Validation of a Novel Mobile Application for Assessing Pediatric Tracheostomy Emergency Simulations.

Objective: Pediatric tracheostomy is associated with high morbidity and mortality, yet clinician knowledge and quality of tracheostomy care may vary widely. In situ simulation is effective at detecting and mitigating related latent safety threats, but evaluation via retrospective video review has disadvantages (eg, delayed analysis, and potential data loss). We evaluated whether a novel mobile application is accurate and reliable for assessment of in situ tracheostomy emergency simulations. Methods: A novel mobile application was developed for assessment of tracheostomy emergency in situ simulation team performance. After 1.25 hours of training, 6 raters scored 10 tracheostomy emergency simulation videos for the occurrence and timing of 12 critical steps. To assess accuracy, rater scores were compared to a reference standard to determine agreement for occurrence or absence of critical steps and a timestamp within ±5 seconds. Interrater reliability was determined through Cohen's and Fleiss' kappa and intraclass correlation coefficient. Results: Raters had 86.0% agreement with the reference standard when considering step occurrence and timing, and 92.8% agreement when considering only occurrence. The average timestamp difference from the reference standard was 1.3 ± 18.5 seconds. Overall interrater reliability was almost perfect for both step occurrence (Fleiss' kappa of 0.81) and timing of step (intraclass correlation coefficient of 0.99). Discussion: Using our novel mobile application, raters with minimal training accurately and reliably assessed videos of tracheostomy emergency simulations and identified areas for future refinement. Implications for Practice: With refinements, this innovative mobile application is an effective tool for real-time data capture of time-critical steps in in situ tracheostomy emergency simulations.

Asymmetric Ocular Vestibular Evoked Myogenic Potentials in Pediatric Vestibular Migraine.

OBJECTIVE: Although ocular vestibular evoked myogenic potentials (oVEMP) abnormalities have been associated with vestibular migraine (VM) in adults, no studies have evaluated this in pediatric patients with VM. oVEMP asymmetry with normal cervical VEMP (cVEMP) findings may be a reliable VM biomarker in adults. We characterize VEMP results among pediatric patients with VM and benign recurrent vertigo of childhood (BRVC), a migraine precursor, and compare these results with VEMP findings from pediatric patients with nonmigrainous vestibular diagnoses. METHODS: Four hundred seventy-four pediatric patients were evaluated over a 3-year period in a multidisciplinary pediatric vestibular clinic, 139 of whom met the inclusion criteria. Records were reviewed for history, audiometry, and vestibular testing results. VEMP testing was performed with a 500-Hz tone burst. Based on adult normative data, oVEMP asymmetry was defined as greater than 33% interaural difference; cVEMP asymmetry was defined as greater than 41%. RESULTS: Eighty subjects had VM or BRVC (mean [standard deviation] 12.8 [3.8] yr; range, 4.3-18.2 yr). Fifty-nine subjects received vestibular diagnoses other than VM or BRVC (nonmigrainous group; mean [standard deviation] age, 13.0 [3.6] yr; range, 5.14-18.9 yr). A greater proportion of the VM/BRVC group demonstrated oVEMP asymmetry with normal cVEMP as compared with the nonmigrainous group (22.5% versus 10.1%; p < 0.05). Zero subjects in the VM/BRVC group demonstrated cVEMP asymmetry versus three subjects (4.9%) of the nonmigrainous group. CONCLUSIONS: VM and BRVC are notable causes of migraine-related vertigo among pediatric patients. Of VM/BRVC patients, 22.5% had oVEMP asymmetry with normal cVEMP. Similar to the adult VM population, this finding may be a useful biomarker in the right clinical setting for pediatric VM. LEVEL OF EVIDENCE: 4.

Endovascular Embolization for Control of Post-Tonsillectomy Hemorrhage.

