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Hearing loss is one of the most common indications for temporal bone imaging in children. Hearing loss may be congenital or acquired, and it may be conductive, sensorineural, or mixed audiologically. Temporal bone imaging plays an important role in the assessment and management of this condition. An understanding of the embryology of ear structures better enables the radiologist to interpret abnormalities on imaging of the temporal bone. Here, we provide a general review of ear development and a description of known genetic defects that contribute to congenital ear anomalies associated with hearing loss. We provide appropriate imaging techniques for the temporal bone depending on the clinical presentation and a systematic approach to imaging for children with hearing loss. Diagnostic imaging for developmental anomalies of the ear and cholesteatoma will be discussed.

Introduction: Non-tuberculous mycobacterial (NTM) infection commonly manifests as subacute or chronic cervicofacial lymphadenitis in immunocompetent children. The optimal management of this pathology remains controversial. Objectives: This international consensus guideline aims to understand the practice patterns for NTM cervicofacial lymphadenitis and to address the primary diagnostic and management challenges. Methods: A modified three-iterative Delphi method was used to establish expert recommendations on the diagnostic considerations, expectant or medical management, and operative considerations. The recommendations herein are derived from current expert consensus and critical review of the literature. Setting: Multinational, multi-institutional, tertiary pediatric hospitals. Results: Consensus recommendations include diagnostic work-up, goals of treatment and management options including surgery, prolonged antibiotic therapy and observation. Conclusion: The recommendations formulated in this International Pediatric Otolaryngology Group (IPOG) consensus statement on the diagnosis and management of patients with NTM lymphadenitis are aimed at improving patient care and promoting future hypothesis generation. Keywords: Atypical mycobacteria; Cervical lymphadenitis; Consensus recommendations; Non-tuberculous mycobacteria.

To determine predictors of success following Veau 1 and 2 cleft palate repair in patients with and without syndromes.Retrospective review of prospectively collected data.Tertiary care children's hospital.All children18 months of age undergoing Furlow palatoplasty for Veau 1 and 2 cleft repair between 2000 and 2014 with postoperative perceptual speech assessment (PSA).In total, 368 consecutive patients were identified; 95 were excluded, resulting in 273 patients. Median age at surgery was 13.0 months (interquartile range [IQR], 11-15 months) with postoperative PSA at a median of 32.3 months (IQR, 26.3-44.5 months). Fifty patients (18.3%) had syndrome diagnosis; 59 patients (21.6%) had nonsyndromic Robin sequence. Velopharyngeal insufficiency (VPI) occurred in 27 patients (10.5%); 13 underwent secondary speech surgery. Cleft-related speech errors occurred in 46 patients (17.6%). Non-cleft-related speech errors occurred in 155 patients (59.6%) and reduced intelligibility in 127 patients (47.9%). Oronasal fistula occurred in 23 patients (8.8%) and was exclusive to Veau 2 clefts. In multivariate analysis, age13 months at palatoplasty demonstrated a 6-fold higher rate of VPI (hazard ratio [HR], 6.64;VPI occurred in 10% of patients undergoing Furlow palatoplasty repair of Veau 1 or 2 clefts. Age13 months at palatoplasty was associated with improved speech outcomes and lower VPI incidence (2.8% vs 16.2%). Syndromic diagnosis was associated with noncleft speech errors and reduced intelligibility on univariate analysis but not velopharyngeal function after palatoplasty.

OBJECTIVES: To evaluate the performance of 4-dimensional computed tomography (4D-CT) in assessing upper airway obstruction (UAO) in patients with Robin Sequence (RS) and compare the accuracy and reliability of 4D-CT and flexible fiberoptic laryngoscopy (FFL). STUDY DESIGN: Prospective survey of retrospective clinical data. SETTING: Single, tertiary care pediatric hospital. METHODS: At initial and 30-day timepoints, a multidisciplinary group of 11 clinicians who treat RS rated UAO severity in 36 sets of 4D-CT visualizations and FFL videos (dynamic modalities), and static CT images. Raters assessed UAO at the velopharynx and oropharynx (1-none to 5-complete) and noted confidence levels (LOC) of each rating. Intraclass correlation and Krippendorff’s Alpha were used to assess intra- and inter-rater reliability, respectively. Accuracy was assessed by comparing clinician ratings with Quantitative Percent Constriction (QPC) Ratings, calculated based on 4D-CT airway cross-sectional area. Results were compared using Wilcoxon rank-sum and signed-rank tests. RESULTS: 4D-CT and FFL demonstrated similar intra-rater agreement (moderate to substantial) and both demonstrated fair inter-rater agreement. Both modalities underestimated UAO severity, though 4D-CT ratings were significantly more accurate, as determined by QPC similarity, than FFL (−1.06 and −1.46 vs QPC Ratings, p=0.004). Overall LOC was similar for 4D-CT and FFL, but other specialists were significantly less confident in FFL ratings than were otolaryngologists (2.25 and 3.92, p

Purpose: The purpose of this study is to provide updated national estimates on the annual number, educational environments, and secondary school outcomes of students who are D/deaf and hard of hearing (D/HH) receiving special education (SpEd) and related services in the United States. Method: We performed a retrospective cross-sectional descriptive analysis of Individuals with Disabilities Education Act, Part B, Section 618 data from 2012 to 2018. Participants included students 6–21 years old in SpEd with “hearing impairment” reported as their primary disability. The general population of students in secondary school served as a comparator, via Current Population Survey data. We described the annual number of students (a) overall, (b) by educational environment, and (c) by reason for exiting SpEd, including the proportion graduating from and dropping out of secondary school. We described variation over time. Results: The median annual number of students was 67,655, with minimal variation by year. The proportion in general education (GenEd) for ≥ 80% of the day increased by 4.2% over 6 years from 57.8% to 62.0%, whereas the proportions in GenEd for < 40% and 40%–79% of the day decreased by 1.6% and 1.3%, respectively. Proportions in the remainder of the environments changed < 1.0% each. Of exiters, 86.8% of students graduated, whereas 3.9% dropped out, compared to a dropout rate of 5.0% in the general population. Conclusion: From 2012 to 2018, students who are D/HH receiving SpEd in the United States have spent increasingly more time in GenEd, most graduated from high school, and few dropped out, with dropout patterns appearing similar to the general population.

