Study protocol for Hear Me Read (HMR): A prospective clinical trial assessing a digital storybook intervention for young children who are deaf or hard of hearing.

Since the early 2000's, digital reading applications have enhanced the language and literacy skills of typically hearing young children; however, no digital storybook intervention currently exists to scaffold the early language and literacy skills of their peers who are deaf or hard of hearing. To address this gap, our research team developed a novel digital storybook intervention called Hear Me Read with the aim of enhancing the therapeutic, language, and literacy benefits of speech-language therapy. This prospective clinical trial (registered at clinicaltrials.gov, NCT#: 05245799) aims to determine the efficacy of adding Hear Me Read to in-person speech-language therapy for children aged three to five years who are deaf or hard of hearing. Fifty caregivers, their child, and their child's treating speech-language pathologist participate in the trial for 12 months. In the first six months, children attend standard-of-care speech-language therapy sessions. In the second six months, children continue to attend standard-of-care speech-language therapy sessions and use the Hear Me Read application, via a study supplied iPad. The primary outcome of this trial is that, compared to in-person speech-language therapy alone, in-person speech-language therapy with Hear Me Read will improve vocabulary, speech, and language outcomes in children aged three to five years who are deaf or hard of hearing. The secondary outcome is that, compared to in-person speech-language therapy alone, in-person speech-language therapy with Hear Me Read will improve literacy outcomes in children aged three to five years who are deaf or hard of hearing. The goal of this intervention is to help children who are deaf or hard of hearing achieve their vocabulary, speech, language, and literacy goals through interactive digital storybook reading.

Validation of a PHIS Esophageal Atresia and Tracheoesophageal Fistula Cohort in ICD-10.

OBJECTIVE: Validation of a contemporary International Classification of Diseases, 10th Revision, Clinical Modification (ICD-10-CM) congenital esophageal atresia/tracheoesophageal atresia (EA/TEF) cohort in the Pediatric Health Information System (PHIS) database. STUDY DESIGN: Database study, validation. SETTING: Tertiary care center. METHODS: Search methods used to validate an ICD-9-CM EA/TEF cohort in PHIS were modified for ICD-10-CM. A retrospectively and prospectively maintained clinical database at a single high-volume EA/TEF center was used for comparison. Patients treated between October 1, 2015 and July 31, 2022 were included. Searches progressively narrowed the cohort by ICD-10-CM diagnosis codes, expansion to include incorrectly coded as 'iatrogenic, age less than 30 days, and use of at least 1 ICD-10-CM procedure code. Results of PHIS data and institution data were compared for accuracy. RESULTS: The most refined search of PHIS and the EA/TEF clinical database yielded 93 and 84 patients, respectively. The sensitivity was 99% and positive predictive value was 94%. A PHIS search using these methods and encompassing 49 children's hospitals yielded an EA/TEF cohort of 2479 patients. CONCLUSION: We present a validated search method in the PHIS database to identify a high-fidelity cohort of EA/TEF patients for multi-institutional study. We have demonstrated that a carefully maintained clinical database may be used to validate cohorts in PHIS. This cohort allows for improved practice variability and outcomes study of EA/TEF patients. Similar methods may be employed to generate other rare disease cohorts in PHIS. LEVEL OF EVIDENCE: Level 4.

