Haynes, David S.

ABSTRACT Objective Management of vestibular schwannoma (VS) remains controversial. Very small VSs represent a pivotal group: on one hand, they pose the least risk to the patient from the perspective of being small; yet, not recommending treatment may result in missing a critical window for achieving the best outcome. This debate fundamentally hinges on whether treatment improves upon the natural history of the disease. There is an unproven assumption that very small tumors behave similarly to the broader observed population. To address this central question, we conducted a multicenter retrospective review to compare the natural history of tumor growth and hearing for patients with very small VS (1–3 mm) with a referent cohort with larger tumors. Methods All patients diagnosed with sporadic VS 1–3 mm were reviewed at 3 large skull base referral centers and compared with a referent cohort of patients with tumors > 3 mm. Tumor growth‐free survival rates, treatment‐free survival rates, and rates of maintenance of class A, B, or C hearing were estimated using the Kaplan–Meier method. Results In total, 123 patients with very small VS and a referent cohort of 326 patients were studied. Seventeen patients with very small VS experienced tumor growth at a median of 3.2 years after diagnosis (IQR 2.6–5.1), demonstrating a median growth rate of 1.0 mm per year (IQR 0.9–1.8). In comparison, 167 of the patients in the referent cohort experienced tumor growth at a median of 1.7 years after diagnosis (IQR 0.8–3.1). Tumor growth‐free survival rates (95% CI, number still at risk) at 5 years after diagnosis for the 2 cohorts were 84% (75–93, 39) and 45% (40–52, 100), respectively ( p < 0.001). Six patients with very small VS underwent treatment after tumor growth at a median of 3.2 years after diagnosis (IQR 3.2–3.8), compared with 138 patients in the referent cohort at a median of 2.2 years after diagnosis (IQR 1.4–3.6). Treatment‐free survival rates (95% CI, number still at risk) at 5 years after diagnosis for the 2 cohorts were 92% (86–98, 55) and 54% (49–61, 125), respectively ( p < 0.001). Among the 105 patients with very small VS and class A, B, or C hearing at diagnosis, 3 progressed to class D. Comparatively, among the 225 patients in the referent cohort with class A, B, or C hearing at diagnosis, 70 progressed to class D at a median of 2.6 years after diagnosis (IQR 1.0–4.8). Rates of maintenance of class A, B, or C hearing (95% CI, number still at risk) at 5 years after diagnosis were 96% (92–100, 28) for patients with very small VS and 65% (57–73, 70) for the referent cohort ( p < 0.001). Conclusions The natural history of tumor growth and hearing for very small VS diverges favorably from the cumulative natural history data and provides a more appropriate benchmark to compare clinical outcomes of upfront surgery or radiosurgery for this population. At 5 years after diagnosis, 96% of very small VS maintained class A, B, or C hearing and only 8% received treatment for growth within this multicenter cohort. Cumulatively, these data substantiate initial wait‐and‐scan management via size threshold surveillance in this population. Level of Evidence 3.

Objective: The Complete Cochlear Implant Care (CCIC) model is a highly coordinated care delivery model reducing and condensing in-person visits for cochlear implant (CI) patients, leveraging telehealth and remote programming. Clinical, quality-of-life, implementation, patient experience, and financial outcomes of the clinical trial are reported. Study design: Prospective, nonrandomized, 2-arm clinical trial Setting: Tertiary referral CI center Patients: Adult CI candidates Interventions: Cochlear implantation Main outcome measures: Speech perception, quality of life, implementation, patient experience, and financial outcomes for both CCIC and traditional care delivery models. Results: Patients participating in the traditional and CCIC delivery models demonstrated 12-month postoperative speech perception scores comparable to nationally reported outcomes (CNC 61% and 72% and AzBio in quiet 75% and 87% for traditional and CCIC cohorts, respectively). Quality-of-life evaluation using the CIQOL-10 demonstrated similar 12-month postoperative scores between groups (52 and 54 for traditional and CCIC cohorts, respectively). The CI Patient Experience Survey demonstrated high satisfaction outcomes in ease of communication, preparedness for surgery, and overall care in both cohorts. Implementation analysis of the CCIC model revealed perceived benefits from staff and clinicians; however, support for expansion of the program was contingent upon the availability of app-based or web-based remote programming technology. Finally, financial analysis revealed decreased out-of-pocket costs for CCIC patients. Conclusion: The CCIC model has the potential to dramatically streamline hearing health care delivery and reduce out-of-pocket costs for CI recipients. Clinical outcomes between cohorts were comparable in this feasibility study; however, a sufficiently powered trial is required to definitively comment on clinical outcomes with the new care delivery model.

