Spectral Resolution and Speech Perception in Cochlear Implanted School-Aged Children.

OBJECTIVE: Cochlear implantation of prelingually deaf infants provides auditory input sufficient to develop spoken language; however, outcomes remain variable. Inability to participate in speech perception testing limits testing device efficacy in young listeners. In postlingually implanted adults (aCI), speech perception correlates with spectral resolution an ability that relies independently on frequency resolution (FR) and spectral modulation sensitivity (SMS). The correlation of spectral resolution to speech perception is unknown in prelingually implanted children (cCI). In this study, FR and SMS were measured using a spectral ripple discrimination (SRD) task and were correlated with vowel and consonant identification. It was hypothesized that prelingually deaf cCI would show immature SMS relative to postlingually deaf aCI and that FR would correlate with speech identification. STUDY DESIGN: Cross-sectional study. SETTING: In-person, booth testing. METHODS: SRD was used to determine the highest spectral ripple density perceived at various modulation depths. FR and SMS were derived from spectral modulation transfer functions. Vowel and consonant identification was measured; SRD performance and speech identification were analyzed for correlation. RESULTS: Fifteen prelingually implanted cCI and 13 postlingually implanted aCI were included. FR and SMS were similar between cCI and aCI. Better FR was associated with better speech identification for most measures. CONCLUSION: Prelingually implanted cCI demonstrated adult-like FR and SMS; additionally, FR correlated with speech identification. FR may be a measure of CI efficacy in young listeners.

Predictors of Time Spent in Special Education Among Students With Severe-to-Profound Hearing Loss.

OBJECTIVE: Determine if students with severe-to-profound hearing loss with cochlear implants (CIs) mainstream (transition to general education) more than students with hearing amplification at the population level. STUDY DESIGN: Cross-sectional secondary analysis of data from the National Center of Education Statistics. SETTING: Special education (SpEd) students in the United States who had severe to profound "hearing impairment" and were 6 to 16 years old at enrollment from 2000 to 2001. METHODS: We weighted the data to produce national estimates, performed multiple imputations for missingness, and built a multivariate linear regression model, which was cross-validated with a multivariate Poisson regression model. We used a theory-based approach to model-building using a directed acyclic graph to identify the minimally sufficient adjustment set of variables, which included school district urbanicity, student's age when they started SpEd, other disabilities, home language, and caregiver education. RESULTS: We identified 7267 students with CIs and 28,794 students with hearing amplification. CI users mainstreamed more than peers using hearing amplification during secondary school (40.29% less daily time in special education, p = .004) but not during primary school (9.19% less daily time in SpEd, p = .155). Additional significant predictors of mainstreaming varied between the primary and secondary school cohorts and included school district urbanicity and the student's age when they started SpEd. CONCLUSION: CI status predicts daily time spent in SpEd among a secondary school cohort. These findings do not establish causation. The National Center of Education Statistics should consider linking to clinical databases in future studies.

Association of Genetic Diagnoses for Childhood-Onset Hearing Loss With Cochlear Implant Outcomes.

Importance: In the US, most childhood-onset bilateral sensorineural hearing loss is genetic, with more than 120 genes and thousands of different alleles known. Primary treatments are hearing aids and cochlear implants. Genetic diagnosis can inform progression of hearing loss, indicate potential syndromic features, and suggest best timing for individualized treatment. Objective: To identify the genetic causes of childhood-onset hearing loss and characterize severity, progression, and cochlear implant success associated with genotype in a single large clinical cohort. Design, Setting, and Participants: This cross-sectional analysis (genomics) and retrospective cohort analysis (audiological measures) were conducted from 2019 to 2022 at the otolaryngology and audiology clinics of Seattle Children's Hospital and the University of Washington and included 449 children from 406 families with bilateral sensorineural hearing loss with an onset younger than 18 years. Data were analyzed between January and June 2022. Main Outcomes and Measures: Genetic diagnoses based on genomic sequencing and structural variant analysis of the DNA of participants; severity and progression of hearing loss as measured by audiologic testing; and cochlear implant success as measured by pediatric and adult speech perception tests. Hearing thresholds and speech perception scores were evaluated with respect to age at implant, months since implant, and genotype using a multivariate analysis of variance and covariance. Results: Of 406 participants, 208 (51%) were female, 17 (4%) were African/African American, 32 (8%) were East Asian, 219 (54%) were European, 53 (13%) were Latino/Admixed American, and 16 (4%) were South Asian. Genomic analysis yielded genetic diagnoses for 210 of 406 families (52%), including 55 of 82 multiplex families (67%) and 155 of 324 singleton families (48%). Rates of genetic diagnosis were similar for children of all ancestries. Causal variants occurred in 43 different genes, with each child (with 1 exception) having causative variant(s) in only 1 gene. Hearing loss severity, affected frequencies, and progression varied by gene and, for some genes, by genotype within gene. For children with causative mutations in MYO6, OTOA, SLC26A4, TMPRSS3, or severe loss-of-function variants in GJB2, hearing loss was progressive, with losses of more than 10 dB per decade. For all children with cochlear implants, outcomes of adult speech perception tests were greater than preimplanted levels. Yet the degree of success varied substantially by genotype. Adjusting for age at implant and interval since implant, speech perception was highest for children with hearing loss due to MITF or TMPRSS3. Conclusions and Relevance: The results of this cross-sectional study suggest that genetic diagnosis is now sufficiently advanced to enable its integration into precision medical care for childhood-onset hearing loss.