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. METHODS: Children <6 months-old at time of first outpatient, Emergency Department, or inpatient visit were included and followed longitudinally. A time-to-event analysis was performed using a Cox proportional hazards model to estimate hazard ratios for OM incidence based on sociodemographic factors and specific health conditions. RESULTS: The PEDSnet cohort included 910,265 children, 54.3% male, mean age (months) 1.3 [standard deviation (SD) 1.6] and mean follow up (years) 4.3 (SD 3.2). By age 3 years, 39.6% of children had evidence of one OM episode. OM rates decreased following pneumococcal-13 vaccination (PCV-13) and the COVID-19 pandemic. Along with young age, non-Hispanic Black/African American or Hispanic race/ethnicity, public insurance, higher family income, and male sex had higher incidence rates. Health conditions that increased OM risk included cleft palate [adjusted hazard ratio (aHR) 4.0 [95% confidence interval (CI) 3.9-4.2], primary ciliary dyskinesia [aHR 2.5 (95% CI 1.8-3.5)], trisomy 21 [aHR 2.0 (95% CI 1.9-2.1)], atopic dermatitis [aHR 1.4 (95% CI 1.4-1.4)], and gastroesophageal reflux [aHR1.5 (95% CI 1.5-1.5)]. CONCLUSIONS: Approximately 20% of children by age 1 and 40% of children by age 3 years will have experienced an OM episode. OM rates decreased after PCV-13 and COVID-19. Children with abnormal ciliary function or craniofacial conditions, specifically cleft palate, carry the highest risk of OM.

Surgical management of vocal cord nodules in children: Trends and outcomes.

OBJECTIVES: Vocal cord nodules (VCNs) are the most common cause of dysphonia in school-aged children, with potential negative impacts on quality of life including diminished self-esteem and academic performance. The standard of care for VCNs is conservative management which ranges from voice hygiene to speech therapy with a focus on voice otherwise known as voice therapy, with surgical excision reserved for refractory cases. Thus, few studies have analyzed outcomes of surgical management of VCNs. The purpose of this study is to assess the prevalence and efficacy of surgical excision of VCNs when compared to speech therapy. METHODS: Children with VCNs seen at a single tertiary care institution between 2015 and 2020 were identified by ICD-9 code 478.5 and ICD-10 code J38.2. Demographics, objective voice assessment, intervention, and follow-up assessment data were reviewed. Frequencies, medians, and interquartile ranges were calculated. Time to resolution and improvement were assessed by Cox proportional hazards model. Univariate logistic regression was performed. A P value of <0.05 was considered statistically significant. RESULTS: Three hundred sixty-eight patients diagnosed with VCNs were identified. 169 patients received intervention for VCNs, with 159 (43.2 %) receiving speech therapy alone and 5 (1.4 %) receiving surgery alone. On bivariate analysis, there was no significant difference in demographic features between treatment groups, however speech therapy patients did have a longer follow-up time. 154 patients underwent objective voice assessment at the time of VCN diagnosis. Among these patients, 95 (61.7 %) received speech therapy and 59 (40.3 %) received no intervention. Speech therapy patients had significantly higher pVHI scores, however there was no significant difference in CAPE-V Overall Severity scores or computerized voice assessment analysis. On Cox proportional hazards analysis, surgical intervention was associated with faster resolution and faster improvement of dysphonic symptoms. On binary logistic regression, surgery was associated with a significantly greater proportion of patients reporting resolution of dysphonic symptoms, however there was no significant difference in proportion of patients reporting improvement of dysphonia. CONCLUSION: For most patients with VCNs, conservative measures such as voice hygiene and speech therapy remain first line, however certain patients may benefit from the rapid improvement and resolution of symptoms that surgical intervention may provide.

Evidence-Based Medicine in Otolaryngology Part 16: Qualitative and Quantitative Methods-Contrasting and Complementary Approaches.

Qualitative methods have been increasingly applied in our literature, providing richness to data and incorporating the nuances of patient and family perspectives. These qualitative research techniques provide breadth and depth beyond what can be gleaned through quantitative methods alone. When both quantitative and qualitative approaches are coupled, their findings provide complementary information which can further substantiate study conclusions. We thus aim to provide insight into qualitative and quantitative methods in comparison and contrast to each other, as well as guidance on when each approach is most apt. In relation, we also describe mixed methods and the theory supporting their framework. In doing so, we provide the foundation for an ensuing, more detailed exposition of qualitative methods.

Sphincter Pharyngoplasty for Velopharyngeal Dysfunction: Impact of 22q11.2 Deletion Syndrome.

