Hunter, Jacob B.

AbstractObjectiveTo assess the correlation between linear and volumetric changes in vestibular schwannomas (VS).Study DesignRetrospective imaging review was performed on patients diagnosed with sporadic VS from 2000 to 2019 who demonstrated linear growth on observation with serial magnetic resonance imaging (MRI).SettingTwo large tertiary care centers.MethodsChanges in diameter on serial MRI scans, measured by 1995 American Academy of Otolaryngology‐Head and Neck Surgery guidelines, were compared to changes in volume, calculated by segmentation.ResultsNinety‐two patients had VS confined to the internal auditory canal (IAC) with 236 MRIs analyzed, and 108 patients had VS involving the cerebellopontine angle (CPA) with 193 MRIs analyzed. The Spearman rank correlation coefficients between changes in diameter and volume for IAC and CPA tumors were 0.43 (p < .001) and 0.65 (p < .001), respectively. Linear diameter increases of 1 to <2 mm corresponded to a median volume change of 32% (interquartile range [IQR]: 6%‐86%) for IAC tumors, compared to 23% (IQR: 13%‐40%) for CPA tumors. Linear diameter increases of 2 to <3 mm (ie, the minimum linear diameter change classically considered “true growth”) corresponded to a median volume change of 42% (IQR: 23%‐100%) and 47% (IQR: 26%‐69%) for IAC and CPA tumors, respectively.ConclusionChanges in linear diameter significantly correlated with changes in volume for IAC and CPA tumors, although diameter changes that did not meet the definition of linear growth (<2 mm) had corresponding median volume changes in excess of 20% for both IAC and CPA tumors.

Abstract Objective To evaluate the safety and outcomes of cochlear implantation (CI) in patients with ventriculoperitoneal (VP) shunts to inform clinical practice. Study Design Historical cohort study. Setting Tertiary referral centers. Methods A multi‐institutional historical cohort of patients with VP shunts and CI was identified and analyzed. Results A total of 46 patients (median age 8 years [interquratile range, IQR: 2‐46]) with VP shunts and CI were identified. Of these, 41 (89%) patients had a VP shunt prior to CI. Based on institutional preference and individual patient factors, CI was performed contralateral to a pre‐existing VP shunt in 24 of these 41 cases (59%) and ipsilateral in 17 (41%). Furthermore, pre‐CI relocation of the VP shunt was performed in 3 cases (7%), and 2 patients (5%) underwent planned revision of their VP shunt concurrent with CI. In total, 2 of 27 pediatric patients (7%) required unanticipated revision shunt surgery, both contralateral to CI device placement, given VP shunt malfunction. One of 19 adult patients (5%) required shunt revision during CI due to shunt damage noted intraoperatively. Among 43 patients with available follow‐up, 38 (88%) are regular CI users, with a median consonant‐nucleus vowel‐consonant word: score of 58% (IQR: 28‐72). Conclusion CI can be performed at low risk, either contralateral or ipsilateral, to a VP shunt, and does not mandate shunt revision in most cases. Additional considerations regarding CI receiver‐stimulator placement are necessary with programmable shunts to mitigate device interaction. Preoperative planning, including coordination of care with neurosurgery, is important to achieving optimal outcomes.

