Congenital Cytomegalovirus Testing Outcomes From the ValEAR Trial.

OBJECTIVE: To determine the positivity rate of congenital cytomegalovirus (cCMV) testing among universal, hearing-targeted CMV testing (HT-cCMV) and delayed targeted dried blood spot (DBS) testing newborn screening programs, and to examine the characteristics of successful HT-cCMV testing programs. STUDY DESIGN: Prospective survey of birth hospitals performing early CMV testing. SETTING: Multiple institutions. METHODS: Birth hospitals participating in the National Institutes of Health ValEAR clinical trial were surveyed to determine the rates of cCMV positivity associated with 3 different testing approaches: universal testing, HT-cCMV, and DBS testing. A mixed methods model was created to determine associations between successful HT-cCMV screening and specific screening protocols. RESULTS: Eighty-two birth hospitals were surveyed from February 2019 to December 2021. Seven thousand six hundred seventy infants underwent universal screening, 9017 infants HT-cCMV and 535 infants delayed DBS testing. The rates of cCMV positivity were 0.5%, 1.5%, and 7.3%, respectively. The positivity rate for universal CMV screening was less during the COVID-19 pandemic than that reported prior to the pandemic. There were no statistically significant drops in positivity for any approach during the pandemic. For HT-cCMV testing, unique order sets and rigorous posttesting protocols were associated with successful screening programs. CONCLUSION: Rates of cCMV positivity differed among the 3 approaches. The rates are comparable to cohort studies reported in the literature. Universal CMV prevalence decreased during the pandemic but not significantly. Institutions with specific order set for CMV testing where the primary care physician orders the test and the nurse facilitates the testing process exhibited higher rates of HT-cCMV testing.

Opioid stewardship and perioperative management of pediatric tympanoplasty.

OBJECTIVE: To provide insight into the intraoperative management, admission course, pain management, and graft success of microscope- and endoscope-assisted tympanoplasty. STUDY DESIGN: Retrospective Chart Review. METHODS: This study included children 18 years and younger who underwent ambulatory tympanoplasty at a tertiary pediatric hospital between January 2018 and December 2020. Medical records were reviewed and information about intraoperative factors, surgical approach, laterality, complications, and post-operative perforation closure success rates was collected. Multivariate analysis was performed to compare and contrast the two surgical approaches. RESULTS: The review included 321 pediatric patients who underwent a tympanoplasty. Endoscopic tympanoplasty accounted for 17.4%, while microscopic tympanoplasty accounted for 82.6%. In both approaches, the rate of intraoperative complications, postoperative complications, audiological improvements, and perforation closure success rates were statistically similar. However, patients who underwent endoscopic tympanoplasty were 3.96 times less likely to require opioids in the post-anesthesia care unit (PACU) and had a shorter post-operative admission length. This pattern emerged regardless of the type of graft used. Obtaining an autograft was not associated with a higher opioid requirement in the PACU. CONCLUSION: While both approaches are viable, our findings demonstrate the reduced need for opioids with similar success rates following an endoscopic tympanoplasty. Ultimately, the trade-off for the minimally invasive endoscopic approach appears to be a less painful experience for the child while promoting clinically appropriate opioid stewardship in the perioperative setting.

Congenital Cytomegalovirus Infection and Hearing Outcomes in Twins: A Systematic Review of Reported Cases.

OBJECTIVE: Congenital cytomegalovirus infection is the leading nonhereditary cause of pediatric sensorineural hearing loss. This systematic review evaluated infection concordance and comparative hearing abilities in twins/multiple births to model infection patterns. DATABASES REVIEWED: PubMed, Embase, Web of Science, and Google Scholar. METHODS: Studies that reported hearing outcomes of congenital cytomegalovirus infection in at least one multiple birth were eligible. Concordant infections (both twins) and discordant infections (single twin) were included. Multiple reviewers performed data extraction and quality assessment. Analyses involved relative risk of infection concordance by zygosity and chorionicity and odds of hearing loss by infection concordance. Hearing outcomes were compared between siblings. RESULTS: Of 247 studies screened, 31 were included (74.2% high quality). The review captured 40 eligible multiple births. Among infected patients, 42.9% (95% confidence interval, 31.2-55.2%) demonstrated hearing loss. All uninfected twins had normal hearing. Most infections were concordant, and infected patients experienced 4.11 (1.18-14.36) times greater odds of hearing loss if their twin was also infected ( p = .02). Yet siblings' hearing outcomes diverged in over 40% of concordant cases. If either twin is infected, infection risk in the second twin is 3.25 (1.83-5.79) times greater in monozygotic than dizygotic twins and 2.50 (1.61-3.88) times greater in monochorionic than dichorionic twins (both p < .001). We describe a case from our practice. CONCLUSION: Congenital cytomegalovirus infection patterns and hearing outcomes can vary widely even within a shared fetal and postnatal environment. Suspected infection in a twin indicates that both should receive testing and continued monitoring for late-onset sequelae.