OBJECTIVE: Patients with 22q11.2 deletion syndrome (22q11DelS) often present with velopharyngeal dysfunction (VPD). VPD in patients with 22q11DelS is multifactorial beyond velopharyngeal insufficiency (VPI) alone, and differences in surgical outcomes are poorly understood. Our objective was to determine whether patients with 22q11DelS have an increased risk for persistent VPI after sphincter pharyngoplasty compared to patients without 22q11DelS. METHODS: We completed a retrospective cohort study of patients with 22q11DelS undergoing sphincter pharyngoplasty between 1995 and 2019 using a VPD clinic database. Patients with 22q11DelS were compared to a cohort of 2:1 frequency-matched (age, degree of velopharyngeal closure) patients without 22q11DelS. Variables included patient characteristics, surgical history, perceptual speech evaluation, and degree of closure on nasopharyngoscopic evaluations. Primary outcomes included postoperative VPI severity and hypernasality. Speech and nasopharyngoscopic characteristics were compared using Fisher's exact test. Postoperative VPI severity and hypernasality were compared between groups via relative risks (RR) from mixed effects Poisson regression models, with random effects of age and velopharyngeal closure. RESULTS: 134 patients (51 22q11DelS, 83 matched) were included, with mean age of 7.3 years (standard deviation 3.0) and 50% male. Cohorts had similar preoperative speech characteristics and nasopharyngoscopic findings. Patients with 22q11DelS had similar postoperative VP function as patients without 22q11DelS (RR 0.85, CI 0.46-1.57 for VPI severity, RR 0.83, CI 0.45-1.53 for hypernasality). Even after adjusting by preoperative variables, no differences were seen between both groups. CONCLUSION: Matched for age and pre-operative velopharyngeal closure, patients with and without 22q11DelS and VPI had similar benefits after sphincter pharyngoplasty. LEVEL OF EVIDENCE: Non-randomized controlled cohort study, 3 Laryngoscope, 133:2813-2820, 2023.

The December Effect in Pediatric Elective Surgery Utilization: Differences Between Privately and Publicly Insured Children.

OBJECTIVES: The objective of this study was to identify differences in December elective surgery utilization between privately and publicly insured children, given that increases in the prevalence and size of annual deductibles may be driving more families with commercial health insurance to delay elective pediatric surgical procedures until later in the calendar year. STUDY DESIGN: We identified patients aged <18 years who underwent myringotomy, tonsillectomy ± adenoidectomy, tympanoplasty, hydrocelectomy, orchidopexy, distal hypospadias repair, or repair of inguinal, umbilical, or epigastric hernia using the 2012-2019 state inpatient and ambulatory surgery and services databases of 9 states. Log-binomial regression models were used to compare relative probabilities of procedures being performed each month. Linear regression models were used to evaluate temporal trends in the proportions of procedures performed in December. RESULTS: Our study cohort (n = 1 001 728) consisted of 56.7% privately insured and 41.8% publicly insured children. Peak procedure utilization among privately and publicly insured children was in December (10.1%) and June (9.6%), respectively. Privately insured children were 24% (95% CI 22%-26%) more likely to undergo surgery in December (P < .001), with a significant increase seen for 8 of 9 procedures. There was no trend over time in the percentage of procedures performed in December, except for hydrocelectomies, which increased by 0.4 percentage points/year among privately insured children (P = .02). CONCLUSIONS: Privately insured children are >20% more likely than publicly insured children to undergo elective surgery in December. However, despite increases in the prevalence of high deductibles, the proportion of procedures performed in December has not increased over recent years.

Healthcare Equity in Pediatric Otolaryngology.

In almost all areas that have been studied, racial, ethnic, socioeconomic, and other disparities have been identified throughout pediatric otolaryngology. This article focuses on some of the most studied areas, including the use of tonsillectomy, ear tubes, cochlear implants, and tracheostomies. Disparities are best reduced through multilevel interventions that address policy and upstream determinants of health. However, in some cases, standardization of care through clinical practice guidelines or can reduce disparities in care delivery. Future research in pediatric otolaryngology should specifically study disparities, their causes, and how to reduce them.

Avoidance of surgery for head and neck infantile myofibromatosis using imatinib monotherapy.

