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Background In 2013, Utah enacted legislation requiring that infants failing newborn hearing screening be tested for cytomegalovirus infection. As a result, cytomegalovirus-infected infants are being identified because of hearing deficits. The neuroimaging findings in this population have not been characterized. Methods Retrospective medical record review was used to identify patients seen at the University of Utah and Primary Children’s Hospitals in Salt Lake City, Utah, who failed newborn hearing screening. A cohort of patients with congenital cytomegalovirus infection, brain magnetic resonance imaging (MRI), and sedated auditory brainstem response testing was studied. Results Seventeen patients were identified; 11 (65%) were female. Confirmatory auditory brainstem response testing, performed at a median age 29 days, showed profound hearing loss in 8 (47%) subjects, severe loss in two (12%), moderate loss in two (12%), and mild loss in three (18%); two (12%) subjects had normal hearing. The diagnosis of cytomegalovirus infection was made at a median age 23 days. Brain imaging was performed at a median age 65 days. Ten (59%) subjects had one or more neuroimaging abnormality. White matter lesions were found in eight (47%) subjects, cysts in three (18%), and stroke in two (12%). Polymicrogyria was identified in two (12%) subjects. Seven (41%) subjects had normal brain MRIs. Conclusions These results indicate that most infants whose cytomegalovirus infections were identified after failing newborn hearing screening had abnormal brain MRIs. Our results suggest that brain MRIs should be considered in infants with congenital cytomegalovirus infections who are identified through hearing screening programs.

Introduction There is increasing concern regarding the risks associated with the use of general anesthesia in pediatric patients. Many otolaryngologic procedures performed under general anesthesia can also be performed in clinic. We hypothesize that anxiolytics can aid in performing common procedures in clinic thus avoiding the need to undergo general anesthesia in the OR. Methods We performed a retrospective review of patients undergoing inoffice procedures with anxiolytics in our pediatric otolaryngology outpatient clinic between February 2013 and January 2017. Charts were reviewed for age, past medical history, procedure type/duration, and outcome. These results were then compared to a cohort undergoing similar procedures in the OR. Results A total of 34 patients underwent an in-office procedure with an anxiolytic. The success rate was 97% (33/34). The average age was 6.2 years. Six children (17%) had a known history of chromosomal abnormalities and 2 children (6%) had autism. The four most common procedures performed were cerumen impaction removal (8), flexible laryngoscopy (6), ear canal foreign body removal (5), and septal cautery (4). Performing similar procedures in the OR resulted in an average additional cost of $822. Conclusions Performing procedures with anxiolytics in a pediatric otolaryngology clinic is safe, expeditious, and cost-effective. Anxiolytics can provide an effective alternative to general anesthesia.

OBJECTIVES: Congenital human cytomegalovirus (cCMV) is a leading cause of pediatric hearing loss. Recent literature has suggested that valganciclovir (VGCV) therapy can improve hearing outcomes. The objective of this study was to evaluate the long-term hearing outcomes among symptomatic CMV patients treated with VGCV. METHODS: A retrospective chart review of symptomatic CMV patients treated with VGCV was completed. The primary endpoint was the change in best ear hearing scores prior to treatment and after follow-up audiograms. A paired-sample t-test was used to evaluate the data. RESULTS: A total of 16 children were included in the study and participants were followed for an average of 3.2 years. There was a measurable worsening, but not a statistically significant change in the best ear hearing scores, where the mean change was 11.9dB (p-value=0.070). However, 14/16 patients (87.5%, p-value

To compare the costs of disposable laryngoscopes to reusable scopes in outpatient and inpatient settings.The total variable and fixed costs involved in flexible scope reprocessing were collected from two general otolaryngology clinics, a pediatric otolaryngology clinic, and a children's hospital. Variable costs of disposable materials and labor were collected from 65 scope reprocessing events to identify the cost of reprocessing. Fixed costs of scope maintenance, monitors, video towers, and storage equipment were collected from financial records. Fixed and variable costs were analyzed to identify the cost per scope event. The costs were then compared to a theoretical model where disposable scopes were used to meet the volume demands of each clinic and children's hospital setting. The model of disposable scopes was generated after obtaining volume costs specific to each setting from a disposable scope company.The average cost of a reusable scope model per scope event was $66.02 ± 4.49 at the three clinics and $130.66 at the children's hospital. The average cost of the disposable scope model per scope event was $152.55 ± 0.55 in the three clinics and $172.61 in the children's hospital. The cost differences were $86.53 ± 3.96 and $41.95 respectively.In an outpatient clinic, reusable scopes are less expensive than a disposable scope model. In children's hospital inpatient setting, the difference in costs between disposable and reusable scopes is lower. When considering other non-economic factors, disposable scopes may be a feasible option, especially in the children's hospital setting.

