Carlson, Matthew L.

Objective: Reported epidemiologic data surrounding vestibular schwannoma (VS) are controversial. Temporal bone prevalence studies have suggested that VS affects up to 2.4% of the population, whereas magnetic resonance imaging (MRI) studies have reported VS to affect 0.017%. Moreover, existing population-based data seem to underestimate the commonness of VS. In an attempt to reconcile temporal bone, radiologic, and population-based reports regarding VS, the current study was conceived to determine the modern prevalence of VS using a unique epidemiological database. Patients: All persons living in Olmsted County, Minnesota on January 1, 2017 with a confirmed diagnosis of sporadic VS identified using the Rochester Epidemiology Project (REP) medical records-linkage system. Main Outcome Measures: Prevalence of all VS and asymptomatic, incidentally diagnosed VS. Results: Sixty-seven persons from a population of nearly 160,000 were living with VS on January 1, 2017, resulting in a point prevalence of 42.0 per 100,000 persons. The prevalence increased with older age, reaching 212.4 per 100,000 in those ≥ 70 years. Including only persons who have undergone head MRI, the prevalence of asymptomatic, incidentally diagnosed VS was 69.9 per 100,000 among adults age 20 years and older. Conclusions: Using the unique infrastructure of the REP, the current study suggests that the clinical prevalence of sporadic VS approximates 1 in 2,000 adults and 1 in every 500 persons aged 70 years and older, with the prevalence of incidentally diagnosed tumors closely paralleling past MRI studies. These data characterize a shift in the modern patient demographic of sporadic VS, as now many people bearing a diagnosis of VS would have previously died without knowledge of their disease. Moreover, they typify an unfolding transition from an era of microsurgery and radiosurgery to the beginnings of an era that will be largely comprised of “chronic disease management.”

Background Age, tumor size and location, overall health, and patient preference are primary considerations driving treatment decision-making for intracranial meningiomas. However, even for the same individual patient, treatment recommendations may vary between centers and providers. Objective To study associations between geography, disease presentation, and management of intracranial meningioma in the United States. Methods The population-based Surveillance, Epidemiology, and End Results(SEER) data were queried between 2004 and 2014 for cases of intracranial meningioma. Results A total of 65,808 patients with intracranial meningioma were identified. Univariate analyses demonstrated strong associations between geographic location, age, and size of tumor at presentation. The mean age for all registries was 64.2 years, with a range from 62.0 (Utah registry) to 66.6 (Detroit registry). The greatest proportion of small tumors (<1 cm) were identified in the Utah registry (13.9% of tumors), while the greatest proportion of large tumors (> 4cm) were noted in the Hawaii registry (30.7% of tumors). Multivariable analysis demonstrated that the impact of geography on treatment selection was just as important as other established variables. For example, the distribution in tumor size between New Mexico and Greater California registries is nearly identical; however, the odds ratio for surgery was 1.5 times greater for the New Mexico population. Conclusion These data suggest that disease presentation and treatment are significantly influenced by regional referral patterns, provider or institutional treatment preferences, and regional availability of subspecialty expertise. Understanding such biases is important for patients, referring physicians, and treatment providers in an effort to provide balanced counseling and treatment.

Objective/Hypothesis The aim of the study was to determine the impact of race on disease presentation and treatment of intracranial meningioma in the United States. Study Design This study comprised of the analysis of a national population-based tumor registry. Methods Analysis of the surveillance, epidemiology, and end results (SEER) database was performed, including all patients identified with a diagnosis of intracranial meningioma. Associations between race, disease presentation, treatment strategy, and overall survival were analyzed in a univariate and multivariable model. Results A total of 65,973 patients with intracranial meningiomas were identified. Of these, 45,251 (68.6%) claimed white, 7,796 (12%) black, 7,154 (11%) Hispanic, 4,902 (7%) Asian, and 870 (1%) patients reported “other-unspecified” or “other-unknown.” The median annual incidence of disease was lowest among black (3.43 per 100,000 persons) and highest among white (9.52 per 100,000 persons) populations (p < 0.001). Overall, Hispanic patients were diagnosed at the youngest age and white patients were diagnosed at the oldest age (mean of 59 vs. 66 years, respectively; p < 0.001). Compared with white populations, black, Hispanic, and Asian populations were more likely to present with larger tumors (p < 0.001). After controlling for tumor size, age, and treatment center in a multivariable model, Hispanic patients were more likely to undergo surgery than white, black, and Asian populations. Black populations had the poorest disease specific and overall survival rates at 5 years following surgery compared with other groups. Conclusion Racial differences among patients with intracranial meningioma exist within the United States. Understanding these differences are of vital importance toward identifying potential differences in the biological basis of disease or alternatively inequalities in healthcare delivery or access Further studies are required to determine which factors drive differences in tumor size, age, annual disease incidence, and overall survival between races.