Sensitivity to Deaf Culture Among Otolaryngology and Audiology Trainees.

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 < .0001) but significantly lower than that for MS-TDCs at 69.0% (SD 13.0%)(CI -20.3%, -7.6%, P < .0001). Knowledge scores were comparable for OHNS and audiology trainees (P = .09). 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.

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.

Drug-induced sleep endoscopy directed surgery improves polysomnography measures in overweight and obese children with obstructive sleep apnea.

BACKGROUND: Obstructive sleep apnea affects approximately 1-4% of all children, with increased prevalence amongst overweight and obese children. OBJECTIVE: To assess the effects of drug-induced sleep endoscopy (DISE)-directed surgery on polysomnography parameters in obese and overweight children. MATERIAL/METHODS: A retrospective case-series was performed on obese and overweight pediatric patients who underwent clinically indicated DISE-directed surgery. Forty children met the inclusion criteria, including: body mass index ≥85%, DISE-study, and pre- and post-DISE polysomnography. Patients were divided into surgically naïve (n = 23) and prior adenotonsillectomy (n = 17) groups. Demographic and clinical characteristics were examined with chi-square and Wilcoxon rank-sum test. Polysomnography parameters were compared with Wilcoxon signed rank test. RESULTS: Of 40 children with mean BMI 94% and mean age 8 ± 6 years old, 17 (43%) underwent a previous adenotonsillectomy. Overall, significant improvements were observed in the apnea-hypopnea index (AHI; 25.0 to 9.9 events/hour, p < .01) and oxygen nadir (82.7% to 88.5%, p < .01). A similar pattern was observed among the surgically naïve (AHI: 35.9 to 12.7 events/hour, p = .04; oxygen nadir: 79.7% to 86.4%, p = .2) and post-adenotonsillectomy groups (AHI: 10.4 to 6.2 events/hour, p = .02; oxygen nadir: 86.7% to 91.2%, p < .01). CONCLUSIONS/SIGNIFICANCE: Polysomnography parameters significantly improved following DISE-directed interventions in obese and overweight children with obstructive sleep apnea.

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.

Reliability of Measuring Insertion Depth in Cochlear Implanted Infants and Children Using Cochlear View Radiography.

OBJECTIVES: Cochlear implant depth of insertion affects audiologic outcomes and can be measured in adults using plain films obtained in the "cochlear view." The objective of this study was to assess interrater and intrarater reliability of measuring depth of insertion using cochlear view radiography. STUDY DESIGN: Prospective, observational. SETTING: Tertiary referral pediatric hospital. SUBJECTS AND METHODS: Patients aged 11 months to 20 years (median, 4 years; interquartile range [IQR], 1-8 years) undergoing cochlear implantation at our institution were studied over 1 year. Children underwent cochlear view imaging on postoperative day 1. Films were deidentified and 1 image per ear was selected. Two cochlear implant surgeons and 2 radiologists evaluated each image and determined angular depth of insertion. Images were re-reviewed 6 weeks later by all raters. Inter- and intrarater reliability were calculated with intraclass correlation coefficients (ICCs). RESULTS: Fifty-seven ears were imaged from 42 children. Forty-nine ears (86%) had successful cochlear view x-rays. Median angular depth of insertion was 381° (minimum, 272°; maximum, 450°; IQR, 360°-395°) during the first round of measurement. Measurements of the same images reviewed 6 weeks later showed median depth of insertion of 382° (minimum, 272°; maximum, 449°; IQR, 360°-397°). Interrater and intrarater reliability ICCs ranged between 0.81 and 0.96, indicating excellent reliability. CONCLUSIONS: Postoperative cochlear view radiography is a reliable tool for measurement of cochlear implant depth of insertion in infants and children. Further studies are needed to determine reliability of intraoperatively obtained cochlear view radiographs in this population.