Post-operative hemorrhage is a potentially life-threatening complication of tonsillectomy. While standard surgical maneuvers including the use of electrocautery, application of topical hemostatic agents, direct pressure, and suturing of the tonsillar pillars have traditionally been used for the treatment of severe bleeding, endovascular approaches are an important adjunct when other techniques are unsuccessful. Here, we describe the case of a 10-year-old female who presented with severe bleeding four days after tonsillectomy and adenoidectomy for chronic tonsillitis. She was taken emergently to the operating room where pulsatile bleeding was noted from the right inferior tonsillar pole. Hemostasis could not be achieved using electrocautery despite multiple attempts. The patient was taken for emergent angiography, which demonstrated an irregularity of the right tonsillar artery consistent with arterial vasospasm, and which corresponded to the intraoral site of bleeding localized by the surgeon. Coil embolization of the tonsillar artery was successfully performed, and the patient experienced no further bleeding. We conclude that endovascular embolization of branches of the external carotid artery is an effective treatment for severe post-tonsillectomy hemorrhage in children and should be considered when attempts at surgical control are ineffective. This procedure requires exceptional collaboration between the surgical, radiology, and anesthesia teams.

Endoscopic anterior-posterior cricoid split to avoid tracheostomy in infants with bilateral vocal fold paralysis.

INTRODUCTION: Infants with bilateral vocal fold paralysis (BVFP) can present with stridor and respiratory distress necessitating tracheostomy. The endoscopic anterior-posterior cricoid split (APCS) with balloon dilation procedure has been described as an alternative to tracheostomy in these patients. Here, we report our institution's preliminary experience with APCS and evaluate patient factors that may predispose to the success or failure of this procedure in infants with BVFP. METHODS: Electronic charts of patients who underwent APCS with balloon dilation at a single institution were reviewed for the following variables: patient demographics, comorbidities, etiology of vocal fold paralysis, symptoms at presentation, need for respiratory support, intra-operative findings, duration of intubation, perioperative medical treatments, subsequent airway management, and findings of follow-up evaluations. APCS was considered successful if the patient did not undergo tracheostomy. RESULTS: Six patients underwent APCS with balloon dilation between August 2014 and October 2019. Four patients (66.7%) were male, and 5 of 6 (83.3%) were born full term. The etiology of vocal fold paralysis was idiopathic in four patients (66.7%) and associated with a neuromuscular disorder and hydrocephalus in the remaining two patients. Mean age at the time of the procedure was 10.3 weeks. Three infants (50%) avoided tracheostomy and had marked alleviation of airway symptoms. Three patients who required tracheostomy had more severe respiratory symptoms pre-operatively, requiring either intubation or positive pressure support. Among all patients, there were no mortalities in our series. CONCLUSION: APCS is safe and may be effective at the elimination of airway symptoms in select infants with BVFP, avoiding the need for tracheostomy, however more investigation is needed to establish its precise role in this patient population.

Predictors of the need for tracheostomy in the neonatal intensive care unit.

OBJECTIVES: Many infants in the neonatal intensive care unit (NICU) require prolonged periods of respiratory support. Microlaryngoscopy and bronchoscopy (MLB) is performed to evaluate for airway pathology and facilitate decision-making regarding further airway interventions or tracheostomy. The objectives of this study are to describe the operative findings of MLB performed on infants in the NICU and determine which pre-operative characteristics or operative findings are predictive of the need for tracheostomy. METHODS: The medical records of preterm inpatients in the NICU at a single tertiary care hospital who underwent MLB between January 1, 2013 and January 7, 2016 were reviewed. Baseline and demographic characteristics and intra-operative findings were compared between patients who underwent tracheostomy and those who were successfully weaned from respiratory support. RESULTS: Seventy-three preterm patients underwent MLB for respiratory failure, of whom 41 (56.2%) underwent tracheostomy. Patients who underwent tracheostomy had lower mean gestational age (27.4 vs. 30.5 weeks), higher prevalence of bronchopulmonary dysplasia (73.2% vs. 37.5%), lower mean birth weight (1.1 kg vs. 1.6 kg), and a greater number of extubation events (5.2 vs. 3.0) than those who weaned from respiratory support. Abnormal MLB findings were common in both groups, though no single MLB finding differed significantly between groups. CONCLUSIONS: Preterm infants in the NICU with gestational age ≤30 weeks, birth weight <1.5 kg, severe pulmonary disease, and who have failed more than 3 extubation attempts are more likely to require tracheostomy.