Hearing loss is the most common congenital defect. With early diagnosis and intervention, we are able to improve speech and language outcomes in this population. In this article, we discuss the implications of the newborn hearing screen, as well as diagnostic interventions, management, and intervention, and the increasing role of congenital cytomegalovirus screening.

To determine if Gadolinium-based enhanced Magnetic Resonance Imaging (GdMRI) can be used to predict sensorineural hearing loss (SNHL) in pediatric patients diagnosed with bacterial meningitis.Design: Retrospective chart review.Primary Children's Hospital, Salt Lake City, Utah.and Methods: We studied forty-two pediatric patients diagnosed with bacterial meningitis who underwent brain GdMRI during their index hospital admission and for whom ear specific audiometric data were available (August 2008-July 2018). A pediatric neuroradiologist, blinded to both disease and audiometric data, rated cochlear enhancement of each GdMRI (0-3; none to markedly enhanced).Ear specific MRI scores were statistically significantly related to ear specific hearing outcomes (p 0.01). SNHL occurred in 19 out of 82 ears (12 out of 42 patients; 2 ears were excluded due to pre-existing SNHL in one ear and inability to read the GdMRI on the other ear). Ten of 19 ears (53%) that developed SNHL showed mild/moderate/marked enhancement (MRI score of 1, 2, or 3 respectively). Fifty-three of the 63 unaffected ears (84%) showed no enhancement (MRI score of 0). Ten of 13 (77%) ears that developed severe to profound SNHL showed mild/moderate/marked enhancement. GdMRI was 58% sensitive and 84% specific in predicting which ears would develop SNHL. GdMRI was 77% sensitive and 84% specific in identifying severe to profound SNHL.Our study demonstrates that GdMRI is a promising tool for predicting specifically severe-profound hearing loss in pediatric patients following bacterial meningitis infection.

Objectives To determine whether a canal wall down mastoidectomy can provide long-term benefit for children with aural stenosis. Methods Retrospective case series of children with congenital aural stenosis having undergone a canal wall down mastoidectomy over a twelve-year period at a tertiary children's hospital. Results Data from thirteen children who underwent a total of twenty canal wall down mastoidectomies for aural stenosis were reviewed. The mean age at surgery was 7.1 years (range, 3.3–12.3 years). All patients had genetic syndromes including Trisomy 21 (n = 7), Trisomy 21 and Pierre Robin sequence (n = 1), Angelmann (n = 1), Cri-du-chat (n = 1), Branchio-oto-renal syndrome (n = 1), Spina bifida (n = 1) and Nager syndrome (n = 1). Seven (54%) children underwent bilateral canal wall down mastoidectomies. All thirteen ears that could not be visualized preoperatively had improved ease of office examination following surgery. Only one patient required revision surgery and all canals were patent at the last clinic visit. The mean follow-up was 4.9 years. There were no cases of facial nerve injury or cerebrospinal fluid leak. Conclusion Syndromic children with congenital aural stenosis with poorly pneumatized mastoids may benefit from canal wall down mastoidectomy to improve ease of office examinations.