Describe a novel use for a kinase inhibitor, imatinib, in young children with a known activated somatic mutation in PDGFR-beta. Two patients with infantile myofibromatosis treated with imatinib. Case description of evaluation, diagnosis and treatment decisions for infantile myfibromatosis of the head and neck. Description of medical therapy for infantile myofibromatosis in these patients. For function threatening myofibromas of a known genotype, in infants, targeted medical therapy is a treatment option.

Quantifying Upper Aerodigestive Sequelae in Esophageal Atresia/Tracheoesophageal Fistula Neonates.

OBJECTIVES: We examined rates of upper aerodigestive tract (UADT) procedures in a multi-institutional cohort of neonates with esophageal atresia/tracheoesophageal fistula (EA/TEF) to estimate secondary UADT pathology. METHODS: A retrospective cohort study was performed using a previously-validated population of patients with EA/TEF within the Pediatric Health Information System (PHIS) between 2007 and 2015. ICD-9/10-CM codes for aerodigestive procedures were examined from 2007 to 2020: 1) diagnostic direct laryngoscopy and/or bronchoscopy (DLB), 2) DLB with intervention, 3) tracheostomy, 4) gastrostomy, 5) fundoplication, 6) aortopexy, 7) laryngotracheoplasty, and 8) esophageal dilation. Associations between procedures and demographics, length of gestation, and weight were estimated using generalized linear mixed models. RESULTS: We identified 2,509 patients with EA/TEF from 47 hospitals, 56.7% male and 43.3% female. Median length of stay for the first admission was 24 days (interquartile range: 12-55). Of these patients, 1,943 (77.4%) had at least one aerodigestive procedure within 14 admissions. Specifically, 1,635 (65.2%) underwent diagnostic DLB, 85 (3.4%) DLB with intervention, 167 (6.7%) tracheostomy, 1,043 (41.2%) gastrostomy, 211 (11.0%) fundoplication, 52 (2.1%) aortopexy, 161 (6.4%) laryngotracheoplasty, and 207 (8.3%) esophageal dilation. Preterm gestation increased odds of tracheostomy (adjusted odds ratio (OR) 2.4, 95% confidence interval (CI) 1.5-3.7), gastrostomy (OR 2.1, CI 1.7-2.7), fundoplication (OR 1.7, CI 1.1-2.4), aortopexy (OR 5.8, CI 2.1-16.1), and esophageal dilation (OR 2.0, CI 1.4-3.0). Very low birth weight (<1,500 g) increased odds of gastrostomy (OR 2.5, CI 1.6-3.8). CONCLUSION: Patients with EA/TEF frequently have aerodigestive sequelae. This work helps quantify aerodigestive needs in neonates with EA/TEF, suggesting early otolaryngology evaluation in their care. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:695-700, 2022.

Validation of the Seattle Suprastomal Safety Score (5S): A Novel Measure in Pediatric Tracheostomy-Dependent Patients.

OBJECTIVE: Suprastomal collapse and granulation are common sequelae of pediatric tracheostomy. We present the first measure of suprastomal obstructive pathology, the Seattle Suprastomal Safety Score (5S), an instrument with 2 domains: collapse and granulation. STUDY DESIGN: Cross-sectional repeated testing survey. SETTING: Electronic survey. METHODS: A library of images was assembled from still pictures of the suprastomal area in 50 patients who previously underwent trachea-bronchoscopy at a quaternary children's hospital. Five pediatric otolaryngologists and 2 pediatric pulmonologists reviewed the images in random, blinded fashion and provided 5S scores. Participants repeated this process 2 to 4 weeks later. Interrater agreement was calculated with an intraclass correlation coefficient (ICC) with a 2-way random-effects model and Fleiss's κ. Intrarater agreement was measured with an ICC using a 2-way mixed-effects model as well as with test-retest correlations using Spearman rank coefficient. All measures were performed separately on collapse and granulation domains. RESULTS: ICC for interrater agreement was 0.88 (95% CI, 0.82-0.93) for collapse and 0.97 (95% CI, 0.96-0.98) for granulation, indicating almost perfect agreement. Fleiss's κ demonstrated moderate agreement for collapse and almost perfect agreement for granulation. ICC for intrarater agreement was 0.95 (95% CI, 0.93-0.97) and 0.99 (95% CI, 0.98-0.99) for collapse and granulation, respectively, indicating almost perfect agreement. Spearman rank correlation for test-retest demonstrated substantial agreement for collapse and almost perfect agreement for granulation. CONCLUSION: The 5S demonstrates excellent interrater and intrarater agreement, making it highly reliable as a novel measure of suprastomal collapse and granulation in tracheostomy-dependent pediatric patients.