Opioid Prescribing Trends After Major Pediatric Ear Surgery: A 12-Year Analysis.

OBJECTIVE: Postoperative opioid prescriptions tend to exceed children's analgesic needs, but awareness of the opioid epidemic may have driven changes in prescribing behaviors. This study evaluated opioid prescribing patterns after major pediatric ear surgery. METHODS: This study reviewed all cases of tympanoplasty, tympanomastoidectomy, mastoidectomy, cochlear implantation, otoplasty, and aural atresia repair at a pediatric hospital during 2010-2021. Regressions were conducted to identify opioid prescribing trends over time. Potential covariates were assessed. Returns to the system were reviewed as a balancing measure. RESULTS: Even without a targeted protocol, opioid prescribing declined significantly. After prescribing peaked in 2012-2013, significant negative trends yielded lower rates of opioid prescriptions, fewer doses per prescription, smaller patient-weight-standardized dose sizes, and less variability (all p < 0.001). In 2012, 96.1% of patients received opioid prescriptions; the rate fell to 13.5% by 2021. For patients ages, 0-6, the annual rate of opioid prescriptions dropped from a maximum of 96.3% in 2012 to 0.0% in 2021. The annual average supply of doses per prescription decreased by 68% between 2013 and 2021, reducing the total days' supply to an evidence-based 3.1 ± 1.6 days. Regressions did not detect changes in returns to the system. Pain-related returns were rare (0.9%) and did not vary by opioid prescriptions (p = 0.37). Prescribing trends were closely correlated with a tonsillectomy-focused protocol that our institution implemented in 2019. CONCLUSION: Surgeon-driven opioid stewardship has improved with no resultant change in revisit rates. Procedure-specific quality improvement interventions may have broader off-target effects on prescribing behaviors. LEVEL OF EVIDENCE: IV Laryngoscope, 133:1987-1992, 2023.

Sustaining standardized opioid prescribing practices after pediatric tonsillectomy.

INTRODUCTION: Opioid prescribing patterns after pediatric tonsillectomy are highly variable, and opioids may not improve pain control compared to over-the-counter pain relievers. We evaluated whether a standardized, opioid-sparing analgesic protocol effectively reduced opioid prescriptions without compromising patient outcomes. METHODS: A quality improvement project was initiated in July 2019 to standardize analgesic prescribing after hospital-based tonsillectomy with/without adenoidectomy. An electronic order set provided weight-based dosing and defaulted to non-opioid prescriptions (acetaminophen and ibuprofen). Patients ages 0-6 received non-opioid analgesics alone. Patients ages 7-18 received non-opioid analgesics as first-line pain control, and providers could manually add hydrocodone-acetaminophen for breakthrough pain. Opioid prescriptions and quantities were compared for 18 months of cases pre- versus post-standardization. Postoperative returns to the system were reviewed as a balancing measure. RESULTS: From 2018 through 2020, 1817 cases were reviewed. The frequency of opioid prescriptions decreased significantly post-standardization, from 64.9% to 33.5% of cases (P < .001). Opioid prescribing for young children steadily decreased from over 50% to 2.4%. Protocol adherence improved over time; outlier prescriptions were eliminated. Opioid quantities per prescription decreased by 16.3 doses on average (P < .001), and variance decreased significantly post-standardization (P < .001). The incidence of returns to the system did not change (P = .33), including returns for pain or decreased intake (P = .28). CONCLUSION: An age-based and weight-based analgesic protocol reduced post-tonsillectomy opioid prescriptions without a commensurate increase in returns for postoperative complaints. Standardized protocols can facilitate sustained changes in prescribing patterns and limit potentially unnecessary pediatric opioid exposure.