Objective: The Deaf community is an ethnolinguistic minority group. Low sensitivity to Deaf culture contributes to health disparities among Deaf patients. This study determines the level of sensitivity to Deaf culture among otolaryngology-head and neck surgery (OHNS) and audiology trainees. Methods: Cross-sectional survey study of OHNS and audiology trainees from 10 large US institutions. Trainees were queried on their exposure to and comfort with Deaf patients and their education on, attitude toward, and awareness and knowledge of Deaf culture. Sensitivity to Deaf culture was operationalized as awareness and knowledge of Deaf culture. These were assessed using a 35-item instrument that was previously developed using a d/Deaf community-based participatory approach to research. We used T-tests to compare the sample to previous samples of medical students with training in Deaf culture (MS-TDCs) and general practitioners (GPs). Results: There were 91 completed surveys (response rate 44.5%). Almost all were aware of Deaf culture (97.8%). The mean knowledge score was 55.0% (standard deviation (SD) 13.4%), which was significantly higher than that for GPs at 43.0% (SD 15.0%) (95% confidence interval 8.1%, 15.8%, P Conclusion: This sample of OHNS and audiology trainees was more sensitive to Deaf culture than GPs but less sensitive than MS-TDCs. Developing specialty-specific education may be warranted. Level of evidence: 4.

Purpose of review The management of patients with unilateral microtia and aural atresia is complex. Recent literature suggests significant strides in hearing habilitation and ear reconstruction. Recent findings Several options of hearing management are available and are associated with improved outcomes. Timelines for hearing habilitation and ear reconstruction vary by institution. We offer our timeline as a reference. Three dimensional (3D) printed models are increasingly used for training and reconstruction. Bioprinting is on the horizon, though safety and effectiveness studies are pending. Lastly, application of qualitative methods has provided a foundation on which to improve communication between physicians and patients and their families. Better understanding of the patient and family experiences will provide opportunities to target interventions to improve care. Summary Current developments include expanding options for hearing management, changing approaches to timing of atresiaplasty, utilization of 3D printed models, and focus on patient and family experience to improve reconstructive outcomes.

Objective To date, the recording of outcomes of interventions for velopharyngeal dysfunction (VPD) has not been standardized. This makes a comparison of results between studies challenging. The aim of this study was to develop a core outcome set (COS) for reporting outcomes in studies examining the management of VPD. Design A two-round Delphi consensus process was used to develop the COS. Patients, Participants The expert Delphi panel comprised patients and caregivers of patients with VPD, surgeons and speech and language therapists specializing in cleft palate, and researchers with expertise in VPD. Interventions A long list of outcomes was derived from the published literature. In each round of a Delphi survey, participants were asked to score outcomes using the Grading of Recommendations, Assessment, Development, and Evaluations scale of 1 to 9, with 1 to 3 labeled “not important,” 4 to 6 labeled “important but not critical,” and 7 to 9 labeled “critical.” Main outcome measure Consensus criteria were specified a priori. Outcomes with a rating of 75% or more of the panel rating 7 to 9 and 25% or fewer rating 1 to 3 were included in the COS. Results A total of 31 core outcomes were identified from the Delphi process. This list was condensed to combine topic areas to produce a final COS of 10 outcomes, including both processes of care and patient-reported outcomes that should be considered for reporting in future studies of VPD. Conclusions Implementation of the COS-VPD will facilitate consistency of outcomes data collection and comparison of results across studies.

Objectives/hypothesis A tracheal cartilaginous sleeve (TCS) is a rare anomaly characterized by anterior fusion of tracheal cartilages. TCS is associated with syndromic craniosynostoses including Apert, Crouzon and Pfeiffer syndromes and FGFR2, FGFR3, and TWIST1 variants. This study presents a 30-year review of patients with syndromic craniosynostosis and TCS and describes diagnostic methods, genetic variants, surgical interventions, and long-term outcomes. Study design Retrospective, single-institution review. Methods This review included patients with syndromic craniosynostosis and TCS treated at Seattle Children's Hospital from 1990 to 2020. Tracheostomy, genetic variants, and additional surgery were primary measures. Fisher's exact test compared need for tracheostomy in patients with proposed high-risk (FGFR2 p.W290 or FGFR2 p.C342) versus low-risk genetic variants. Results Thirty patients with TCS were identified. Average age at diagnosis was 12 months (range 2-weeks to 7.9-years; standard deviation 19.8 months). Syndromes included Pfeiffer (37%), Apert (37%), and Crouzon (26%). Severe obstructive sleep apnea was present in 76% of patients. Tracheostomy was performed in 17 patients (57%); five were successfully decannulated. Additional interventions included adenotonsillectomy (57%), nasal (20%), laryngeal (17%), and craniofacial skeletal surgery (87%). All patients with Pfeiffer syndrome and FGFR2 p.W290C variants and 83% of patients with FGFR2 p.C342 variants required tracheostomy, differing from other variants (P = .02, odds ratio 33, 95% confidence interval 1.56-697.96). One patient (3%) died. Conclusion TCS contributes to multilevel airway obstruction in patients with syndromic craniosynostosis. Genetic testing in patients with FGFR2-related syndromic craniosynostoses may identify those at risk of TCS and facilitate early intervention. A better understanding of this patient population may foster individualized airway management strategies and improve outcomes. Level of evidence 4 Laryngoscope, 2021.

Objectives/hypothesis Language barriers may impact family experience, which is a key measure of healthcare quality. We compared family satisfaction between Spanish-speaking families (SSF) and English-speaking families (ESF) in pediatric otolaryngology. Study design Retrospective cohort study. Methods Responses from the Family Experience Survey (FES), a hospital quality benchmarking survey, were analyzed from 2017 to 2019 at one academic pediatric otolaryngology practice. Question responses were compared between SSF versus ESF using mixed effect logistic regression models, adjusting for patient age, medical complexity, and insurance. Results A total of 4,964 FES survey responses were included (14% SSF). In multivariate analysis adjusting for age, medical complexity, and insurance, SSF were 1.7 times more likely than ESF to rate their provider with the highest rating (i.e. 9-10/10; 95% confidence interval [CI] 1.24-2.22). However, SSF were less likely than ESF to provide the highest rating on many individual aspects of care, including whether providers explained things intelligibly (odds ratio [OR] 0.43, 95% CI 0.25-0.74), listened carefully (OR 0.36, 95% CI 0.28-0.47), knew their medical child's history (OR 0.53, 95% CI 0.44-0.64), provided understandable information (OR 0.36, 95% CI 0.16-0.83), spent sufficient time with them (OR 0.38, 95% CI 0.31-0.48), allowed them to discuss their questions (OR 0.57, 95% CI 0.47-0.70), or had enough input in their children's' care (OR 0.46, 95% CI 0.26-0.80). Conclusions In a large cohort of pediatric otolaryngology patients, SSF rated many individual aspects of their child's care less positively compared to ESF, despite rating their provider highly. Further research is needed to explore the reasons for these differences and how they can be improved. Level of evidence 4 Laryngoscope, 131:E2393-E2401, 2021.

