Hearing Loss in Children with 22q11.2 Deletion Syndrome.

Objectives: Hearing loss is considered common in children with 22q11.2 deletion syndrome (22q11.2DS), with a few prior studies reporting a 32%-78% prevalence; mild-moderate conductive hearing loss has been reported most commonly.

Weight Gain After Adenotonsillectomy in Children With Mild Obstructive Sleep-Disordered Breathing: Exploratory Analysis of the PATS Randomized Clinical Trial.

Importance: It is unknown whether adenotonsillectomy causes undesirable weight gain in children with mild obstructive sleep-disordered breathing (oSDB).

Objective: To compare changes in anthropometric measures in children with mild oSDB treated with adenotonsillectomy vs watchful waiting.

Design, Setting, And Participants: This was an exploratory analysis of the Pediatric Adenotonsillectomy Trial for Snoring (PATS) randomized clinical trial of adenotonsillectomy vs watchful waiting for mild oSDB (snoring with obstructive apnea-hypopnea index of <3 events/hour) that took place at 7 pediatric tertiary care centers across the US and included 458 children aged 3.

Multi-institutional Assessment of Otitis Media Epidemiology Using Real-world Data.

Objectives: To determine rates and risk factors of pediatric otitis media (OM) using real-world electronic health record (PEDSnet) data from January 2009 through May 2021.

Study Design: Retrospective cohort study.

Setting: Seven pediatric academic health systems that participate in PEDSnet.

Recurrent missense variant identified in two unrelated families with MPZL2-related hearing loss, expanding the variant spectrum associated with DFNB111.

MPZL2-related hearing loss is a rare form of autosomal recessive hearing loss characterized by progressive, mild sloping to severe sensorineural hearing loss. Thirty-five previously reported patients had biallelic truncating variants in MPZL2, with the exception of one patient with a missense variant of uncertain significance and a truncating variant. Here, we describe the clinical characteristics and genotypes of five patients from four families with confirmed MPZL2-related hearing loss.

Clinical Characteristics of Primary Snoring vs Mild Obstructive Sleep Apnea in Children: Analysis of the Pediatric Adenotonsillectomy for Snoring (PATS) Randomized Clinical Trial.

Importance: It is unknown whether children with primary snoring and children with mild obstructive sleep apnea (OSA) represent populations with substantially different clinical characteristics. Nonetheless, an obstructive apnea-hypopnea index (AHI) of 1 or greater is often used to define OSA and plan for adenotonsillectomy (AT).

Objective: To assess whether a combination of clinical characteristics differentiates children with primary snoring from children with mild OSA.

Adenotonsillectomy for Snoring and Mild Sleep Apnea in Children: A Randomized Clinical Trial.

Importance: The utility of adenotonsillectomy in children who have habitual snoring without frequent obstructive breathing events (mild sleep-disordered breathing [SDB]) is unknown.

Objectives: To evaluate early adenotonsillectomy compared with watchful waiting and supportive care (watchful waiting) on neurodevelopmental, behavioral, health, and polysomnographic outcomes in children with mild SDB.

Design, Setting, And Participants: Randomized clinical trial enrolling 459 children aged 3 to 12.

Associations of actigraphy measures of sleep duration and continuity with executive function, vigilance, and fine motor control in children with snoring and mild sleep-disordered breathing.

Study Objectives: Children with snoring and mild sleep-disordered breathing may be at increased risk for neurocognitive deficits despite few obstructive events. We hypothesized that actigraphy-based sleep duration and continuity associate with neurobehavioral functioning and explored whether these associations vary by demographic and socioeconomic factors.

Methods: 298 children enrolled in the Pediatric Adenotonsillectomy Trial, ages 3 to 12.

Vinblastine/Methotrexate for Debilitating and Progressive Plexiform Neurofibroma in Children and Young Adults with Neurofibromatosis Type 1: A Phase 2 Study.

Limited therapies exist for neurofibromatosis type 1 (NF1)-associated plexiform neurofibroma (PN). For this reason, the activity of vinblastine (VBL) and methotrexate (MTX) was evaluated in children and young adults with NF1 and PN. Patients ≤ 25 years of age with progressive and/or inoperable NF1-PN received VBL 6 mg/m and MTX 30 mg/m weekly for 26 weeks, followed by every 2 weeks for 26 weeks.

A Trial of Intranasal Corticosteroids to Treat Childhood OSA Syndrome.

Background: Intranasal corticosteroids (INCS) are frequently used to treat OSA syndrome (OSAS) in children. However, their efficacy has not been rigorously tested.

Research Question: Do INCS result in improved OSAS symptoms, polysomnography findings, behavior, and quality of life compared with placebo?

Study Design And Methods: In this randomized, double-blind, placebo-controlled trial, children with OSAS aged 5 to 12 years (N = 134) were randomized 2:1 to receive 3 months of INCS or placebo.

Neurobehavioral morbidity of pediatric mild sleep-disordered breathing and obstructive sleep apnea.