Prevalence and surgical management of cholesteatoma in Down Syndrome children.

OBJECTIVE: The objective was to report the outcomes of surgical management of cholesteatoma in Down Syndrome (DS) children and to determine the prevalence of cholesteatoma in DS children at three academic tertiary care centers. METHODS: A retrospective chart review was performed of all DS children diagnosed with cholesteatoma from three tertiary care academic centers. Demographic data, comorbidities, imaging findings, surgical management, and pre- and postoperative pure tone averages were collected. RESULTS: The prevalence of cholesteatoma in DS patients at these three institutions was calculated to be 8/802 (0.998%), 4/448 (0.893%), and 4/1016 (0.394%), with an average prevalence of 0.762%. Of the 16 patients with cholesteatoma identified, the average age of diagnosis was 10.8 years, and 5/16 (31%) had at least three sets of pressure equalizing tubes (PETs) placed. Of the 13 patients with preoperative CTs, 4/13 (30.8%) had blunting of the scutum, 4/13 (30.8%) had tegmen involvement, 1/13 (7.7%) had erosion of the semicircular canal, 2/13 had facial nerve dehiscence, and 7/13 (53.8%) had ossicular chain erosion. For surgical management, disease control was attained in 4/16 children with tympanoplasty only and canal wall up (CWU) tympanomastoidectomy in 11/16 patients. Available pre- and postoperative audiograms were evaluated; however, there was insufficient audiological data to contribute to outcomes. CONCLUSION: Cholesteatoma occurs in nearly 1% of DS patients, and the disease tends to be advanced with tegmen involvement in nearly a third of patients, and ossicular erosion seen in more than half the patients. A CWU procedure can be successful in managing DS patients with cholesteatoma when consistent long term follow-up is planned. Close monitoring and a high index of suspicion for cholesteatoma are essential to prevent delays in diagnosis and treatment of DS patients with a history of multiple sets of PET placement.

Tracheostomy decannulation to noninvasive positive pressure ventilation in congenital central hypoventilation syndrome.

PURPOSE: Noninvasive positive pressure ventilation (NPPV) may permit tracheostomy decannulation (TD) in patients with congenital central hypoventilation syndrome (CCHS) requiring nocturnal positive pressure ventilation via tracheostomy (PPV-T). There is limited evidence on optimal strategies for transitioning patients from PPV-T to NPPV. This study aimed to describe the clinical course and outcome of children with CCHS who underwent TD and transitioned from PPV-T to NPPV. METHODS: Retrospective review was conducted on patients with CCHS using nocturnal PPV-T who underwent TD to NPPV. The results of clinical evaluations, airway endoscopy, polysomnography, and clinical course leading to TD were analyzed. RESULTS: We identified 3 patients with CCHS aged 8-17 years who required PPV-T only during sleep. Patients underwent systematic multidisciplinary evaluations with a pediatric psychologist, pulmonologist, sleep physician, and otolaryngologist utilizing a TD algorithm. These included evaluation in the sleep clinic, NPPV mask fitting and desensitization, endoscopic airway evaluation, daytime tracheostomy capping, acclimatization to low-pressure NPPV, polysomnography with capped tracheostomy and NPPV titration, and if successful, TD. All patients underwent successful TD following optimal titration of NPPV during polysomnography. The duration to TD from decision to pursue NPPV was between 2.4 and 10.6 months, and the duration of hospitalization for TD was between 4 and 5 days. There were no NPPV-related complications; however, all patients required surgical closure of tracheocutaneous fistula. CONCLUSION: NPPV may be an effective and feasible option for patients with CCHS requiring PPV-T during sleep and permits TD. In patients with CCHS, a systematic multidisciplinary algorithm may optimize successful transition to NPPV and TD.

Effects of Age on Delayed Facial Palsy After Otologic Surgery: A Systematic Review and Meta-Analysis.