OBJECTIVES: Determine whether a murine model of cytomegalovirus (CMV) and CMV- infected children show evidence of synaptopathy. STUDY DESIGN: Murine model of CMV infection and case series SUBJECTS AND METHODS: C57 BL/6 mice were inoculated with murine-CMV (mCMV). Auditory function was assessed using Auditory Brainstem Response (ABR) and distortion product otoacoustic emission (DPOAE) testing. Temporal bones from mCMV-infected mice were used for both ribbon synapse and hair cell quantification. Four groups of children (non-CMV normal hearing, non-CMV hearing impaired, CMV normal hearing and CMV hearing impaired) underwent ABRs between 2014-2018. The outcomes included raw amplitude, wave I:V amplitude ratio, absolute latency, and interpeak latency. RESULTS: Mice at 8 weeks post mCMV infection had higher ABR and DPOAE (P < 0.05) thresholds and increased outer hair cell loss compared to uninfected mice and mCMV-infected mice at 4- and 6-weeks post infection, indicating progressive hearing loss. A reduction in the wave I amplitude and synaptic counts were noted earlier at 4 weeks in CMV-infected mice (P < 0.05). The human data indicated that the wave I:V amplitude ratio was lower on average in CMV-infected groups when compared to the uninfected cohorts. The wave I:V amplitude ratio for the click and 4k stimuli were not significantly different between the congenital CMV-infected and uninfected children with normal or with hearing loss. CONCLUSION: This study suggests mCMV infection results in a synaptopathy before hair cell damage. Additional studies need to be performed to determine whether this effect is also observed in CMV-infected children. LEVEL OF EVIDENCE: animal studies and basic science- NA; human studies: level 4.

Since 2013, after Utah became the first state to implement hearing targeted early CMV screening, a national debate has been percolating about whether this approach should be introduced nationally. Currently Utah, Iowa, Connecticut, and New York have passed legislation mandating early CMV screening, and over 100 birth hospitals across the United States have voluntarily implemented early CMV screening programs as part of their standard of care. We reviewed the evidence related to this approach and used the Wilson and Jungner (1968) criteria to evaluate this method of screening. Based on these criteria, there is substantial rationale and evidence to support a hearing targeted approach to screen for congenital CMV. Given this evidence, we currently recommend that infants who fail newborn hearing screen should undergo CMV screening.

Objective To determine whether a community engagement approach can provide feedback for implementation of valuable measures to improve the outcome of a clinical trial. Methods Review of the results from a Community Engagement Studio (CE Studio) for the ValEAR trial: an NIH-funded, multi-institutional study designed to research the efficacy of valganciclovir in the treatment of congenital cytomegalovirus (cCMV)-induced hearing loss. Participants were given information about the trial then asked a series of questions to assess their input on the merits or weaknesses affecting their participation in the trial. Results Thirteen parents whose children have congenital CMV infection were recruited for the CE Studio. The overall theme from the responses was a desire to advance the field but a need to clearly understand the risks and benefits of participation. Many requested more educational resources, more printed materials, or greater access to researchers if questions arose. Many welcomed having patient stories and information displayed in a dedicated website or through social media. Conclusion This community engagement approach provided useful feedback from families similar to those expected to be potential enrollees in the CMV ValEAR trial. In response to parental comments, two educational videos were created: one on the general topic of cCMV and the other specific to the CMV ValEAR trial. Researchers who wish to optimize their clinical trial's success should consider incorporating a CE Studio into their study design.

Valganciclovir (VGC) is an orally available mono-valyl ester pro drug of the nucleoside analog (NA) ganciclovir (GCV) used to treat cytomegalovirus (CMV). Congenital CMV infection in the newborn is associated with progressive sensorineural hearing loss; however, effective CMV therapy with VGC can improve audiologic outcomes. Ongoing studies to demonstrate the effect of VGC in this setting are hampered by a poor understanding of the pharmacology of VGC and GCV in newborns, and the low blood volumes that can be safely collected from this population. We describe a simple method for determining systemic GCV concentrations using dried blood spot (DBS) samples. GCV was extracted from a single 6 mm punch via sonication in methanol, then quantified using liquid chromatography-tandem mass spectrometry. The assay was accurate and precise in the dynamic range of 10–10,000 ng/mL. GCV concentrations determined in DBS agreed well with GCV concentrations observed in serum. The assay was successfully applied to patient samples, and will be used to support pharmacokinetic studies in an ongoing clinical trial of VGC in infants with CMV-mediated hearing loss.

