Faculty Bibliography

This multi-center study examined the safety and effectiveness of cochlear implantation of children between 9 and 11 months of age. The intended impact was to support practice regarding candidacy assessment and prognostic counseling of pediatric cochlear implant candidates. Data in the clinical chart of children implanted at 9-11 months of age with Cochlear Ltd devices at five cochlear implant centers in the United States and Canada were included in analyses. The study included data from two cohorts implanted with one or two Nucleus devices during the periods of January 1, 2012-December 31, 2017 (Cohort 1, n = 83) or between January 1, 2018 and May 15, 2020 (Cohort 2, n = 50). Major adverse events (requiring another procedure/hospitalization) and minor adverse events (managed with medication alone or underwent an expected course of treatment that did not require surgery or hospitalization) out to 2 years post-implant were monitored and outcomes measured by audiometric thresholds and parent-reports on the IT-MAIS and LittlEARS questionnaires were collected. Results revealed 60 adverse events in 41 children and 227 ears implanted (26%) of which 14 major events occurred in 11 children; all were transitory and resolved. Improved hearing with cochlear implant use was shown in all outcome measures. Findings reveal that the procedure is safe for infants and that they show clear benefits of cochlear implantation including increased audibility and hearing development.

OBJECTIVE/UNASSIGNED: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/UNASSIGNED: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/UNASSIGNED: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/UNASSIGNED: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/UNASSIGNED: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.

BACKGROUND/UNASSIGNED:Meniere's disease (MD) is defined by episodic vertigo, unilateral sensorineural hearing loss and fluctuating aural symptoms. Due to the variable clinical presentation, objective tests of MD may have significant diagnostic utility. Head kinematics derived from a head-mounted display (HMD) have demonstrated to be sensitive to vestibular dysfunction. The purpose of this pilot study was to investigate whether head sway can differentiate between patients with MD, vestibular hypofunction (VH) and healthy controls. MATERIALS/METHODS/UNASSIGNED:80 adults (30 healthy controls, 32 with VH, and 18 with MD) were recruited from a tertiary vestibular clinic. All underwent a postural control assessment using the HTC Vive Pro Eye HMD that recorded head sway in the anterior-posterior (AP), medio-lateral (ML), pitch, yaw and roll direction. Participants were tested with 2 levels of visual load: a static versus oscillating star display. Each scene lasted 60 s and was repeated twice. Sway in each direction was quantified using root mean square velocity (VRMS) for the first 20 s and full 60 s of each scene. RESULTS/UNASSIGNED:Static visual: participants with VH showed significantly larger head VRMS than controls in the AP (60 s and 20 s) and pitch (20 s) directions. Dynamic visual: participants with VH showed significantly larger head VRMS than controls all directions for both the 60 and 20 s analysis. Participants with MD did not differ significantly from the control or the VH group. CONCLUSION/UNASSIGNED:While limited in numbers, Patients with MD had a high variability in head sway in all directions, and their average head sway was between controls and those with VH. A larger sample as well as patients with worse symptoms at time of testing could elucidate whether head sway via HMD could become a viable test in this population. A similar finding between 20- and 60-s scene and the full portability of the system with an in-clinic testing setup could help these future endeavors. Head sway derived from HMD is sensitive to VH and can be clinically useful as an outcome measure to evaluate sensory integration for postural control.

BACKGROUND/UNASSIGNED:The velocity storage mechanism of the central vestibular system is closely associated with the vestibulo-ocular reflex (VOR), but also contributes to the sense of orientation in space and the perception of self-motion. We postulate that mal de débarquement syndrome (MdDS) is a consequence of inappropriate sensory adaptation of velocity storage. The premise that a maladapted velocity storage may be corrected by spatial readaptation of the VOR has recently been translated into the development of the first effective treatment for MdDS. However, this treatment's initial impact may be reversed by subsequent re-triggering events. Presently, we hypothesized that MdDS symptoms could alternatively be reduced by attenuating the velocity storage contribution in the central vestibular pathways. METHODS/UNASSIGNED:). To attenuate velocity storage, Group 1 underwent a progressively intensifying series of low-frequency earth-vertical oscillatory rotation coupled to conflicting visual stimuli. Group 2 underwent an established protocol combining head tilts and visual stimulation, designed to correct maladapted spatial orientation but not change the velocity storage strength. The symptom severity was self-rated on an 11-point scale and reported before and up to 6 months after the treatment. RESULTS/UNASSIGNED: < 0.001), but paralleling previous findings, symptoms often returned subsequently. CONCLUSION/UNASSIGNED:Attenuation of velocity storage shows promise as a lasting remedy for MdDS that can complement the VOR readaptation approach.

