sino-Nasal Outcome Test (Cohen's d, 0.93). The instrument was slightly more responsive to medically treated patients with CRS without nasal polyps compared with patients with CRS with nasal polyps (Cohen's d, 1.1 vs 0.89, respectively). The change in 12-item CRS-PRO total score has moderate correlation with change in Lund-Mackay computed tomography scores. CONCLUSIONS: The CRS-PRO is a 12-item rigorously developed, responsive, and valid PROM that was developed using extensive input from patients with current definitions of CRS, including its 2 major phenotypes.

Development and Preliminary Validation of a New Patient-Reported Outcome Measure for Chronic Rhinosinusitis (CRS-PRO).

BACKGROUND: Patient-reported outcome (PRO) measures developed and validated on patients with the currently defined phenotypes of chronic rhinosinusitis (CRS) are needed to support clinical trials in CRS. OBJECTIVE: This study developed and examined the initial reliability and validity of the CRS-PRO, a new PRO measure of CRS. METHODS: Instrument development was performed through structured interviews and focus groups with clinical experts and 45 patients with CRS meeting current definitions of disease, 21 patients with CRS without nasal polyps (CRSsNP), and 24 patients with CRS with nasal polyps (CRSwNP) to identify items important to patients. Then another 50 patients (32 with CRSsNP and 18 with CRSwNP) with stable CRS symptoms were enrolled to evaluate the reliability of the instrument. Each patient completed the CRS-PRO, Sinonasal Outcome Test-22 (SNOT-22), and 4 Patient-Reported Outcome Measurement Information System short forms at the baseline visit and then at least 7 days later. RESULTS: After the development process, 21 items were identified from the conceptual domains of physical symptoms, sensory impairment, psychosocial effects, and life impact. Using the responses of the 50 patients with CRS, 21 draft items were further refined to 12 items by eliminating conceptually similar or highly correlated items or those with low mean symptom severity. The 12-item questionnaire was shown to have excellent internal consistency (Cronbach α, 0.86) and test-retest reliability with a high intraclass correlation coefficient (0.89) and Pearson's correlation (r = 0.82, P < .0001). The 12-item CRS-PRO correlated highly with the longer SNOT-22 (r = 0.83, P < .0001) demonstrating its concurrent validity. We also demonstrated validity and reliability in a separate analysis for patients with CRSsNP and CRSwNP. CONCLUSION: The CRS-PRO is a concise, valid, and reliable measure that was developed with extensive input from patients with CRS with current disease definitions.

Predicting Obstructive Sleep Apnea Status With the Reflux Symptom Index in a Sleep Study Population.

OBJECTIVES/HYPOTHESIS: Otolaryngologic symptoms of obstructive sleep apnea (OSA) and their diagnostic utility are not well studied. We aimed to elucidate the prevalence of otolaryngologic symptoms among patients being evaluated for OSA. Given findings that the Reflux Symptom Index (RSI) was strongly associated with OSA status, we evaluated the diagnostic utility of the RSI for predicting OSA status. STUDY DESIGN: Cross-sectional. METHODS: We recruited 101 adults presenting for ambulatory polysomnograms to the Northwestern Sleep Disorders Center from July 2017 to July 2018. The Epworth Sleepiness Scale, Pittsburgh Sleep Quality Index, Leicester Cough Questionnaire (LCQ), RSI, Gastroesophageal Reflux Disease Questionnaire, Sino-Nasal Outcome Test-22, Nasal Obstruction Symptom Evaluation, Eustachian Tube Dysfunction Questionnaire 7, and Headache Impact Test were administered. Polysomnogram results were subsequently obtained. Patients with OSA (apnea-hypopnea index ≥ 5) and without OSA were compared. RESULTS: Of the 101 participants, 98 had valid sleep study results. Of those, 72 were diagnosed with OSA and 26 were not. The two groups differed significantly in age and body mass index (BMI). Of the questionnaires, only the RSI and LCQ means differed significantly, with worse symptoms in the OSA group (P = .003 and .014, respectively). Upon univariate regression, age, BMI, and RSI were associated with OSA status. Using regression coefficients, a clinical score of 2 (RSI) + 1.5 (BMI) + age yielded a diagnostic model (C-statistic = 0.807, P < .001). A threshold score of 104.21 was 76.4% sensitive and 73.1% specific. CONCLUSIONS: Patients with OSA have worse symptoms of laryngopharyngeal reflux as measured by the RSI. The addition of the RSI to the recognized factors of age and BMI improves diagnostic utility for OSA. LEVEL OF EVIDENCE: 2 Laryngoscope, 2020.

