Faculty Bibliography

BACKGROUND:We created a clinical virtual reality application for vestibular rehabilitation. Our app targets contextual sensory integration (C.S.I.) where patients are immersed in safe, increasingly challenging environments while practicing various tasks (e.g., turning, walking). The purpose of this pilot study was to establish the feasibility of a randomized controlled trial comparing C.S.I. training to traditional vestibular rehabilitation. METHODS:Thirty patients with vestibular dysfunction completed the Dizziness Handicap Inventory (DHI), Activities-Specific Balance Confidence Scale (ABC), Visual Vertigo Analog Scale (VVAS), Functional Gait Assessment (FGA), Timed-Up-and-Go (TUG), and Four-Square Step Test (FSST). Following initial assessment, the patients were randomized into 8 weeks (once per week in clinic + home exercise program) of traditional vestibular rehabilitation or C.S.I. training. Six patients had to stop participation due to the covid-19 pandemic, 6 dropped out for other reasons (3 from each group). Ten patients in the traditional group and 8 in the C.S.I group completed the study. We applied an intention to treat analysis. RESULTS:Following intervention, we observed a significant main effect of time with no main effect of group or group by time interaction for the DHI (mean difference - 18.703, 95% CI [-28.235, -9.172], p = 0.0002), ABC (8.556, [0.938, 16.174], p = 0.028), VVAS, (-13.603, [-25.634, -1.573], p = 0.027) and the FGA (6.405, [4.474, 8.335], p < 0.0001). No changes were observed for TUG and FSST. CONCLUSION:Patients' symptoms and function improved following either vestibular rehabilitation method. C.S.I training appeared comparable but not superior to traditional rehabilitation. TRIAL REGISTRATION:This study (NCT04268745) was registered on clincaltrials.gov and can be found at https://clinicaltrials.gov/ct2/show/NCT04268745 .

OBJECTIVE/UNASSIGNED:To report our institutional experience in diagnosing and surveilling patients with infantile subglottic hemangioma (SGH) using in-office flexible fiberoptic laryngoscopy (FFL) with video technology, without requiring operative endoscopy in the era of propranolol use. METHODS/UNASSIGNED:A retrospective case series was conducted on 4 children diagnosed with SGH between 2016 and 2022 at our institution. RESULTS/UNASSIGNED:Awake FFL with video technology provided adequate visualization of SGH lesions for diagnosis, without any complications. Serial examinations of the airway were performed in the outpatient setting and each SGH gradually regressed, with marked improvement in respiratory symptoms within 48 hours of oral propranolol initiation. CONCLUSION/UNASSIGNED:Our findings showed that in select patients, FFL with video technology can successfully identify SGH lesions without general anesthesia exposure. FFL may be used as a low-risk screening tool for propranolol therapy initiation in some patients, but operative endoscopy should remain the gold standard procedure for others. By utilizing FFL in this manner, it is possible to diagnose SGH lesions and start propranolol therapy without exposing all patients to the risks of operative endoscopy.

BACKGROUND:Prospective data on maintenance therapy with bevacizumab for persons with NF2-related schwannomatosis (NF2-SWN) is lacking. In this prospective multicenter phase II study, we evaluated the efficacy, safety, and tolerability of bevacizumab for maintenance therapy in children and adults with NF2-SWN and hearing loss due to vestibular schwannomas (VS). METHODS:Following induction therapy, participants received bevacizumab 5 mg/kg every 3 weeks for 18 months. Participants were monitored for changes in hearing, tumor size, and quality of life (QOL), and for adverse events. Hearing loss was defined as a statistically significant decline in word recognition score (WRS) or pure-tone average compared to the study baseline; tumor growth was defined as >20% increase in volume compared to baseline. RESULTS:Twenty participants with NF2-SWN (median age 23.5 years; range, 12.5-62.5 years) with hearing loss in the target ear (median WRS 70%, range 2%-94%) received maintenance bevacizumab. Freedom from hearing loss in the target ear was 95% after 48 weeks, 89% after 72 weeks, and 70% after 98 weeks. Freedom from tumor growth in the target VS was 94% after 48 weeks, 89% after 72 weeks, and 89% after 98 weeks. NF2-related QOL remained stable for 98 weeks whereas tinnitus-related distress decreased. Maintenance bevacizumab was well tolerated, with 3 participants (15%) discontinuing treatment due to adverse events. CONCLUSIONS:Maintenance bevacizumab (5 mg/kg every 3 weeks) is associated with high rates of hearing and tumor stability during 18 months of follow-up. No new unexpected adverse events related to bevacizumab were identified in this population.

