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Objective To determine if differences in patient characteristics, treatments, and outcomes exist between children with sepsis who arrive by emergency medical services (EMS) versus their own mode of transport (self-transport). Methods Retrospective cohort study of patients who presented to the Emergency Department (ED) of two large children's hospitals and treated for sepsis from November 2013 to June 2017. Presentation, ED treatment, and outcomes, primarily time to first bolus and first parental antibiotic, were compared between those transported via EMS versus patients who were self-transported. Results Of the 1813 children treated in the ED for sepsis, 1452 were self-transported and 361 were transported via EMS. The EMS group were more frequently male, of black race, and publicly insured than the self-transport group. The EMS group was more likely to have a critical triage category, receive initial care in the resuscitation suite (51.9 vs. 22%), have hypotension at ED presentation (14.4 vs. 5.4%), lactate >2.0 mmol/L (60.6 vs. 40.8%), vasoactive agents initiated in the ED (8.9 vs. 4.9%), and to be intubated in the ED (14.4 vs. 2.8%). The median time to first IV fluid bolus was faster in the EMS group (36 vs. 57 min). Using Cox LASSO to adjust for potential covariates, time to fluids remained faster for the EMS group (HR 1.26, 95% CI 1.12, 1.42). Time to antibiotics, ICU LOS, 3- or 30-day mortality rates did not differ, yet median hospital LOS was significantly longer in those transported by EMS versus self-transported (6.5 vs. 5.3 days). Conclusions Children with sepsis transported by EMS are a sicker population of children than those self-transported on arrival and had longer hospital stays. EMS transport was associated with earlier in-hospital fluid resuscitation but no difference in time to first antibiotic. Improved prehospital recognition and care is needed to promote adherence to both prehospital and hospital-based sepsis resuscitation benchmarks.

Adolescents with autism spectrum disorder (ASD) without an intellectual disability have daily living skills (DLS) impairments. An initial feasibility pilot of Surviving and Thriving in the Real World (STRW), a group intervention that targets DLS, demonstrated significant improvements. A pilot RCT of STRW was conducted to extend these findings. Twelve adolescents with ASD were randomized to the treatment or waitlist groups. The treatment group had significant DLS improvements on the Vineland Adaptive Behavior Scales, 3(rd) Edition and the DLS goal attainment scale. Four adolescents from the waitlist crossed over and completed STRW. Entire sample analyses with 10 participants demonstrated large DLS gains. Results provide further evidence of the efficacy of STRW for closing the gap between DLS and chronological age.

Background:Infants with single ventricle congenital heart disease demonstrate increasing head growth after bidirectional Glenn; however, the expected growth trajectory has not been well described.Aims:1) We will describe the pattern of head circumference growth in the first year after bidirectional Glenn. 2) We will determine if head growth correlates with motor developmental outcomes approximately 12 months after bidirectional Glenn.Methods:Sixty-nine single ventricle patients underwent bidirectional Glenn between 2010 and 2016. Patients with structural brain abnormalities, grade III–IV intra-ventricular haemorrhage, significant stroke, or obstructive hydrocephalus were excluded. Head circumference and body weight measurements from clinical encounters were evaluated. Motor development was measured with Psychomotor Developmental Index of the Bayley Scales of Infant Development, Third Edition. Generalised estimating equations assessed change in head circumference z-scores from baseline (time of bidirectional Glenn) to 12 months post-surgery.Results:Mean age at bidirectional Glenn was 4.7 (2.3) months and mean head circumference z-score based on population-normed data was −1.13 (95% CI −1.63, −0.63). Head circumference z-score increased to 0.35 (95% CI −0.20, 0.90) (p < 0.0001) 12 months post-surgery. Accelerated head growth, defined as an increase in z-score of >1 from baseline to 12 months post-surgery, was present in 46/69 (66.7%) patients. There was no difference in motor Psychomotor Developmental Index scores between patients with and without accelerated head growth.Conclusion:Single ventricle patients demonstrated a significant increase in head circumference after bidirectional Glenn until 10–12 months post-surgery, at which time growth stabilised. Accelerated head growth did not predict sub-sequent motor developmental outcomes.

PURPOSE: There is a gap in knowledge regarding the use of emergency services by pediatric spina bifida patients. The goal of this study was to describe Emergency Department utilization patterns in this population. METHODS: Through a retrospective observational study, patients with spina bifida who visited the emergency department during a four-year period were identified; medical and demographic information was obtained though the Centers for Disease Control National Spina Bifida Patient Registry. Chief complaints and final diagnoses of visits were classified and related to medical needs of spina bifida to determine the appropriate care level. RESULTS: Among 303 children within the registry, 161 patients (53%) accounted for 579 visits. 70% of visits were for spina bifida-related complaints. Approximately half (51.7%) had a shunt-related chief complaint, although final diagnosis was largely unrelated to the shunt. Admission rate was 39%, higher than institutional baseline, and largely represented by genitourinary (GU) complaints. CONCLUSION: Pediatric patients with spina bifida presenting to a single center emergency department were most likely to present with shunt and urinary concerns; these patients were most likely to be admitted. This potentially suggests that improving outpatient care for bladder management may decrease emergency department use among this population.

Background Smart pumps have been widely adopted but there is limited evidence to understand and support their use in pediatric populations. Our objective was to assess whether smart pumps are effective at reducing medication errors in the neonatal population and determine whether they are a source of alert burden and alert fatigue in an intensive care environment. Methods Using smart pump records, over 370,000 infusion starts for continuously infused medications used in neonates and infants hospitalized in a level IV NICU from 2014 to 2016 were evaluated. Attempts to exceed preset soft and hard maximum limits, percent variance from those limits, and pump alert frequency, patterns and salience were evaluated. Results Smart pumps prevented 160 attempts to exceed the hard maximum limit for doses that were as high as 7–29 times the maximum dose and resulted in the reprogramming or cancellation of 2093 infusions after soft maximum alerts. While the overall alert burden from smart pumps for continuous infusions was not high, alerts clustered around specific patients and medications, and a small portion (17%) of infusions generated the majority of alerts. Soft maximum alerts were often overridden (79%), consistent with low alert salience. Conclusions Smart pumps have the ability to improve neonatal medication safety when compliance with dose error reducing software is high. Numerous attempts to administer high doses were intercepted by dosing alerts. Clustered alerts may generate a high alert burden and limit safety benefit by desensitizing providers to alerts. Future efforts should address ways to improve alert salience.

OBJECTIVES. To compare the percentage and mean age of children with Down syndrome (DS) who underwent polysomnography (PSG) to evaluate for obstructive sleep apnea (OSA) before and after the introduction of the American Academy of Pediatrics guidelines recommending universal screening by age 4 years. STUDY DESIGN. Retrospective cohort study. SETTING. Single tertiary pediatric hospital. METHODS. This study is a review of patients with DS seen in a subspecialty clinic. Children born preguidelines (2000-2006) were compared with children born postguidelines (2007-2012) regarding percentage receiving PSG, age at first PSG, and rate of OSA. RESULTS. We included 766 children with DS; 306 (40%) were born preguidelines. Overall, 61% (n = 467) underwent PSG, with a mean ± SD age of 4.2 ± 2.9 years at first PSG; 341 (44.5%) underwent first PSG by age 4 years. The rate of OSA (obstructive index ≥1 event/hour) among children undergoing first PSG was 78.2%. No difference was seen in the percentage receiving PSG preguidelines (63.4%) versus postguidelines (59.4%, P = .26). The mean age at the time of first PSG was 5.3 ± 3.5 years preguidelines versus 3.4 ± 2.0 years postguidelines (P < .0001). Children in the postguidelines cohort were more likely to undergo first PSG during the ages of 1 through 4 years (67.4% vs 52.1%, P < .0001). There was no difference in rates of OSA between the pre- and postguidelines cohorts (79.8% vs 75.9%, P = .32). CONCLUSIONS. Nearly two-thirds of children with DS (61%) underwent PSG overall, with a significant shift toward completion of PSG at an earlier age after the introduction of the American Academy of Pediatrics guidelines for universal screening for OSA.

