Search

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.

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: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.

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.

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.

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.

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.

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.

To compare the prevalence of congenital cytomegalovirus (CMV) infection in Washington State in children with hearing loss (HL) and the general population and to compare the characteristics of HL in children with and without congenital CMV infection.Matched case-control; case cohort.Regional pediatric hospital, Washington State Department of Health (WSDOH).Cases were children 4 years and older with HL born in Washington State. Control individuals matched for demographic characteristics were identified at random through the WSDOH.Congenital CMV status determined using quantitative polymerase chain reaction testing on newborn heel stick blood spots archived by the WSDOH. Audiologic data were used to characterize HL.Congenital CMV testing was performed for 222 matched cases and controls. Congenital CMV infection was detected in 1.4% of controls and in 9.9% of cases (odds ratio, 10.5; 95% confidence interval, 2.6-92.4). An estimated 8.9% of HL in children in Washington can be attributed to CMV infection. After inclusion of an additional 132 children with HL (for a total of 354 cases in the case cohort), we observed that children with congenital CMV had more severe HL (P.001) and higher proportions of progressive (P = .02) and unilateral (P = .002) HL compared with children without congenital CMV infection. In the 35 children with congenital CMV infection, there was no relationship between neonatal CMV load and severity of HL.In Washington State, children with HL had a far higher prevalence of congenital CMV viremia than did the general pediatric population, and CMV infection seems to be responsible for an appreciable fraction of pediatric HL in Washington State.

To study routine culture-negative persistent cervical lymphadenitis in children treated surgically during a 10-year period (December 26, 1997, to October 1, 2007) at a single institution.Retrospective case series.Tertiary university-based pediatric referral center.Patients 18 years or younger with cervical lymphadenitis managed surgically (incision and drainage, curettage, and/or excisional lymphadenectomy) and medically (antibiotic therapy), culture-negative after 48 hours, and subsequently diagnosed using the polymerase chain reaction, extended culture incubation, and/or histopathologic evaluation.Number of surgical interventions, causative organisms, histopathologic features, and resolution of lymphadenitis.Ninety surgical procedures were performed in 60 patients. The cure rate was 23% (approximately 14 patients) with incision and drainage, 58% (approximately 35 patients) with curettage, and 95% (57 patients) with excisional lymphadenectomy. Nontuberculous mycobacteria were the most prevalent causative organisms, followed by Bartonella and Legionella organisms. Four of 6 patients with Bartonella infection had a history of cat exposure, and 4 of 6 patients with Legionella infection had a history of hot tub exposure.Excisional lymphadenectomy is the preferred treatment of mycobacterial persistent cervical lymphadenitis in children. Sufficient data are lacking for similar recommendations in patients with disease caused by Bartonella organisms, whereas for neck disease caused by Legionella organisms, excisional lymphadenectomy may be superior to incision and drainage. The polymerase chain reaction is useful for pathogen identification in pediatric cervical lymphadenitis, although it is less sensitive in identification of mycobacteria. To our knowledge, our study is the first to report multiple cases of legionellosis in otherwise healthy children. Legionella seems to be a previously unrecognized but relatively common pathogen in culture-negative persistent cervical lymphadenitis in children.