Study Objectives: Obstructive sleep apnea is associated with neurobehavioral dysfunction, but the relationship between disease severity as measured by the apnea-hypopnea index and neurobehavioral morbidity is unclear. The objective of our study is to compare the neurobehavioral morbidity of mild sleep-disordered breathing versus obstructive sleep apnea.

Methods: Children 3-12 years old recruited for mild sleep-disordered breathing (snoring with obstructive apnea-hypopnea index < 3) into the Pediatric Adenotonsillectomy Trial for Snoring were compared to children 5-9 years old recruited for obstructive sleep apnea (obstructive apnea-hypopnea 2-30) into the Childhood Adenotonsillectomy Trial.

Endovascular Embolization for Control of Post-Tonsillectomy Hemorrhage.

Post-operative hemorrhage is a potentially life-threatening complication of tonsillectomy. While standard surgical maneuvers including the use of electrocautery, application of topical hemostatic agents, direct pressure, and suturing of the tonsillar pillars have traditionally been used for the treatment of severe bleeding, endovascular approaches are an important adjunct when other techniques are unsuccessful. Here, we describe the case of a 10-year-old female who presented with severe bleeding four days after tonsillectomy and adenoidectomy for chronic tonsillitis.

Magnetic resonance angiography (MRA) in preoperative planning for patients with 22q11.2 deletion syndrome undergoing craniofacial and otorhinolaryngologic procedures.

Introduction: Patients with 22q11.2 deletion syndrome (22q11.2DS) have a variety of anatomic anomalies.

Recorded Flexible Nasolaryngoscopy for Neonatal Vocal Cord Assessment in a Prospective Cohort.

Objective: Assessing vocal cord mobility by flexible nasolaryngoscopy (FNL) can be difficult in neonates. To date, prospective studies evaluating the incidence and diagnostic accuracy of vocal cord paralysis (VCP) after surgical patent ductus arteriosus (PDA) ligation are limited. It is unknown whether video FNL improves diagnosis in this population.

Algorithm-Based Pediatric Otolaryngology Management During the COVID-19 Global Pandemic: A Children's Hospital of Philadelphia Clinical Consensus.

Objective: The coronavirus disease 2019 (COVID-19) pandemic requires clinicians to explore alternatives to routine patient management. Otolaryngologists caring for children commonly depend on physical examination, laboratory data, and ambulatory surgical procedures. Limiting patient care, mindful allocation of resources, and concern for safety have challenged all aspects of our health care system.

Reliability and Accuracy of Remote Fiberoptic Nasopharyngolaryngoscopy in the Pediatric Population.

Introduction: Telemedicine is an increasingly prevalent component of medical practice. In otolaryngology, there is the potential for telemedicine services to be performed in conjunction with device use, such as with a nasolaryngoscope. This study evaluates the reliability of remote examinations of the upper airway through an iPhone recording using a coupling device attached to a nasopharyngolaryngoscope (NPL).

Defining Risk of Postoperative Obstructive Sleep Apnea in Patients With 22q11.2DS Undergoing Pharyngeal Flap Surgery for Velopharyngeal Dysfunction Using Polysomnographic Evaluation.

Objective: To determine pre- and postoperative prevalence of obstructive sleep apnea (OSA) in patients with 22q11.2 deletion syndrome (DS) undergoing wide posterior pharyngeal flap (PPF) surgery for velopharyngeal dysfunction (VPD).

Design: Retrospective study using pre- and postoperative polysomnography (PSG) to determine prevalence of OSA.

Lateral Neck Radiography in Preoperative Evaluation of Adenoid Hypertrophy.

Objective: To assess the value of lateral neck radiographs in quantifying adenoid hypertrophy to help guide treatment decisions in patients with symptoms of nasal obstruction.

Study Design: Retrospective review.

Methods: Quantitative radiologic grading of adenoids was correlated with the intraoperative grading to select cases in agreement between the two methods.

New treatments for obstructive sleep apnea in children.

Obstructive sleep apnea (OSA) has a prevalence of up to 5% in children and 50% in obese children. OSA is associated with various comorbidities in the general population and in the perioperative period. In this review, we will provide background of OSA and insights into the available treatment options both surgically and medically.

Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis.

Background: Recurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of papillomas in the airway and especially the larynx. The clinical course is highly variable among individuals and there is poor understanding of the factors that drive an aggressive vs an indolent course.

Methods: A convenience cohort of 339 affected subjects with papillomas positive for only HPV6 or HPV11 and clinical course data available for 1 year or more, from a large multicenter international study were included.

Depressive symptomatology in school-aged children with obstructive sleep apnea syndrome: incidence, demographic factors, and changes following a randomized controlled trial of adenotonsillectomy.

Study Objectives: Depressive symptoms following adenotonsillectomy (AT) relative to controls were examined in children with obstructive sleep apnea syndrome (OSAS).

Methods: The Childhood Adenotonsillectomy Trial (CHAT) multisite study examined the impact of AT in 453 children aged 5 to 9.9 years with polysomnographic evidence of OSAS without prolonged desaturation, randomized to early adenotonsillectomy (eAT) or watchful waiting with supportive care (WWSC).