PURPOSE:Early hearing detection and intervention (EHDI) programs using audiometric screening techniques alone have a limited ability to detect noncongenital childhood permanent hearing loss (PHL). In 2019, Ontario launched universal newborn screening (NBS) for PHL risk factors, including congenital cytomegalovirus and 22 common variants in GJB2 and SLC26A4. Here, we describe our experience in screening for genetic risk factors. METHODS:Ontario newborns who participated in universal newborn hearing screening (UNHS) were offered risk factor screening using dried blood spots (DBS) collected for conventional newborn screening. The screening was conducted using a custom MassArray assay, and positive results were confirmed by Sanger sequencing or polymerase chain reaction. Diagnostic audiological assessments were performed for all screen-positive infants. RESULTS:Of the 412,424 infants screened, 93 had 2 variants in GJB2 or SLC26A4. Of these, 72 had confirmed PHL, 20 had normal hearing, and 1 declined follow-up. Thirteen infants with PHL (1 in 31,724; 11.8% of screen positives) were not identified through audiometric testing as they passed (3) or missed (10) the screening. Importantly, among infants who ultimately received cochlear implants, the detection of genetic etiology through NBS led to an accelerated time to diagnosis, assessment, and intervention. CONCLUSION:Genetic screening has strengthened UNHS and care for infants with or at risk of PHL in Ontario. This study is a step toward the broader inclusion of genomic testing in NBS.

ImportanceDetection of congenital cytomegalovirus (cCMV) infection has previously relied on targeted screening programs or clinical recognition; however, these approaches miss most cCMV-infected newborns and fail to identify those infants who are asymptomatic at birth but at risk for late-onset sensorineural hearing loss.ObjectiveTo determine the feasibility of using routinely collected newborn dried blood spots (DBS) in a population-based cCMV screen to identify infants at risk for hearing loss and describe outcomes of infants screened.Design, Setting, and ParticipantsThis diagnostic study of a population-based screening program in Ontario, Canada, took place from July 29, 2019, to July 31, 2023. All newborns with a DBS sample collected as part of routine care were screened using polymerase chain reaction (PCR) analysis for cCMV as a risk factor for hearing loss. Infants with positive DBS PCR results for cCMV were referred for confirmation of infection by urine PCR (the gold standard), as well as complete medical and audiologic assessments for sequelae of cCMV infection. Infants with possible or confirmed symptomatic cCMV were referred to pediatric infectious disease specialists for evaluation for potential treatment with valganciclovir.ExposureDetection of cCMV by polymerase chain reaction assay on a newborn DBS.Main Outcomes and MeasuresNumber of infants with positive screening results successfully retrieved and confirmed to have cCMV and the timeliness of retrieval and symptomatic evaluation.ResultsOf 565 987 infants born in the screening period, 551 034 (97.4%) received cCMV screening on the DBS (45.7% female, 54.3% male). Of these infants, 689 (0.13%) screened positive for cCMV; 601 (87.2%) had cCMV infection confirmed and a complete assessment of sequelae of their congenital infection. Ninety-six infants with completed assessments (16.0%) were deemed to have cCMV symptoms, and 63 of these (65.6%) began valganciclovir treatment. Sensorineural hearing loss was confirmed in 34 of 96 infants (35.4%).Conclusions and RelevanceThis program found acceptable and feasible implementation of a population-based screening program using routinely collected DBS samples, suggesting that it may serve as a template for jurisdictions considering universal cCMV screening. The program had a much lower than expected prevalence of cCMV-positive screens but still identified many children who would otherwise not have been diagnosed and who would benefit from ongoing audiologic surveillance.