OBJECTIVES: To report 4 cases of delayed facial palsy (DFP) after pediatric middle ear (ME) surgery and systematically review and analyze the associated literature to evaluate the effects of age on DFP etiology, management, and prognosis. METHODS: Systematic review of PubMed, Cochrane Library, and Embase for articles related to DFP after cochlear implantation (CI) was performed. These articles were assessed for level of evidence, methodological limitations, and number of cases. Meta-analysis was performed to assess the effects of age on DFP incidence. Furthermore, a comprehensive list of all pediatric DFP cases after otologic surgery was assembled through a multi-institutional retrospective review and systematic review of the literature. RESULTS: Twenty-nine articles fit the criteria for inclusion in the meta-analysis. The incidence of DFP after CI was 0.23% and 1.01% for pediatric and adult cases, respectively. This difference was statistically significant (P < .001, odds ratio 4.36). Twenty-three cases, adding to the 4 presented herein, were suitable for a comprehensive list. The mean age was 6.9 years. Average postoperative day of paresis onset was 5.4, with an average maximum House-Brackmann grade of 3.5. All patients obtained full facial recovery after an average of 23.5 days. CONCLUSIONS: The systematic review demonstrates that DFP after pediatric CI is rare and occurs at a significantly lower rate than in adults, further supporting the viral reactivation hypothesis of DFP. The prognosis for pediatric DFP after otologic surgery is excellent, with a high rate of full recovery in a short time frame. However, steroid administration can be considered. LEVEL OF EVIDENCE: IIa.

Bullying and Children who are Deaf or Hard-of-hearing: A Scoping Review.

OBJECTIVE/HYPOTHESIS: The goal of this scoping review is to evaluate and synthesize the published research regarding bullying and children who are deaf or hard-of-hearing (DHH). STUDY DESIGN: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for scoping review (PRISMA-ScR) statement was used as the guideline for conducting this review. METHODS: This review included studies assessing pediatric (0-21 years) DHH children. Seventy records underwent the initial title-abstract screening, 33 underwent full-text review, and 23 studies met inclusion criteria. During the data extraction process, an additional six were excluded, resulting in a total of 17 evaluated studies. RESULTS: Of the 17 studies assessed, nine compared peer victimization of DHH children and their hearing peers. Of those, seven studies reported that hearing loss (HL) is significantly associated with increased victimization. Two studies found that HL is significantly associated with decreased bullying perpetration. Notably, bullying in DHH children was not associated with a visible sign of disability, such as a hearing assistive device. Rather, variables such as educational setting, parental, and peer support are more likely to mediate bullying in this population. CONCLUSIONS: DHH children have a higher risk of peer victimization and may bully others less often than their hearing peers. Studies described the health consequences of bullying in children who are DHH, including sleep issues and anxiety. These consequences may have implications for the patients' overall HL management. Additional research regarding bullying interventions and prevention in this population should be conducted. LEVEL OF EVIDENCE: NA Laryngoscope, 131:1884-1892, 2021.

Social Isolation and Loneliness in the Hearing-Impaired Pediatric Population: A Scoping Review.

OBJECTIVE: To review existing publications in order to evaluate the effect of hearing loss on social isolation and loneliness in the pediatric population. METHODS: Preferred Reporting Items for Systematic Reviews and Meta-Analyses for Scoping Review (PRISMA-ScR) guidelines were followed. Eight databases were searched. Studies were independently screened and analyzed by two reviewers. Publications were included if pediatric hearing-impaired individuals and social isolation or loneliness were studied. Discrepancies were resolved by a team of five reviewers. RESULTS: Thirty-three studies were included in this review. Sixty percent of studies (12/20) found that hearing loss was related to loneliness and 64.7% found that children with hearing loss experienced more social isolation (11/17). The Asher Loneliness and Dissatisfaction Questionnaire was commonly used to assess loneliness. No commonly used tool for assessing social isolation was found. Six articles found that school type was not associated with loneliness. Difficulty communicating was the most mentioned factor leading to loneliness and social isolation. Frequent recommendations to improve social integration included facilitating interactions with the hearing-impaired and educating the nonhearing-impaired to normalize the disability. CONCLUSION: The majority of studies found that hearing impairment results in a higher prevalence of social isolation and loneliness. However, an association cannot be definitively claimed due to the lack of uniform assessment tools. This review emphasizes the need for standardized methods to assess loneliness and social isolation and highlights methods to improve social integration for the hearing impaired. Laryngoscope, 131:1869-1875, 2021.