Objective Spontaneous tumor shrinkage during wait-and-scan management of sporadic vestibular schwannoma is generally considered an uncommon phenomenon. However, most data informing this understanding stem from single-slice linear tumor measurements taken in the axial imaging plane. The objective of the current work was to characterize the regression capacity of sporadic vestibular schwannomas using volumetric tumor measurements. Study Design Retrospective cohort study using slice-by-slice, three-dimensional volumetric tumor measurements. Setting Three tertiary referral centers. Patients Patients with sporadic vestibular schwannoma. Interventions Wait-and-scan. Main Outcome Measures Regression-free survival rates with regression defined as a decrease of at least 20% of the tumor volume. Results Among 952 patients undergoing a total of 3,505 magnetic resonance imaging studies during observation, 123 experienced volumetric tumor regression after diagnosis at a median of 1.2 years (interquartile range, 0.6–2.9 yr). Volumetric regression-free survival rates (95% confidence interval; number still at risk) at 1, 3, and 5 years after diagnosis were 94% (92–95%; 662), 86% (83–89%; 275), and 78% (73–82%; 132), respectively. Among 405 patients who demonstrated an initial period of tumor growth but continued wait-and-scan management, 48 experienced volumetric regression at a median of 1.2 years (interquartile range, 0.8–2.6 yr) after initial growth. Volumetric regression-free survival rates at 1, 3, and 5 years after initial growth were 94% (92–97%; 260), 84% (79–89%; 99), and 75% (67–83%; 43), respectively. Ultimately, only 82 of the 952 patients studied showed exclusively volumetric tumor regression (i.e., without any periods of tumor growth) by the time of last follow-up. Conclusion Spontaneous volumetric tumor shrinkage during wait-and-scan management occurs more frequently than suggested by previous studies using linear tumor measurements and can even occur after previous episodes of documented tumor growth. These data further highlight the dynamic nature of vestibular schwannoma growth. To this end, the application of natural history data to patient management requires a nuanced approach that parallels the complex tumor behavior of vestibular schwannoma.

Abstract Background The current study aims to characterize the natural history of sporadic vestibular schwannoma volumetric tumor growth, including long-term growth patterns following initial detection of growth. Methods Volumetric tumor measurements from 3505 serial MRI studies were analyzed from unselected consecutive patients undergoing wait-and-scan management at three tertiary referral centers between 1998 and 2018. Volumetric tumor growth was defined as a change in volume ≥20%. Results Among 952 patients undergoing observation, 622 experienced tumor growth with initial growth-free survival rates (95% CI) at 1, 3, and 5 years following diagnosis of 66% (63–69), 30% (27–34), and 20% (17–24). Among 405 patients who continued to be observed despite demonstrating initial growth, 210 experienced subsequent tumor growth with subsequent growth-free survival rates at 1, 3, and 5 years following initial growth of 77% (72–81), 37% (31–43), and 24% (18–31). Larger tumor volume at initial growth (HR 1.13, P = .02) and increasing tumor growth rate (HR 1.31; P &lt; .001) were significantly associated with an increased likelihood of subsequent growth, whereas a longer duration of time between diagnosis and detection of initial growth was protective (HR 0.69; P &lt; .001). Conclusions While most vestibular schwannomas exhibit an overall propensity for volumetric growth following diagnosis, prior tumor growth does not perfectly predict future growth. Tumors can subsequently grow faster, slower, or demonstrate quiescence and stability. Larger tumor size and increasing tumor growth rate portend a higher likelihood of continued growth. These findings can inform timing of intervention: whether upfront at initial diagnosis, after detection of initial growth, or only after continued growth is observed.

