Link, Michael J.

Abstract BACKGROUND Low-grade intracranial dural arteriovenous fistulas (dAVF) have a benign natural history in the majority of cases. The benefit from treatment of these lesions is controversial. OBJECTIVE To compare the outcomes of observation versus intervention for low-grade dAVFs. METHODS We retrospectively reviewed dAVF patients from institutions participating in the CONsortium for Dural arteriovenous fistula Outcomes Research (CONDOR). Patients with low-grade (Borden type I) dAVFs were included and categorized into intervention or observation cohorts. The intervention and observation cohorts were matched in a 1:1 ratio using propensity scores. Primary outcome was modified Rankin Scale (mRS) at final follow-up. Secondary outcomes were excellent (mRS 0-1) and good (mRS 0-2) outcomes, symptomatic improvement, mortality, and obliteration at final follow-up. RESULTS The intervention and observation cohorts comprised 230 and 125 patients, respectively. We found no differences in primary or secondary outcomes between the 2 unmatched cohorts at last follow-up (mean duration 36 mo), except obliteration rate was higher in the intervention cohort (78.5% vs 24.1%, P < .001). The matched intervention and observation cohorts each comprised 78 patients. We also found no differences in primary or secondary outcomes between the matched cohorts except obliteration was also more likely in the matched intervention cohort ( P < .001). Procedural complication rates in the unmatched and matched intervention cohorts were 15.4% and 19.2%, respectively. CONCLUSION Intervention for low-grade intracranial dAVFs achieves superior obliteration rates compared to conservative management, but it fails to improve neurological or functional outcomes. Our findings do not support the routine treatment of low-grade dAVFs.

Objectives/HypothesisTo examine the hearing status and aural rehabilitative profile in a national cohort of patients with sporadic vestibular schwannoma (VS).Study DesignCross‐sectional surveyMethodsA cross‐sectional survey of Acoustic Neuroma Association members diagnosed with sporadic VS was performed from February 2017 through January 2019. Self‐reported results were used to determine the aural rehabilitative profile of respondents.ResultsAmong survey respondents, 62.2% (546/878) were not using any hearing‐assistive device at time of survey. For the 37.8% (332/878) that were utilizing hearing‐assistive devices, 32.8% (109/332) reported using a behind‐the‐ear hearing aid, 23.8% (79/332) used a contralateral routing of signal (CROS) hearing aid, and 21.7% (72/332) used a bone conduction device. Notably, 41.9% (278/663) of patients who previously underwent tumor treatment reported utilizing a hearing rehabilitation device at some point during VS management compared to 27.0% (58/215) of those undergoing observation with serial imaging (P < .001). Of 275 patients with functional hearing in the ipsilateral ear, 26.5% (73/275) reported having used at least one type of hearing device; 24.0% (66/275) reported use of a conventional hearing aid, 0.7% (2/275) a CROS aid, and 0.4% (1/275) a bone conduction device. Among respondents reporting ipsilateral nonfunctional hearing, 44.9% (258/575) reported having used at least one type of hearing device; 13.0% (75/575) a CROS aid, and 12.3% (71/575) a bone conduction device.ConclusionsEven among a cohort with presumably elevated literacy surrounding hearing rehabilitation options, few patients with a history of unilateral vestibular schwannoma ultimately use hearing assistive devices long‐term, suggesting that most patients sufficiently adjust to unilateral hearing loss or are unsatisfied with the benefits achieved with current device options.Level of Evidence4 Laryngoscope, 131:1378–1381, 2021

Abstract BACKGROUND Hypopituitarism is the most frequent complication after pituitary adenoma stereotactic radiosurgery (SRS) and is correlated with increasing radiation to the pituitary gland. Biological effective dose (BED) is a dosimetric parameter that incorporates a time component to adjust for mechanisms of deoxyribonucleic acid repair activated during treatment. OBJECTIVE To assess mean gland BED as a predictor of post-SRS hypopituitarism, as compared to mean gland dose, in a contemporary cohort study of patients undergoing single-fraction SRS for pituitary adenoma. METHODS Cohort study of 97 patients undergoing single-fraction SRS from 2007 to 2014. Eligible patients had no prior pituitary irradiation, normal pre-SRS endocrine function, and >24 mo of endocrine follow-up. Cox proportional hazards analysis was used to assess mean gland dose and BED as predictors of new post-SRS hypopituitarism. RESULTS Median post-SRS follow-up was 48 mo (interquartile range [IQR], 34-68). A total of 27 patients (28%) developed new hypopituitarism at a median 22 mo (IQR, 12-36). Actuarial rates of new endocrinopathy were 17% at 2 yr (95% CI 10%-25%) and 31% at 5 yr (95% CI 20%-42%). On univariate analysis, sex ( P = .02), gland volume ( P = .03), mean gland dose ( P < .0001), and BED significantly predicted new hypopituitarism ( P < .0001). After adjusting for sex and gland volume, BED > 45 Gy 2.47 and mean gland dose > 10 Gy were significantly associated increased risk of hypopituitarism (hazard ratio [HR] = 14.32, 95% CI = 4.26-89.0, P < .0001; HR = 11.91, 95% CI = 3.54-74.0, P < .0001). CONCLUSION BED predicted hypopituitarism more reliably than mean gland dose after pituitary adenoma SRS, but additional studies are needed to confirm these results.