Rapid telemedicine implementation in the context of the COVID-19 pandemic in an academic pediatric otolaryngology practice.

OBJECTIVE: To describe the implementation of telemedicine in a pediatric otolaryngology practice during the coronavirus disease 2019 (COVID-19) global pandemic. METHODS: A descriptive paper documenting the development and application of telemedicine in a tertiary academic pediatric otolaryngology practice. RESULTS: A total of 51 established patients were seen via telemedicine within the first 2 weeks of telemedicine implementation. Seven (7) patients were no shows to the appointment. The median patient age was 5 years old, with 55% male patients. Common diagnoses for the visits included sleep disordered breathing/obstructive sleep apnea (25%) and hearing loss (19.64%). Over half (50.98%) of visits were billed at level 4 visit code. DISCUSSION: The majority (88%) of visits during the first 2 weeks of telemedicine implementation in our practice were completed successfully. Reasons that patients did not schedule telemedicine appointments included preference for in person appointments, and lack of adequate device at home to complete telemedicine visit. Limitations to our telemedicine practice included offering telemedicine only to patients who had home internet service, were established patients, and English-speaking. Trainees were not involved in this initial implementation of telemedicine. CONCLUSIONS: COVID-19 has driven the rapid adoption of telemedicine in outpatient medicine. Our group was able to institute an effective telemedicine practice during this time.

Assessment and Treatment of Behavioral Disorders in Children with Hearing Loss: A Systematic Review.

OBJECTIVE: There is evidence that children who are deaf and hard of hearing (DHH) have a higher incidence of behavioral disorders. Assessment of behavioral health in this population is often complicated by language developmental delays, which may result in unrecognized and untreated behavioral problems. The purpose of this study is to assess the association of behavioral disorders among children who are DHH and to explore behavioral interventions for children in this population. DATA SOURCES: PubMed, CINALH, PsychINFO, and Web of Science. REVIEW METHODS: Search terms included the following: problem behavior, child behavior disorders/diagnosis, child behavior disorders/psychology coupled with hearing loss, cochlear implants, hearing aids, or deafness. Studies from the last 30 years (1985-2016) were included. The articles were reviewed independently by 3 reviewers. RESULTS: Thirty-six articles met criteria. There was an association between internalizing behaviors and hearing loss among children, which may persist after cochlear implantation. These problems may be more pronounced for children with additional disabilities. Conduct and hyperactivity disorders as well as emotional and executive function problems among children who are DHH may be related to poor language development. There was limited evidence regarding interventions to address the behavioral disorders of DHH children. CONCLUSIONS: There is a significant body of evidence demonstrating behavioral problems among DHH children but a lack of clear understanding of the mechanisms involved. There is limited evidence on interventions to address the behavioral problems of DHH children. Future research is warranted to mitigate the long-term effects of disruptive behavior among these children.

Upper aerodigestive tract frostbite from inhalation of automotive nitrous oxide.

Nitrous oxide, a cryogenic gas, may be abused as an inhalant for its euphoric properties. If inhaled, nitrous oxide may cause frostbite to the oral cavity and upper aerodigestive tract, with possible airway compromise due to edema. In this article we describe what is, to the best of our knowledge, the first case of intentional inhalation of nitrous oxide from an automotive nitrous oxide canister and discuss the management and mechanism of the patient's injury.

Awake vs Sedated Tracheostomies: A Review and Comparison at a Single Institution.