Objective: Active treatment of small- or medium-sized vestibular schwannoma during wait-and-scan management is currently recommended at most centers globally once growth is detected. The primary aim of the current study was to characterize the natural history of growing sporadic vestibular schwannoma during observation. Study Design: Cohort study. Setting: Four tertiary referral centers across the United States and Denmark. Patients: Patients with two prior MRI scans demonstrating ≥2 mm of linear growth who continued observational management. Intervention: Observation with serial imaging. Main Outcome Measure: Subsequent linear growth-free survival (i.e., an additional ≥2 mm of growth) following initial growth of ≥2 mm from tumor size at diagnosis. Results: Among 3,402 patients undergoing observation, 592 met inclusion criteria. Median age at initial growth was 66 years (IQR 59–73) for intracanalicular tumors (N = 65) and 62 years (IQR 54–70) for tumors with cerebellopontine angle extension (N = 527). The median duration of MRI surveillance following initial detection of tumor growth was 5.2 years (IQR 2.4–6.9) for intracanalicular tumors and 1.0 year (IQR 1.0–3.3) for cerebellopontine angle tumors. For intracanalicular tumors, subsequent growth-free survival rates (95% CI; number still at risk) at 1, 2, 3, 4, and 5 years following the initial MRI that demonstrated growth were 77% (67–88; 49), 53% (42–67; 31), 46% (35–60; 23), 34% (24–49; 17), and 32% (22–47; 13), respectively. For cerebellopontine angle tumors, subsequent growth-free survival rates were 72% (68–76; 450), 47% (42–52; 258), 32% (28–38; 139), 26% (21–31; 82), and 22% (18–28; 57), respectively. For every 1 mm increase in magnitude of growth from diagnosis to tumor size at detection of initial growth, the HRs associated with subsequent growth were 1.64 (95% CI 1.25–2.15; p < 0.001) for intracanalicular tumors and 1.08 (95% CI 1.01–1.15; p = 0.02) for cerebellopontine angle tumors. Conclusions: Growth detected during observation does not necessarily portend future growth, especially for slowly growing tumors. Because early treatment does not confer improved long-term quality of life outcomes, toleration of some growth during observation is justifiable in appropriately selected cases.

ObjectiveTo ascertain the prevalence of and associations with distress and professional burnout among academic otolaryngology attending physicians.Study DesignCross‐sectional survey.SettingTwelve US academic otolaryngology programs.MethodsA questionnaire was administered that encompassed sociodemographic and professional features, the Expanded Physician Well‐being Index for distress, the 2‐item Maslach Burnout Inventory for professional burnout, the Patient Health Questionnaire–2 screen for major depressive disorder, and the Generalized Anxiety Disorder–2 screen for generalized anxiety disorder.ResultsThe survey response rate was 56% and included 186 attending physicians. The average respondent age was 47 years; 72% were men; 93% were married or partnered; and 86% had children. Distress was present in 40%, professional burnout in 26%, positive depression screening in 8%, and positive anxiety screening in 11%. In a univariable setting, age, hours worked in a typical week, nights on call in a typical week, and years of practice were significantly associated with distress, although in a multivariable setting, only hours worked in a typical week remained significantly associated with a positive Expanded Physician Well‐being Index screen (odds ratio for each 10‐hour increase, 2.61; 95% CI, 1.73‐3.93; P <. 001). In a univariable setting, hours worked in a typical week was significantly associated with a positive Maslach Burnout Inventory screen.ConclusionDistress or professional burnout occurs in more than a quarter of academic otolaryngology attending physicians, whereas the prevalence of depression or anxiety is approximately 10%. The number of hours worked per week had the strongest association with distress and burnout. These findings may be used to develop and implement programs to promote physician well‐being and mitigate professional burnout.

ObjectiveTo assess the prevalence of distress and burnout in otolaryngology trainees, including associations with relevant sociodemographic and professional factors, and to compare these results with those of attending otolaryngologists.Study DesignA cross‐sectional survey of trainees and attending physicians.SettingTwelve academic otolaryngology programs.MethodsDistress and burnout were measured with the Expanded Physician Well‐being Index and the 2‐item Maslach Burnout Inventory. The Patient Health Questionnaire–2 and Generalized Anxiety Disorder–2 were used to screen for depressive disorders and anxiety disorders, respectively. Associations with sociodemographic and professional characteristics were assessed.ResultsOf the 613 surveys administered to trainees and attending physicians, 340 were completed (56%). Among 154 trainees, distress was present in 49%, professional burnout in 35%, positive depressive disorder screening in 5%, and positive anxiety disorder screening in 16%. In univariable analysis, female gender, hours worked in a typical week (HW), and nights on call in a typical week (NOC) were significantly associated with distress. In multivariable analysis, female gender (odds ratio, 3.91; P =. 001) and HW (odds ratio for each 10 HW, 1.89; P =. 003) remained significantly associated with distress. Female gender, HW, and NOC were significantly associated with burnout univariably, although only HW (odds ratio for each 10 HW, 1.92; P =. 003) remained significantly associated with burnout in a multivariable setting. Attending physicians had less distress than trainees (P =. 02) and felt less callous and less emotionally hardened than trainees (P <. 001).ConclusionOtolaryngology trainees experience significant work‐place distress (49%) and burnout (35%). Gender, HW, and NOC had the strongest associations with distress and burnout.

