Neff, Brian A.

Abstract BACKGROUND Vestibular schwannomas (VS) have a peak incidence in the sixth and seventh decades of life. Stereotactic radiosurgery is often the preferred treatment for VS among patients of advanced age. The fraction of elderly patients potentially requiring consideration for surgical treatment is anticipated to expand, mandating an update to management paradigms in this population. OBJECTIVE To describe our experience with surgical management of VS in patients aged 75 yr and older. METHODS Cohort study of all patients aged ≥75 yr with sporadic VS requiring surgical treatment at our institution between 1999 and 2020. Data included preoperative baseline characteristics and outcome data including extent of resection, facial nerve and hearing status, functional outcome, length of stay, and complications. RESULTS A total of 24 patients were included, spanning an age range of 75 to 90 yr. Average tumor size was 2.76 ± 1.04 cm, and average baseline Modified 5-item Frailty Index (mFI-5) score was 1.08 ± 0.93. Extent of resection was gross total in 5 (20.8%), near total in 3 (12.5%), and sub-total resection in the remaining 16 (66.7%). One patient died in the postoperative period because of an acute sub-dural hematoma. Favorable facial nerve function (HB1-2) was preserved in 12 patients (75%) between 75 and 79 yr and 2 patients (28.6%) aged ≥ 80 yr. No cerebrospinal fluid leak or surgical site infection was observed; 3 patients developed hydrocephalus requiring ventriculo-peritoneal shunt placement. Nine patients required out-of-home disposition; all patients eventually returned to independent living. CONCLUSION Microsurgical resection of VS can be safely undertaken in patients greater than 75 y/o but may carry an increased risk of poor facial function.

Objective: To compare the prevalence of surgical, anesthetic, and device-related complications among infants and older children receiving cochlear implantation (CI). Study Design: Retrospective chart review. Setting: Tertiary academic referral center. Patients: Pediatric patients who underwent CI from November 1990 to January 2020. Intervention: CI. Main Outcome Measures: Surgical, anesthetic, and device-related complication rates were compared by age group (<12 versus 12–23 versus 24+ months with subset analysis of <9 versus 9–11 months). Results: A total of 406 primary pediatric CI surgeries encompassing 482 ears were analyzed, including 45 ears in 23 patients implanted less than 9 months and 89 ears in 49 patients less than 12 months. No anesthetic complications occurred. Postoperative surgical and device-related complication rates were not significantly different among the less than 12, 12 to 23, and 24+ month groups (16% versus 16% versus 12%; p = 0.23) or between the less than 9 and 9 to 11 month groups (22% versus 9%; p = 0.09). Thirty-day readmission was significantly higher for patients less than 12 months compared with patients 24+ months (6% versus <1%; p = 0.011), but was not significantly higher compared with patients 12 to 23 months (6% versus 3%; p = 0.65). Reoperation rates did not differ significantly among the less than 12, 12 to 23, and 24+ month groups (10% versus 7% versus 6%; p = 0.31). Conclusions: The prevalence of surgical, anesthetic, and device related complications was not significantly different among infants implanted less than 9 or less than 12 months of age when compared with older children. These data provide evidence for the continued expansion of pediatric cochlear implant candidacy criteria to include appropriately selected infants less than 9 months of age.

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.

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 evaluate adverse events and feasibility of performing 1.5-T MRI in patients with cochlear implants (CI) and auditory brainstem implants (ABI). Setting: Single tertiary academic referral center. Patients: CI and ABI recipients undergoing 1.5-T MRI without internal magnet removal. Intervention(s): MRI after tight headwrap application. Main Outcome Measures: Adverse events, patient tolerance. Results: A total of 131 MR studies in 79 patients were performed, with a total of 157 study ears. Sixty-one patients (77%) had unilateral devices. Four patients (5%) underwent MRI with ABI magnets in place. Sixteen patients (20%) had MRI-compatible devices that did not require a head wrap. There were no instances of device stimulation, device malfunction, or excessive heating of the receiver-stimulator package. Magnet tilt requiring manual repositioning occurred during seven studies (4.5%) and magnet displacement requiring operative intervention occurred during seven studies (4.5%). Significant pain where imaging had to be discontinued occurred during three episodes (2%). No adverse events were noted among patients who underwent MRI with an MRI-compatible magnet. Conclusions: MRI with CI or ABI magnets in place is associated with a low prevalence of adverse events when performed in a controlled setting. Many partial magnet displacements can be corrected with firm manual pressure. Devices with magnets that align with the field within their housing were not associated with any adverse events and do not require immobilization of the magnet during the scan. These may be valuable in patients with known or anticipated need for MRI.