Defining Essential Services for Deaf and Hard of Hearing Children during the COVID-19 Pandemic.

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.

Pediatric Otolaryngology Divisional and Institutional Preparatory Response at Seattle Children's Hospital after COVID-19 Regional Exposure.

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.

A Systematic Review of Cochlear Implant Outcomes in Prelingually-deafened, Late-implanted Patients.

OBJECTIVE: To analyze outcomes of cochlear implantation (CI) in prelingually-deafened, late-implanted patients. DATA SOURCES: A search of MEDLINE and EMBASE was performed in February 2018 using SCOPUS for the intersection of "cochlear implant," "prelingual," "deaf," and "delay." REVIEW METHODS: Two independent reviewers screened all abstracts and titles for relevance, with conflicts resolved by either the primary or senior author. All articles passing this screen were subjected to a full-text review, during which the primary and senior author each examined manuscripts for inclusion and exclusion criteria. The Cochrane Risk of Bias Assessment Tool was used to assess potential sources of systematic error, and postoperative clinical outcomes were collected at the latest clinical follow-up. RESULTS: Twenty-eight articles were yielded in the final systematic review, accounting for 542 patients. For open-set sentence scores, 10 studies representing 240 patients showed an overall estimated improvement of 44.6% (95% CI: 38.0-51.2%). In terms of quality of life, studies generally showed improvement when looking at specific emotional, social, or hearing-specific domains, but not in global measures. Nonuser rates ranged from 0 to 9.5%. CONCLUSION: Despite performance that is generally poorer than what is generally seen in "traditional" candidates, prelingually-deafened, late-implanted (PL-LI) CI users can experience benefit in terms of both QOL and audiometric scores. The wide range of performance that is reported in the literature highlights the importance of careful patient counseling.

Creation of a head and neck Keloid quality of life questionnaire.

OBJECTIVES: Create a head-and-neck keloid quality of life (QOL) questionnaire. Demonstrate the reliability of the keloid-specific QOL scale according to test-retest and internal consistency standards. STUDY DESIGN: Prospective cohort study. METHODS: Patients presenting to a keloid clinic in a tertiary referral institution between April 2012 and February 2013 were enrolled on their first visit. They were administered a questionnaire assessing demographics and a 21-item Likert keloid-specific survey assessing symptomatology, self-esteem, social functioning, and therapeutic motivation during their first three visits. Reliability of the keloid questionnaire was assessed for internal consistency (Cronbach's alpha) and test-retest correlations. Patients were treated with Kenalog steroid injections at each visit. The 126-point total score from the questionnaire was then compared to each subscale (physical symptoms, self-esteem, social function, and medical motivation) using a Pearson coefficient. RESULTS: The keloid QOL questionnaire showed a Cronbach's alpha of 0.87 for the overall questionnaire and ranged from 0.66 to 0.86 for individual questions. The test-retest Pearson's R was 0.70 between visits 1 and 2 and 0.77 between visits 2 and 3. The Pearson correlation between symptoms, self-esteem, social functioning, and medical motivation subscales and the overall scale were 0.77, 0.73, 0.72, and 0.57, respectively. CONCLUSION: This head and neck keloid-specific QOL questionnaire proved to be a reproducible method of reliably assessing QOL burden on patients with head and neck keloids. LEVEL OF EVIDENCE: N/A.