Marinelli, John P.

Objective: Despite the growing emphasis on healthcare costs, limited data address this aspect of care within the vestibular schwannoma (VS) literature. We sought to determine which strategy confers the lowest lifetime cost and greatest quality-adjusted life-years (QALYs) for patients with small- to medium-sized sporadic VS tumors. Study Design: A Markov model was created to determine the most cost-effective management algorithm. Tumor characteristics, magnetic resonance imaging surveillance schedule, treatment outcomes, and health-related quality of life values were derived from previously published data. Cost estimates were based on CMS Fee Schedule reimbursement rates. Setting: Economic Evaluation Service within the Kern Center for the Science of Healthcare Delivery. Patients: Patients diagnosed with small- to medium-sized sporadic VS. Interventions: Upfront microsurgery following diagnosis, upfront radiosurgery following diagnosis, observation with microsurgery reserved for observed tumor growth, and observation with radiosurgery reserved for observed tumor growth. Results: Across patient ages at time of diagnosis ranging from 18 to 70 years, observation with subsequent radiosurgery used for tumor growth was the most cost-effective management algorithm while upfront microsurgery was the least. When presented with a hypothetical 50-year-old patient, the strategy with the lowest lifetime cost and highest QALYs was observation with subsequent radiosurgery reserved for tumor growth ($32,161, 14.11 QALY), followed by observation with microsurgery reserved for tumor growth ($34,503, 13.94 QALY), upfront radiosurgery ($43,456, 14.02 QALY), and lastly, upfront microsurgery ($47,252, 13.60 QALY). Sensitivity analyses varying mortality rates, estimated costs, health-related quality of life, and progression to nonserviceable hearing demonstrated consistent ranking among treatments. Conclusions: When considering initial management of small- and medium-sized sporadic VSs, neither lifetime cost nor QALYs support upfront microsurgery or radiosurgery, even for younger patients. Initial observation with serial imaging, reserving radiosurgery or microsurgery for patients exhibiting tumor growth, confers the greatest potential for optimized lifetime healthcare cost and QALY outcomes.

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.

Objective: Reports using large brain tumor and cancer registries suggest that the incidence of vestibular schwannoma is considerably lower in the United States compared with other countries. The current study compares the incidence and disease characteristics of vestibular schwannoma within a large tertiary referral center's brain tumor and cancer registry using nationally mandated reporting protocols to disease incidence in the same population using an externally validated population-based consortium. Study Design: Population-based study spanning 1,945,007 person-years. Setting: Large tertiary referral center. Patients: Adults with sporadic vestibular schwannoma. Main Outcome Measure: Disease incidence rates from 2004 to 2016. Results: From 2004 to 2016, the incidence of vestibular schwannoma in the tumor registry was 1.3 per 100,000 person-years whereas the population-based cohort had an incidence of 4.4 per 100,000 person-years. From 2012 to 2016, the incidence in the tumor registry was 1.4 per 100,000 person-years compared with 5.2 in the population-based cohort. Patients within the population-based cohort were significantly more likely to have smaller tumors at diagnosis (78% intracanalicular versus 45%; p = 0.004) and consequently more likely to undergo management consisting of observation with serial imaging as opposed to treatment with either microsurgery or radiosurgery (71% versus 28%; p = 0.001). Conclusions: The reliance on pathology specimens and cancer-related treatment data for the national registration of new cancer and brain tumor diagnoses may introduce selection bias and underreporting of benign brain tumors that frequently involve observation as a primary treatment modality. This selection bias likely accounts for the discrepant incidence rates of vestibular schwannoma reported between the United States and other countries.

Objectives/Hypothesis To describe the pre‐implant audiometric profile of adult cochlear implant (CI) recipients to investigate whether current binaural candidacy requirements prevent access to patients who could benefit from CI. Study Design Retrospective case series. Methods Retrospective review from 2016 to 2018 evaluating preoperative pure‐tone thresholds and speech perception scores in the ipsilateral and contralateral ear. Results A total of 252 adult CI recipients undergoing 270 implants were identified. Median age at time of implantation was 70.5 years (IQR 61.3–78.3) for those undergoing unilateral implantation and 59.0 (IQR 48.0–72.3) for those undergoing bilateral implantation ( P < .01). For unilateral implantation, median pre‐implantation speech perception performance in the ear to be implanted was 8.0% (IQR 0%–26.0%) for CNC word scores, and 9.0% (IQR 0%–34.0%) for AzBio sentence scores in quiet. Median speech perception performance in the contralateral ear was 36.0% (IQR 14.0%–60.0%) on CNC word scores, and 48.5% (IQR 17.5%–76.0%) on AzBio sentence tests. Speech perception scores were significantly different between ears for word and sentence tests ( P < .01). Patients older than age 65 were significantly less likely to undergo bilateral implantation ( P = .03). Conclusions Adult CI recipients exhibit substantially poorer pre‐implant speech perception scores than the commonly utilized ipsilateral qualifying threshold of 50% on sentence testing. Yet, existing insurance paradigms limit patients by excessively stringent binaural best‐aided requirements. This limitation likely leads to worse device performance as patients often wait years for their binaural hearing to qualify while their ear to be implanted potentially experiences an unnecessarily extended duration of deafness. Older patients also experience a unique delay in referral for cochlear implantation. Level of Evidence 3‐ Retrospective review Laryngoscope , 131:E2007–E2012, 2021

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

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.

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 This study sought to determine whether a history of pregnancy or bilateral oophorectomy is associated with subsequent otosclerosis development or disease severity. Study Design Population‐based case‐control study. Setting Olmsted County, Minnesota. Methods Women diagnosed with otosclerosis were matched to 3 women without otosclerosis based on age and historical depth of medical records. Associations of prior delivery and bilateral oophorectomy with subsequent development of otosclerosis and with pure‐tone average (PTA) at the time of otosclerosis diagnosis were evaluated. Results We studied 1196 women: 299 cases of otosclerosis and 897 matched controls. The odds ratio for the association of ≥1 delivery with otosclerosis was 1.16 (95% confidence interval [CI] 0.85‐1.60; P =. 35). Odds ratios for the associations of 1, 2, 3, or ≥4 deliveries with otosclerosis were 1.22 (0.83‐1.80), 1.09 (0.71‐1.68), 1.28 (0.77‐2.12), and 1.00 (0.54‐1.84), respectively. The odds ratio for the association of prior bilateral oophorectomy with otosclerosis was 1.12 (0.58‐2.18; P =. 73). In cases with otosclerosis, PTA at diagnosis was not significantly higher for women with ≥1 delivery as compared with those without (median 45 dB hearing loss [HL] [interquartile range {IQR} 36‐55] vs 43 [IQR 34‐53]; P = 0.18) but was significantly higher for women with bilateral oophorectomy compared with those without (median 54 dB HL [IQR 44‐61] vs 44 [IQR 34‐53]; P =. 03). Conclusion These data do not support a relationship between endogenous estrogen exposure and development of otosclerosis. Women with otosclerosis who had a history of pregnancy did not have significantly worse hearing at the time of diagnosis, suggesting that pregnancy is not associated with disease severity.