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.

Polysomnography outcomes of sleep endoscopy-directed intervention in surgically naïve children at risk for persistent obstructive sleep apnea.

PURPOSE: Drug-induced sleep endoscopy (DISE) is useful in children with obstructive sleep apnea (OSA) that persists after adenotonsillectomy (AT), but its utility in surgically naïve children is unclear. We report polysomnography outcomes of surgically naïve children who underwent DISE-directed intervention because they were considered high risk for persistent OSA after adenotonsillectomy. METHODS: This study is a case series of 62 surgically naïve children with OSA who were considered high risk for persistence after AT and underwent DISE-directed intervention with pre- and postoperative polysomnography between 2012 and 2016. Analysis was performed with the paired t test. RESULTS: Children were on average 5.9 (± 5.5, 0.2-18.6) years old at the time of surgery, 68% male, 18% obese, and 60% white. Thirty-eight percent had a syndromic diagnosis: 19% trisomy 21, 11% hypotonic neuromuscular disorder, and 8% craniofacial condition. The remaining 62% were non-syndromic but underwent DISE because they had at least one risk factor for OSA persistence after AT (age > 7 years, black race, 1+ tonsils, obesity, and/or severe OSA). Forty-two percent underwent AT, while 58% underwent treatment other than AT, including 18% who had multilevel surgery. Children improved significantly in 4 out of 5 polysomnography parameters tested, including obstructive apnea-hypopnea index (oAHI; 22.2 to 7.2, p < 0.01) and oxygen nadir (82 to 87, p < 0.01). Thirty-eight (61%) had a postoperative oAHI < 5; 16 (21%) had a postoperative oAHI < 2. CONCLUSION: DISE resulted in intervention other than AT in 58% of surgically naïve children at high risk for persistent OSA after AT. DISE-directed intervention resulted in significant mean improvement in postoperative OSA.

Auditory comprehension outcomes in children who receive a cochlear implant before 12 months of age.

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.

Reliability and Construct Validity of the Penetration-Aspiration Scale for Quantifying Pediatric Outcomes after Interarytenoid Augmentation.

OBJECTIVE: To assess the reliability and construct validity of the Penetration-Aspiration Scale in children. STUDY DESIGN: This was a retrospective cohort study of pre- and postoperative video modified barium swallow studies from children who underwent interarytenoid injection augmentation for unexplained persistent pharyngeal dysphagia. Two pediatric speech and language pathologists reviewed each study twice in a blinded and randomized fashion. SETTING: Tertiary academic pediatric hospital. SUBJECTS AND METHODS: Thirty children were identified with adequate pre- and postoperative modified barium swallow studies within 4 weeks of intervention. Children were separated into clinical outcome groups based on ability to advance to thinner diet consistencies postoperatively. Construct validity was assessed with a mixed linear model to test the hypothesis that only the clinically improved group would receive better Penetration-Aspiration Scale scores after surgery. Reliability was assessed by calculating chance-corrected agreement between raters (interrater) and raters' repeat evaluations (intrarater). RESULTS: Inter- and intrarater reliabilities (Cohen's κ) were both excellent. Results of the mixed model revealed a significant interaction between outcome group and pre- and postoperative time interval. As hypothesized, this involved a significant improvement in Penetration-Aspiration Scale score only in the improved group. CONCLUSIONS: These findings suggest that the Penetration-Aspiration Scale is a reliable and valid measure of clinical response to interarytenoid injection augmentation in children.

Pediatric dysphagia: Is interarytenoid mucosal height significant?