Objective: To inform the future development of a pediatric prehospital sepsis tool, we sought to 1) describe the characteristics, emergent care, and outcomes for children with septic shock who are ...

OBJECTIVES. To compare the demographic and clinical characteristics of children with Down syndrome who did and did not receive polysomnography to evaluate for obstructive sleep apnea after publication of the American Academy of Pediatrics’ guidelines recommending universal screening by age 4 years. STUDY DESIGN. Retrospective cohort study. SETTING. Single tertiary pediatric hospital. METHODS. Review was conducted of children with Down syndrome born between 2007 and 2012. Children who obtained polysomnography were compared with children who did not, regarding demographic data, socioeconomic status, and comorbidities. RESULTS. We included 460 children with Down syndrome; 273 (59.3%) received at least 1 polysomnogram, with a median age of 3.6 years (range, 0.1–8.9 years). There was no difference in the distribution of sex, insurance status, or socioeconomic status between children who received polysomnography and those who did not. There was a significant difference in race distribution (P = .0004) and distance from home to the medical center (P < .0001) between groups. Among multiple medical comorbidities, only children with a history of hypothyroidism (P = .003) or pulmonary aspiration (P = .01) were significantly more likely to have obtained polysomnography. CONCLUSIONS. Overall, 60% of children with Down syndrome obtained a polysomnogram. There was no difference between groups by payer status or socioeconomic status. A significant difference in race distribution was noted. Proximity to the medical center and increased medical need appear to be associated with increased likelihood of obtaining a polysomnogram. This study illustrates the need for improvement initiatives to increase the proportion of patients receiving guideline-based screening.

OBJECTIVE To describe the characteristics of treatment decisions to address challenging behaviors in children with autism spectrum disorder (ASD). METHODS Parents of children aged 4 to 15 years with ASD seen in a developmental behavioral pediatric (DBP) clinic completed validated measures to characterize their child's behaviors and their own level of stress. Parents reported their treatment priority before the visit. During the visit, we assessed shared decision making (SDM) using the Observing Patient Involvement (OPTION) scale and alignment of the clinician's treatment plan with the parent's priority. Before and after the visit, parents rated their uncertainty about the treatment plan using the Decisional Conflict Scale (DCS). We calculated descriptive statistics for the measures. RESULTS Fifty-four families participated. Children were a mean (SD) age of 8.8 (3.3) years, and 87% were male. Children had a variety of behavioral challenges, and parents reported high levels of stress. Commonly reported parent treatment priorities were hyperactivity, tantrums, anxiety, and poor social skills. Levels of SDM were low, with a mean (SD) OPTION score of 24.5 (9.7). Parent priorities were addressed in 65% of treatment plans. Approximately 69% of parents had elevated DCS scores before the visit. Although levels of decisional conflict were lower after the visit compared with before the visit (p < 0.03), 46% of parents continued to report high scores on the DCS. CONCLUSION Parents leave DBP visits with feelings of uncertainty about treatment decisions and with treatment plans that do not always address their priorities. SDM interventions hold promise to improve the quality of ASD treatment decisions.

There is a significant lack of evidence guiding our understanding of the needs of families of children who are deaf/hard of hearing (Deaf/HH) with an autism spectrum disorder (ASD). Much of our current knowledge is founded in case report studies with very small numbers of children with the dual diagnosis.The purpose of this study was to gain an understanding of the factors relating to caregiver stress and needs (i.e., supports and interventions) in families of children who are Deaf/HH with ASD.Comparison groups of families of children who were Deaf/HH, families with a hearing child with ASD, and families of children who were Deaf/HH with ASD were administered standardized questionnaires of stress with brief qualitative questionnaires focusing on family-identified needs.Six families of children with the dual diagnosis, four families of children who were Deaf/HH, and three families of children with ASD.Surveys included demographic and support questionnaires, the Parenting Stress Index (PSI), the Pediatric Hearing Impairment Caregiver Experience, and a qualitative questionnaire.Families of children who were Deaf/HH with ASD had a higher median total stress score on the PSI as compared to families of children who were Deaf/HH only (58.5 versus 41.5, respectively; p = 0.02) and higher Child Domain scores (60 versus 43, respectively; p = 0.02), indicating higher levels of stress in families of children with the dual diagnosis. The families of children who were Deaf/HH with ASD reported similar levels of stress as families of children with ASD.Families of children who are Deaf/HH with an ASD experience stress and describe similar needs and priorities as families of hearing children with ASD. This suggests the needs related to having an autism spectrum disorder are of high priority in families of children with the dual diagnosis.

Objective Home oximetry is commonly used to screen for obstructive sleep apnea (OSA) in children; however, normal oxygen desaturation levels by disease severity are not well known. It was our objective to determine if oxygen saturation levels differed by OSA severity category in children and if these differences were similar for preteen children and adolescents. Study Design Retrospective case series of children undergoing polysomnography from September 2011 to July 2015. Setting Tertiary pediatric hospital. Subjects and Methods Six- to 18-year-olds (preteen, 6-12 years old; adolescent, 13-18 years old). Chi-square, Wilcoxon rank sum test, and Kruskal-Wallis testing were used to compare variables between age groups. Results The study included 342 children with a mean age of 11.3 ± 2.4 years (range, 6.5-17.5) and a mean body mass index of 25.6 ± 9.2 kg/m2 (78 ± 29 percentile); 61% were white, 35% were black, and 4% were other or unknown. Of the children, 48% were female, and this was not a significant difference between age groups ( P = .81). Overall, 50% of the children had no OSA, 32% mild, 10% moderate, and 8% severe. When compared with the younger children, the adolescents had a longer sleep time ( P = .014) and a higher mean obstructive apnea-hypopnea index (3.53 ± 5.1 vs 3.03 ± 6.1 events per hour, P = .02). The 3% and 4% oxygen desaturation indices were not significantly different between age groups when accounting for OSA severity. Conclusion Adolescents have longer sleep times and higher obstructive apnea-hypopnea indexes than preteens, but oxygen saturations and desaturation indices were similar. This supports current triage algorithms for children with OSA, as we found no significant age-based differences.

OBJECTIVE Psychotropic medications are frequently prescribed to children with autism spectrum disorder (ASD), but little is known about the prescribing practices of developmental-behavioral pediatricians (DBPs). Our objective was to determine whether clinical site, age, insurance, or comorbidities influenced DBPs prescribing psychotropic medication for children with ASD. METHODS A retrospective analysis was performed using electronic health record data of all patients with ASD seen at 3 academic developmental-behavioral pediatrics (DBP) clinical programs from January 2010 to December 2011. Data included age, diagnoses, primary insurance, and medications prescribed. Factors associated with prescribing psychotropic medication were examined using generalized estimating equations. RESULTS Sites varied in the frequency with which they prescribed psychotropic medication for children with ASD (site 1: 33.1%, site 2: 49.3%, site 3: 4.0%; p < .001). We found that the following factors predicted prescribing of psychotropic medications: comorbidities (odds ratio [OR]: 2.87; 95% confidence interval [CI], 2.58-3.18), age, and primary insurance. However, the impact of insurance depended on age. For 3- to 5-year-old children, those on Medicaid were more likely to be prescribed psychotropic medications than those with private insurance (OR: 1.65; 95% CI, 1.29-2.12). This was particularly true for alpha-2-adrenergic agonists (OR: 2.48; 95% CI, 1.56-3.92) and atypical antipsychotics (OR: 2.57; 95% CI, 1.46-4.55). CONCLUSION There are large variations in prescribing psychotropic medication to children with ASD at 3 academic DBP programs. Further research is needed to understand factors that contribute to higher use of psychotropic medication in young children with Medicaid.