Introduction Optimal management of vestibular schwannoma (VS) demands involvement of an experienced multidisciplinary team. As the number of training programs in neurotology and skull base neurosurgery continues to rise, ensuring that trainees are capable of evidence-based decision-making and treatment, whether microsurgical or radiosurgical, is of paramount importance. The purpose of this study is to characterize the landscape of neurotologic and neurosurgical fellowship training programs in North America, with special reference to VS management. Methods A 64-item web-based survey assessing VS practice trends was devised by members of the North American Skull Base Society (NASBS) Research Task Force and distributed electronically to NASBS membership via SurveyMonkey as a cross-sectional study. Participation was entirely voluntary and there was no remuneration for survey completion. The survey link was active from November 29 to December 14, 2016. Results Of 719 members of the NASBS who were emailed a survey link, a total of 57 were returned (8%) completed surveys. Of all respondents, 51 (89%) claimed to have formal training in skull base neurosurgery or neurotology. Thirty-three respondents (65%) were skull base neurosurgeons while the remainder were neurotologists (n = 18; 35%). Institutions with fellowship programs tended to have a higher surgical, radiosurgical, and overall case volume than those with a residency program alone. However, 20% of respondents at institutions with fellowship programs reported evaluating less than 50 new diagnoses of VS per year and 12% reported a surgical case volume of less than 10 cases per year. Conclusion As the number of skull base training programs expands, it is our duty to ensure that trainees gain sufficient experience to enter independent practice with the ability to exercise informed decision-making and safely perform VS surgery and radiosurgery. In the current training climate, implementing multidisciplinary care models, formalized training requirements, and emerging surgical simulators will support the development of minimum proficiencies in VS care.

Objectives Although vestibular schwannomas (VS) are known to cause cranial nerve deficits, cerebellar symptoms, and hydrocephalus, the role of these symptoms as the key driver of presentation from the patient's perspective has not been described. Our objective was to survey a large, retrospective VS cohort to document the patient-reported principal initial symptom, and self-reported tumor size, and to study trends in VS patient presentation. Methods Patients diagnosed with VS at our tertiary referral center and belonging to the Acoustic Neuroma Association (ANA) answered a questionnaire between 2015 and 2017. Demographic data, self-reported tumor size, and symptomatology were analyzed. Results 1,304 patients completed the questionnaire. Tumors were diagnosed from 1966 to 2017 at a mean 51.8 years (range: 8–86 years); 66% were female, and 1.1% had confirmed neurofibromatosis type 2 (NF2). Tumor size was reported using a 6-point scale: 0 to 1 cm (22.9%), 1 to 2 cm (28.7%), 2 to 3 cm (20.5%), 3 to 4 cm (10%), greater than 4 cm (7.2%), and unknown (10.6%). Hearing loss was the most common symptom that led to diagnosis (51.5%), followed by dizziness (17%), tinnitus (11.2%), and incidental diagnosis (10.2%); a fraction that has increased significantly in the last decade (p = 0.022). Larger tumors and NF2 were significantly associated with young age (p < 0.001). Conclusion Our large-scale questionnaire-driven review of 1,304 patients confirms that VS presentations are stereotypical, with most individuals recalling hearing loss, dizziness, or tinnitus as their chief complaint. Many tumors were incidentally diagnosed; an expanding population, attributable to increased access to magnetic resonance imaging (MRI). Large tumors were significantly more prevalent among younger patients at diagnosis, excluding NF2 patients, suggesting a more aggressive tumor biology that remains incompletely understood.

Objective: To evaluate quality-of-life (QOL) in patients with sporadic vestibular schwannoma (VS) with particular focus on those recently diagnosed. Study Design: Cross-sectional survey, Penn Acoustic Neuroma Quality of Life (PANQOL) instrument. Setting: Acoustic Neuroma Association and a single tertiary referral center. Patients: Patients with sporadic VS. Main Outcome Measures: Domain-specific and total PANQOL scores comparing treatment modalities after adjusting for baseline covariates of interest. Results: Among all 1,288 respondents there were 229 (18%) who were recently diagnosed and had not yet selected a treatment modality, 303 (24%) who were observed, 185 (14%) who underwent radiosurgery alone, 507 (39%) who underwent microsurgery alone, and 64 (5%) who underwent radiosurgery and microsurgery. After adjusting for covariates of interest, total PANQOL scores were highest for the observation cohort (65; 95% CI 62–68), lowest for patients treated with multimodality therapy (56; 51–61) and those recently diagnosed (58; 55–62), and intermediate for those who received microsurgery alone (60; 58–62) and radiosurgery alone (61; 57–64) (global comparison, p = 0.001). When comparing groups that received single-modality therapy, there were no statistically significant differences in total PANQOL scores at short (0–5 yr), intermediate (6–10 yr), or long-term (≥11 yr) follow-up after adjusting for baseline covariates (all, p > 0.05). Conclusion: A new diagnosis of VS frequently imparts significant anxiety on the patient and leads to a temporary reduction in QOL. Notably, anxiety domain QOL scores among patients with newly diagnosed VS are poorer than patients who have observed their tumor for 6 months or longer. When feasible, an initial period of observation may afford patients valuable time to reconcile with the new diagnosis, to learn more about treatment options, and to discover whether there is tumor growth. In turn, this valuable information can be used to inform sound decision-making regarding tumor management.