BACKGROUND:Adenotonsillectomy and tonsillectomy (referred to as tonsillectomy hereafter) are common pediatric surgeries. Postoperative complications include hemorrhage requiring surgery (2 to 3% of cases) and pain. Although nonsteroidal anti-inflammatory drugs are commonly administered for postsurgical pain, controversy exists regarding bleeding risk with cyclooxygenase-1 inhibition and associated platelet dysfunction. Preliminary evidence suggests selective cyclooxygenase-2 inhibitors, for example celecoxib, effectively manage pain without adverse events including bleeding. Given the paucity of data for routine celecoxib use after tonsillectomy, this study was designed to investigate the association between postoperative celecoxib prescription and post-tonsillectomy hemorrhage requiring surgery using chart-review data from the Children's Hospital of Eastern Ontario. METHODS:After ethics approval, a retrospective single-center observational cohort study was performed in children less than 18 yr of age undergoing tonsillectomy from January 2007 to December 2017. Cases of adenoidectomy alone were excluded due to low bleed rates. The primary outcome was the proportion of patients with post-tonsillectomy hemorrhage requiring surgery. The association between a celecoxib prescription and post-tonsillectomy hemorrhage requiring surgery was estimated using inverse probability of treatment weighting based on propensity scores and using generalized estimating equations to accommodate clustering by surgeon. RESULTS:An initial patient cohort of 6,468 was identified, and 5,846 children with complete data were included in analyses. Median (interquartile range) age was 6.10 (4.40, 9.00) yr, and 46% were female. In the cohort, 28.1% (n = 1,644) were prescribed celecoxib. Among the 4,996 tonsillectomy patients, 1.7% (n = 86) experienced post-tonsillectomy hemorrhage requiring surgery. The proportion with post-tonsillectomy hemorrhage requiring surgery among patients who had a tonsillectomy and were or were not prescribed celecoxib was 1.94% (30 of 1,548; 95% CI, 1.36 to 2.75) and 1.62% (56 of 3,448; 95% CI, 1.25 to 2.10), respectively. Modeling did not identify an association between celecoxib prescription and increased odds of post-tonsillectomy hemorrhage requiring surgery (odds ratio = 1.4; 95% CI, 0.85 to 2.31; P = 0.20). CONCLUSIONS:Celecoxib does not significantly increase the odds of post-tonsillectomy hemorrhage requiring surgery, after adjusting for covariates. This large pediatric cohort study of celecoxib administered after tonsillectomy provides compelling evidence for safety but requires confirmation with a multisite randomized controlled trial. EDITOR’S PERSPECTIVE:

Purpose To evaluate the progress and challenges of a hearing screening program as well as review the incidence of pediatric hearing loss in grade school children participating in this program. Methods Medical students from the University of Ottawa established iHear, a grade school hearing assessment program that uses novel tablet audiometry. Over 3 years, children in grades 1 and 2 were assessed and those found to have abnormal results on iHear assessment were then referred to audiology for formal testing, and to otolaryngology if needed. Results From 2014 to 2017, 753 children aged 5–9 years old were assessed for hearing loss. Mean age of participants was 6.7 years, 51.9% of whom were female. Of the children assessed, 86 (11.4%) had abnormal results and 6 (0.8%) had inconsistent results, necessitating 92 referrals for assessment by a professional audiologist. Of the 65 participants who completed secondary audiologic assessment, 54 (83.1%) were normal and 11 (16.9%) had a definitive hearing loss or abnormal tympanometry. A total of 32 children were lost to follow-up. A total of 118 medical students were involved in the iHear program. Conclusions Hearing loss in grade school populations continues to go undetected across Canada. Programs such as iHear demonstrate that gaps in the provision of hearing assessment can be filled effectively by medical students equipped with tablet audiometry. Medical student exposure to audiology and otolaryngology increased through the iHear program. Graphical Abstract

Background Piriform sinus fistulas occur due to developmental abnormalities of the third and fourth branchial arches, and almost always occur unilaterally. They generally present as recurrent abscesses in the anterior-inferior neck, with concurrent thyroiditis. They have conventionally been managed with complete removal of the sinus tract, and thyroidectomy if required; however, endoscopic approaches have been increasingly favored. Herein we describe a case of bilateral piriform sinus fistulas, and present a review of the literature concerning their endoscopic management. Case presentation Our patient was determined to have bilateral piriform sinus fistulas based on computer tomography, magnetic resonance imaging and microlaryngoscopy. We performed electrocauterization of the proximal fistula tracts, followed by injection of fibrin sealent. Our patient has not had a recurrence in the ten months since his procedure. There were no complications. Twenty-three articles describing an endoscopic approach to these fistulas were identified through PubMed, and a search through the references of related articles was completed. Conclusion Of one hundred and ninety-five patient cases we reviewed, an endoscopic procedure success rate of 82% and complication rate of 5.6% was determined. Piriform sinus fistulas that occur bilaterally are a rare congenital abnormality of the neck. Endoscopic approaches are an acceptable alternative option to open procedures, with similar success and a lower rate of complications.

