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.

Association of Diet Patterns and Post-Operative Tympanostomy Tube Otorrhea: A Pilot Study.

OBJECTIVE: The objective of this study was to explore diet patterns in children with tympanostomy tube placement (TTP) complicated by postoperative tympanostomy tube otorrhea. STUDY DESIGN: Cross-sectional survey and retrospective cohort study. METHODS: Caregivers of children (0-12 years old), at a tertiary-care pediatric hospital who underwent TTP within 6 months to 2 years prior to enrollment were included. Children with a history of Down syndrome, cleft palate, craniofacial syndromes, known immunodeficiency, or a non-English-speaking family were excluded. Our primary outcome variable was the number of otorrhea episodes. The primary predictor was diet patterns, particularly dessert intake, which was captured through a short food questionnaire. RESULTS: A total of 286 participants were included in this study. The median age was 1.8 years (IQR, 1.3, 2.9). A total of 174 (61%) participants reported at least one episode of otorrhea. Children who consumed dessert at least two times per week had a higher risk of otorrhea compared to children who consumed one time per week or less (odds ratio [OR], 3.22, 95% Confidence Interval [CI]: 1.69, 6.12). The odds ratio increase continued when considering more stringent criteria for otorrhea (multiple episodes or one episode occurring 4 weeks after surgery), with a 2.33 (95% CI: 1.24, 4.39) higher odds of otorrhea in children with dessert intake at least 2 times per week. CONCLUSIONS: Our pilot data suggest that episodes of otorrhea among children with TTP were associated with more frequent dessert intake. Future studies using prospectively administered diet questionnaires are necessary to confirm these findings. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:3575-3581, 2023.

High-Dose Celecoxib for Pain After Pediatric Tonsillectomy: A Randomized Controlled Trial.

OBJECTIVE: Pediatric tonsillectomy causes significant postoperative pain. Newer nonsteroidal anti-inflammatory drugs such as celecoxib control pain without increasing bleeding risk, but in prior studies provided only modest pain reduction at standard doses. We aimed to determine if high-dose celecoxib (double the usual pediatric dose) is effective for pain, without increasing bleeding or other risks. STUDY DESIGN: Randomized double-blind trial. SETTING: Pediatric tertiary center. METHODS: Children aged 3 to 11 years undergoing total tonsillectomy were randomized to receive celecoxib (6 mg/kg/dose) or placebo, twice daily, for up to 10 days. All cases were supplemented with acetaminophen and oxycodone as needed. All participants and personnel were blinded to treatment group. Subjects recorded coanalgesic consumption, pain, diet, and activity. RESULTS: The celecoxib group (n = 68) consumed 0.72 mg/kg of oxycodone, as compared with 1.12 mg/kg in the placebo group (n = 62), a 36% difference that was not significant. However, multivariate analysis by treatment group, separate from pain levels, confirmed that this reduction was due to celecoxib treatment (P = .03). In subjects with more prolonged pain (n = 88), celecoxib reduced consumption by 52% (P = .02). Celecoxib showed greater benefit for subjects in the prolonged pain group than for those in the lesser pain group (P = .006). Incidence of adverse events was similar between groups. Minor hemorrhage occurred in 4.6% (5 placebo, 3 celecoxib). CONCLUSION: High-dose celecoxib is effective in controlling pain after tonsillectomy, with no adverse effects in this relatively small sample. It reduces narcotic consumption, and its impact appears greater in children with higher degrees of pain. Celecoxib can be considered an effective alternative to ibuprofen after tonsillectomy. This trial was registered at ClinicalTrials.gov: NCT02934191.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient.

OBJECTIVE: To provide recommendations for the workup of hearing loss in the pediatric patient. METHODS: Expert opinion by the members of the International Pediatric Otolaryngology Group. RESULTS: Consensus recommendations include initial screening and diagnosis as well as the workup of sensorineural, conductive and mixed hearing loss in children. The consensus statement discusses the role of genetic testing and imaging and provides algorithms to guide the workup of children with hearing loss. CONCLUSION: The workup of children with hearing loss can be guided by the recommendations provided herein.

Unilateral cochlear nerve deficiency in children.