Some forms of sensorineural hearing loss involve damage or degenerative changes to the stria vascularis and/or other vascular structures in the cochlea. In animal models, many methods for anatomical assessment of cochlear vasculature exist, each with advantages and limitations. One methodology, corrosion casting, has proved useful in some species, however in the mouse model this technique is difficult to achieve because digestion of non vascular tissue results in collapse of the delicate cast specimen. We have developed a partial corrosion cast method that allows visualization of vasculature along much of the cochlear length but maintains some structural integrity of the specimen. We provide a detailed step-by-step description of this novel technique. We give some illustrative examples of the use of the method in mouse models of presbycusis and cytomegalovirus (CMV) infection.

Objectives In this study, we estimate an ex ante cost–benefit analysis of a Utah law directed at improving early cytomegalovirus (CMV) detection. Study design We use a differential cost of treatment analysis for publicly insured CMV-infected infants detected by a statewide hearing-directed CMV screening program. Methods Utah government administrative data and multi-hospital accounting data are used to estimate and compare costs and benefits for the Utah infant population. Results If antiviral treatment succeeds in mitigating hearing loss for one infant per year, the public savings will offset the public costs incurred by screening and treatment. If antiviral treatment is not successful, the program represents a net cost, but may still have non-monetary benefits such as accelerated achievement of diagnostic milestones. Conclusions The CMV education and treatment program costs are modest and show potential for significant cost savings.

Objective To review our experience treating hypopharyngeal branchial anomalies utilizing an open transcervical approach that: (1) includes recurrent laryngeal nerve (RLN) monitoring and identification if needed; (2) resection of tract if present; and (3) a superiorly based sternothyroid muscle flap for closure. Methods A retrospective chart review was performed to identify all patients at a tertiary level children's hospital with branchial anomalies from 2005 to 2014. The clinical presentation, evaluation, treatment and outcome were analyzed for those patients with hypopharyngeal branchial anomalies. Results Forty-seven patients who underwent excision of branchial anomalies with a known origin were identified. Thirteen patients had hypopharyngeal branchial anomalies. Six of these patients were treated by the authors of this study and are the focus of this analysis. All six underwent an open transcervical procedure with a sternothyroid muscle flap closure of a piriform sinus opening over a nine year period. Definitive surgery included a microlaryngoscopy and an open transcervical approach to close a fistula between the piriform sinus and neck with recurrent laryngeal nerve monitoring or dissection. A superiorly based sternothyroid muscle flap was used to close the sinus opening. There were no recurrences, recurrent laryngeal nerve injuries or other complications from these procedures. Conclusions This study supports complete surgical extirpation of the fistula tract using an open cervical approach, recurrent laryngeal nerve monitoring or identification, and rotational muscle flap closure to treat patients with hypopharyngeal branchial anomalies.

Objectives Determine whether the outcome of tympanoplasty in preschool children is different from that of older children. Study design Retrospective case series. Methods Retrospective review of children having undergone a primary tympanoplasty by 4 surgeons for a tympanic membrane perforation between 2002 and 2013. Results Data from 50 children age 2–4, 130 children age 5–7 and 105 children age 8–13 years old were reviewed. Median follow-up was 7.5 months. On crude analysis, the incidence of anatomical success was not significantly different between the different age groups (p = 0.38), the success rate was respectively 69.4%, 68.5% and 79.1% with an overall rate of 72.5%. 5.9% of all children required later insertion of tympanostomy tubes, 10.2% in preschool children. The post-operative audiology results were similar for all groups with a mean improvement of 9 dB in the air-bone gap. When limiting the analysis to the 155 children having at least 6 months of follow-up, the rate of success was respectively 50.0%, 60.8% and 74.0% (p = 0.10). After multivariate analysis controlling for the effect of surgeon, approach and etiology, the odds ratio of perforation was respectively 5.48, 2.27 and 1.00 for the different age groups. Conclusion Children younger than 4 years of age have the worst outcome after tympanoplasty. It remains uncertain whether the benefits of hearing improvement and quality of life may outweigh that of a high rate of a residual, usually smaller, perforation. Prospective studies are needed to confirm these results and delineate the patient characteristics and technique most likely to lead to successful results.