OBJECTIVES/HYPOTHESIS To investigate perceptual speech outcomes following sphincter pharyngoplasty (SP) and to identify patient characteristics associated with velopharyngeal insufficiency (VPI) resolution or improvement. METHODS Retrospective review of prospectively collected data was performed of consecutive patients that underwent SP for management of VPI between 1994 and 2016 at a single tertiary care pediatric hospital. Demographic data, nasendoscopic findings, and speech characteristics were recorded using a standardized protocol. Pre- and post-operative VPI was graded on a five-point Likert scale. Frequency of post-operative VPI resolution and improvement was assessed and associations with patient characteristics were analyzed. The association between odds of VPI resolution or improvement and five patient characteristics identified a priori was performed controlling for confounding factors. RESULTS Two-hundred ninety-six subjects were included. All patients had at least minimal VPI pre-operatively; 72% were graded moderate or severe. Sixty-four percent experienced resolution and 83% improved at least one point on the VPI-severity scale. Of the five patient characteristics, only history of cleft palate repair was significantly associated with decreased odds of VPI improvement but not resolution when controlling for other variables. CONCLUSIONS Sphincter pharyngoplasty resulted in resolution of VPI in 64% and improvement in 83% of subjects. Children with a history of cleft palate had significantly decreased odds of VPI improvement compared to those without a history of cleft palate. Neither syndrome diagnosis nor 22q11 deletion had a significant association with speech outcomes after sphincter pharyngoplasty. LEVEL OF EVIDENCE 4 Laryngoscope, 131:E2046-E2052, 2021.

Objective: The study aim was to assess behavioral adjustment in preschool children with and without craniofacial microsomia (CFM). Design: Multisite cohort study of preschoolers with CFM (“cases”) or without CFM (“controls”). Participants: Mothers (89%), fathers (9%), and other caregivers (2%) of 161 preschoolers. Outcome Measure: Child Behavior Check List (CBCL 1.5-5); linear regressions with standardized effect sizes (ES) adjusted for sociodemographic confounds. Results: Child Behavior Check Lists for 89 cases and 72 controls (average age 38.3 ± 1.9 months). Children were male (54%), white (69%), and of Latino ethnicity (47%). Cases had microtia with mandibular hypoplasia (52%), microtia only (30%), or other CFM-associated features (18%). Nearly 20% of cases had extracranial anomalies. Composite CBCL scores were in the average range compared to test norms and similar for cases and controls. On the subscales, cases’ parents reported higher Anxious/Depressed scores (ES = 0.35, P = .04), Stress Problems (ES = 0.40, P = .04), Anxiety Problems (ES = 0.34, P = .04), and Autism Spectrum Problems (ES = 0.41, P = .02); however, the autism subscale primarily reflected speech concerns. Among cases, more problems were reported for children with extracranial anomalies and certain phenotypic categories with small ES. Conclusions: Behavioral adjustment of preschoolers with CFM was comparable to peers. However, parental reports reflected greater concern for internalizing behaviors; thus, anxiety screening and interventions may benefit children with CFM. Among cases, more problems were reported for those with more complex presentations of CFM. Craniofacial microsomia–related speech problems should be distinguished from associated psychosocial symptoms during developmental evaluations.

COVID-19 is a rapidly growing global pandemic caused by a novel coronavirus. With no vaccine or definitive treatment, public health authorities have recommended a strategy of "social distancing," reducing individual interaction, canceling elective procedures, and limiting nonessential services. Health care providers must determine what procedures are considered "elective," balancing risk of treatment delays with that of coronavirus exposure to patient, family, and providers. Given critical periods for language development and the long-term impact of auditory deprivation, some audiologic and otologic services should be considered essential. In this article, we describe the experience of a quaternary referral pediatric hospital in Seattle, the epicenter of COVID-19 in the United States, and share strategies for risk minimization employed by Seattle Children's Hospital. We hope that this work can be a reference for other centers continuing care for children who are deaf and hard of hearing during the COVID-19 and future resource-limiting crises.

Coronavirus disease 2019 (COVID-19) is a novel coronavirus resulting in high mortality in the adult population but low mortality in the pediatric population. The role children and adolescents play in COVID-19 transmission is unclear, and it is possible that healthy pediatric patients serve as a reservoir for the virus. This article serves as a summary of a single pediatric institution's response to COVID-19 with the goal of protecting both patients and health care providers while providing ongoing care to critically ill patients who require urgent interventions. A significant limitation of this commentary is that it reflects a single institution's joint effort at a moment in time but does not take into consideration future circumstances that could change practice patterns. We still hope dissemination of our overall response at this moment, approximately 8 weeks after our region's first adult case, may benefit other pediatric institutions preparing for COVID-19.

Objective To develop an expert-based consensus of recommendations for the diagnosis and management of pediatric obstructive sleep apnea. Methods A two-iterative Delphi method questionnaire was used to formulate expert recommendations by the members of the International Pediatric Otolaryngology Group (IPOG). Results Twenty-six members completed the survey. Consensus recommendations (>90% agreement) are formulated for 15 different items related to the clinical evaluation, diagnosis, treatment, postoperative management and follow-up of children with OSA. Conclusion The recommendations formulated in this IPOG consensus statement may be used along with existing clinical practice guidelines to improve the quality of care and to reduce variation in care for children with OSA.