Objective: Limited data currently characterize management trends of sporadic vestibular schwannoma (VS) in the United States over recent decades. Serving as the primary patient support organization in the United States, the Acoustic Neuroma Association (ANA) facilitates amalgamation of data from patients treated from 1970 through January 2019. The primary objective of the current study was to characterize the evolution in the management of sporadic VS among ANA survey respondents treated over the last half-century. Study Design/Intervention: Cross-sectional survey. Setting/Patients: ANA survey respondents diagnosed with sporadic VS. Main Outcome Measures: Trends in rates of microsurgery, radiosurgery, and observation from 1970 to 2019. Results: Among 953 patients with VS responding during the study period, 876 (92%) were included. Subjects reported the following management strategies: 220 observation (25%), 454 microsurgery (52%), and 193 radiosurgery (22%). The rate of microsurgery decreased from 86–88% from 1970 to 2000 to 44% in the last decade. The proportion of VS observed increased from 7% of all tumors in the 1990s, to 14% in the 2000s, to 33% in the 2010s. The rate of radiosurgery has increased to 28% in the last decades compared with 7% in the 1990s. The rate of subtotal resection in tumors > 2 cm increased from 34% in the 2000s to 60% in the 2010s (p = 0.0011). Conclusions: Overall, these data support a progression toward fewer patients treated with microsurgery in recent decades. Radiosurgery and observation became increasingly more common after the turn of the century, possibly due to better detection of small and asymptomatic tumors and a greater understanding of the natural history of disease.

Background: Previous cross-sectional studies analyzing quality of life (QOL) outcomes in patients with sporadic vestibular schwannoma (VS) have shown surprisingly little difference among treatment modalities. To date, there is limited prospective QOL outcome data available comparing baseline to posttreatment scores. Study Design: Prospective longitudinal study using the disease-specific Penn Acoustic Neuroma Quality of Life (PANQOL) scale. Setting: Large academic skull base center. Patients: Patients diagnosed with unilateral VS who completed a baseline survey before treatment and at least one posttreatment survey. Main Outcome Measures: Change in PANQOL scores from baseline to most recent survey. Results: A total of 244 patients were studied, including 78 (32%) who elected observation, 118 (48%) microsurgery, and 48 (20%) stereotactic radiosurgery. Patients who underwent microsurgery were younger (p < 0.001) and had larger tumors (p < 0.001) than those who underwent observation or radiosurgery; there was no significant difference in duration of follow-up among management groups (mean 2.1 yrs; p = 0.28). When comparing the total PANQOL score at baseline to the most recent survey, the net change was only –1.1, –0.1, and 0.3 points on a 100-point scale for observation, microsurgery, and radiosurgery, respectively (p = 0.89). After multivariable adjustment for baseline features, there were no statistically significant changes when comparing baseline to most recent scores within each management group for facial function, general health, balance, hearing loss, energy, and pain domains or total score. However, the microsurgical group experienced a 10.8-point improvement (p = 0.002) in anxiety following treatment, compared with 1.5 (p = 0.73) and 5.3 (p = 0.31) for observation and radiosurgery, respectively. Conclusions: In this prospective longitudinal study investigating differences in QOL outcomes among VS treatment groups using the disease-specific PANQOL instrument, treatment did not modify QOL in most domains. Microsurgery may confer an advantage with regard to patient anxiety, presumably relating to the psychological benefit of “cure” from having the tumor removed.