Objective To determine common polysomnographic (PSG) diagnoses for children referred by otolaryngologists. Study Design Retrospective case series with chart review. Setting Single tertiary pediatric hospital (2010-2015). Subjects and Methods Review of the medical records of 1258 patients undergoing PSG by otolaryngology referral. Patients who underwent previous otolaryngologic surgery were excluded. Data distributions were evaluated using means with standard deviations for continuous variables and frequencies with percentages for categorical variables. Results A total of 1258 patients were included; 55.9% were male, 64.5% were Caucasian, 16.6% had Down syndrome, and 48% had public insurance. The median age at the time of PSG was 5.2 years (range = 0.2-18.94). Indications for PSG were sleep-disordered breathing (SDB; 69.4%), restless sleep (12.7%), airway anomalies (7.5%), and laryngomalacia (7.2%). SDB was seen in 73.4%, obstructive sleep apnea (OSA) in 53.2%, OSA + central sleep apnea (CSA) in 4.5%, CSA in 0.9%, and non-OSA snoring in 15%. Other diagnoses included periodic limb movements of sleep (PLMS; 7.4%), hypoventilation (6.8%), and nonapneic hypoxemia (2.6%). SDB was more common in younger children and seen in 91.4% of children

Objectives/Hypothesis Tracheotomy is a common procedure. A reliable method of securing the tracheotomy tube is essential to minimize accidental decannulation. However, skin breakdown has been reported in ∼30% of patients. We sought to evaluate rates of skin-related complications and accidental decannulation with the use of Velcro ties compared to twill ties. Study Design A nonblinded, randomized controlled trial comparing Velcro versus twill ties in patients undergoing tracheotomy between July 1, 2014 and January 31, 2016. Methods Patients ≤21 years of age were recruited and randomized to receive either Velcro or twill ties. The primary outcome measure was skin-related complications. The secondary outcome measure was accidental decannulation. Outcome measures were followed through postoperative day 5. Results Ninety-three patients were eligible, and 63 were enrolled. No patients were withdrawn. Fifty-seven patients were included in the analysis. Twenty-seven (47.4%) received Velcro, and 30 (52.6%) received twill. Five enrolled patients did not undergo tracheotomy (one Velcro, four twill). One was diagnosed with a genetic skin condition after enrollment but prior to undergoing tracheotomy. Patient characteristics were similar between groups. No significant differences were found with respect to skin-related complications (P = .59). Six patients (20%) with twill ties required early tie change compared to two (7.4%) with Velcro ties (P = .5). Two accidental decannulations occurred in the twill group compared to one in the Velcro group (P = 1.0). Conclusions Our study demonstrated no differences in skin-related complications or accidental decannulation between Velcro and twill tracheotomy ties in the immediate postoperative period following tracheotomy. Our study suggests that Velcro ties are a viable alternative to twill ties. Level of Evidence 1b Laryngoscope, 127:1996–2001, 2017

Objectives (1) Describe longitudinal trends in annual prevalence of hospital admission for pediatric acute otitis media (AOM) and complications of AOM (CAOM) since introduction of pneumococcal vaccination in 2000 and (2) describe the longitudinal trend of prevalence of hospital admission for pneumococcal meningitis in children with AOM-related diagnoses in the postvaccination era. Study Design Retrospective analysis of Kids' Inpatient Database from 2000 to 2012. Setting Community, nonrehabilitation hospitals. Subjects and Methods To determine annual prevalence of admission for AOM/CAOM, nationally weighted frequencies of children aged

Objectives/Hypothesis Lingual tonsil hypertrophy is a common cause of persistent airway obstruction in patients with Down syndrome (DS) following adenotonsillectomy (TA however, little is known about the effect of lingual tonsillectomy (LT) on polysomnographic outcomes in these patients. Our objective was to describe changes in sleep-related respiratory outcomes following LT in children with DS and persistent obstructive sleep apnea (OSA) following T&A. Study Design Retrospective case series. Methods We included all children with DS who underwent polysomnography before and after LT at a tertiary care center from 2003 to 2013. Nonparametric analysis of variables was performed. Results Forty patients with DS underwent LT; 21 met inclusion criteria. The mean age at surgery was 9.3 ± 4.3 years and 47.6% were female. The median apnea-hypopnea index (AHI) was 9.1 events/hour (range, 3.8 to 43.8 events/hour) before surgery and 3.7 events/hour (range, 0.5 to 24.4 events/hour) after surgery. The median improvement in overall AHI and the obstructive AHI (oAHI) were 5.1 events/hour (range, −2.9 to 41) and 5.3 events/hour (range, −2.9 to 41), respectively (P 50 mm Hg, central index, and percentage of rapid eye movement sleep were not significantly different. After surgery, the oAHI was

BACKGROUND AND PURPOSE: Infants with congenital diaphragmatic hernia are reported to have evidence of brain MR imaging abnormalities. Our study aimed to identify perinatal clinical factors in infants with congenital diaphragmatic hernia that are associated with evidence of brain injury on MR imaging performed before hospital discharge. MATERIALS AND METHODS: MRIs performed before hospital discharge in infants with congenital diaphragmatic hernia were scored for brain injury by 2 pediatric neuroradiologists. Perinatal variables and clinical variables from the neonatal intensive care unit stay were analyzed for potential associations with brain MR imaging findings. RESULTS: Fifty-three infants with congenital diaphragmatic hernia (31 boys) were included. At least 1 abnormality was seen on MR imaging in 32 infants (60%). The most common MR imaging findings were enlarged extra-axial spaces (36%), intraventricular hemorrhage (23%), ventriculomegaly (19%), white matter injury (17%), and cerebellar hemorrhage (17%). The MR imaging brain injury score was associated with extracorporeal membrane oxygenation ( P = .0001), lack of oral feeding at discharge ( P = .012), use of inotropes ( P = .027), and gastrostomy tube placement before hospital discharge ( P = .024). The MR imaging brain injury score was also associated with a large diaphragmatic defect size ( P = .011). CONCLUSIONS: Most infants with congenital diaphragmatic hernia have at least 1 abnormality identified on MR imaging of the brain performed before discharge. The main predictors of brain injury in this population are a requirement for extracorporeal membrane oxygenation, large diaphragmatic defect size, and lack of oral feeding at discharge.