Reconstruction with autologous costochondral cartilage is one of the mainstays of surgical management of congenital microtia. We review the literature, present our current technique for microtia reconstruction with autologous costochondral graft, and discuss the evolution of our technique over the past 20 years. We aim to minimize donor site morbidity and create the most durable and natural appearing ear possible using a stacked framework to augment the antihelical fold and antitragal-tragal complex. Assessment of outcomes is challenging due to the paucity of available objective measures with which to evaluate aesthetic outcomes. Various instruments are used to assess outcomes, but none is universally accepted as the standard. The challenges we continue to face are humbling, but ongoing work on tissue engineering, application of 3D models, and use of validated questionnaires can help us get closer to achieving a maximal aesthetic outcome.

Assess interrater agreement of endoscopic assessment of velopharyngeal (VP) function before and after viewing the video instruction tool (VIT). We hypothesized improvement in interrater agreement using the Golding-Kushner scale (GKS) after viewing the VIT.Prospective study.Multi-institutional.Sixteen fellowship-trained pediatric otolaryngologists who treat velopharyngeal insufficiency (VPI) rated 50 video segments using the GKS before and after watching the VIT. Raters assessed gap size percentage and lateral pharyngeal wall (LPW), soft palate (SP), and posterior pharyngeal wall (PPW) movement. Intraclass correlation coefficient was estimated for these continuous measures. Raters also indicated the presence of a palatal notch, Passavant's ridge, and aberrant pulsations (categorical variables). Fleiss κ coefficient was used for categorical variables. Wilcoxon signed-rank test was performed on the difference between the pre/post individual video ratings.Reliability improved for all continuous variables after watching the instructional video. The improvement was significant for PPW (0.22-0.30, P.001), SP (left: 0.63-0.68, P.001 and right: 0.64-0.68, P = .001), and LPW (left: 0.49-0.54, P = .01 and right: 0.49-0.54, P = .09) but not significant for gap size (0.65-0.69, P = .36). Among categorical variables, agreement on Passavant's ridge significantly improved (0.30-0.36, P = .03).Exposure to a video instruction tool improves interrater agreement of endoscopic assessment of VP function. Significant improvement was observed in our primary end points, specifically posterior pharyngeal wall movement, soft palate movement, and lateral pharyngeal wall movement. There was less impact of the VIT on the interrater agreement of the categorical variables, palatal notch, Passavant's ridge, and aberrant pulsations.

Objective:The Craniofacial microsomia: Longitudinal Outcomes in Children pre-Kindergarten (CLOCK) study is a longitudinal cohort study of neurobehavioral outcomes in infants and toddlers with craniofacial microsomia (CFM). In this article, we review the data collection and methods used to characterize this complex condition and describe the demographic and clinical characteristics of the cohort.Setting:Craniofacial and otolaryngology clinics at 5 study sites.Participants:Infants with CFM and unaffected infants (controls) ages 12 to 24 months were recruited from the same geographical regions and followed to age 36 to 48 months.Methods:Phenotypic, neurodevelopmental, and facial expression assessments were completed during the first and third waves of data collection (time 1 and time 3, respectively). Medical history data were taken at both of these time points and during an intermediate parent phone interview (time 2).Results:Our cohort includes 108 cases and 84 controls. Most cases and controls identified as white and 55% of cases and 37% of controls identified as Hispanic. Nearly all cases had microtia (95%) and 59% had mandibular hypoplasia. Cases received extensive clinical care in infancy, with 59% receiving care in a craniofacial clinic and 28% experiencing at least one surgery. Study visits were completed at a study site (92%) or at the participant’s home (8%).Conclusions:The CLOCK study represents an effort to overcome the challenges of characterizing the phenotypic and neurodevelopmental outcomes of CFM in a large, demographically and geographically diverse cohort.

Hearing loss associated with craniofacial microsomia (CFM) requires a team approach for evaluation, diagnosis, aural rehabilitation, and surgical intervention. Age-appropriate hearing assessment is essential to appropriately diagnose associated hearing loss and provide early intervention services. Therapy is based on the anomalies associated with the craniofacial microsomia and can vary from ear-level hearing aids to bone conduction sound processors (BCSP), using either band or osseointegrated implant-retention systems to atresia repair. For many patients with favorable anatomy, aural atresia repair allows for improved conductive hearing and quality of life. In summary, unilateral hearing loss (UHL) should be treated early and appropriately to aid in optimal speech and language development, as well as age-appropriate school performance.

Objective The U.S. Food and Drug Administration guidelines for cochlear implantation (CI) include age greater than 12 months. Studies have suggested that implantation in children younger than 12 months with congenital deafness may be associated with better spoken language outcomes. Compare auditory comprehension (AC) outcomes for children with congenital deafness who received CI less than 12 months of age to those implanted at 12 to 24 months of age. Methods Retrospective review of prospectively collected data in consecutively implanted patients under 2 years of age who received CI and had post-CI Preschool Language Scale (PLS)-AC scores. Receptive language was assessed with the AC subtest of the PLS. Patients without pre-CI PLS-AC scores were excluded. The association between age at implantation and post-CI PLS-AC scores up to 2 years after CI surgery was modeled using a linear mixed-effects model. Time from CI surgery, number of implants, risk factors for language delay, pre-CI PLS-AC score, and sex were included in the model. Patients implanted less than 12 months of age were compared to those implanted between 12 and 24 months. Results Twenty-nine patients who had CI surgery by 12 months and 82 who had CI surgery between 12 and 24 months were included in the analysis. Younger age at implantation and better pre-CI PLS-AC scores were significantly associated with better post-CI PLS-AC scores. Conclusion Cochlear implantation in children with congenital deafness less than 12 months of age was associated with better PLS-AC than in children implanted over 12 months of age up to 2 years after implantation. Level of evidence 4 Laryngoscope, 130:776-781, 2020.

The Facial Asymmetry Collaborative for Interdisciplinary Assessment and Learning (FACIAL) network applies key principles of established team science while using equity-based approaches that advance career development and accelerating collaborative research in craniofacial microsomia. Team science is an effort to leverage a challenge in science by using professionals of different backgrounds and expertise.