Objectives: To highlight superior semicircular canal dehiscence (SSCD) involving the superior petrosal sinus (SPS), and to propose a novel classification system for SPS associated SSCD with potential surgical implications. Study Design: Multicenter retrospective review. Setting: Three tertiary referral centers. Patients: All patients diagnosed with SPS associated SSCD (1/2000 to 8/2016). Radiographic findings and clinical symptoms were analyzed. Intervention: Surgical repair or observation. Main Outcome Measure: Radiographic findings and clinical symptoms were analyzed. Results: Thirty-three dehiscences (30 patients) involving the SPS were identified. The average age at the time of presentation was 52.5 years (median, 56.9; range, 4.9–75.3 yr), and 53.3% of patients were men. Three patients had bilateral SPS associated SSCD. The most common associated symptoms at presentation were episodic vertigo (63.6%), subjective hearing loss (60.6%), and aural fullness (57.6%). Four distinct types of dehiscence were identified: class Ia. SSCD involving a single dehiscence into an otherwise normal appearing SPS; class Ib. SSCD involving a single dehiscence into an apparent venous anomaly of the SPS; class IIa. SSCD involving two distinct dehiscences into the middle cranial fossa and the SPS; class IIb. SSCD involving a single confluent dehiscence into the middle cranial fossa and the SPS. Conclusions: SSCD involving the SPS represents a small but distinct subset of SSCD cases. This scenario can create a unique set of symptoms and surgical challenges when intervention is sought. Clinical findings and considerations for surgical intervention are provided to facilitate effective diagnosis and management.

AbstractA cochlear implant (CI) is a surgically implanted device for the treatment of severe to profound sensorineural hearing loss in children and adults. It works by transducing acoustic energy into an electrical signal, which is used to stimulate surviving spiral ganglion cells of the auditory nerve. The past 2 decades have witnessed an exponential rise in the number of CI surgeries performed. Continual developments in programming strategies, device design, and minimally traumatic surgical technique have demonstrated the safety and efficacy of CI surgery. As a result, candidacy guidelines have expanded to include both pre and postlingually deaf children as young as 1 year of age, and those with greater degrees of residual hearing. A growing proportion of patients are undergoing CI for off-label or nontraditional indications including single-sided deafness, retrocochlear hearing loss, asymmetrical sensorineural hearing loss (SNHL) in adults and children with at least 1 ear that is better than performance cut-off for age, and children less than 12 months of age. Herein, we review CI design, clinical evaluation, indications, operative technique, and outcomes. We also discuss the expanding indications for CI surgery as it relates to lateral skull base pathology, comparing CI to auditory brainstem implants, and address the concerns with obtaining magnetic resonance imaging (MRI) in CI recipients.

Abstract BACKGROUND The diagnosis of vestibular schwannomas (VS) is associated with reduced patient quality of life (QOL). Minimal clinically important difference (MCID) was introduced as the lowest improvement in a patient-reported outcome (PRO) score discerned as significant by the patient. We formerly presented an MCID for the Penn Acoustic Neuroma QOL (PANQOL) battery based on cross-sectional data from 2 tertiary referral centers. OBJECTIVE To validate the PANQOL MCID values using prospective data. METHODS A prospective registry capturing QOL was queried, comprising patients treated at the authors’ institution and Acoustic Neuroma Association members. Anchor- and distribution-based techniques were utilized to determine the MCID for domain and total scores. We only included anchors with Spearman's correlation coefficient larger than 0.3 in the MCID threshold calculations. Most domains had multiple anchors with which to estimate the MCID. RESULTS A total of 1254 patients (mean age: 57.4 yr, 65% females) were analyzed. Anchor-based methods produced a span of MCID values (median, 25th-75th percentile) for each PANQOL domain and the total score: hearing (13.1, 13-16 points), balance (14, 14-19 points), pain (21, 20-28 points), face (25, 16-36 points), energy (16, 15-18 points), anxiety (16 [1 estimate]), general (13 [1 estimate]), and total (12.5, 10-15 points). CONCLUSION Current findings corroborate our formerly shared experience using multi-institutional, cross-sectional information. These MCID thresholds can serve as a pertinent outcome when deciphering the clinical magnitude of VS QOL endpoints in cross-sectional and longitudinal studies.