OBJECTIVE: The literature surrounding awake tracheostomies is sparse, particularly comparing awake tracheostomy patients to that of the sedated tracheostomy population. This study sought to compare tracheostomy patient demographics, indications, and outcomes of the 2 populations. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care center. MATERIALS AND METHODS: All tracheostomies performed at our tertiary academic medical institution between January 2013 through November 2015 were reviewed. The data collected included demographics, comorbidity, anticoagulation, and outcomes. RESULTS: A total of 978 tracheostomies performed during this period met inclusion criteria, with 78 (8.0%) on awake patients. Most awake procedures were performed by otolaryngology (97.4%). Male sex predominated (73.1% awake vs 57.8% sedated). Forty-four patients (56.4%) were smokers in the awake group vs 326 of 900 (36.2%) in the sedated group. Malignancy was the primary indication for awake tracheostomy (68/78, 87.1%). One patient (1.3%) had significant postoperative bleeding compared to 26 of 900 (2.9%) of the sedated tracheostomy patients ( P = .406). Only 9 (11.4%) were ever decannulated. Thirty-one (39.2%) patients ultimately underwent total laryngectomy, 3 could not be decannulated secondary to anatomical causes (stenosis or vocal fold paralysis), and 19 were lost to follow-up after discharge. There were 12 of 78 (15.4%) overall deaths in the awake cohort, with 215 of 900 (23.9%) in the sedated cohort ( P = .088). CONCLUSION: Despite all the differences between the 2 patient populations, the urgent awake tracheostomy appears to be safe and its complications do not appear significantly different from the sedated population.

Barriers to Rehabilitation Care in Pediatric Cochlear Implant Recipients.

OBJECTIVE: The purpose of this study was to assess barriers to rehabilitation care for pediatric cochlear implant (CI) recipients. STUDY DESIGN: Cross-sectional questionnaire study. SETTING: Tertiary medical center. PATIENTS: Parents of children who received a CI from October 1996 to June 2013. MAIN OUTCOME MEASURE(S): Factors related to access to and barriers in audiology and speech therapy services, factors related to CI use, and performance with CI using the Parents' Evaluation of Aural/Oral Performance of Children (PEACH). RESULTS: Thirty-five parents participated in the study (21 rural residents and 14 urban residents). Distance was a significant barrier to audiology services for rural participants compared with urban participants (p = 0.01). Consistent CI use was complicated by mechanical complications or malfunction in 70% of rural children compared with 33% of urban children (p = 0.05). Only 10% of rural children were able to access speech therapy services at diagnosis compared with 42% of urban children (p = 0.04). Low socioeconomic (SES) status and Medicaid insurance were associated with a lack of local speech therapists and medical/mechanical CI complications. Higher parental educational attainment was associated with higher PEACH scores in quiet conditions compared with families with lower parental education (p = 0.04). CONCLUSIONS: Rural children are often delayed in receipt of CI rehabilitation services. Multiple barriers including low SES, insurance type, and parental education can affect utilization of these services and may impact the recipient language development. Close follow-up and efforts to expand access to care are needed to maximize CI benefit.

Otolaryngology consultation tracheostomies and complex patient population.

PURPOSE: To assess for the differences in patients undergoing tracheostomy by the otolaryngology consult service versus other specialties. MATERIALS AND METHODS: A series of 1035 tracheostomies performed at our institution from January 2013 through November 2015 was retrospectively reviewed. Patient-related factors that contribute to procedural difficulty were reviewed. RESULTS: 805 consecutive tracheostomies were included. Otolaryngology performed 176/805 (21.8%) tracheostomies as a consulting service. Morbidly obese patients were three times as likely to be referred to otolaryngology as other services (adjusted OR: 3.23; 95% CI: 2.21-4.72). Mean BMI was 36.38kg/m2 for Consults vs. 28.69kg/m2 for Others and morbidly obese patients had a mean BMI of 49.84kg/m2 vs. 42.68kg/m2 for Consults and Others respectively (p<0.001). Patients with upper airway compromise (8.5% of Consults vs. 1.6% for Others) had 5.5 times higher odds to be performed by otolaryngology (adjusted OR: 5.46; 95% CI: 2.24-13.28). Otolaryngology performed 81.8% of awake tracheostomies (n=9/11). There were significantly higher proportions of patients with diabetes, renal, pulmonary and cardiovascular disease in the Consults groups vs. Others (p<0.05). CONCLUSIONS: More complex tracheostomies are being referred to and performed by otolaryngology at our institution. Difficult and challenging tracheostomies seem to be the "standard" for otolaryngologists.

Audible sphenoid-sellar fistula: An unusual complication of transphenoidal surgery.

Eustachian tube problems are relatively common complaints to otolaryngologists' offices. However, clinicians should consider other possibilities when traditional therapies fail to improve symptoms. We present a previously not described case of sphenoid-sellar fistula after transphenoidal surgery causing objective and subjective clicking. Laryngoscope, 126:E314-E316, 2016.