Objectives/Hypothesis In this meta-analysis, we reviewed observational studies investigating differences in intelligence quotient (IQ) scores of children with unilateral hearing loss compared to children with normal hearing. Data Sources PubMed Medline, Cumulative Index to Nursing and Allied Health Literature, Embase, PsycINFO. Methods A query identified all English-language studies related to pediatric unilateral hearing loss published between January 1980 and December 2014. Titles, abstracts, and articles were reviewed to identify observational studies reporting IQ scores. Results There were 261 unique titles, with 29 articles undergoing full review. Four articles were identified, which included 173 children with unilateral hearing loss and 202 children with normal hearing. Ages ranged from 6 to 18 years. Three studies were conducted in the United States and one in Mexico. All were of high quality. All studies reported full-scale IQ results; three reported verbal IQ results; and two reported performance IQ results. Children with unilateral hearing loss scored 6.3 points lower on full-scale IQ, 95% confidence interval (CI) [−9.1, −3.5], P value < 0.001; and 3.8 points lower on performance IQ, 95% CI [−7.3, −0.2], P value 0.04. When investigating verbal IQ, we detected substantial heterogeneity among studies; exclusion of the outlying study resulted in significant difference in verbal IQ of 4 points, 95% CI [−7.5, −0.4], P value 0.028. Conclusions This meta-analysis suggests children with unilateral hearing loss have lower full-scale and performance IQ scores than children with normal hearing. There also may be disparity in verbal IQ scores. Laryngoscope, 126:746–754, 2016

(1) To define the minimal clinically important difference (MCID) of the Velopharyngeal Insufficiency (VPI) Effects on Life Outcomes (VELO) instrument, and (2) to test for the change in quality of life (QOL) after VPI surgery.Prospective observational cohort.VPI clinic at a tertiary pediatric medical center.Children with VPI and their parents completed the VELO instrument (higher score is better QOL) at enrollment and then underwent VPI surgery (Furlow palatoplasty or sphincter pharyngoplasty, n = 32), other treatments (obturator or oronasal fistula repair, n = 7), or no treatment (n = 18). They completed the VELO instrument again and an instrument of global rating of change in QOL at 1 year. The MCID was anchored to the global change instrument scores corresponding to "a little" or "somewhat" better. Within-group (paired t test) and between-group (Student t test) changes in VELO scores were tested for the VPI surgery and no treatment groups. The association between treatment group and change in VELO scores was tested with multivariate linear regression, adjusting for confounders.Follow-up was obtained for 37 of 57 (65%) patients. The mean (±standard deviation) change in VELO scores corresponding to the MCID anchor was 15 ± 13. The VELO score improved significantly more in the VPI surgery group (change, 22 ± 15; P.001) than in the no treatment group (change, 9 ± 12; P = .04), after adjusting for confounders (P = .007 between groups).VPI surgery using the Furlow palatoplasty or sphincter pharyngoplasty improves VPI-specific QOL, and the improvement is clinically important.

Objectives/Hypothesis To examine the relationship between bony cochlear nerve canal (BCNC) width, degree of hearing loss, and speech discrimination in children with unilateral sensorineural hearing loss (USNHL). Study Design Retrospective chart review (case-control study). Methods Audiometric database was cross-referenced with radiologic database at pediatric tertiary care facility to identify children with USNHL and temporal bone computed tomography. BCNC widths were measured independently by two radiologists blinded to affected ear. Regression analyses investigated associations among variables. Results One hundred and sixty children with USNHL had temporal bone imaging. Mean BCNC width was significantly smaller in affected ears, P = 0.0001. Narrower width was associated with more severe hearing loss, P = 0.01. Among children who had narrower cochlear nerve canals in affected ears compared to unaffected ears, smaller width was associated with lower speech discrimination score, P = 0.03. Increasing asymmetry in BCNC width between affected and unaffected ears was associated with poorer discrimination scores, P = 0.02. Among ears with asymmetrically smaller cochlear nerve canals, a 1-mm reduction in cochlear canal width between the normal and affected ear was associated with 30.4% lower word recognition score percentage in the affected ear, P =

The clinical presentation of microtia varies widely from minimal morphological abnormalities to complete absence of the ear. In this study we sought to identify and characterize sub-groups of microtia using a statistical and a clinical approach. Photographs of 86 ears were classified in relation to all the external ear components. We used cluster analysis and rater's clinical opinion to identify groups with similar phenotypes in two separate analyses. We used Cramer's Phi coefficient of association to assess the similarity among the clinician's groupings as well as among the statistical sub-phenotypic groups and each of the clinician's groupings. The cluster analysis initially divided the 86 ears into a more and a less severe group. The less severe group included two sub-groups that included ears classified as normal and a group that had very few anomalous components. The group of 48 more affected ears all had abnormalities of the helix crus; antihelix-stem, -superior crus and -inferior crus; and antitragus. These were further divided into 4 sub-phenotypes. There was a moderate degree of association among the raters' groupings (Cramer's Phi: 0.64 to 0.73). The statistical and clinical groupings had a lower degree of association (Cramer's Phi: 0.49 to 0.58). Using standardized characterization of structural abnormalities of the ear we identified six distinct phenotypic groups; correlations with clinicians' groupings were moderate. These clusters may represent groups of ear malformations associated with the same etiology, similar time of insult or target cell population during embryonic development. The results will help inform investigations on etiology. © 2015 Wiley Periodicals, Inc.

Craniofacial microsomia (CFM) is a complex condition associated with microtia, mandibular hypoplasia, and preauricular tags. It is the second most common congenital facial condition treated in many craniofacial centers and requires longitudinal multidisciplinary patient care. The purpose of this article is to summarize current recommendations for clinical management and discuss opportunities to advance clinical research in CFM.

Management of children with unilateral hearing loss is not standardized. The primary goal of this study was to elicit patient- and parent-reported perspectives regarding usage of hearing devices in pediatric UHL and to suggest a basic algorithmic approach to management.Our tertiary care center recruited families of youth ages 5-19 years with unilateral hearing loss from January 2014 through October 2015. Parents of all youths completed a 36-item survey, and some youth ages 11-19 years participated in hour-long interviews. We assessed patterns of hearing device usage among participants, and performed qualitative data analysis to understand factors considered by youths when deciding whether or not to use a hearing device.Survey information was collected for 50 patients. Distribution of hearing loss severity in affected ear was mild 14%, moderate 26%, severe 22%, and profound 38%. The majority of children had sensorineural hearing loss (57%), followed by mixed (32%), and then conductive (11%). 34 children (68%) had tried a hearing device; 20 continued to use the device. Retention rates were similar among children with different degrees of hearing loss: mild 66%, moderate 50%, severe 60%, profound 64%. Sixteen children tried a wireless contralateral routing of signal (CROS) device, and 15 tried a behind-the-ear (BTE) hearing aid. Retention rates for CROS and BTE devices were 69% and 47%, respectively. The most common reason for cessation of use was discomfort, followed by lack of benefit.A majority of children with unilateral hearing loss who tried a hearing device continued to use it, and retention rates were similar across all degrees of hearing loss. These findings suggest that personal hearing devices should be included in management protocols.