OBJECTIVE:In certain cases, clinicians may consider continued observation of a vestibular schwannoma after initial growth is detected. The aim of the current work was to determine if patients with growing sporadic vestibular schwannomas could be stratified by the likelihood of subsequent growth based on initial growth behavior. STUDY DESIGN/METHODS:Slice-by-slice volumetric tumor measurements from 3,505 serial magnetic resonance imaging studies were analyzed from 952 consecutively treated patients. SETTING/METHODS:Three tertiary-referral centers. PATIENTS/METHODS:Adults with sporadic vestibular schwannoma. INTERVENTIONS/METHODS:Wait-and-scan. MAIN OUTCOME MEASURES/METHODS:Composite end point of subsequent growth- or treatment-free survival rates, where growth is defined as an additional increase of at least 20% in tumor volume from the volume at the time of initial growth. RESULTS:Among 405 patients who elected continued observation despite documented growth, stratification, of volumetric growth rate into less than 25% (reference: n = 107), 25 to less than 50% (hazard ratio [HR], 1.39; p = 0.06; n = 96), 50 to less than 100% (HR, 1.71; p = 0.002; n = 112), and at least 100% (HR, 2.01; p < 0.001; n = 90) change per year predicted the likelihood of future growth or treatment. Subsequent growth- or treatment-free survival rates (95% confidence interval) at year 5 after detection of initial growth were 31% (21-44%) for those with less than 25% growth per year, 18% (10-32%) for those with 25 to less than 50%, 15% (9-26%) for those with 50 to less than 100%, and 6% (2-16%) for those with at least 100%. Neither patient age ( p = 0.15) nor tumor volume at diagnosis ( p = 0.95) significantly differed across stratification groups. CONCLUSIONS:At the time of diagnosis, clinical features cannot consistently predict which tumors will ultimately display aggressive behavior. Stratification by volumetric growth rate at the time of initial growth results in a stepwise progression of increasing likelihood of subsequent growth. When considering continued observation after initial growth detection, almost 95% of patients who have tumors that double in volume between diagnosis and the first detection of growth demonstrate further tumor growth or undergo treatment if observed to 5 years.

OBJECTIVE/UNASSIGNED:To report patient tolerance in office-based blue laser therapy for vocal fold lesions. METHODS/UNASSIGNED:A retrospective review of the medical records of patients who underwent office-based blue laser therapy for lesions of the vocal folds between November 2021 and February 2023 was conducted. A total of 48 patients were included. Patient tolerance was assessed using the Iowa Satisfaction with Anesthesia Scale (IOWA). Patient tolerance was analyzed in relation to patients' demographic characteristics, disease type and severity, and procedure-related factors. RESULTS/UNASSIGNED:value .041). There was a moderate and significant negative correlation between vocal fold movement VAS, swallowing VAS, and tolerance score. The mean total duration of the procedures was 10.38 ± 4.8 min. There was a mild negative correlation between the duration of the procedure and tolerance. CONCLUSION/UNASSIGNED:Office-based blue laser therapy for vocal fold lesions is a well-tolerated procedure. Patient should be instructed how to breathe quietly and avoid swallows to improve tolerance to surgery. LEVEL OF EVIDENCE/UNASSIGNED:4.