Objectives/Hypothesis To establish the prevalence of abnormal vestibular test findings in children with enlarged vestibular aqueduct (EVA) and determine if these findings correlate with clinical symptoms, radiographic findings (EVA size and laterality), audiometric findings, and genetic testing in these patients. Study Design Prospective cohort. Methods Patients 3 to 12 years of age with hearing loss and imaging findings consistent with EVA treated at our tertiary care institution were sequentially enrolled from 2009 to 2011. The following six outcome measurements were analyzed: audiometric findings, EVA laterality, temporal bone measurements, genetic testing, vestibular testing (cervical-evoked myogenic potentials, posturography, rotational chair, and calorics), and vestibular symptoms. Results Twenty-seven patients with EVA (mean age 9.2 years, 48% female) were enrolled in and completed the study. Vertigo was reported in six patients. Twenty-four of 27 (89%) had at least one abnormal vestibular test result. Midpoint and operculum size correlated with directional preponderance (P = .042 and P = .032, respectively). Also, high-frequency pure tone average (HFPTA) correlated with unilateral weakness (P = .002). Walking at a later age correlated with abnormal posturography results. There was no correlation between EVA laterality and vestibular test findings. Conclusion We found a high rate of vestibular pathology in children with EVA; however, the prevalence of abnormal vestibular test findings in this patient population was not correlated with vestibular symptoms. Enlarged vestibular aqueduct size, HFPTA, and walking at a later age were correlated with abnormal vestibular test findings. In view of these results, it may be prudent to consider vestibular testing in children with these clinical characteristics. Level of Evidence 2b. Laryngoscope, 126:2344–2350, 2016

Objective The abnormality of intrinsic brain activity in autism spectrum disorders (ASDs) is still inconclusive. Contradictory results have been found pointing towards hyper-activity or hypo-activity in various brain regions. The present research aims to investigate the spatial and spectral signatures of aberrant brain activity in an unprecedented frequency range of 1–2884 Hz at source levels in ASD using newly developed methods. Materials and methods Seven ASD subjects and age- and gender-matched controls were studied using a high-sampling rate magnetoencephalography (MEG) system. Brain activity in delta (1–4 Hz), theta (4–8 Hz), alpha (8–12 Hz), beta (12–30 Hz), low gamma (30–55 Hz), high gamma (65–90 Hz), ripples (90–200 Hz), high-frequency oscillations (HFOs, 200–1000 Hz), and very high-frequency oscillations (VHFOs, 1000–2884 Hz) was volumetrically localized and measured using wavelet and beamforming. Results In comparison to controls, ASD subjects had significantly higher odds of alpha activity (8–12 Hz) in the sensorimotor cortex (mu rhythm), and generally high-frequency activity (90–2884 Hz) in the frontal cortex. The source power of HFOs (200–1000 Hz) in the frontal cortex in ASD was significantly elevated as compared with controls. Conclusion The results suggest that ASD has significantly altered intrinsic brain activity in both low- and high-frequency ranges. Increased intrinsic high-frequency activity in the frontal cortex may play a key role in ASD.

To describe the use of stimulants and alpha-2 agonists (A2As) for the treatment of preschool-aged children with attention-deficit hyperactivity disorder (ADHD) at 2 Developmental-Behavioral Pediatrics Research Network sites.Demographic information, diagnoses, and medications prescribed by developmental-behavioral pediatricians (DBPs) were extracted from the electronic health record for all outpatient visits from January 1, 2010, to December 31, 2011. The subset of visits for children aged 2 to 5 years who had a diagnosis of ADHD was included in this analysis. Multivariable models were constructed to identify factors associated with prescribing stimulants and A2As.Over the 2-year period, 984 children with a diagnosis of ADHD were seen at 1779 visits. Of the 984 children, 342 (34.8%) were prescribed a stimulant, and 243 (24.7%) were prescribed an A2A. Both medications were prescribed at the same visit at least once during the 2-year period for 97 children (9.9%). Alpha-2 agonists were prescribed more often at site 2 than site 1 (OR [odds ratio] = 1.62, p = 0.015). Stimulants were more likely to be prescribed for older preschool-aged children (OR = 1.66, p0.001), and A2As were more likely to be prescribed for younger children (OR = 0.82, p = 0.02). Both stimulants and A2As were more likely to be prescribed to children with ADHD and comorbid conditions.Alpha-2 agonists are commonly used by some DBPs for preschool ADHD. Variation in the use of A2As across sites may indicate a lack of consensus on when to use these medications and suggests a need for comparative effectiveness research to better define the relative benefits and side effects of A2As and stimulants for the treatment of preschool ADHD.

Objectives/Hypothesis Determine the prevalence of high-frequency sensorineural hearing loss (HFSNHL) in our hearing loss population and a diagnostic algorithm for these patients. Study Design Retrospective case series. Methods We identified patients diagnosed with sensorineural hearing loss (SNHL) at our pediatric tertiary care institution from 1981 to 2010. Based on audiometric profiles, these patients were subdivided into those with a flat SNHL configuration and those with HFSNHL. Imaging and genetic testing data and data regarding age at diagnosis, laterality, and risk factors were obtained for both groups. Comparisons were then made between the two groups. Results Of 2,867 patients included in the study, 7.6% had HFSNHL. Age at diagnosis was significantly higher in HFSNHL patients (8.3 years vs. 6.1 years; P

Objectives/Hypothesis With the prevalence of head and neck squamous cell carcinoma (HNSCC) nearly twice as high in veterans (6%) than general populations (3%), the noted problems of long waits and access to care in United States Veterans Affairs (VA) hospitals across the country are pressing. We examined primary outcome measures of timeliness and access to care for our patients with HNSCC assessing a multidisciplinary team approach at our VA hospital. Study Design Retrospective chart review. Methods Our patients newly diagnosed with HNSCC were identified from two 24-month periods: diagnosis before (group 1, 2005–2006) and after (group 2, 2008–2009) implementing our multidisciplinary team in 2007. No significant differences in age (P = .13) or disease stage (P = .18) occurred between groups. Primary and secondary outcomes (i.e., treatment modality, imaging, completion of treatment, survival) were compared. Results Timeliness to care improved for all measures. Improvement was significant for times from consult placed to seen in clinic (27.5–16.5 days; P < 0.0001) and from positive biopsy reported to date of initiating definitive treatment (35–27 days; P = 0.04). Pretreatment consults to various services represented by the multidisciplinary team increased from one to four (P < 0.0001). Two-year mortality was approximately the same between group 1 (33%) and group 2 (36%) (P = 0.035). Five-year mortality was slightly better in group 2 (50%) versus group 1 (61%), although not statistically significant. Conclusion Our veteran population with HNSCC had improved timeliness and access to care with our multidisciplinary approach. Level of Evidence 4. Laryngoscope, 2015

Objectives/Hypothesis Although adenotonsillectomy is accepted as a first-line therapy for pediatric obstructive sleep apnea (OSA), there is currently no consensus regarding optimal methods for identifying the sites of obstruction or treatment of children with persistent disease after surgery. With this in mind, our aim was to systematically review the English-language literature pertaining to these issues. Study Design Systematic review. Methods We searched all indexed years of Pubmed, Cochrane CENTRAL, DynaMed, UpToDate, CINAHL, and Scopus for English-language articles containing original human data, with ≥ 7 participants, all

With a high rate of additional learning needs in children with permanent hearing loss, this study sought to understand their educational and support needs. School information on 62 children with varying degrees of hearing loss attending an urban public school during a 5-year period was analysed to understand types and amounts of services over time. Individualized Education Programs and Multifactored Evaluation Plans reported the number of minutes provided by various providers, the types of services provided, and the goals set for each student. Although all students had a documented hearing loss, the services and intensity of services varied widely based on primary educational label. This is characterized by changes in goals, services, and intensity (minutes provided) from one year to the next if a child experienced an educational label change. Implications are far reaching for educators and administrators regarding determination of educational label for students with comorbid conditions.