OBJECTIVE: Cochlear nerve deficiency (CND) is increasingly diagnosed in children with sensorineural hearing loss (SNHL). We sought to determine the prevalence of CND, its imaging characteristics, and correlations with audiologic phenotype in children with unilateral SNHL. DESIGN: Case series with chart review. SETTING: Tertiary pediatric hospital. SUBJECTS/METHODS: In 128 consecutive children with unilateral SNHL who underwent high-resolution magnetic resonance imaging, the diameters, area, and signal intensity of the cochlear nerve (CN) were measured and normalized to the ipsilateral facial nerve. Presence of CND was determined by comparison to normative data. Relationships among hearing loss severity, progression, and nerve size were investigated. RESULTS: Cochlear nerve deficiency was present in 26% of children with unilateral SNHL. Its prevalence was higher (48%) in severe to profound SNHL, especially when in infants (100%). Width of the bony cochlear nerve canal (BCNC) correlated strongly with relative CN diameter, density, and area (R = 0.5); furthermore, a narrow BCNC (<1.7 mm) strongly predicted CND. Severity of hearing loss modestly correlated with nerve size, although significant variability was observed. Progression never occurred unless there were other inner ear malformations, whereas in the non-CND group, it occurred in 22%. Ophthalmologic abnormalities were very common (67%) in CND children, particularly oculomotor disturbances. CONCLUSION: Cochlear nerve deficiency is a common cause of unilateral SNHL, particularly in congenital unilateral deafness. Width of the BCNC effectively predicts CND, a finding useful when only computed tomography imaging is available. In an ear with CND, hearing can be expected to remain stable over time. Diagnosis should prompt evaluation by an ophthalmologist.

Venous malformations of the temporal bone are a common feature in CHARGE syndrome.

OBJECTIVES/HYPOTHESIS: CHARGE (Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and/or development, Genital and/or urinary abnormalities, and Ear abnormalities and/or deafness) syndrome is a genetic disorder with prominent otolaryngologic features including choanal atresia and inner ear malformations. Recent experience with venous malformations during cochlear implant surgery prompted this study to define the spectrum of venous abnormalities in CHARGE and their surgical implications in otology. STUDY DESIGN: Retrospective review of medical and radiologic records from databases of patients with CHARGE syndrome from three tertiary care academic medical centers. METHODS: Eighteen patients with CHARGE for whom temporal bone CT scans were available were included in the review. RESULTS: Venous anomalies of the temporal bone were present in 10 of 18 (56%) patients. The most common were large emissary veins (n = 5). In two of these cases, these veins were associated with an ipsilateral a hypoplastic sigmoid sinus or jugular foramen. Other abnormalities included an aberrant petrosal sinus, venous lakes in proximity to the lateral venous sinus, condylar canal veins, and jugular bulb abnormalities, including a high riding bulb obscuring the round window niche and a dehiscent jugular bulb. In four of six patients undergoing cochlear implantation, the course of the aberrant vessel necessitated a change in the surgical approach, either during mastoidectomy or placement of the cochleostomy. CONCLUSIONS: Temporal bone venous abnormalities are a common feature in CHARGE syndrome. The pattern of venous abnormality suggests that there is a failure of the sigmoid sinus/jugular bulb to fully develop, resulting in persistence of emissary veins. Recognition of these abnormal venous structures during otologic surgery is critical to avoiding potentially catastrophic bleeding.

Early prediction of postmeningitic hearing loss in children using magnetic resonance imaging.

OBJECTIVE: To determine whether early gadolinium-enhanced magnetic resonance imaging (GdMRI) can reliably detect meningitic labyrinthitis and thereby predict which children are at high risk for hearing loss. Permanent sensorineural hearing loss (SNHL) remains a common sequela of bacterial meningitis, and early diagnosis of the associated suppurative labyrinthitis can be difficult, especially in critically ill, sedated patients and young children. DESIGN: Retrospective cohort study. SETTING: Tertiary pediatric hospital. PARTICIPANTS: Twenty-three survivors of bacterial meningitis (median age, 15 months [range, 3 months-14 years]) who had undergone brain GdMRI during the acute disease and had subsequent ear-specific audiometric data. MAIN OUTCOME MEASURE: Blinded to disease and outcome, a neuroradiologist rated the relative enhancement of each cochlea on T1-weighted images using a 4-point scale. Scores were then correlated with the degree of hearing loss on subsequent testing. RESULTS: Sensorineural hearing loss occurred in 15 of 46 ears (8 of 23 patients). Enhancement on GdMRI was detected in 13 of the 15 ears that later developed SNHL but was absent in all 31 unaffected ears. Thus, GdMRI was 87% sensitive and 100% specific for predicting which ears would develop permanent SNHL. In the subgroup with pneumococcal meningitis (n = 15), GdMRI was 100% sensitive and 100% specific. Labyrinthine enhancement was detectable as early as 1 day after diagnosis. CONCLUSION: Gadolinium-enhanced MRI detected meningitic labyrinthitis at early stages and accurately predicted which patients would later develop hearing loss.