OBJECTIVES: The clinical significance of the interarytenoid mucosal height (IAMH) in pediatric dysphagia, ranging from normal anatomy to a laryngeal cleft, is unknown. This study seeks to evaluate a cohort of patients who underwent evaluation of their IAMH during microdirect laryngoscopy (MDL) for associations between IAMH and dysphagia as diagnosed on preoperative videofluoroscopic swallow study (VFSS). METHODS: A retrospective case series of 1,351 patients who underwent MDL between 2011 and 2016 were reviewed for intraoperative evaluation of IAMH using our interarytenoid assessment protocol. After exclusions, 182 patients were divided into three groups: 1) thickened diet: VFSS with recommendation for thickened liquids (n = 82 of 182; 45.1%), 2) normal diet: VFSS with allowance of thin liquids (n = 19 of 182; 10.4%), and 3) control: no VFSS performed (n = 81 of 182; 44.5%). RESULTS: There was no difference in IAMH between groups (P = 0.35). Power analysis was able to achieve > 80% power to detect an effect size of ≥ 0.5 (1-5 mucosal height scale). The majority of patients in each group had an IAMH above the false vocal folds (thickened diet: 57.3%, normal diet: 57.9%, control: 64.2%). There were similar percentages of patients in each group with an IAMH at or below the true vocal folds (thickened diet: 4.9%, normal diet: 5.3%, control: 6.1%). CONCLUSION: There was no significant association between IAMH and preoperative thickened liquid recommendation in this cohort. This data fails to support the hypothesis that the IAMH is an independent etiological factor for pediatric pharyngeal dysphagia. Further studies comparing IAMH with outcomes after feeding therapy and surgery may better clarify this relationship between anatomy and physiology. LEVEL OF EVIDENCE: 4. Laryngoscope, 129:2588-2593, 2019.

Polysomnography outcomes in children with small tonsils undergoing drug-induced sleep endoscopy-directed surgery.

OBJECTIVES/HYPOTHESIS: The optimal surgical treatment to improve sleep apnea in children with small tonsils is not known. Drug-induced sleep endoscopy (DISE) may be useful in selecting effective surgical procedures for this patient population. This study compared polysomnography (PSG) measures before and after DISE-directed surgery in children with small tonsils. We hypothesize that DISE-directed surgery improves PSG measures in children with small tonsils. We also aimed to identify the most common surgery performed in this population. STUDY DESIGN: Retrospective chart review. METHODS: A retrospective review was performed of subjects who underwent DISE at a single pediatric tertiary care center over a 6-year period. Inclusion criteria were 1+ tonsils and PSG performed before and after DISE-directed surgery. Exclusion criteria were previous tonsillectomy and tonsils score 2+ or greater. Pre- and postoperative PSG parameters were compared using paired t tests. RESULTS: Average age was 7 ± 4 years old at the time of surgery. The most common DISE-directed intervention was supraglottoplasty (n = 23). DISE-directed surgery significantly improved mean apnea-hypopnea index (AHI) from 14.4 to 8.0 (P = .02). Although improvements were seen in mean obstructive AHI (12.5 to 5.5), O2 nadir (87.0 to 88.3), and ODI (10.6 to 5.8), these measures did not reach statistical significance. CONCLUSIONS: DISE-directed surgery significantly improves AHI in children with small tonsils. The most common intervention performed on these children was supraglottoplasty. Interestingly, adenotonsillectomy was rarely performed in this cohort. Additionally, supraglottic collapse, prompting supraglottoplasty, is difficult to accurately assess in an awake child, supporting use of a DISE-directed approach in this patient population. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:2771-2774, 2019.

Unilateral Hearing Loss in Youth: Development of Candidate Items for a Condition-Specific Validated Instrument.

OBJECTIVE: This study interviewed youth with unilateral hearing, utilizing their responses to generate candidate items for a condition-specific patient-reported instrument. STUDY DESIGN: Mixed methods, cross-sectional. SETTING: Tertiary care children's hospital. PARTICIPANTS AND METHODS: Youth with unilateral hearing loss and normal hearing in the contralateral ear were identified and recruited for participation through query of an audiometric database and through hearing loss clinics. Interviews with the youth were qualitatively analyzed to identify common themes and generate items related to functional impact. A multi-institutional expert panel reviewed items with prespecified item selection criteria. Participants rated items for impact on daily life. For preliminary criterion validity assessment, statistical analyses explored correlations between functional scores and type and severity of hearing loss. RESULTS: Thirty-nine youth aged 9 to 18 years with unilateral hearing loss participated; 31% used a hearing device. Fifteen youth participated in interviews; thematic analysis, item crafting, and expert panel item review resulted in 41 items. Twenty-six youth responded to the items, reporting low functional scores in the domains of sound localization, ear positioning, and noise environment. They reported better levels of function in carrying out group conversations, focusing on schoolwork, and feeling safe during activities. Multivariate linear regression found that youth scored 0.4 points (or approximately 8%) lower on the functional impact scale with every 20-dB HL increase in pure tone average in the abnormal ear. CONCLUSION: Youth with unilateral hearing loss report functional impact, particularly related to sound localization, ear positioning, and noise environment; therefore, they may benefit from a condition-specific functional assessment instrument.