Objectives/Hypothesis To examine the ability of the OSA−18 to predict Obstructive Sleep Apnea (OSA) in a racially diverse population when compared to overnight polysomnography (PSG). Study Design Cross-sectional retrospective. Methods Children 2 to 12 years of age diagnosed with OSA who were treated at a tertiary care institution between 2008 and 2013 and had complete PSG and OSA-18 data were included. We performed logistic regression with OSA as the dependent variable and the OSA-18 total symptom score (TSS), age, gender, race, asthma, and body mass index (BMI) as independent variables. Results Seventy-nine children (32 females) were included (mean age 5.2 ± 2.4 years). The positive predictive value (PPV) was greater than 90 for an obstructive apnea–hypopnea index (oAHI) ≥ 1. The PPV and specificity were higher for white than for nonwhite children; however, sensitivity and negative predictive value (NPV) of OSA-18 TSS were low for mild, moderate, and severe OSA regardless of race. Age, race, and BMI were not significantly associated with oAHI. Conclusions This study, conducted in a racially diverse cohort, examined the ability of the OSA-18 to predict OSA when compared to PSG—the gold standard—and found that sensitivity and NPV were extremely low for both white and nonwhite children. This suggests that the OSA-18 is not sufficiently sensitive to detect OSA nor sufficiently specific to determine the absence of OSA. The OSA-18 should be used as a quality-of-life indicator and is not a reliable substitute for PSG. Level of Evidence 4. Laryngoscope, 125:1491–1495, 2015

Few studies have addressed the benefits of cochlear implantation for the small group of patients with bilateral, end-stage Meniere's disease, or unilateral disease with contralateral hearing loss from another cause. Our retrospective study evaluates the effectiveness and post-operative performance in these Meniere's disease patients and discusses these findings relative to other post-lingually deafened adults.Among 456 adults who received cochlear implants, we identified eight (1.7%) patients with Meniere's disease who met clinical indications for implantation and a control group of seven non-Meniere's patients of comparable age and deafness. Data gathered included demographics, auditory and vestibular symptoms pre-implantation, and clinical course after implantation, including audiometric scores (consonant-nucleus-consonant (CNC), hearing-in-noise testing (HINT), and AzBio score) and audiologic evaluations.Comparing the Meniere's and non-Meniere's patients, pre- to post-implant scores averaged 15 and 16%-57 and 76% for CNC; and 4.5 and 40.5%-78 and 81% for AzBio and/or HINT tests, respectively; scores between groups did not statistically differ. Of note, five of eight patients had 0% word recognition scores pre-operatively. Fluctuations in CI performance were seen in five patients with Meniere's disease but not for those without the disease.Audiologic testing found a strong likelihood that CIs did benefit our subset of Meniere's patients with severe to profound sensorineural hearing loss. Fluctuations in hearing sensitivity (perhaps owing to physical changes relative to the implant array with the spiral ganglion neurons during an acute attack or changes in spiral ganglion neurons because of the hydropic state) can often be resolved by subsequent reprogramming.

Objectives/Hypothesis The objective was to describe trends in the annual prevalence of hospitalization for pediatric acute mastoiditis since introduction of the 7-valent pneumococcal vaccine in 2000 and the 13-valent vaccine in 2010. Study Design Cross-sectional retrospective data analysis. Methods The Kids' Inpatient Database from years 2000 to 2012 was analyzed. To determine the annual prevalence of hospitalization for acute mastoiditis, nationally weighted frequencies of hospitalization for children

The purpose of this study was to evaluate pressurised wideband acoustic immittance (WAI) tests in children with Down syndrome (DS) and in typically developing children (TD) for prediction of conductive hearing loss (CHL) and patency of pressure equalising tubes (PETs).Audiologic diagnosis was determined by audiometry in combination with distortion-product otoacoustic emissions, 0.226 kHz tympanometry and otoscopy. WAI results were compared for ears within diagnostic categories (Normal, CHL and PET) and between groups (TD and DS).Children with DS (n = 40; mean age 6.4 years), and TD children (n = 48; mean age 5.1 years) were included.Wideband absorbance was significantly lower at 1-4 kHz in ears with CHL compared to NH for both TD and DS groups. In ears with patent PETs, wideband absorbance and group delay (GD) were larger than in ears without PETs between 0.25 and 1.5 kHz. Wideband absorbance tests were performed similarly for prediction of CHL and patent PETs in TD and DS groups.Wideband absorbance and GD revealed specific patterns in both TD children and those with DS that can assist in detection of the presence of significant CHL, assess the patency of PETs, and provide frequency-specific information in the audiometric range.

Objective The aim of this study is to determine the validity and reliability of neutrophil CD64 in identifying infected infants and to evaluate the impact of this marker on clinical care. Study Design Neutrophil CD64 index was incorporated in 371 infection evaluations in 234 infants (ages 1–293 days) from 2005 to 2009 and the impact of this change on clinical care was evaluated. Results The sensitivity of the neutrophil CD64 assay was 87% in identifying 31 episodes of culture positive sepsis and 83% in identifying 12 infants with ventilator-associated pneumonia. There was no difference in the mean number of antibiotic days in infants with a normal CD64 versus those with a normal complete blood count (CBC) ( p = 0.89), but twofold more infants were identified as “not infected” by CD64 than by CBC. Conclusion CD64 had a high sensitivity for identifying infected infants while also decreasing the number of infants that were exposed to unnecessary antibiotic use.

Objectives Approximately 4% of children who are deaf or hard of hearing have co-occurring autism spectrum disorder (ASD). Making an additional diagnosis of ASD in this population can be challenging, given the complexities of determining whether speech/language and social delays can be accounted for by their hearing loss, or whether these delays might be indicative of a comorbid ASD diagnosis. This exploratory study described a population of 24 children with the dual diagnosis of ASD and hearing loss. Methods Children completed a comprehensive ASD evaluation using standardized autism diagnostic instruments (Autism Diagnostic Observation Schedule, language and psychological testing). Children with permanent hearing loss who had a developmental evaluation between 2001 and 2011 and were diagnosed with an ASD based on the results of that evaluation were included. Information on communication modality, language and cognitive abilities was collected. Results The median age of diagnosis was 14 months (range 1–71) for hearing loss and 66.5 months (range 33–106) for ASD. Only 25% (n = 6) children were diagnosed with ASD ≤48 months of age and 46% by ≤6 years. Twelve (50%) children were diagnosed with ASD, 11 were diagnosed with pervasive developmental disorder not otherwise specified and 1 child had Asperger's. Most (67%) had profound degree of hearing loss. Fourteen (58%) children had received a cochlear implant, while 3 children had no amplification for hearing loss. Nine (38%) of the 24 children used speech as their mode of communication (oral communicators). Conclusions Communication delays in children who are deaf or hard of hearing are a serious matter and should not be assumed to be a direct consequence of the hearing loss. Children who received cochlear implants completed a multidisciplinary evaluation including a developmental pediatrician, which may have provided closer monitoring of speech and language progression and subsequently an earlier ASD diagnosis. Because children who are deaf or hard of hearing with ASD are challenging to evaluate, they may receive a diagnosis of ASD at older ages.

Background No clear consensus yet defines the endpoints for operative learning curves in the transition to minimally invasive endoscopic techniques. This retrospective review of our first 202 patients who underwent endoscopic pituitary resection examines the statistical learning curve related to operative times—a measure of our surgical team's efficiency and complication rate, a reflection of surgical skill and maturity. Methods Retrospective chart review included patient demographic data, tumor type, operative time, complications, and follow-up. During the 5-year study period, surgeries were performed by an otolaryngology-neurosurgery team. Statistical analysis by Pearson's correlation delineated a learning curve for operative time and complications. Results Our learning curve showed comparable plateaus: 120 cases (48% males, 52% females) for operative time (mean, 134 minutes; range, 62–307 minutes) and 100 cases for incidence of cerebrospinal fluid (CSF) leak. The risk of CSF leak declined significantly with the surgeon's increasing experience. Complication rates were as follows: temporary nasal obstruction, 9.9%; CSF leak, 8.4%; postoperative epistaxis, 7%; sinusitis, 4.5%; septal osteomyelitis, 2.4%; postoperative sellar hematoma, 1.5%; anosmia, 0.5%; and septal perforation, 0.5%. The overall CSF leak rate included 5.5% intraoperative and 2.9% postoperative; most cases resolved with a lumbar drain. Four patients (2%) underwent postoperative surgical repair and lumbar drainage. Conclusion Our learning curve–defined endpoints for 2 measures, operative time and complication rates, support improved outcomes for reduced CSF leaks, the most common complication, with increasing operative experience. We will continue to examine the implications related to safety, efficacy, and the need for subspecialization in this minimally invasive surgery.