The present state of vestibular schwannoma (VS) management is the product of over a century of technical progress by revolutionary surgeons who transformed a once perilous operation. At the beginning of the 1900s, patients who did not succumb to their disease were treated exclusively with surgery, which itself was almost assuredly devastating. Through the pioneering work of surgeons such as Harvey Cushing, Walter Dandy, William House, and others, safer surgical approaches were established with concurrent advances in neuromonitoring, neuroanesthesia, radiology, and adoption of the operating microscope. Owing to refinements in radiosurgical treatment and a greater understanding of the natural history of disease, there has been a dramatic shift toward more conservative management in recent years. For more than 100 years, the Mayo Clinic in Rochester, MN, has maintained an active and uninterrupted VS practice with activities that are well documented and preserved through the Mayo Clinic historical archives. We herein report representative early cases to illustrate the fascinating evolution in VS surgery over the past century at a single-tertiary referral center. Original clinical, imaging, pathology, and operative reports are presented from each era of management. To accurately portray the medical context of each era, antiquated terminology is intentionally preserved as originally transcribed.

Objective:To address variance in clinical care surrounding sporadic vestibular schwannoma, a modified Delphi study was performed to establish a general framework to approach vestibular schwannoma care. A multidisciplinary panel of experts was established with deliberate representation from key stakeholder societies. External validity of the final statements was assessed through an online survey of registered attendees of the 8th Quadrennial International Conference on Vestibular Schwannoma.Study Design:Modified Delphi method.Methods:The panel consisted of 16 vestibular schwannoma experts (8 neurotology and 8 neurosurgery) and included delegates representing the AAOHNSF, AANS/CNS tumor section, ISRS, and NASBS. The modified Delphi method encompassed a four-step process, comprised of one prevoting round to establish a list of focus areas and three subsequent voting rounds to successively refine individual statements and establish levels of consensus. Thresholds for achieving moderate consensus, at ≥67% agreement, and strong consensus, at ≥80% agreement, were determined a priori. All voting was performed anonymously via the Qualtrics online survey tool and full participation from all panel members was required before procession to the next voting round.Results:Through the Delphi process, 103 items were developed encompassing hearing preservation (N = 49), tumor control and imaging surveillance (N = 20), preferred treatment (N = 24), operative considerations (N = 4), and complications (N = 6). As a result of item refinement, moderate (4%) or strong (96%) consensus was achieved in all 103 final statements. Seventy-nine conference registrants participated in the online survey to assess external validity. Among these survey respondents, moderate (N = 21, 20%) or strong (N = 73, 71%) consensus was achieved in 94 of 103 (91%) statements, and no consensus was reached in 9 (9%). Of the four items with moderate consensus by the expert panel, one had moderate consensus by the conference participants and three had no consensus.Conclusion:This modified Delphi study on sporadic vestibular schwannoma codifies 100% consensus within a multidisciplinary expert panel and is further supported by 91% consensus among an external group of clinicians who regularly provide care for patients with vestibular schwannoma. These final 103 statements address clinically pragmatic items that have direct application to everyday patient care. This document is not intended to define standard of care or drive insurance reimbursement, but rather to provide a general framework to approach vestibular schwannoma care for providers and patients.

Objective: To assess vestibular schwannoma (VS) practice patterns among providers. Study Design: Cross-sectional survey. Setting: 8th Quadrennial International Conference on Vestibular Schwannoma and Other CPA Tumors. Subjects: Clinicians who specialize in the management of VS. Main Outcome Measures: Responses to questions on the management and anticipated outcomes of VS for a series of common clinical scenarios were compared by specialty (otolaryngology versus neurosurgery), level of experience, scope of practice (surgery versus radiation and surgery), and geographic location of practice (United States versus international). Results: Responses from 110 participants were analyzed. Overall, 53% of respondents were otolaryngologists, 60% had greater than 10 years of experience, and 57% practiced within the United States. In total, 86% of respondents would pursue initial observation for themselves if diagnosed with a 4 mm distal intracanalicular VS; however, practicing radiosurgeons were more likely to select stereotactic radiosurgery for this scenario compared with providers who solely practice surgery (14 versus 0%; p = 0.032). Otolaryngologists and neurosurgeons alike report that radiosurgery should not be considered a long-term hearing preservation strategy. Otolaryngologists were more optimistic regarding microsurgical hearing preservation outcomes for small distal intracanalicular tumors compared with neurosurgeons (11 versus 3% selected a high likelihood of maintaining class A/B hearing; p = 0.007). Ninety-five percent of respondents prioritized facial nerve outcome over complete disease removal in the context of microsurgical resection of large tumors. Conclusions: Management decision-making and expected outcomes for various clinical scenarios were largely similar among providers; however, variances in several key clinical areas exist. This study points to the feasibility of developing a widely accepted consensus statement among VS experts across specialties.