Objective Optimize the diagnosis of pediatric Sjogren's syndrome in children who present with parotitis. Methods Twenty children presented to a pediatric otolaryngology or rheumatology clinic with recurrent parotitis. Presenting symptoms, serologies, sialendoscopy findings, and minor salivary gland biopsy pathology results were reviewed. Results Twenty patients aged 3–17 years presented with recurrent parotitis. Ten percent of this cohort met the American-European Consensus Group adult diagnostic criteria for Sjogren's syndrome. Forty percent of this cohort met diagnosis of Sjogren's syndrome when utilizing Bartunkova's proposed pediatric criteria for diagnosis of Sjogren's syndrome. Conclusion Sjogren's syndrome is surprisingly common in pediatric patients who present with recurrent parotitis. Otolaryngologists who treat pediatric parotitis should have a high index of suspicion for Sjogren's syndrome. Level of Evidence 4.

Determine clinical signs or symptoms associated with a more complicated clinical course in patients with retropharyngeal abscesses (RPAs).Retrospective chart review at a tertiary care level Children's hospital. Main Outcome Measures Age, presenting signs and symptoms, laboratory tests, imaging results, antibiotic therapy, surgical approach, pathogens isolated, and duration of hospitalization were evaluated to determine any factors associated with a more complicated clinical course (CCC).Fifteen of one hundred thirty pediatric patients with RPA were identified with a complicated clinical course (CCC). Eight of the fifteen required more than one procedure before the abscess resolved. Patients with multiple abscess sites had a statistically significantly greater chance of requiring multiple procedures to clear the infections (p0.001). Another seven presented with airway obstruction requiring an admission into the Pediatric Intensive Care (PICU) and/or intubation. All the patients requiring admission to the PICU presented with signs or symptoms of airway obstruction compared to ten of the one hundred fifteen patients (8.7%) with a smooth clinical course (SCC) (p0.001). Five patients from the CCC group required a bronchoscopy to secure the airway; seven patients required intubation following incision and drainage of the abscess for an average of 5+/-3 days. There was no statistically significant difference between the two groups with respect to pathogens isolated, or antibiotics used.Our study suggests that patients with a CCC are more likely to present with airway obstruction or multiple abscess sites than patients with SCC.

Objectives The objective of this study is to examine the correlation between enlarged vestibular aqueduct (EVA) anomaly and other inner ear anomalies such as cochlear dysplasia, vestibulocochlear dysplasia and modiolar hypoplasia. Methods Retrospective chart review, with institutional review board approval, of patients with EVA who received treatment at Primary Children's Medical Center or University Hospital at the University of Utah, between 1997 and 2006. Review of radiographs was done to evaluate for the presence of EVA and other inner ear anomalies. Results Twenty patients (40 ears) were included in the study, 17 patients had bilateral EVA and three patients had unilateral EVA. There were 10 females and 10 males. The average age of all patients at the time of initial diagnosis was 1.7 years (0–6 years). Thirty-seven ears were shown to have EVA (92.5%). Of those ears with EVA, 29 (78.4%) had one or more inner ear anomalies. Twenty-three (62.2%) ears had cochlear dysplasia, six (16.2%) had vestibulocochlear dysplasia, and 18 (48.7%) had modiolar hypoplasia. Conclusions Using small field of view, thin section CT and/or MR imaging, cochleovestibular abnormalities are commonly identified in association with EVA.

To describe a different surgical approach to treat pediatric orbital floor fractures. We report the case of a 14-year-old boy who underwent a microscope-assisted repair of his orbital floor fracture 6 weeks after his injury. The microscope provided excellent visualization of the entire orbital floor defect and facilitated placement of a polyethylene implant. The repair was achieved without complications, and follow-up examinations demonstrated improvement in patient's enophthalmos and diplopia. We noted significant advantages with a microscope-assisted repair over an endoscopic technique for this condition. The operating microscope allows for two-handed surgery, does not require withdraw of the endoscope for cleaning, and provides depth of field. A larger patient population and longer follow-up are needed to better assess the clinical efficacy of this technique.