Does drug induced sleep endoscopy-directed surgery improve polysomnography measures in children with Down Syndrome and obstructive sleep apnea?

BACKGROUND: Children with Down Syndrome (DS) and obstructive sleep apnea (OSA) are difficult to treat, as first line therapies may not lead to significant improvement. Drug-induced sleep endoscopy (DISE) directed surgery may be particularly beneficial for these patients. OBJECTIVE: To assess change in polysomnography (PSG) measures of patients with DS who underwent DISE-directed surgery. METHODS: Retrospective chart review was performed on patients with DS who underwent DISE-directed surgery and had pre- and post-surgery PSG. Patients were analyzed in groups defined by previous adenotonsillectomy. Two-sided t-tests with equal variances were used to assess statistical significance. RESULTS: Of 24 patients reviewed, 14 were surgically naïve and 10 had undergone prior adenotonsillectomy. The primary outcome was change in PSG parameters including apnea hypopnea index, obstructive apnea hypopnea index, oxygen nadir, oxygen desaturation index, and mean carbon dioxide level. While improvement was seen in all PSG parameters, only improvement in oxygen nadir in children who had undergone prior adenotonsillectomy was statistically significant (88.5% to 90.9%, p = .04). CONCLUSIONS AND SIGNIFICANCE: DISE-directed surgery may be beneficial for children with DS and OSA, with improvement in the means of main PSG measures observed. A larger, prospective study is warranted to further explore DISE utility.

Candidemia and invasive candidiasis among hospitalized neonates and pediatric patients.

OBJECTIVE: To investigate the epidemiology, treatment, length of stay (LOS) and costs for neonatal and pediatric inpatients with invasive candidiasis (IC). METHODS: The Cerner Health Facts Database was used to assess inpatients (2005-2014) identified by positive blood or cerebrospinal fluid (CSF) Candida cultures. Log-transformed LOS and cost were examined in candidemia-only patients (n = 191) using multivariable linear regression. RESULTS: A total of 202 patients had a positive culture (blood: n = 192; CSF: n = 10; both: n = 2). The most prevalent species were C. parapsilosis (n = 70, 34.7%), and C. albicans (n = 66, 32.7%). Mean (SD) age was 5 (5.5) years; 30 (14.9%) patients were <4 months. Comorbidities included sepsis (n = 85, 42.1%), coagulation disorders (n = 57, 28.2%), cancer (n = 64, 31.7%), and low birthweight (n = 26, 12.9%). Antifungals (AFs) included azoles (57.4%), polyenes (28.7%), and echinocandins (35.1%); 20.8% of patients received no AF during their encounter. The mean (SD) cost per encounter was $97,392 ($149,253), with a mean (SD) LOS of 45.6 (59.5) days and 9.9% mortality at discharge. Results were similar across Candida species. In regression analysis, intensive care unit (ICU) exposure, central catheter, sepsis, AF >48 hours prior to index culture, and age <4 months were associated with increased LOS; treatment at a non-teaching hospital was associated with reduced LOS (p < .05). AF use >48 hours before index, in-hospital mortality, Midwest region and ventricular shunt were associated with increased cost (p ≤ .05). CONCLUSIONS: This analysis confirms the association between pediatric candidemia and increased resource utilization and LOS. Given high observed rates of potential under-treatment, an opportunity may exist to improve AF therapy in this population.

Immunomodulatory oligonucleotide IMT504: Effects on mesenchymal stem cells as a first-in-class immunoprotective/immunoregenerative therapy.

The immune responses of humans and animals to insults (i.e., infections, traumas, tumoral transformation and radiation) are based on an intricate network of cells and chemical messengers. Abnormally high inflammation immediately after insult or abnormally prolonged pro-inflammatory stimuli bringing about chronic inflammation can lead to life-threatening or severely debilitating diseases. Mesenchymal stem cell (MSC) transplant has proved to be an effective therapy in preclinical studies which evaluated a vast diversity of inflammatory conditions. MSCs lead to resolution of inflammation, preparation for regeneration and actual regeneration, and then ultimate return to normal baseline or homeostasis. However, in clinical trials of transplanted MSCs, the expectations of great medical benefit have not yet been fulfilled. As a practical alternative to MSC transplant, a synthetic drug with the capacity to boost endogenous MSC expansion and/or activation may also be effective. Regarding this, IMT504, the prototype of a major class of immunomodulatory oligonucleotides, induces in vivo expansion of MSCs, resulting in a marked improvement in preclinical models of neuropathic pain, osteoporosis, diabetes and sepsis. IMT504 is easily manufactured and has an excellent preclinical safety record. In the small number of patients studied thus far, IMT504 has been well-tolerated, even at very high dosage. Further clinical investigation is necessary to demonstrate the utility of IMT504 for resolution of inflammation and regeneration in a broad array of human diseases that would likely benefit from an immunoprotective/immunoregenerative therapy.