Are chronic cough and laryngopharyngeal reflux more common in obstructive sleep apnea patients?

OBJECTIVES/HYPOTHESIS: To assess if there is a significant difference in the prevalence and severity of chronic cough symptoms in obstructive sleep apnea (OSA) patients versus non-OSA patients and examine this relationship in regard to laryngopharyngeal reflux (LPR) symptoms. STUDY DESIGN: Prospective cohort study. METHODS: Patients referred to Northwestern Medicine Sleep Lab for home sleep testing were enrolled. Patients filled out the Leicester Cough Questionnaire (LCQ) and Reflux Symptom Index (RSI) before completing sleep testing. Home sleep testing results were reviewed, and patients were separated into non-OSA and OSA groups by standard Apnea-Hypopnea Index (AHI) criteria. Demographic characteristics and questionnaire scores of the two groups were compared. The relationship between OSA severity, as determined by AHI, and LCQ and RSI scores was assessed. RESULTS: Of the 52 patients enrolled, 33 patients met criteria for OSA and 19 patients did not. Comparing patients without OSA versus those with OSA, there was a significant difference in mean LCQ score (129.9 vs. 120.0, respectively; P = .02), implying worse cough symptoms among OSA patients, and mean RSI score (3.2 vs. 11.2, respectively; P = .0013), implying worse upper-airway reflux symptoms among OSA patients. There was a significant correlation between LCQ score and AHI (r = -0.39, P = .0061) and between RSI score and AHI (r = 0.37, P = .0078). CONCLUSIONS: OSA patients demonstrate worse chronic cough and LPR-related quality of life versus non-OSA patients. Furthermore, the severity of these quality-of-life measures was correlated with the severity of the AHI. Chronic cough and particularly the pharyngeal LPR symptoms may be associated with the presence and severity of OSA. LEVEL OF EVIDENCE: 2 . Laryngoscope, 129:1244-1249, 2019.

Challenges and outcomes of cholesteatoma management in children with Down syndrome.

INTRODUCTION: The high incidence of chronic otitis media with effusion and Eustachian tube dysfunction in children with Down syndrome (DS) may predispose them to cholesteatoma formation. Establishing the diagnosis, choosing the appropriate operative intervention, and post-operative care can be challenging. OBJECTIVE: To describe management strategies for cholesteatoma diagnosis, surgical treatment, and post-operative management in children with Down syndrome. METHODS: Retrospective case series of 14 patients (17 total ears) with Down syndrome diagnosed with cholesteatoma over a 9-year period. RESULTS: A total of 14 patients with cholesteatoma (3 with bilateral disease) were analyzed. Thirteen ears (76.5%) had ≥2 tympanostomy tubes insertions prior to cholesteatoma diagnosis, and otorrhea and hearing loss were the most common presenting symptoms. Common pre-operative CT scan findings included mastoid sclerosis and ossicular erosion. The average age at first surgery was 9.8 years, and the average follow-up was 4.3 years. For acquired cholesteatoma, most ears were managed with canal wall up (CWU) approaches, but ultimately 6/15 (40.0%) required canal wall down (CWD) approaches. Postoperatively, 3 (20.0%) ears developed new tympanic membrane retraction pockets, but no recurrent cholesteatoma. Four (26.7%) ears developed recurrent disease, and 3 (20.0%) had residual disease at secondary procedures. Ossiculoplasty was performed in 4 ears. Twelve (70.6%) ears were rehabilitated with hearing aids or FM systems. CONCLUSIONS: The diagnosis of cholesteatoma in Down syndrome was associated with otorrhea, hearing loss, and CT scan findings of ossicular erosion and mastoid sclerosis. Most cases were managed with CWU surgical approaches. Hearing aid use was common post-operatively.

Outcomes of tympanoplasty in children with down syndrome.