Etiology of unilateral neural hearing loss in children.

OBJECTIVE: Unilateral sensorineural hearing loss (SNHL) can be caused by a variety of lesions of the inner ear and central nervous system. An inner hair cell or neural site of pathology must be suspected when otoacoustic emissions (OAEs) are present, and inconsistent with audiologic data. We reviewed unilateral neural hearing loss (UNHL) in children, to better understand its etiology, clinical and audiologic features. DESIGN: Retrospective series. SETTING: Tertiary pediatric center. METHODS: From a database of 480 children with unilateral SNHL, 148 had OAE data. Patients with a neural pattern (present OAEs in the affected ear) were reviewed. OUTCOME MEASURES: Clinical course, audiologic data, imaging findings. RESULTS: Of 148 patients with OAE data, 11 (7.4%) had the unilateral neural phenotype. Most had stable, severe-to-profound loss in the affected ear. MRI determined an etiology in all 10 patients who received it. Absent cochlear nerves were remarkably common, being found in eight patients (73%). Tumors, previously unsuspected, were identified in the other two patients who received MRI. CONCLUSIONS: Cochlear nerve aplasia appears by far the most common cause of UNHL in children. As in adults, mass lesions must also be considered in children with unilateral SNHL with a neural pattern. As both lesions elude diagnosis on CT, MRI is the better modality for evaluating this condition.

Real-time intraoperative computed tomography to assist cochlear implant placement in the malformed inner ear.

OBJECTIVE: Cochlear implantation is increasingly being performed in children with inner ear malformations. In severe cochleovestibular anomalies, such as severe partitioning defects and common cavity dysplasia, positioning of the electrode array can be hazardous, with inadvertent placement into the internal auditory canal (IAC) or carotid canal being well known. We describe a case in which real-time intraoperative computed tomographic scanning was used to help achieve proper electrode positioning in a child with a severe malformation. PATIENT: Child with common cavity malformations undergoing cochlear implantation. INTERVENTION: Intraoperative computed tomography used during implantation procedure. MAIN OUTCOME MEASURE: Use of technique in determining electrode position. RESULTS: A 10-year-old patient with bilateral common cavity malformations presented with declining performance in a functioning implant placed 7 years earlier. The family elected implantation of the contralateral ear. Via a posterior labyrinthotomy approach, a straight array was placed into the common cavity. Intraoperative computed tomographic scanning was immediately performed on the operating room table, showing that the array was in the IAC. A second attempt with a different insertion angle also resulted in IAC placement. In a third attempt, the electrode was advanced as a loop, grasping the tip through an adjacent second labyrinthotomy. Computed tomography confirmed good position against the outer wall of the cavity. CONCLUSION: Real-time intraoperative computed tomography is a new technology with many potential applications in surgery. In our patient, it allowed rapid and accurate determination of electrode position and helped achieve ideal placement in a severely malformed inner ear.

Intracranial complications of sinusitis in children and adolescents and their outcomes.