The aim of this study is 1) to determine the sensitivity and specificity of continuous heart rate characteristics (HRC) monitoring in detection of infections and 2) to evaluate whether HRC monitoring detects infections prior to onset of clinical symptoms in very low birth weight (VLBW) infants. A retrospective cohort study was conducted analyzing HRC scores and episodes of infection for VLBW infants in the Neonatal Intensive Care Unit (NICU) at Cincinnati Children’s Hospital Medical Center from January 2015 through May 2016. HRC scores were acquired using the HRC monitor system and entered into the electronic medical record by bedside staff. Culture-positive and culture-negative clinical infections were recorded. Positive HRC scores were defined as an increase 1 point above the baseline or the first rise above 2. HRC scores within 24 hours and also within the 5-day period before the start of antibiotics for infections were analyzed for sensitivity, specificity, positive predictive value and negative predictive value for detection of neonatal infection. The HRC score increase 1 point above the baseline or the first rise above 2 in the 24 hours before the start of antibiotics for infectious events had a sensitivity of 68.0%, a specificity of 10.8%. Positive predictive value (PPV) and negative predictive value (NPV) were 34.0% and 33.3%, respectively. The PPV and NPV were modestly higher for elevated HRC scores during the 5-day period before infections, 41.1% and 66.7%, respectively. In our single-center retrospective study, elevated HRC scores had limited ability to detect infection. More than half of the positive monitor events were not related to infection. The potential clinical impact of the monitor to detect infection before the onset of clinical symptoms was limited and the risk for unnecessary evaluation and treatment was high.

Objective. To examine the association of intracranial radiographic abnormalities and developmental measures with outcomes in children with congenital symptomatic cytomegalovirus (CMV) and cochlear implants (CI). Design/Methods. It was a retrospective review of 15 children implanted from 2004 to 2010. Preimplant nonverbal intelligence quotient/developmental quotient (IQ/DQ) and head circumference (HC) were obtained. Computed tomography and magnetic resonance imaging of the brain and post-CI audiometry and language assessments were reviewed. Results. Eleven children (73%) had cognitive delay. Most had >1 developmental disability. Median IQ/DQ was 65 (23–90). All had imaging abnormalities. Most imaging abnormalities were in parietal (60%) and temporal (60%) lobes. Children with HC < 5th percentile had poorer median post-CI PTA (38 dB versus 27 dB, ). Periventricular calcifications were associated with lower receptive (, ) and expressive (, ) language. Because IQ/DQ was associated with periventricular calcifications (, ) and small HC (, ), their relationships with language appear partially driven by IQ/DQ. Conclusions. The location of brain abnormalities appears to correlate with worse outcomes after CI. These findings may allow for more accurate counseling of parents regarding anticipated postimplantation performance.

Lingual tonsillar hypertrophy is recognized as a cause of persistent obstructive sleep apnea (OSA) after adenotonsillectomy in children. However, little has been reported regarding the complications, postoperative course and effectiveness of lingual tonsillectomy (LT). Our objective was to review the safety and effectiveness of LT in children.Retrospective review of children undergoing LT from January 2009 to December 2015 at a tertiary children's hospital. Complications, postoperative course and polysomnographic (PSG) outcomes were recorded for all patients.We identified 92 children (mean age = 8.6 years, 50% female) who underwent LT; 43.5% had a syndromic diagnosis. The most common complications were emergency department presentation for bleeding (4.4%) and poor oral intake (3.3%). The readmission rate was 4.4% including 2 children (2.2%) who required operative control of hemorrhage. No children required unplanned reintubation or ICU admission. In children with PSG data (n = 18), the median apnea-hypopnea index (AHI) decreased from 8.5 to 3.8 events/hour (p = 0.022) and the median obstructive AHI (oAHI) decreased from 8.3 to 3.1 events/hour (p = 0.021). In addition, the oxygen saturation nadir increased from 83.8% to 89.0% (p = 0.0007). After surgery the percentage of patients with oAHI5 events/hour increased from 27.8% to 61.1% (p = 0.08).Readmission and bleeding rates after lingual tonsillectomy in children were similar to that seen with tonsillectomy. Polysomnographic data showed that lingual tonsillectomy resulted in a significant reduction of both AHI and oAHI with a postoperative oAHI5 achieved in 61% of patients.

Background Acute chorioamnionitis contributes to premature birth, and is associated with post-birth complications. How chorioamnionitis impacts neonate’s developing immune system has not been well defined. Methods Blood from extremely preterm infants (≤28 weeks gestation) was drawn at the 1st, 2nd, and 4th week of life. Blood was either left unstimulated or stimulated for 4 hours with PMA/ionomycin. mRNA expression of transcription factors in unstimulated cells (RORC, TBET, GATA3, FOXP3) and inflammatory cytokines (IFN-γ, TNF-α, IL-2, IL-4, IL-5, and IL-6) in unstimulated and stimulated cells were analyzed. Data were analyzed based on the diagnosis of chorioamnionitis, funisitis and bronchopulmonary dysplasia (BPD). Results At 1 week of life, exposure to funisitis, but not maternal chorioamnionitis was associated with an increased expression of RORC and RORC/FOXP3 ratio. These increases in RORC and RORC/FOXP3 ration were sustained over the 4 weeks of follow-up. Leukocytes from infants who developed BPD had increased stimulated and unstimulated IL-4 at the 1st week of life, but these increases were not sustained over time. In contrast, infants with mild BPD had a sustained decrease in stimulated IL-2. Conclusion Chorioamnionitis exposure, in particular to funisitis, lead to enhanced Th17-like responses that persist for 4 weeks after birth. Infants who later developed BPD did not exhibit a strikingly distinct immune profile.

Objectives/Hypothesis: Evaluate sexual dysfunction in patients after treatment for head and neck cancer. Study Design: Single-institution cross-sectional study. Methods: Our modified Sexual Adjustment Questionnaire was administered to 42 patients (mean age, 55.1 years) and included seven questions (total score, 7–35). Based on sexual satisfaction ratings, we categorized three groups as unsatisfied (7–16), satisfied (17–25), and very satisfied (26–35). Clinical information was obtained by reviewing medical records. Bivariate analysis tested associations between sexual satisfaction and patient-related factors (e.g., sex, age, site/cancer stage, treatment, time between treatment and survey, partner, alcohol/tobacco consumption). Pearson correlation was used to analyze two continuous variables, and multivariate logistic regression analysis was used to evaluate the independent impact of each factor. Results: All 42 patients rated that head and neck cancer negatively impacted their sexual relationships, including 21 (50%) rating effects as negative or extremely negative. Men reported higher satisfaction scores with sexual function (mean ± standard deviation) than women (19.9 ± 5.0 vs. 16.3 ± 6.5, respectively; P = .06). Respondents with partners reported higher scores than those without partners (19.9 ± 5.3 vs. 14.1 ± 4.4, respectively; P = .01). When the survey was administered (median, 12 months; range, 4–33 months) after the first treatment, mean score was 19; 57% of respondents were sexually satisfied, 31% were unsatisfied, and 12% were very satisfied. Instrument reliability was .82 (Cronbach alpha). Conclusions: Patients who are male and ≤60 years have a higher probability of sexual satisfaction during recovery. Our sexual dysfunction questionnaire will be administered in further prospective studies in patients with head and neck cancer. Laryngoscope, 2012

Aims To determine whether extremely low birth weight (ELBW) infants with bilateral compared to unilateral intraventricular hemorrhage (IVH) have worse neurodevelopmental outcomes at 18–22 months.