INTRODUCTION: The prevalence of chronic otitis media with effusion (COME), and Eustachian tube dysfunction (ETD) is high in Down syndrome (DS) patients. This often necessitates multiple tympanostomy tube (TT) placements resulting in a higher rate of persistent tympanic membrane (TM) perforation requiring tympanoplasty for repair. OBJECTIVES: To assess risk factors for persistent perforation and outcomes of tympanoplasty in DS patients. METHODS: Retrospective case series of 91 ears in 69 DS patients with TM perforations, who were either observed or underwent tympanoplasty. Clinical features, surgical outcomes, and hearing outcomes were assessed. RESULTS: 91 ears were evaluated. Sixty perforations were observed, and 31 perforations were repaired. The closure rate was 54.8% for primary surgery, and 70.9% after secondary surgical interventions in the Tympanoplasty Group, compared to 33.0% spontaneous closure rate in the Observation Group (p < 0.001). The only risk factor for failed tympanoplasty repair was persistent COME/ETD (OR 27.2, p = 0.001). In the Observation Group perforations diagnosed at an older age, with >3 TT insertions, and with persistent COME/ETD were less likely to close spontaneously. Patients undergoing tympanoplasty had worse preoperative pure tone averages than those being observed, but significant improvement in air-bone gaps were noted in the Tympanoplasty Group (p = 0.02) post-operatively. Patients were often rehabilitated with hearing aids regardless of intervention (53.3% Observation Group, 48.4% Tympanoplasty Group). CONCLUSIONS: Persistent TM perforation in children with Down syndrome was associated with a history of COME/ETD, and multiple prior TT insertions. Tympanoplasty was successful for repair in most patients who underwent surgical intervention, but residual hearing loss was common.

Contemporary Pharmacotherapy for Allergic Rhinitis and Chronic Rhinosinusitis.

Chronic rhinosinusitis (CRS) and allergic rhinitis (AR) are chronic conditions causing nasal inflammation. CRS is increasingly recognized as a chronic inflammatory process rather than a chronic infection. Although the primary initiating factors in CRS remain unclear, AR is driven by IgE-mediated hypersensitivity to environmental allergens. Understanding the underlying inflammatory pathways and disease endotypes are driving innovation toward novel pharmacotherapies targeting critical mediators implicated in CRS and AR, including IL-4, IL-13, IL-5, IgE, and epithelial initiators IL-33 and TSLP. Extensive investigations are needed to determine the role, timing, predictive prognostic factors and long-term safety and efficacy of these agents.

Endoscopic transcanal approach to the middle ear for management of pediatric cholesteatoma.

OBJECTIVES: To describe outcomes for endoscopic ear surgery (EES) for pediatric cholesteatoma at a tertiary pediatric hospital. METHODS: Retrospective case series of 65 pediatric cholesteatoma cases in 38 ears. Subgrouping based on cholesteatoma type and EES type. Surgical findings, outcomes, and demographic data were evaluated. RESULTS: Endoscopes were used in 65 pediatric cholesteatoma cases in 38 primary ears (34 patients), followed for an average of 2.6 years (9 months to 4.6 years). The endoscope was used as the primary visualization tool in 31 (81.6%) ears (EES 2 or 3), and as an adjunct to the microscope in seven ears (EES 1). Twenty-two (57.9%) ears and 35 (53.4%) cases were transcanal endoscopic ear surgery (EES 3 or TEES). Overall, there was recurrence in five (13.2%) ears and residual in four (10.5%) ears. Cholesteatoma was acquired in 27 ears, with average age 10.9 years; and congenital in 11 ears, with average age 3.8 years. Surgical time was longer for acquired cases (226 vs. 154 minutes). Hearing outcomes were comparable for both cholesteatoma types. Residual disease was seen in three (11.1%) acquired ears and one (9.1%) congenital ear. Overall, the lowest rates of recurrent and residual disease were seen in EES 3 cases, and relatively low rates in EES 2 and 3 ears, including four (12.9%) recurrences and two (6.5%) ears with residual disease. CONCLUSION: The endoscopes are a viable tool for resection of pediatric cholesteatoma and provide excellent visualization of the middle ear and associated recesses. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:2653-2658, 2017.