OBJECTIVE: To gain insight into patterns of presentation, imaging, microbiological aspects, therapy, disease course, and outcome of intracranial complications of sinusitis (ICS), which are challenging conditions with the potential to cause significant morbidity and mortality. We reviewed our experience with ICS in children and adolescents. DESIGN: Consecutive case series with a mean follow-up of 12 months. SETTING: Tertiary pediatric referral center. PATIENTS: Consecutive sample of 25 children and adolescents treated for 35 intracranial complications (mean age, 13.2 years [range, 4-18 years]). INTERVENTIONS: Medical and surgical management. MAIN OUTCOME MEASURES: Survival and temporary and permanent neurologic sequelae. RESULTS: Most patients were adolescents (n = 19; 76%) and male (n = 19; 76%). Epidural abscess was most common (13 complications), followed by subdural empyema (n = 9), meningitis (n = 6), encephalitis (n = 2), intracerebral abscess (n = 2), and dural sinus thrombophlebitis (n = 2). Abscesses were primarily located in the frontal or frontoparietal regions. Magnetic resonance imaging was extensively used and was superior to contrast computed tomography in diagnosis. All patients received intravenous antibiotics, 21 underwent endoscopic sinus surgery, and 13 underwent neurosurgical drainage. Only 1 death occurred from sepsis secondary to meningitis (mortality, 4%). Overall, neurologic outcome was excellent. Although 10 patients (40%) had neurologic deficits, most resolved within 2 months. Only 2 patients had permanent neurologic sequelae. Among ICS, epidural abscess appeared to be a distinct clinical entity. Epidural abscesses typically presented without specific neurologic symptoms or signs, were more often associated with orbital complications, and had outcomes considerably better than the other ICS. CONCLUSION: Intracranial complications of sinusitis are challenging, but prognosis can be favorable in children and adolescents by using aggressive medical and surgical management.

Chronic Pseudomonas infections of cochlear implants.

OBJECTIVE: To discuss chronic, refractory Pseudomonas infections of cochlear implants and their management. DESIGN: Retrospective case series. SETTING: Two university-based cochlear implant programs. PATIENTS: Twenty-eight-year-old (Case 1) and 4-year-old (Case 2), different devices. INTERVENTIONS: Medical and surgical management. MAIN OUTCOME MEASURES: Clinical course. RESULTS: Both patients had delayed presentations, 4 months and 3 years postimplantation, respectively, with fluctuating scalp edema and pain resistant to multiple courses of oral antibiotics. Infections began as localized granulation and progressed to complete encasement of both devices with rubbery, poorly vascularized tissue. In each case, two different strains of multiresistant Pseudomonas aeruginosa were cultured. Infections progressed despite local debridement and targeted antipseudomonal antibiotic coverage, and sensitive organisms continued to appear in cultures of refractory granulation tissue. Both patients underwent partial explantation, with the electrode array left in the cochlea, then received 2 to 3 more months of further medical therapy and observation and then were reimplanted successfully with new devices. Both have shown excellent performance and no sign of recurrent infection. CONCLUSIONS: Infections of cochlear implants are uncommon, and cases of successful conservative management without device explantation have been reported. However, our experience and the implanted device literature suggest that chronic Pseudomonas infections may represent a distinct clinical entity, likely to fail protracted therapy and ultimately require device removal. Fortunately, successful reimplantation is possible.

Molecular characterization of conditionally immortalized cell lines derived from mouse early embryonic inner ear.

Inner ear sensory hair cells (HCs), supporting cells (SCs), and sensory neurons (SNs) are hypothesized to develop from common progenitors in the early embryonic otocyst. Because little is known about the molecular signals that control this lineage specification, we derived a model system of early otic development: conditionally immortalized otocyst (IMO) cell lines from the embryonic day 9.5 Immortomouse. This age is the earliest stage at which the otocyst can easily be separated from surrounding mesenchymal, nervous system, and epithelial cells. At 9.5 days post coitum, there are still pluripotent cells in the otocyst, allowing for the eventual identification of both SN and HC precursors--and possibly an elusive inner ear stem cell. Cell lines derived from primitive precursor cells can also be used as blank canvases for transfections of genes that can affect lineage decisions as the cells differentiate. It is important, therefore, to characterize the "baseline state" of these cell lines in as much detail as possible. We characterized seven representative "precursor-like" IMO cell populations and the uncloned IMO cells, before cell sorting, at the molecular level by polymerase chain reaction (PCR) and immunocytochemistry (IHC), and one line (IMO-2B1) in detail by real-time quantitative PCR and IHC. Many of the phenotypic markers characteristic of differentiated HCs or SCs were detected in IMO-2B1 proliferating cells, as well as during differentiation for up to 30 days in culture. These IMO cell lines represent a unique model system for studying early stages of inner ear development and determining the consequences of affecting key molecular events in their differentiation.