OBJECTIVE:To provide recommendations for a comprehensive management approach for infants and children presenting with symptoms or signs of aspiration. METHODS:Three rounds of surveys were sent to authors from 23 institutions worldwide. The threshold for the critical level of agreement among respondents was set at 80 %. To develop the definition of "intractable aspiration," each author was first asked to define the condition. Second, each author was asked to complete a 5-point Likert scale to specify the level of agreement with the definition derived in the first step. RESULTS:Recommendations by the authors regarding the clinical presentation, diagnostic considerations, and medical and surgical management options for aspiration in children. CONCLUSION/CONCLUSIONS:Approach to pediatric aspiration is best achieved by implementing a multidisciplinary approach with a comprehensive investigation strategy and different treatment options.

BACKGROUND:The prognosis for Li-Fraumeni syndrome (LFS) patients with medulloblastoma (MB) is poor. Comprehensive clinical data for this patient group is lacking, challenging the development of novel therapeutic strategies. Here, we present clinical and molecular data on a retrospective cohort of pediatric LFS MB patients. METHODS:In this multinational, multicenter retrospective cohort study, LFS patients under 21 years with MB and class 5 or class 4 constitutional TP53 variants were included. TP53 mutation status, methylation subgroup, treatment, progression free- (PFS) and overall survival (OS), recurrence patterns, and incidence of subsequent neoplasms were evaluated. RESULTS:The study evaluated 47 LFS individuals diagnosed with MB, mainly classified as DNA methylation subgroup "SHH_3" (86%). The majority (74%) of constitutional TP53 variants represented missense variants. The 2- and 5-year (y-) PFS were 36% and 20%, and 2- and 5y-OS were 53% and 23%, respectively. Patients who received post-operative radiotherapy (RT) (2y-PFS: 44%, 2y-OS: 60%) or chemotherapy before RT (2y-PFS: 32%, 2y-OS: 48%) had significantly better clinical outcome then patients who were not treated with RT (2y-PFS: 0%, 2y-OS: 25%). Patients treated according to protocols including high-intensity chemotherapy and patients who received only maintenance-type chemotherapy showed similar outcomes (2y-PFS: 42% and 35%, 2y-OS: 68% and 53%, respectively). CONCLUSIONS:LFS MB patients have a dismal prognosis. In the presented cohort use of RT significantly increased survival rates, whereas chemotherapy intensity did not influence their clinical outcome. Prospective collection of clinical data and development of novel treatments are required to improve the outcome of LFS MB patients.

BACKGROUND AND OBJECTIVES/OBJECTIVE:Clinical registries are critical for modern surgery and underpin outcomes research, device monitoring, and trial development. However, existing approaches to registry construction are labor-intensive, costly, and prone to manual error. Natural language processing techniques combined with electronic health record (EHR) data sets can theoretically automate the construction and maintenance of registries. Our aim was to automate the generation of a spine surgery registry at an academic medical center using regular expression (regex) classifiers developed by neurosurgeons to combine domain expertise with interpretable algorithms. METHODS:We used a Hadoop data lake consisting of all the information generated by an academic medical center. Using this database and structured query language queries, we retrieved every operative note written in the department of neurosurgery since our transition to EHR. Notes were parsed using regex classifiers and compared with a random subset of 100 manually reviewed notes. RESULTS:A total of 31 502 operative cases were downloaded and processed using regex classifiers. The codebase required 5 days of development, 3 weeks of validation, and less than 1 hour for the software to generate the autoregistry. Regex classifiers had an average accuracy of 98.86% at identifying both spinal procedures and the relevant vertebral levels, and it correctly identified the entire list of defined surgical procedures in 89% of patients. We were able to identify patients who required additional operations within 30 days to monitor outcomes and quality metrics. CONCLUSION/CONCLUSIONS:This study demonstrates the feasibility of automatically generating a spine registry using the EHR and an interpretable, customizable natural language processing algorithm which may reduce pitfalls associated with manual registry development and facilitate rapid clinical research.

OBJECTIVE:The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN/METHODS:Survey. SETTING/METHODS:A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS:House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS:Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.