Congenital soft tissue stenosis of the external auditory canal with canal cholesteatoma: Case report and literature review.

Congenital external auditory canal stenosis (EACS) is a spectrum of abnormalities affecting the external and middle ear. We report a 6 year-old patient with EACS affecting the lateral fibrocartilaginous canal that was successfully repaired. This patient highlights a variant of EACS characterized by lateral soft tissue narrowing with normal osseous development. Most previous studies of CAA have described severe forms associated with complete atresia, bony stenosis, and middle ear malformations. Stenosis affecting only the fibrocartilaginous canal is a milder form resulting from premature arrest of the canalization process during embryologic development, and may predispose to cholesteatoma formation.

Corrigendum: Stereocilia-staircase spacing is influenced by myosin III motors and their cargos espin-1 and espin-like.

This corrects the article DOI: 10.1038/ncomms10833.

Stereocilia-staircase spacing is influenced by myosin III motors and their cargos espin-1 and espin-like.

Hair cells tightly control the dimensions of their stereocilia, which are actin-rich protrusions with graded heights that mediate mechanotransduction in the inner ear. Two members of the myosin-III family, MYO3A and MYO3B, are thought to regulate stereocilia length by transporting cargos that control actin polymerization at stereocilia tips. We show that eliminating espin-1 (ESPN-1), an isoform of ESPN and a myosin-III cargo, dramatically alters the slope of the stereocilia staircase in a subset of hair cells. Furthermore, we show that espin-like (ESPNL), primarily present in developing stereocilia, is also a myosin-III cargo and is essential for normal hearing. ESPN-1 and ESPNL each bind MYO3A and MYO3B, but differentially influence how the two motors function. Consequently, functional properties of different motor-cargo combinations differentially affect molecular transport and the length of actin protrusions. This mechanism is used by hair cells to establish the required range of stereocilia lengths within a single cell.

Rare Upper Airway Anomalies.

A broad spectrum of congenital upper airway anomalies can occur as a result of errors during embryologic development. In this review, we will describe the clinical presentation, diagnosis, and management strategies for a few select, rare congenital malformations of this system. The diagnostic tools used in workup of these disorders range from prenatal tests to radiological imaging, swallowing evaluations, indirect or direct laryngoscopy, and rigid bronchoscopy. While these congenital defects can occur in isolation, they are often associated with disorders of other organ systems or may present as part of a syndrome. Therefore workup and treatment planning for patients with these disorders often involves a team of multiple specialists, including paediatricians, otolaryngologists, pulmonologists, speech pathologists, gastroenterologists, and geneticists.

Superior canal dehiscence length and location influences clinical presentation and audiometric and cervical vestibular-evoked myogenic potential testing.