AIMSTo examine the effect of a pre-operative DVD on parents' knowledge, participation and anxiety and on children's distress, pain, analgesic requirements and length of recovery after same-day surgery.BACKGROUNDVery few parents are adequately prepared to participate in their child's care during a same-day surgery. An educational DVD was developed to educate parents on how to actively support their child in the recovery room.DESIGNSingle-blind, post-test randomized controlled trial. Study is registered at ClinicalTrials.gov NCT02766452.METHODSBetween September 2011-September 2012, 123 parent-child dyads where the child underwent an ENT or dental same-day surgery were recruited in a Canadian paediatric hospital. Dyads were randomly assigned to either the intervention (DVD and standard preparation) or control group (standard preparation). Parents and children were videotaped in the recovery room where parental participation and anxiety and children's distress were measured. Data on parents' knowledge, children's postoperative pain, analgesic requirements and length of recovery were measured. Independent and paired t-tests, chi square and repeated measures anova were used to analyse the data.RESULTSParents in the intervention group gained greater knowledge of and used more positive reinforcement and distraction and relaxation methods than those in the control group. Children's postoperative pain in the day-care surgery unit was significantly lower among the intervention group compared with the control group.CONCLUSIONA pre-operative DVD can increase parents' participation in the recovery room and decrease children's postoperative pain.

BACKGROUND:Otitis media with effusion (OME) causes significant morbidity in children, but the causes of OME and methods for prevention are unclear. To look for potential infectious etiologies, we performed a pilot study using multiple-target real-time polymerase chain reaction (qPCR) for 27 infectious agents, including nine bacterial organisms and 18 respiratory viruses in middle ear fluids (MEFs) from children with OME. QPCR was also performed for the 13 Streptococcus pneumoniae serotypes contained in the current vaccine.RESULTS:Forty-eight MEF samples were obtained and qPCR detected bacterial nucleic acid (NA) in 39/48 (81%) and viral NA in 7/48 (15%). Alloiococcus otitidis and S. pneumoniae were both detected in 15/48 (31%) MEFs, followed by M. catarrhalis in 14/48 (29%), H. influenzae in 5/48 (10%) and M. pneumoniae in 4/48 (8%). Rhinoviruses were most common virus type detected, found in 4/48 (8%) MEFs. Serotypes included in the current 13-serotype vaccine were detected in only 3/15 (20%) S. pneumoniae qPCR-positive MEFs.CONCLUSIONS:Bacteria may play an important role in OME, since over 80% of MEFs contained bacterial NA. Further research into the role of A. otitidis in OME will be helpful. Serotypes of S. pneumoniae not included in the current 13-serotype vaccine may be involved in OME. Larger studies of OME S. pneumoniae serotypes are needed to help determine which additional serotypes should be included in future vaccine formulations in order to try to prevent OME.

Pediatric adenotonsillectomy (A&T) is associated with prolonged pain and functional limitation. Celecoxib is an effective analgesic in adult surgery patients; however, its analgesic efficacy on pain and functional recovery in pediatric A&T patients is unknown.

A male infant was delivered at 28 weeks and one day gestation by emergency Caesarean section to a gravida 1 mother with pre-eclampsia and HELLP (hemolysis, elevated liver enzymes and low platelets) syndrome. In early life, he had symptoms of respiratory distress, presumably due to respiratory distress syndrome, and was promptly intubated with surfactant administered. Other comorbidities included hyperbilirubinemia, which was treated with a short course of phototherapy; ventilator-associated pneumonia, treated with cloxacillin and gentamicin; and a small patent ductus arteriosus, treated with indomethacin. All of these issues resolved with treatment. Subsequently, the infant underwent several unsuccessful trials of extubation but, on day of life 19, was extubated to noninvasive ventilation. He was started on caffeine to treat apnea of prematurity. The baby appeared well; however, there was a red, demarcated, mammillated oval plaque on the anterior neck. According to the parents, the lesion had increased in size over the previous days to weeks. As noninvasive support was weaned, biphasic stridor was apparent. A bedside flexible nasopharyngolaryngoscopy performed by otolaryngology ruled out a supraglottic cause. To exclude a subglottic lesion, a bronchoscopy was performed in the operating room and showed a deformation of the trachea below the vocal cords, with a lesion in the trachea (Figure 1). Figure 1) Bronchoscopic view of the subglottic region. The arrow identifies the deformation on the posterior tracheal wall