Objective To review the presentation, evaluation, and treatment of children with vallecular cysts and introduce a new technique of transoral excision for this entity. Methods Retrospective case series of children diagnosed with vallecular cyst between 2001 and 2008 at a single tertiary care children's hospital. Data collected, including age at diagnosis, presenting symptoms, additional diagnoses, diagnostic modality, prior and subsequent surgical therapy, length of hospital stay, length of follow-up, and recurrence were analyzed with descriptive statistics. Results Seven children (mean age 198 days, range 2 days to 2.9 years) were included in this series. Five children presented with respiratory distress and/or swallowing difficulties. Vallecular cyst was diagnosed by initial flexible fiberoptic laryngoscopy (5/7), MRI (1/7), and intubating laryngoscopy (1/7). All children underwent complete cyst excision via transoral surgical approach. Two children underwent additional supraglottoplasty for concomitant laryngomalacia, one of whom underwent tracheotomy for persistent respiratory distress and vocal cord immobility. The average length of hospital stay postoperatively was 9.5 days, and four patients stayed less than 2 days. No patients experienced recurrence of the vallecular cyst at last follow-up (range 4–755 days, mean 233 days). Conclusions Vallecular cysts are rare but should be considered in children with respiratory distress and dysphagia. Awake, flexible fiberoptic laryngoscopy with particular attention to the vallecular region should be performed on any child presenting with these symptoms. Direct, transoral approach for excision of the vallecular cyst is our preferred method of treatment with no recurrences to date.

Velopharyngeal closure is essential for normal speech production. Velopharyngeal insufficiency is characterized by nasal air emissions and hypernasal resonance. The most common surgical procedures used to treat velopharyngeal insufficiency include posterior pharyngeal flap, palatoplasty, and sphincter pharyngoplasty. The sphincter pharyngoplasty is used when fiberoptic nasal endoscopy reveals velopharyngeal inadequacy associated with adequate velar movement but poor lateral wall motion. It is suited for both coronal and circular closure patterns. In our experience, it may be considered for all patients with velopharyngeal inadequacy and transverse orientation of the levator veli palatini. 1 This article describes the senior author's method for performing a sphincter pharyngoplasty.

Objective To (1) update the technique of endoscopic electrocauterization of patients with pyriform fossa sinus tracts and (2) evaluate its effectiveness as a definitive treatment. Methods Retrospective case series with nine patients (age range, 3.3–16.1 years) who were diagnosed with pyriform fossa sinus tracts between 2000 and 2007 at a single tertiary care children's hospital and underwent endoscopic electrocauterization of the sinus tract. Data collected including age of diagnosis, presenting symptoms, time from presentation to diagnosis, diagnostic studies, prior and subsequent treatments, length of hospital stay, and recurrence, were reviewed and analyzed with descriptive statistics. Results All nine patients presented with recurrent left or midline neck masses or abscesses. Seven patients (78%) had at least one previous incision and drainage procedures for abscess treatment. All patients had a computed tomography scan with findings suspicious for left-sided pyriform fossa sinus tract. The diagnosis was confirmed with laryngoscopy. For seven patients (78%), endoscopic electrocauterization was definitive treatment with no recurrences to date. Two patients (22%) had recurrent left neck abscesses after endoscopic treatment; these patients ultimately underwent excision of sinus tract with left thyroid lobectomy without complications or further recurrences. Conclusions Endoscopic electrocauterization of pyriform fossa sinus tracts is a safe and definitive treatment for most patients. We advocate this minimally invasive procedure as first line of treatment for pyriform fossa sinus tracts, reserving open excision with or without thyroid lobectomy for failures.

To discuss principles of evaluation and treatment of children with velopharyngeal insufficiency, and to review recent reports of surgical outcomes.Velopharyngeal insufficiency significantly impacts both quality-of-life and speech intelligibility in children. Diagnosis is made through history and physical, perceptual speech assessment, and instrumental measures including nasendoscopy and radiographic multiplanar videofluoroscopy. Treatment options for velopharyngeal insufficiency consist of prosthetic management or surgery, supplemented with speech therapy when appropriate. Surgical interventions are palatal, palatopharyngeal or pharyngeal in nature. Despite some controversy, most recent reports identify no significant difference in outcomes following pharyngeal flap or sphincter pharyngoplasty. Complications of surgical therapy relate to postoperative obstructive breathing or persistent velopharyngeal insufficiency.Although there is no universally accepted measure to assess velopharyngeal insufficiency severity, nasendoscopy and multiplanar videofluoroscopy are most commonly used for clinical diagnosis. A speech pathologist is an integral member of the velopharyngeal insufficiency team, and momentum toward a standardized reporting system of perceptual speech measurements is increasing. Treatment of velopharyngeal insufficiency should be tailored to the specific needs of the child and family. Surgical therapy may improve velopharyngeal function but may negatively impact upper airway patency and respiration during sleep. The otolaryngologist should be familiar with strengths and limitations of different surgical options for velopharyngeal insufficiency.

Because a tracheal cartilaginous sleeve (TCS) confers a significant mortality risk that can be mitigated with appropriate intervention, we sought to describe the prevalence and associated genotypes in a large cohort of children with syndromic craniosynostosis. Chart review of patients with syndromic craniosynostosis across two institutions. In a cohort of 86 patients with syndromic craniosynostosis, 31 required airway evaluation under anesthesia. TCS was found in 19, for an overall prevalence of 22%. FGFR2, TWIST1, and FGFR3 mutations were identified in children with TCS. All five children with a W290C mutation in FGFR2 had TCS, and most previously reported children with W290C had identification of TCS or early death. In contrast, TCS was not associated with other mutations at residue 290. There is an association between TCS and syndromic craniosynostosis, and it appears to be particularly high in individuals with the W290C mutation in FGFR2. Referral to a pediatric otolaryngologist and consideration of operative airway evaluation (i.e., bronchoscopy or rigid endoscopy) in all patients with syndromic craniosynostosis should be considered to evaluate for TCS. Results from genetic testing may help providers weigh the risks and benefits of early airway evaluation and intervention in children with higher-risk genotypes. Genet Med 19 1, 62–68.