Clinically small tonsils are typically not obstructive in children during drug-induced sleep endoscopy.

OBJECTIVE: To determine whether the degree of lateral pharyngeal wall (LPW) obstruction on pediatric drug-induced sleep endoscopy (DISE) correlates with preprocedure tonsillar hypertrophy score on physical examination, and to determine if clinically small tonsils are obstructive. METHODS: Retrospective review of 154 patients who underwent DISE at a single pediatric tertiary care center over a 4-year period. Inclusion criteria were documentation of Brodsky tonsil score on preoperative physical examination. Exclusion criteria were previous tonsillectomy, adenoidectomy, or adenotonsillectomy. Lateral pharyngeal wall obstruction was graded for each patient from 0 (no obstruction) to 3 (severe obstruction) using a validated pediatric DISE scoring system known as the Chan-Parikh scoring system (C-P). Data were analyzed using multivariate linear regression controlling for age at time of DISE and presence of comorbid conditions. RESULTS: One hundred fifteen patients met criteria for analysis. Median age at DISE was 5.1 years. A moderate positive correlation was calculated between Brodsky score and DISE score, Spearman correlation coefficient 0.55, P = < 0.001. Linear regression modeling determined that for every 1-point increase in tonsil score, there was a 0.7-point increase in C-P LPW score (95% confidence interval [0.45, 0.92]). Sensitivity analysis did not detect a difference in correlation between children with comorbid conditions and children who were otherwise in good health. Of the 65 children with a pre-DISE Brodsky tonsil score of 1, 39 (60%) had a LPW score of 0 (no obstruction); nine (14%) had a score of 1 (< 50% obstruction); 11 (17%) had a score of 2 (> 50% obstruction); and six (9%) had a score of 3 (100% obstruction). CONCLUSION: There is a positive correlation between Brodsky Score and DISE LPW score. The majority of children with sleep-disordered breathing with a Brodsky score of 1 did not demonstrate LPW obstruction. These children may benefit from DISE for identification of alternative sites of upper airway obstruction. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:1943-1949, 2017.

Airway Obstruction during Drug-Induced Sleep Endoscopy Correlates with Apnea-Hypopnea Index and Oxygen Nadir in Children.

OBJECTIVE: To determine if standardized intraoperative scoring of anatomic obstruction in children with obstructive sleep apnea correlates with the apnea-hypopnea index (AHI) and lowest oxygen saturation on preprocedural polysomnogram (PSG). A secondary objective was to determine if age, presence of a syndrome, or previous adenotonsillectomy affect this correlation. STUDY DESIGN: Case series with chart review. SETTING: Two tertiary care children's hospitals. SUBJECTS: Patients with a preprocedural PSG who underwent drug-induced sleep endoscopy (DISE) over a 4-year period. METHODS: All DISEs were graded in a systematic manner with the Chan-Parikh (C-P) scoring system. AHI and nadir oxygen saturations were extracted from preprocedural PSG. Data were analyzed with a multivariate linear regression model that controlled for age at time of sleep endoscopy, syndrome diagnosis, and previous adenotonsillectomy. RESULTS: A total of 127 children underwent PSG prior to DISE: 56 were syndromic, and 21 had a previous adenotonsillectomy. Mean AHI was 13.6 ± 19.6 (± SD), and mean oxygen nadir was 85.4% ± 9.4%. Mean C-P score was 5.9 ± 2.7. DISE score positively correlated with preoperative AHI (r = 0.36, P < .0001) and negatively correlated with oxygen nadir (r = -0.26, P = .004). The multivariate linear regression models estimated that for every 1-point increase in C-P score, there is a 2.6-point increase in AHI (95% confidence interval: 1.4-3.8, P < .001) and a 1.1% decrease in the lowest oxygen saturation (95% confidence interval: -1.7 to -0.6, P < .001). CONCLUSION: The C-P scoring system for pediatric DISE correlates with both AHI and lowest oxygen saturation on preprocedural PSG.