OBJECTIVE The object of this study was to ascertain outcomes of cochlear implantation (CI) following stereotactic radiosurgery (SRS) for vestibular schwannoma (VS). METHODS The authors conducted a retrospective chart review of adult patients with VS treated with SRS who underwent CI between 1990 and 2019 at a single tertiary care referral center. Patient demographics, tumor features, treatment parameters, and pre- and postimplantation audiometric and clinical outcomes are presented. RESULTS Seventeen patients (18 ears) underwent SRS and ipsilateral CI during the study period. Thirteen patients (76%) had neurofibromatosis type 2 (NF2). Median age at SRS and CI were 44 and 48 years, respectively. Median time from SRS to CI was 60 days, but notably, 4 patients underwent SRS and CI within 1 day and 5 patients underwent CI more than 7 years after SRS. Median marginal dose was 13 Gy. Median treatment volume at the time of SRS was 1400 mm 3 (range 84–6080 mm 3 , n = 15 patients). Median post-CI PTA was 28 dB HL, improved from 101 dB HL preoperatively (p < 0.001). Overall, 11 patients (12 ears) exhibited open-set speech understanding. Sentence testing was performed at a median of 10 months (range 1–143 months) post-CI. The median AzBio sentence score for patients with open-set speech understanding was 76% (range 19%–95%, n = 10 ears). Two ears exhibited Hearing in Noise Test (HINT) sentence scores of 49% and 95%, respectively. Four patients achieved environmental sound awareness without open-set speech recognition. Two had no detectable auditory percepts. CONCLUSIONS Most patients who underwent CI following SRS for VS enjoyed access to sound at near-normal levels, with the majority achieving good open-set speech understanding. Implantation can be performed immediately following SRS or in a delayed fashion, depending on hearing status as well as other factors. This strategy may be applied to cases of sporadic or NF2-associated VS.

Objective: Definitive treatment of sporadic vestibular schwannoma (VS) following documented growth is common practice at most centers in the United States. However, as a natural extension of this paradigm, very little evidence exists surrounding the natural history of growing tumors. The primary objective of the current work was to describe the natural history of sporadic VS following documentation of initial tumor growth. Study Design: Retrospective cohort study. Setting: Tertiary referral center. Patients: Patients diagnosed with sporadic VS between January 1, 2001 and December 31, 2015 who elected continued observation despite having volumetric growth ≥20% of original tumor volume on interval magnetic resonance imaging (MRI). Main Outcome Measure: Survival free of subsequent volumetric growth. Results: Of 361 patients undergoing observation with serial imaging during the study period, 85 patients met inclusion criteria at a median age of 66 years (interquartile ranges [IQR] 55–71). Within this cohort, 40 patients demonstrated subsequent volumetric growth at a median of 1.7 years (IQR 1.0–2.6) from the date of initial MRI that documented growth. The median volumetric growth was 43% (IQR 28–57), and the median growth rate was 0.026 cm3 per year (IQR 0.009–0.107). Survival free of subsequent volumetric growth rates (95% CI; number still at risk) at 1, 2, 3, 4, and 5 years were 93% (87–99; 75), 67% (58–79; 45), 54% (43–67; 29), 44% (33–59; 19), and 41% (30–56; 12), respectively. Conclusions: In a cohort exclusively comprised of sporadic VS with documented growth, over 40% of tumors demonstrated no subsequent volumetric growth after 5 years of continued MRI surveillance. These data challenge the supposition that once growth occurs, all tumors will exhibit sustained growth. Continued observation after documented growth of sporadic VS is a reasonable consideration in appropriately selected cases.