We sought to determine the incidence of necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) in surviving extremely low-birth-weight (ELBW

Objective To investigate the outcomes after prolonged empirical antibiotic administration to premature infants in the first week of life, and concluding subsequent late onset sepsis (LOS), necrotizing enterocolitis (NEC), and death. Study design Study infants were ≤32 weeks gestational age and ≤1500 g birth weight who survived free of sepsis and NEC for 7 days. Multivariable logistic regression was conducted to determine independent relationships between prolonged initial empirical antibiotic therapy (≥5 days) and study outcomes that control for birth weight, gestational age, race, prolonged premature rupture of membranes, days on high-frequency ventilation in 7 days, and the amount of breast milk received in the first 14 days of life. Results Of the 365 premature infants who survived 7 days free of sepsis or NEC, 36% received prolonged initial empirical antibiotics, which was independently associated with subsequent outcomes: LOS (OR, 2.45 [95% CI, 1.28-4.67]) and the combination of LOS, NEC, or death (OR, 2.66 [95% CI, 1.12-6.3]). Conclusions Prolonged administration of empirical antibiotics to premature infants with sterile cultures in the first week of life is associated with subsequent severe outcomes. Judicious restriction of antibiotic use should be investigated as a strategy to reduce severe outcomes for premature infants.

To compare continuous positive airway pressure (CPAP) vs. traditional mechanical ventilation (MV) at 24 h of age as predictors of neurodevelopmental (ND) outcomes in extremely low birth weight (ELBW) infants at 18–22 months corrected gestational age (CGA). Infants ≤1000 g birth weight born from January 2000 through December 2006 at two hospitals at the Cincinnati site of the National Institute of Child Health and Human Development Neonatal Research Network were evaluated comparing CPAP (n = 198) vs. MV (n = 109). Primary outcomes included the Bayley Score of Infant Development Version II (BSID-II), presence of deafness, blindness, cerebral palsy, bronchopulmonary dysplasia and death. Ventilatory groups were similar in gender, rates of preterm prolonged rupture of membranes, antepartum hemorrhage, use of antenatal antibiotics, steroids, and tocolytics. Infants receiving CPAP weighed more, were older, were more likely to be non-Caucasian and from a singleton pregnancy. Infants receiving CPAP had better BSID-II scores, and lower rates of BPD and death. After adjusting for acuity differences, ventilatory strategy at 24 h of age independently predicts long-term neurodevelopmental outcome in ELBW infants.

Objectives: Cricotracheal resection (CTR) is an effective treatment for moderate-to-severe laryngotracheal stenosis (LTS) in adults. However, one of the potentially significant sequela of this procedure is postoperative dysphonia and permanent voice alteration. The objective of this study is to characterize voice changes in adult patients with subglottic stenosis who have undergone CTR. Study Design: Retrospective case-series. Methods: Acoustic, aerodynamic, and consensus auditory-perceptual evaluation of voice (CAPE-V) data were reviewed for consecutive adult patients undergoing CTR and perioperative voice evaluation from 2000 to 2010. Results: Sixteen patients (median age 44 years, 94% female) underwent CTR and voice evaluation during the study period. Thirteen patients underwent postoperative evaluation and had a mean overall CAPE-V score of 47.5/100, mean fundamental frequency (F0) of 156.7 Hz, average estimated subglottic pressure of 10.0 cm H2O, and an average airflow of 224 mL/sec. Seven patients underwent pre- and postoperative evaluations and had a significant reduction in mean F0 (206.5 vs. 151.1 Hz, P = .002) and mean F0 for connected speech (194.9 vs. 152.7 Hz, P = .047), but not in median intensity, range, estimated subglottic pressure, or airflow. Median overall CAPE-V scores worsened, but did not reach statistical significance (12 vs. 61.5, P = .28). Seventy-one percent of patients underwent an increase in acoustic signal typing postoperatively. Conclusions: Although CTR is an effective treatment for adult LTS, it results in significant alteration of the adult voice. In particular, CTR decreases the fundamental frequency of connected speech and vowel phonation and changes the acoustic signal type. Patients should be counseled about these likely voice alterations prior to undergoing surgery.

To develop a specific, targeted intervention strategy for reducing preterm birth through use of geographic analysis. We utilized Hamilton County, Ohio vital records and Census data from 2003 to 2006. Spatial scanning statistics allowed determination of the prevalence of preterm birth for any geographical point. Attributable risk calculations demonstrated heterogeneity of risk factors within areas of high or low preterm birth prevalence. Three geographically separate areas with high preterm birth proportions (>16%) had differing primary risk factors for preterm birth, including short interpregnancy interval, previous preterm birth, and low prepregnancy weight, despite similarities in demographics and physical location. Primary risk factors also differed when comparing areas with high and low preterm birth proportions, with diabetes and smoking having primary associations in the lower risk areas. Each local region of high preterm birth proportion as well as those with average or low proportion displayed distinct hierarchies of attributable risk. The heterogeneous distribution of preterm birth proportion within an urban county is complex and requires location specific analysis to develop targeted interventions.

The objectives of this review are to provide the reader with a current and concise review of the data and trends in universal newborn hearing screening. Within a relatively short period of time, the concept of screening all infants for hearing loss at the time of birth has evolved from a nascent process to a truly universal system in most developed countries. As a result, the focus and challenges of universal newborn hearing screening have shifted to topics of developing even more efficient and cost-effective approaches, and potentially melding physiologic hearing screenings with ancillary screening techniques.Enhancement of the universal newborn hearing screening process is likely to be accomplished by implementation of novel tools such as wideband reflectance technologies and intelligent incorporation of screening for common genetic and viral causes of congenital hearing loss.With such a rapidly evolving process, it will be critical for clinicians to understand the benefits and limitations of various newborn hearing screening methodologies in order to determine the most appropriate management of children referred from their universal newborn hearing screening. This will entail a working knowledge of emerging audiologic tools as well as infectious and genetic causes of pediatric hearing loss.

Objectives/Hypothesis: Our objectives were to determine genotype-phenotype correlations in patients with sensorineural hearing loss (SNHL) who undergo testing for GJB2 mutations and to examine the relationship of temporal bone anomalies seen on computed tomography (CT) and GJB2 mutations. Study design: We conducted a retrospective review of all children diagnosed with SNHL and who underwent GJB2 testing from 1997 to 2006. Results: Of 840 patients, 146 (17.4%) had mutations. Seventy-six (9.1%) had biallelic GJB2 mutations and 70 (8.3%) had heterozygous mutations. When biallelic mutations were categorized as missense or nonsense mutations, the presence of at least one missense mutation was associated with mild or moderate SNHL. Biallelic nonsense mutations were associated with severe to profound SNHL. Among patients with GJB2 mutations, those with heterozygous mutations (n = 14 [20%]) had a higher rate of asymmetric SNHL loss than those with biallelic mutations (n = 6 [7.9%], P = .03). Those with heterozygous mutations were more likely to experience progression than were those with biallelic mutations, though this difference was only marginally significant (26.5% vs. 12.3%, respectively; P = .06). Patients who were wild type for GJB2 were more likely to have an enlarged vestibular aqueduct (EVA) than were those with biallelic and heterozygous mutations (29% vs. 11.9%, respectively; P = .004). Compared to patients who were wild type, those with biallelic mutations had a significantly lower rate of EVA. Conclusions: This is the largest single-institution study of pediatric patients with GJB2 mutations and SNHL. The functional consequences of GJB2 mutations correlated with the degree of hearing loss. Patients with M34T mutations and/or mild SNHL had a low risk of progression. Temporal bone anomalies were uncommon in patients with GJB2 mutations. Laryngoscope, 119:554–558, 2009