BACKGROUND:Cochlear implants (CI) are the treatment of choice for individuals with severe to profound sensorineural hearing loss. A small group of patients, with pathology central to the cochlea, cannot benefit from CI. Examples in children include absence of the cochlear-nerve or cochlear aplasia. In these cases, implantation of an auditory brainstem implant (ABI), directly stimulating the cochlear nucleus, bypassing the inner-ear and auditory-nerve, may be beneficial. OBJECTIVES/OBJECTIVE:Describe a series of children with ABI's treated in Shaare-Zedek, including the first ABI implantation in Israel (2017). METHODS:Of 9 patients with ABI's treated in Shaare Zedek Medical Center ,7 were children implanted between ages 2-8.6 years. Five boys and two girls. Surgeries were conducted in collaboration between neurosurgeons, neurotologists and audiologists (five implanted in Shaare-Zedek and two in New-York University). Follow-up was between 2-6 years. Hearing evaluation was conducted, mainly, with audiograms, categories of auditory performance (CAP), speech perception testing when possible and estimation of device use per day. RESULTS:Six of the seven children, who initially underwent unsuccessful CI, had deficient auditory-nerves. One child had cochlear-aplasia. In 3 children hearing loss was part of the CHARGE syndrome. CAP scores ranged from 0-7 (0,1,3,5,5,7). One child was able to achieve open-set speech perception. CONCLUSIONS:Although functional auditory outcomes for children with ABI are inferior to CI recipients and are highly variable, some children were able to obtain significant benefit. In these children, who are not candidates for CI, the ABI presents the only chance for auditory awareness and may be recommended. DISCUSSION/CONCLUSIONS:John Thomas Roland is a consultant and recipient of research support from Cochlear Americas.

OBJECTIVES/OBJECTIVE:Lacrimal gland cancer is a rare malignancy with little data known about its pathologic characteristics or optimal management. We performed a large database analysis using the National Cancer Database (NCDB) to elucidate this unusual condition. METHODS:Patients with lacrimal gland cancer diagnosed between 2004 and 2018 were included in the analysis. Using available clinical data, we excluded all patients with histologies likely reflective of lacrimal sac or duct cancer, which are coded similarly to lacrimal gland cancer in the NCDB. Kaplan-Meier analysis was used to estimate overall survival (OS), and Cox proportional hazards models were used to indicate covariates associated with survival. RESULTS:A total of 440 cases of lacrimal gland cancer were included in the analysis, with a median follow-up of 52.9 months. The five-year OS for the entire cohort was 65.0%. Adenoid cystic carcinoma was the predominant histology (47.3%). Cox models showed that improved OS was associated with surgical resection (UVA: p < 0.001; MVA: p = 0.035). A detriment in OS was associated with increasing age, Charlson-Deyo score of 1, T4 stage, and positive margins and on UVA for adenocarcinoma and malignant mixed tumor histology. CONCLUSION/CONCLUSIONS:Adenoid cystic carcinoma comprises the plurality of lacrimal gland cancers. About half of patients with lacrimal gland carcinoma will live beyond 10 years, underscoring the importance of reduced morbidity of treatment. Surgical management is associated with improved prognosis. Further study will elucidate the role of surgical excision and radiotherapy in lacrimal gland cancer.

OBJECTIVES/HYPOTHESIS/UNASSIGNED:Systemic glucocorticoids (GC)s are employed to treat various voice disorders. However, GCs have varying pharmacodynamic properties with adverse effects ranging from changes in epithelial integrity, skeletal muscle catabolism, and altered body weight. We sought to characterize the acute temporal effects of systemic dexamethasone and methylprednisolone on vocal fold (VF) epithelial glucocorticoid receptor (GR) nuclear translocation, epithelial tight junction (ZO-1) expression, thyroarytenoid (TA) muscle fiber morphology, and body weight using an established pre-clinical model. We hypothesized dexamethasone and methylprednisolone will elicit changes in VF epithelial GR nuclear translocation, epithelial ZO-1 expression, TA muscle morphology, and body weight compared to placebo-treated controls. METHODS/UNASSIGNED: = 15) into the iliocostalis/longissimus muscle for 6 consecutive days. Vocal folds from 5 rabbits from each treatment group were harvested at 1-, 3-, or 7 days following the final injection and subjected to immunohistochemistry for ZO-1 and GR as well as TA muscle fiber cross-sectional area (CSA) measures. RESULTS/UNASSIGNED: = .004). CONCLUSIONS/UNASSIGNED:Systemic dexamethasone may more efficiently activate GR in the VF epithelium with a lower risk of body weight loss, suggesting a role for more refined approaches to GC selection for laryngeal pathology.