Objective To assess Advanced Bionics (AB) HiRes Ultra and Ultra 3D initial version (V1) cochlear implant electrode failures over time at four large cochlear implant programs. Study Design Retrospective cohort. Setting Four tertiary referral centers. Patients Patients receiving AB HiRes Ultra and Ultra 3D initial version (V1) devices as of December 31, 2022. Main Outcome Measures Failure rate, revision surgery, speech recognition scores. Results Over a span of 7 years, 205 (42.6%) of 483 implanted V1 devices have failed. Device failure rate varied across institutions from 32 to 67%. Of the 205 detected failures, 163 (79%) have undergone revision surgery, with 94% of revisions being performed with Advanced Bionics devices. Average time from implantation to diagnosis of device failure was 2.7 ± 1.2 years. Most patients (89.8%) with available testing were matched or exceeded their best prefailure speech performance after implant revision. After revision, patients had an average CNC score improvement of 24.7 percentage points over their most recent pre-revision scores and demonstrated average datalogging of 12.7 ± 4.1 h/d at most recent evaluation. Conclusions Comparison of patients across multiple high-volume implant centers confirms the presence of ongoing device failures. There is variability across institutions in the rate of revision surgery once a patient is diagnosed with a V1 device failure, as well as in the rate of device failure detection. Inter-institutional variability in failure rates may be explained by the variation in the routine use of electrical field imaging. Reimplantation with a new device typically results in a return to prefailure peak performance. Professional Practice Gap & Educational Need To our knowledge, a comparison of AB HiRes (V1) device failures across multiple institutions has not been conducted. Learning Objective To identify device failure rate across multiple institutions with different testing protocols. Desired Result Providers will have an improved understanding of the trajectory of device failures for HiRes (V1) devices over time. Level of Evidence Level IV—Historical cohort or case-controlled studies. Indicate IRB or IACUC IRB #230017; IRB #22-000183; IRB #STU-032018-085; IRB #H-49479.

Objective To introduce and discuss implementation strategy for the Complete Cochlear Implant Care (CCIC) model, a highly-coordinated cochlear implant (CI) care delivery model requiring a single on-site visit for preoperative workup, surgery, and postoperative programming. Study Design Prospective, nonrandomized, two-arm clinical trial. Setting Tertiary referral CI center. Patients Adults who meet audiologic criteria for cochlear implantation. Interventions Cochlear implantation, coordinated care delivery, including remote programming. Main outcome measures Care delivery model feasibility and process implementation. Results Patients determined to be likely CI candidates based on routine audiometry are eligible for enrollment. The CCIC model uses telemedicine and electronic educational materials to prepare patients for same-day on-site consultation with CI surgery, same or next-day activation, and postoperative remote programming for 12 months. Implementation challenges include overcoming inertia related to the implementation of a new clinical workflow, whereas scalability of the CCIC model is limited by current hardware requirements for remote programming technology. A dedicated CCIC process coordinator is critical for overcoming obstacles in implementation and process improvement through feedback and iterative changes. Team and patient-facing materials are included and should be tailored to fit each unique CI program looking to implement CCIC. Conclusion The CCIC model has the potential to dramatically streamline hearing healthcare delivery. Implementation requires an adaptive approach, as obstacles may vary according to institutional infrastructure and policies.

ObjectiveTo characterize the catchment area and patient profile of large cochlear implant (CI) centers in the United States.Study DesignMulti‐institutional retrospective case series.SettingTertiary referral CI centers.MethodsPatients who underwent CI surgery at 7 participating CI centers between 2015 and 2020 were identified. Patients’ residential zip codes were used to approximate travel distances and urban vs rural residential areas.ResultsOver the 6‐year study period (2015‐2020), 6313 unique CI surgical procedures occurred (4529 adult, 1784 pediatric). Between 2015 and 2019, CI procedures increased by 43%. Patients traveled a median 52 miles (interquartile range, 21‐110) each way; patients treated at rural CI centers traveled greater distances vs those treated at urban centers (72 vs 46 miles, P <. 001). Rural residents represented 61% of the patient population and traveled farther than urban residents (73 vs 24 miles, P <. 001). Overall, 91% of patients lived within a 200‐mile radius of the institution, while 71% lived within a 100‐mile radius. In adults, multiple regression analysis redemonstrated an association between greater travel distances and (1) older age at the time of CI and (2) residential rural setting (both P <. 001, r2 = 0.2).ConclusionsWhile large CI centers serve geographically dispersed populations, most patients reside within a 200‐mile radius. Strategies to expand CI utilization may leverage remote programming, telemedicine, and strategic placement of new centers and satellite clinics to ameliorate travel burden.

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: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.