The objective of this study was to investigate trends in the referral process among pediatric cochlear implant candidates. Medical and audiologic charts between 2003 and 2005 were reviewed, and children five years and younger with moderately-severe or worse sensorineural hearing loss were included. Of the 105 audiograms meeting the inclusion criteria, 69% were referred for a cochlear implant, and 52% were considered as definite candidates for an implant by audiologists with expertise in cochlear implant technology. Children referred for an implant, compared to children who were not referred, were more likely to have married parents (91% vs. 70%, p=0.02) and more likely to have private insurance (56% vs. 29%, p=0.02). Multivariable regression results were consistent with the unadjusted findings regarding marital status, but not insurance status. Children with sensorineural hearing loss are inconsistently referred to cochlear implant teams despite similar audiologic findings. To reach the Healthy People 2010 goals, this disparity should be addressed. A further understanding of the population of children not referred is important in diminishing inconsistencies and understanding barriers to care.

Objectives: We sought to understand auditory skills outcomes in young children with cochlear implants and developmental delay. Methods: Children who received cochlear implants at less than 36 months of age were identified via chart review. Their postimplant auditory skills outcomes were measured with the Auditory Skills Checklist. Results: Of 35 children who received cochlear implants before the age of 36 months, 14 children (40%) had additional disabilities or some form of developmental delay. The 12-month postimplant data indicated progress in all groups of children. Children with additional disabilities had the same rate of auditory skills progress as children with no additional disabilities (β = 9.3 versus 9.3; p = 0.5). However, the children with additional disabilities tended to start at a lower baseline skills set (approximately 6 points lower) on the Auditory Skills Checklist. For children with average developmental quotients (at least 80), the rate of progress was twice that of children with a developmental quotient of less than 80, irrespective of a developmental disability (β = 9.9 versus 4.8; p = 0.03). Children with a developmental quotient of less than 80 were less likely to gain skills in discrimination and identification after the first postimplant year. Conclusions: Children with additional disabilities make progress in auditory skills, but may not develop higher auditory skills of identification and comprehension within the first 6 months after implantation. Categorizing children according to a cognitive developmental quotient may provide more predictive ability than does categorizing them by disability type.

OBJECTIVE. Because most internationally adopted children come from areas of high tuberculosis prevalence, an initial tuberculin skin test is recommended after arrival to the United States. We evaluated whether repeat testing of children ≥3 months after arrival to the United States would identify additional children with latent tuberculosis infection.METHODS. Internationally adopted children who were seen at our International Adoption Center and had a tuberculin skin test within 2 months of arrival to the United States were eligible for the study. Children not diagnosed with tuberculosis with initial testing were retested at least 3 months later. The prevalence of tuberculosis on arrival and after repeat testing was determined, and potential risk factors for infection were examined.RESULTS. Of the 527 internationally adopted children with an initial tuberculin skin test completed, 111 (21%) had evidence of latent tuberculosis infection. Repeat tuberculosis testing was complete for 191 internationally adopted children (46.9% of those who had an initially negative tuberculin skin test). Latent tuberculosis infection was found in 20% of those who were retested. No children were found to have active tuberculosis disease. Children with an initially positive tuberculin skin test result had slightly higher weight-for-age z scores at their initial clinic visit, whereas those whose tuberculin skin test result was positive after repeat testing had slightly lower weight-for-age z scores. A strong correlation between BCG immunization and tuberculin skin test result was observed.CONCLUSIONS. Latent tuberculosis infection is common in internationally adopted children. A high proportion of internationally adopted children had an initially false-negative tuberculin skin test. Repeat tuberculosis testing of all internationally adopted children with an initially negative tuberculin skin test should be the standard of care for identifying tuberculosis infection and preventing tuberculosis disease in this high-risk population.

Objectives: To evaluate rates of long-term survival in a prospective, longitudinal, closed HIV cohort in Africa between 1986 and 2006. Methods: A total of 548 HIV-infected Rwandan women were recruited from prenatal clinics in Kigali and followed at 3-6 month intervals to February 2006. Overall, 401 women (73%) were HIV positive at initial cross-sectional testing in 1986 (seroprevalent cohort) and 147 women (27%) were initially HIV negative but seroconverted during follow-up from 1986 to 1993 (seroincident cohort). Kaplan-Meier survival methods were used to calculate survival times censored in mid-2003. Results: In February 2006, 109 women (20%) remained alive in the cohort. Time to 50% non-genocide mortality was 11.9 years among seroincident women and 8.9 years among seroprevalent women. Smoothed mortality rates increased with duration of follow-up to a peak of 0.12 deaths per person-year at 9.5 years of follow-up but subsequently declined. After 15 years of follow-up (pre-HAART introduction), the survival probability was 36% for seroincident women and 26% for seroprevalent women. Most survivors had virological and immunological evidence of disease progression. The median CD4 cell count of survivors declined from 447 cells/μl in 1998 to 268 cells/μl in 2003. Among survivors, 57 women (52%) met treatment criteria and initiated antiretroviral treatment by 2006. Conclusion: Although median survival times in this cohort were similar to those observed in high-income countries, the rates of long-term survival after 15 years of follow-up were higher than expected. A levelling off of mortality rates during the late stages of follow-up may explain this high rate of long-term survival.

Objectives:The Auditory Skills Checklist© (ASC) was developed to address the need for tools to evaluate functional auditory skill progress in very young children with sensorineural hearing loss. We describe the development, validation, and utility of the ASC for use in young children with cochlear implants.Methods:Using the ASC, we measured auditory skills in 37 subjects who received cochlear implants at no more than 36 months of age. Repeated measures analysis was conducted to determine expected auditory skill development after implantation. Interrater reliability was tested on a small subset. The ASC was compared to the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) to determine its validity in measuring functional auditory skills.Results:The ASC had excellent internal consistency (Cronbach's alpha, 0.98) and interrater reliability (intraclass correlation coefficient, 0.99), and was highly correlated with the IT-MAIS (r = 0.90). According to the repeated measures analysis, children who received a cochlear implant at 36 months of age or earlier were expected to increase their ASC score by 8 points every 3 months (beta coefficient, 8.3; p < .001).Conclusions:The ASC is a clinically relevant and easily administered tool for assessing the functional auditory skills of young children with a cochlear implant. By assessing auditory skill development over time with the ASC, we can better realize expectations for a particular child based on his or her age, hearing loss level, and management strategies in place.

Objective Inner ear inflammation triggered by CMV infection may play a role in CMV-related auditory pathogenesis. The purpose of the study was to determine if a virally encoded macrophage inflammatory protein played a role in CMV-related hearing loss. Design Mutagenesis was performed with deletion of a guinea pig CMV macrophage inflammatory protein. Intracochlear inoculations were performed on three groups of animals (n = 18). Group 1 received sterile viral media, Group 2 received wild-type CMV virus, and Group 3 received “knockout” (KO) virus with a deleted immunomodulation gene. Baseline and postinoculation ABRs were obtained. ELISA and PCR were performed and temporal bones examined. Subjects Eighteen guinea pigs. Results The KO group had significantly better hearing than the WT group. There were no significant differences between the KO and sham groups. The WT group had significant hearing loss at all frequencies. Inflammation and fibrosis were noted in the WT temporal bones only. Conclusions Virally encoded macrophage inflammatory proteins appear to play a significant role in CMV-related hearing loss.