Deletions affecting the terminal end of chromosome 3p result in a characteristic set of clinical features termed 3p-- syndrome. Bilateral, sensorineural hearing loss (SNHL) has been found in some but not all cases, suggesting the possibility that it is due to loss of a critical gene in band 3p25. To date, no genetic locus in this region has been shown to cause human hearing loss. However, the ATP2B2 gene is located in 3p25.3, and haploinsufficiency of the mouse homolog results in SNHL with similar severity. We compared auditory test results with fine deletion mapping in seven previously unreported 3p-- syndrome patients and identified a 1.38Mb region in 3p25.3 in which deletions were associated with moderate to severe, bilateral SNHL. This novel hearing loss locus contains 18 genes, including ATP2B2. ATP2B2 encodes the plasma membrane calcium pump PMCA2. We used immunohistochemistry in human cochlear sections to show that PMCA2 is located in the stereocilia of hair cells, suggesting its function in the auditory system is conserved between humans and mice. Although other genes in this region remain candidates, we conclude that haploinsufficiency of ATP2B2 is the most likely cause of SNHL in 3p-- syndrome.

In diastrophic dysplasia, auricular swelling commonly occurs in early infancy, inevitably leading to deformity. Till date, no description exists in the literature for the initial treatment of auricular swelling in this population. We present two siblings with diastrophic dysplasia on whom auricular swelling was treated with incision and drainage or conforming auricular molds. The ear treated with incision and drainage had worse outcome than those treated with pressure alone. This paper presents a novel but simple approach to the compression of auricular swelling in the setting of diastrophic dysplasia, using conforming molds with the goal of preventing permanent deformity.

This study describes the presentation of first branchial cleft anomalies and compares outcomes of first branchial cleft with other branchial cleft anomalies with attention to otologic findings.Case series with chart review.Pediatric tertiary care facility.Surgical databases were queried to identify children with branchial cleft anomalies. Descriptive analysis defined sample characteristics. Risk estimates were calculated using Fisher's exact test.Queries identified 126 subjects: 27 (21.4%) had first branchial cleft anomalies, 80 (63.4%) had second, and 19 (15.1%) had third or fourth. Children with first anomalies often presented with otologic complications, including otorrhea (22.2%), otitis media (25.9%), and cholesteatoma (14.8%). Of 80 children with second branchial cleft anomalies, only 3 (3.8%) had otitis. Compared with children with second anomalies, children with first anomalies had a greater risk of requiring primary incision and drainage: 16 (59.3%) vs 2 (2.5%) (relative risk [RR], 3.5; 95% confidence interval [CI], 2.4-5; P.0001). They were more likely to have persistent disease after primary excision: 7 (25.9%) vs 2 (2.5%) (RR, 3; 95% CI, 1.9-5; P=.0025). They were more likely to undergo additional surgery: 8 (29.6%) vs 3 (11.1%) (RR, 2.9; 95% CI, 1.8-4.7; P=.0025). Of 7 persistent first anomalies, 6 (85.7%) were medial to the facial nerve, and 4 (57.1%) required ear-specific surgery for management.Children with first branchial cleft anomalies often present with otologic complaints. They are at increased risk of persistent disease, particularly if anomalies lie medial to the facial nerve. They may require ear-specific surgery such as tympanoplasty.

A retrospective study was undertaken to assess speech outcomes in patients undergoing Furlow palatoplasty. Since 1994, the authors have used the position of the levator veli palatini musculature to determine type of surgical intervention recommended for the management of velopharyngeal insufficiency. Furlow palatoplasty has been used in patients with clinical evidence of sagittally oriented levator veli palatini musculature. Forty-eight patients who underwent a Furlow palatoplasty between June of 1994 and August of 1998 were included. All patients underwent preoperative and postoperative perceptual speech analyses to describe velopharyngeal insufficiency severity, nasal air emissions, and resonance, and preoperative nasendoscopy to assess velopharyngeal gap size and palatal and lateral pharyngeal wall movement. Other patient characteristics considered included gender, age at time of surgery, previously repaired cleft palate, submucous cleft palate, and syndrome diagnosis. Speech outcomes were determined on the basis of postoperative perceptual speech analyses and were categorized in one of three ways: (1) complete resolution of velopharyngeal insufficiency, (2) substantial improvement of velopharyngeal insufficiency, and (3) audible residual velopharyngeal insufficiency. Complete resolution of velopharyngeal insufficiency was defined as normal resonance and an absence of nasal air emissions. Substantial improvement of velopharyngeal insufficiency was defined as an improvement of at least two categories in velopharyngeal insufficiency severity in those patients without complete resolution. Audible residual velopharyngeal insufficiency refers to patients with postoperative velopharyngeal insufficiency severity ratings of mild, moderate, or severe. The male:female ratio in the study was 27:21. Twelve patients were syndromic; three had velocardiofacial syndrome. The median age at surgery was 6.5 years (range, 2 to 22 years). The average duration of follow-up was 14.7 months (range, 1.3 to 58.6 months). Postoperatively, the severity of velopharyngeal insufficiency was rated as none in 19 of the 48 patients (39.6 percent), minimal in eight (16.7 percent), mild in six (12.5 percent), moderate in nine (18.75 percent), and severe in six (12.5 percent). Substantial improvement was seen in seven of the 29 patients without complete resolution. There was a significant association between male gender and complete resolution of velopharyngeal insufficiency (p < 0.05). Presence of syndrome and female gender was associated with audible residual velopharyngeal insufficiency (p < 0.05). The main complication was palatal fistula (two cases). In conclusion, most patients who underwent a Furlow palatoplasty had a complete resolution or substantial improvement of velopharyngeal insufficiency postoperatively, and there were few surgical complications.

Once a prevalent disease, acute epiglottitis in children has become a rare entity. The introduction of the Haemophilus influenzae type b vaccine has had a dramatic impact on the number of invasive infections caused by this organism. However, physicians must be aware that epiglottitis may result from vaccine failures or from infection with other pathogenic organisms. Vaccinated children with epiglottitis present in a similar fashion to those who are not vaccinated. We present a rare case of acute epiglottitis in a fully vaccinated child due to nontypeable H. influenzae and discuss the clinical presentation and management.