Objectives: To review the outcomes of supraglottoplasty performed in children with severe laryngomalacia at a pediatric university teaching hospital and to determine if these outcomes are influenced by gender, age at time of supraglottoplasty, or medical comorbidities.Methods: Retrospective chart review of children who underwent supraglottoplasty for severe laryngomalacia between 2001 and 2010 at the Children's Hospital of Eastern Ontario in Ottawa, Canada. Statistical significance was obtained using 2-tailed Fisher's exact test. The outcome measures were resolution of laryngomalacia symptoms or persistence of laryngomalacia symptoms with or without additional surgery.Results: Among 26 post-primary supraglottoplasty patients, 46.2% had resolution and 53.8% had persistence, including 19.2% with partial improvement and 34.6% requiring revision supraglottoplasty and/or tracheostomy. Resolution was present in 35.7% of males and 58.3% of females (p = 0.43); in 33.3% of all patients <= 2 months old and 52.9% of all patients >2 months old (p = 0.43); and in 50.0% of non-comorbid patients <= 2 months old and 80.0% of non-comorbid patients >2 months old (p = 0.52). 71.4% of persistence cases were found in comorbid patients. 83.3% of comorbid patients had persistence, compared to 28.6% of non-comorbid patients (p = 0.008). Of the 3 patients with overlapping comorbid categories, 2 required at least 2 revision supraglottoplasties and ultimately required a tracheostomy.Conclusions: Persistence of laryngomalacia symptoms post-primary supraglottoplasty was not associated with gender or age at time of surgery, and was more commonly found in children with comorbidities. Patients with coexisting medical conditions appear to require more aggressive surgical management, beyond one revision supraglottoplasty. (C) 2013 Published by Elsevier Ireland Ltd.

Preoperative oral acetaminophen (30 mg·kg-1) was compared with topical 2% lidocaine ear drops for postoperative analgesia following bilateral myringotomy and tube placement (BMT) in children.

La lidocaïne topique et l'acétaminophène orale en dose de 30 mg·kg procurent une analgésie similaire à la suite d'une MBA.

Foreign bodies in the oropharynx of the pediatric population are a well-known subject within the medical field. This report deals with two cases of a very unusual foreign body: an umbrella. Case 1 A 5-year-old boy was brought to the emergency room at the Children's Hospital of Eastern Ontario (CHEO) with the handle of an umbrella protruding through his mouth. The child had fallen face first on the handle while at school. On physical examination, the child was quiet and calm. His vital signs were all stable with oxygen saturation 100% on room air. The umbrella was protruding through his mouth., with minimal bleeding around its handle (Figure 1). Plain film radiography was not helpful because the intraoral part was radiolucent. The patient was rushed to the operating room. Under intravenous sedation, the umbrella was manipulated out of the child's mouth, and then he was endotracheally intubated. A thorough examination under anesthesia and exploration of the oropharynx revealed a 4 cm laceration in the right floor of the mouth, sparing the submandibular duct. There were no other observed injuries. The laceration was repaired primarily. The patient was discharged home on the same day once oral intake was established and covered with oral antibiotics. No long-term sequelae resulted.

Chronic sinusitis, otitis media with effusion, and upper respiratory tract infections are commonly found in patients with Down syndrome. These diseases are generally felt to be secondary to depressed immune function and altered craniofacial dimensions. Recently, a cilia ultrastructure abnormality was found in a child with Down syndrome. This study is the first to be carried out to determine if cilia ultrastructure abnormalities are prevalent in the population with Down syndrome. Four of 10 patients had documented cilia abnormalities, but these were present in the background of normal cilia, suggesting that they were the result rather than the cause of chronic sinusitis. Similarly, nasal epithelium metaplasia was detected in 50% of the patients. Chronic sinusitis, otitis media with effusion, and recurrent upper respiratory tract infections in children with Down syndrome cannot generally be attributed to primary cilia ultrastructure abnormalities.

Objective: Adequate biopsy specimens that clearly demonstrate cilia, and therefore enable the determination of the presence or absence of primary cilia dyskinesia, may be difficult to obtain. This study is an attempt to identify the optimal sampling technique to best examine respiratory tract cilia.Design: A prospective comparison of the four sampling techniques was carried out: nasal brushing, nasal biopsy, bronchial brushing, and tracheal biopsy.Setting: Tertiary care pediatric hospital: Children's Hospital of Eastern Ontario.Methods: Ten consecutive patients booked for bronchoscopy and tracheal biopsy underwent all four procedures. Specimens were examined under light microscopy for an assessment of quality.Results: The nasal brushing and tracheal biopsy specimens provide superior quality (p = .22); however, nasal brushing is more cost efficient. Nasal biopsy samples frequently are metaplastic and therefore are inferior to nasal brushing samples (p = .02).Conclusion: With equal efficiency demonstrated, the reduction in potential morbidity and health care costs suggests nasal brushings to be the optimal initial investigation for primary ciliary dyskinesia.