Summary Objective To determine whether a subtotal bovie tonsillectomy decreases postoperative pain following adenotonsillectomy. Methods A prospective, randomized, double-blinded clinical trial was undertaken at a University Pediatric Children's Hospital and included 39 otherwise healthy children, aged 2–12 years, undergoing adenotonsillectomy. Patients were randomized to receive either a subtotal or total removal of the tonsils utilizing an electrocautery technique. The main outcome measures included a visual analog scale (VAS) at rest and while eating, time to take 100 cm 3 of fluid, throat, neck, and ear pain, quantity of liquids, activity level and incidence of emesis and retching. Results A repeated measures analysis using a repeated measures ANOVA failed to demonstrate a statistically significant impact with either treatment for VAS at rest or while eating ( p = 0.52 and 0.48, respectively). A repeated measures analysis did not show either procedure significantly affecting throat, neck or ear pain, or liquid quantity. Time to take 100 cm 3 liquids and the incidence of emesis or retching were found not to be statistically significantly different between the treatment groups. Conclusion Subtotal tonsillectomy via an electrocautery technique does not reduce postoperative pain or improve outcome parameters. Subtotal tonsillectomy with this technique is not recommended for this patient population.

Myxomas are benign, slow growing neoplasms derived from mesenchyme. While these tumors most frequently occur in the myocardium, the other sites most commonly affected are the maxilla and mandible. Nevertheless, myxoma is a very uncommon lesion of the midface, particularly in the pediatric population. We present two reports of infant children with midfacial myxomas. The clinical features, radiographic evaluation and treatment of these cases will be presented.

There is little consensus on the optimal management of postparotidectomy salivary fistulas. Timely treatment is important since fistulas may result in wound dehiscence and infection. Management options include pressure dressings, total parotidectomy, tympanic neurectomy, graft interpositioning, surgical closure of the tract, radiation therapy, and pharmacotherapy. Unfortunately, many therapies require weeks to months for resolution and possess additional risks. The affected patient often suffers social embarrassment from the drainage. Through our work with neurologically impaired children with sialorrhea, we have had success with using glycopyrrolate, an anticholinergic frequently used to decrease salivary secretions. We present a case of a patient with a postparotidectomy fistula which was successfully treated with glycopyrrolate and pressure dressings. The rationale and potential use of glycopyrrolate for the treatment of a salivary fistula are the focus of this presentation.

There have been numerous reports of various types of heterotopic tissue in the head and neck. Heterotopic cartilage, gastric tissue, thyroid, and salivary gland in such various locations as tongue, gingiva, palate, nasopharynx, parapharyngeal space, and neck have been frequently reported, Heterotopic brain in the parapharyngeal space causing airway obstruction in the neonate has been rarely described. These benign masses are capable of expansion and because of their location, can lead to significant airway and feeding difficulties. We describe 3 cases of heterotopic brain tissue in the parapharyngeal space causing feeding difficulties and airway obstruction in the neonatal period. Two were initially misdiagnosed as lymphatic malformations. In the third, a nine month delay in diagnosis occurred. The diagnostic features of heterotopic brain in this location and some management suggestions in treating such a lesion are discussed.

The great majority of cases of sinusitis are uncomplicated. These infections, however, can result in significant morbidity and often mortality. One must be vigilant in detecting orbital or intracranial spread and iatrogenic injury from surgery because manifestations of these complications can be protean. A good knowledge of the anatomy, the clinical signs, and some key laboratory data is crucial to successful treatment of this challenging disease.

A technique to surgically manage neonatal subglottic stenosis is described in this report. Our extubation and decannulation rate in 17 patients who have undergone this procedure has been higher than that with the anterior cricoid split procedure. Early accidental extubation has not been problematic; this complication has been a difficulty with the anterior cricoid split procedure. The indications of this technique are being expanded to address subglottic pathology in olden children and for posterior cricoid augmentation.

Endoscopic sinus surgery in the sphenoid sinus can be complicated by vision loss and severe life-threatening hemorrhage. In many cases, injury to the carotid artery leads to massive brain injury and death. However, injury to the carotid artery can be treated successfully if the surgeon follows an appropriate protocol for management. This report reviews a successful case study and discusses how to find the sphenoid sinus and avoid the carotid artery. It outlines a protocol for treatment of carotid injuries based on hemorrhage control and neuroradiologic embolization.