Hemangiomas and vascular malformations may involve any part of the airway, from the nares to the tracheobronchial tree. As mass lesions impinging on the airway, either lesion can cause nonspecific signs and symptoms of airway obstruction. Specifically, the patients may have stridor, retractions, feeding difficulties, sleep disturbance, and failure to thrive. The distinction between hemangioma and vascular or lymphatic malformations usually can be made based upon history and physical examination. This article is an overview of the common congenital vascular lesions that may affect the airway. The clinical presentation, laboratory evaluation, management options, and expected outcomes are discussed. HEMANGIOMAS Hemangiomas are the most common tumors of infancy, affecting 1 % to 10% of children. The typical presentation of a cutaneous hemangioma is an erythematous macular lesion at birth, which undergoes a rapid proliferative phase in the first 1 to 2 months of life. The cutaneous lesions become cherry-colored and raised, whereas subdermal lesions may increase in size without changes of the overlying skin. About one third of these lesions are observed first in the newborn period; up to 90% become

Ototoxicity associated with cis-platinum administration commonly presents as hearing loss and tinnitus. The hearing loss is usually an irreversible, high-frequency sensorineural loss. Histologic studies in humans and animals suggest that the outer hair cells (OHCs) are most susceptible to cis-platinum. Evoked otoacoustic emissions (EOAE), as a measure of outer hair cell function, are potentially useful in following ototoxic insults involving OHCs. Distortion-product otoacoustic emissions (DPOAE) test frequency-specific regions of the cochlea and therefore may be particularly well suited for monitoring ototoxic injuries. We measured distortion product otoacoustic emissions, at f2 = 2, 4, 6, 8, 10, and 12 kHz, in gerbils after a single large dose of cis-platinum. Animals treated with saline served as controls. The findings were compared to auditory brain stem evoked response (ABR) thresholds, using tone pips of the same frequencies. The DPOAE and ABR thresholds were measured before treatment and again 2, 5, and 14 days after drug administration. The changes in DPOAE were compared with the changes in ABR. No treatment effect was noted in the 2-day group. Animals treated with c/s-platinum demonstrated significant elevation of DPOAE and ABR thresholds compared with control animals at 5 and 14 days. There was no significant difference between the threshold changes in the 5-and 14-day groups.

Objective: To propose a staging system for patients with lymphatic malformations of the head and neck. Design: Retrospective chart review. Patients: Fifty-six patients were treated for lymphatic malformations from 1983 to 1993 at Children's Hospital and Medical Center, Seattle, Wash. The charts were reviewed for anatomic location of the lesion, preoperative and postoperative complications, number of procedures to control disease, long-term sequelae, and persistence of disease. Lesions were characterized as being unilateral or bilateral and suprahyoid and/or infrahyoid. The five patient groups were then compared with respect to the above categories. Results: Preoperative complications reviewed include preoperative infection, respiratory embarassment necessitating airway intervention, and feeding difficulties. Postoperative complications assessed were cranial nerve injury, wound infection, and seroma formation. Long-term sequelae included malocclusion, speech delay, and cosmetic deformity. The rate of persistent disease was also assessed. A staging system was developed based on a progression of extent of disease. Stage I patients (n=12) had unilateral infrahyoid disease and a 17% incidence of complications overall. Stage II patients (n=17) had unilateral suprahyoid disease and a 41% incidence of complications. Stage III patients (n=15) had unilateral suprahyoid and infrahyoid disease and a complication rate of 67%. Stage IV patients (n=5) with bilateral suprahyoid disease had a complication rate of 80%, while stage V patients (n=6) with bilateral suprahyoid and infrahyoid disease had a 100% incidence of complications. Conclusion: Anatomic location of lymphatic malformations of the head and neck can be used to predict prognosis and outcome of surgical intervention. (Arch Otolaryngol Head Neck Surg. 1995;121:577-582)

Objective To generate consensus ratings of velopharyngeal function on nasendoscopy (NE) with the goal of creating a video instruction tool. Methods The American Society of Pediatric Otolaryngology Velopharyngeal Insufficiency Study Group convened to identify NE segments to be included in an instructional video. Of 24 segments reviewed, 11 were selected based on the quality of the examinations and spectrum of closure patterns. Participating otolaryngologists independently rated NE segments using the Golding-Kushner scale. The participants then convened and rated each of the NE segments as a group. Thirty-nine members of the American Society of Pediatric Otolaryngology met and agreed with the group ratings, creating a consensus standard. Results Individual scores for palate and lateral wall motion showed high variability, ranging from 0 to 6 points difference from the consensus. Variability was also seen for the following qualitative findings: the Passavant ridge, aberrant pulsations, and dorsal palatal notch. The individual ratings are presented graphically to demonstrate the range of individual responses as well as to compare responses to the consensus ratings. No further changes were made to the proposed consensus ratings when reviewed by the larger group. Conclusions Rating of NE evaluations of velopharyngeal function was variable among a group of pediatric otolaryngologists experienced in treating velopharyngeal insufficiency. These results highlight the need to develop a standardized method of reporting NE findings for velopharyngeal insufficiency. Despite this, consensus ratings were achieved that will facilitate development of a video instruction tool.

Objective: In a study of patients with velopharyngeal insufficiency (VPI) diagnosed by pediatric speech pathologists, test the VPI Effects on Life Outcomes (VELO) instrument for criterion validity, construct validity, reliability, and responsiveness (ability to detect change in quality of life).Method: VPI subjects/parents completed the VELO at baseline, 2 weeks later, and 3 months posttreatment. Criterion-validity tested correlation between VELO score and VPI severity; construct-validity tested correlation with speech intelligibility. Test-retest reliability tested intraclass-correlation between baseline VELO score and 2-week score. Responsiveness tested mean baseline and posttreatment scores with the paired t test.Results: Parental responses are reported (n = 83); youth responses (obtained in 8+ year-olds only; n = 24) showed similar results. VPI severity (minimal n = 11, mild n = 31, moderate n = 27, severe n = 14) and speech intelligibility were rated by pediatric speech pathologists. The VELO instrum...