Superior canal dehiscence (SCD) is caused by an absence of bony covering of the arcuate eminence or posteromedial aspect of the superior semicircular canal. However, the clinical presentation of SCD syndrome varies considerably, as some SCD patients are asymptomatic and others have auditory and/or vestibular complaints. In order to determine the basis for these observations, we examined the association between SCD length and location with: (1) auditory and vestibular signs and symptoms; (2) air conduction (AC) loss and air-bone gap (ABG) measured by pure-tone audiometric testing, and (3) cervical vestibular-evoked myogenic potential (cVEMP) thresholds. 104 patients (147 ears) underwent SCD length and location measurements using a novel method of measuring bone density along 0.2-mm radial CT sections. We found that patients with auditory symptoms have a larger dehiscence (median length: 4.5 vs. 2.7 mm) with a beginning closer to the ampulla (median location: 4.8 vs. 6.4 mm from ampulla) than patients with no auditory symptoms (only vestibular symptoms). An increase in AC threshold was found as the SCD length increased at 250 Hz (95% CI: 1.7-4.7), 500 Hz (95% CI: 0.7-3.5) and 1,000 Hz (95% CI: 0.0-2.5), and an increase in ABG as the SCD length increased at 250 Hz (95% CI: 2.0-5.3), 500 Hz (95% CI: 1.6-4.6) and 1,000 Hz (95% CI: 1.3-3.3) was also seen. Finally, a larger dehiscence was associated with lowered cVEMP thresholds at 250 Hz (95% CI: -4.4 to -0.3), 500 Hz (95% CI: -4.1 to -1.0), 750 Hz (95% CI: -4.2 to -0.7) and 1,000 Hz (95% CI: -3.6 to -0.5) and a starting location closer to the ampulla at 250 Hz (95% CI: 1.3-5.1), 750 Hz (95% CI: 0.2-3.3) and 1,000 Hz (95% CI: 0.6-3.5). These findings may help to explain the variation of signs and symptoms seen in patients with SCD syndrome.

Identification of inputs to olivocochlear neurons using transneuronal labeling with pseudorabies virus (PRV).

Olivocochlear (OC) neurons respond to sound and provide descending input that controls processing in the cochlea. The identities of neurons in the pathways providing inputs to OC neurons are incompletely understood. To explore these pathways, the retrograde transneuronal tracer pseudorabies virus (Bartha strain, expressing green fluorescent protein) was used to label OC neurons and their inputs in guinea pigs. Labeling of OC neurons began 1 day after injection into the cochlea. On day 2 (and for longer survival times), transneuronal labeling spread to the cochlear nucleus, inferior colliculus, and other brainstem areas. There was a correlation between the numbers of these transneuronally labeled neurons and the number of labeled medial (M) OC neurons, suggesting that the spread of labeling proceeds mainly via synapses on MOC neurons. In the cochlear nucleus, the transneuronally labeled neurons were multipolar cells including the subtype known as planar cells. In the central nucleus of the inferior colliculus, transneuronally labeled neurons were of two principal types: neurons with disc-shaped dendritic fields and neurons with dendrites in a stellate pattern. Transneuronal labeling was also observed in pyramidal cells in the auditory cortex and in centers not typically associated with the auditory pathway such as the pontine reticular formation, subcoerulean nucleus, and the pontine dorsal raphe. These data provide information on the identity of neurons providing input to OC neurons, which are located in auditory as well as non-auditory centers.

Auditory brainstem circuits that mediate the middle ear muscle reflex.

The middle ear muscle (MEM) reflex is one of two major descending systems to the auditory periphery. There are two middle ear muscles (MEMs): the stapedius and the tensor tympani. In man, the stapedius contracts in response to intense low frequency acoustic stimuli, exerting forces perpendicular to the stapes superstructure, increasing middle ear impedance and attenuating the intensity of sound energy reaching the inner ear (cochlea). The tensor tympani is believed to contract in response to self-generated noise (chewing, swallowing) and non-auditory stimuli. The MEM reflex pathways begin with sound presented to the ear. Transduction of sound occurs in the cochlea, resulting in an action potential that is transmitted along the auditory nerve to the cochlear nucleus in the brainstem (the first relay station for all ascending sound information originating in the ear). Unknown interneurons in the ventral cochlear nucleus project either directly or indirectly to MEM motoneurons located elsewhere in the brainstem. Motoneurons provide efferent innervation to the MEMs. Although the ascending and descending limbs of these reflex pathways have been well characterized, the identity of the reflex interneurons is not known, as are the source of modulatory inputs to these pathways. The aim of this article is to (a) provide an overview of MEM reflex anatomy and physiology, (b) present new data on MEM reflex anatomy and physiology from our laboratory and others, and (c) describe the clinical implications of our research.