Objective: To date, prediction models for estimating risk of acquiring non-serviceable hearing in subjects with observed vestibular schwannoma (VS) have evaluated outcomes primarily based on features at initial diagnosis. Herein, we evaluate the association of rate of hearing decline during the initial period of observation with time to non-serviceable hearing. If significant, rate of hearing decline may inform decision making after an introductory period of observation. Setting: Two tertiary care centers. Patients: VS patients with serviceable hearing who underwent at least three audiograms and two magnetic resonance imaging (MRI) studies before intervention or being lost to follow-up. The rate of change in pure-tone average (PTA) and word recognition score (WRS) was calculated as the score from the second audiogram minus the score from the first audiogram, divided by the duration in months between the two. Main Outcome Measure(s): Serviceable hearing, defined as PTA ≤50 dB HL and WRS ≥50%. Results: Among 266 patients meeting inclusion criteria, 52 developed non-serviceable hearing at last follow-up. Kaplan–Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years were 97% (95–100; 206), 78% (72–85; 98), 68% (60–77; 39), 60% (50–73; 17), and 44% (29–67; 2), respectively. In a univariable setting, each 1 dB increase per month in the rate of initial PTA change was associated with a 96% increased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 1.96; 95% CI 1.44–2.68; p < 0.001). Each 1% increase per month in the rate of initial WRS change was associated with a decreased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 0.79; 95% confidence interval [CI] 0.66–0.94; p = 0.009). After multivariable adjustment, both rate of PTA change (HR 2.42; 95% CI 1.72–3.41; p < 0.001) and rate of WRS change (HR 0.81; 95% CI 0.67–0.99; p = 0.043) remained statistically significantly associated with time to non-serviceable hearing. Conclusion: Rate of early PTA and WRS decline during the initial period of observation are significantly associated with time to development of non-serviceable hearing. This information may facilitate accurate patient counseling and inform decision-making regarding prospective disease management.

Objective: To characterize the risk of progression to nonserviceable hearing in patients with sporadic vestibular schwannomas (VS) who elect initial observation. Study Design: Retrospective case series. Setting: Two tertiary care centers. Patients: VS patients with serviceable hearing who underwent at least two audiograms and two MRI studies before intervention or loss to follow-up. Main outcome measure(s): Serviceable hearing, defined as the pure tone average ≤ 50 dB HL and word recognition score ≥ 50%. Results: Four-hundred sixty-six patients (median age of 57 yr and median tumor diameter of 7.3 mm) had serviceable hearing at presentation and were followed for a median of 2.3 years (IQR 1.0 – 4.0). Kaplan–Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years following diagnosis were 94% (91–96; 357), 77% (73–82; 172), 66% (60–73; 81), 56% (49–65; 31), and 44% (33–59; 10), respectively. Each 10-dB increase in pure-tone averages at diagnosis was associated with a 2-fold increased likelihood of developing nonserviceable hearing (hazard ratio 2.07; p < 0.001). Each 10% decrease in word recognition score was associated with a 1.5-fold increased likelihood of developing nonserviceable hearing (hazard ratio 1.48; p < 0.001). Conclusions: Among patients with sporadic VS, good baseline word recognition score and low pure-tone average are jointly associated with maintenance of serviceable hearing. These data may be used to guide patient counseling and optimize management.