Repeat Radiosurgery for Sporadic Vestibular Schwannoma After Primary Radiosurgical Failure: An International Multi-institutional Investigation.

OBJECTIVE: To describe outcomes of patients with sporadic vestibular schwannoma (VS) who underwent repeat stereotactic radiosurgery (SRS) after primary SRS failure. STUDY DESIGN: Multi-institutional historical cohort study. SETTING: Five tertiary care referral centers. PATIENTS: Adults ≥18 years old with sporadic VS. INTERVENTION: Primary and repeat treatment with SRS. MAIN OUTCOME MEASURE: Microsurgery-free survival after repeat SRS. RESULTS: Across institutions, 32 patients underwent repeat SRS after primary SRS. Most patients (74%) had tumors with cerebellopontine angle extension at primary SRS (median size, 13.5 mm [interquartile range, 7.5-18.8] mm). After primary SRS, patients underwent repeat SRS at a median of 4.8 years (interquartile range, 3.2-5.7 yr). For treatment modality, 30 (94%) patients received gamma knife for primary treatment and 31 (97%) patients received gamma knife as their repeat treatment. Median tumor volume increased from 0.970 cm3 at primary SRS to 2.200 cm3 at repeat SRS. Facial nerve function worsened in two patients after primary SRS and in two patients after repeat SRS. There were no instances of intracranial complications after repeat SRS. Microsurgery-free survival rates (95% confidence interval; number still at risk) at 1, 3, and 5 years after repeat SRS were 97% (90-100%, 24), 84% (71-100%, 13), and 68% (48-96%, 6), respectively. There was one occurrence of malignancy diagnosed after repeat radiosurgery. CONCLUSION: Overall, repeat SRS for sporadic VS has comparable risk profile, but lower rates of tumor control, compared with primary SRS.

Salvage Microsurgery Following Failed Primary Radiosurgery in Sporadic Vestibular Schwannoma.

Importance: Management of sporadic vestibular schwannoma with radiosurgery is becoming increasingly common globally; however, limited data currently characterize patient outcomes in the setting of microsurgical salvage for radiosurgical failure. Objective: To describe the clinical outcomes of salvage microsurgery following failed primary stereotactic radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSRT) among patients with sporadic vestibular schwannoma. Design, Setting, and Participants: This was a cohort study of adults (≥18 years old) with sporadic vestibular schwannoma who underwent salvage microsurgery following failed primary SRS/FSRT in 7 vestibular schwannoma treatment centers across the US and Norway. Data collection was performed between July 2022 and January 2023, with data analysis performed between January and July 2023. Exposure: Salvage microsurgical tumor resection. Main Outcomes and Measures: Composite outcome of undergoing less than gross total resection (GTR) or experiencing long-term facial paresis. Results: Among 126 patients, the median (IQR) age at time of salvage microsurgery was 62 (53-70) years, 69 (55%) were female, and 113 of 117 (97%) had tumors that extended into the cerebellopontine angle at time of salvage. Of 125 patients, 96 (76%) underwent primary gamma knife SRS, while 24 (19%) underwent linear accelerator-based SRS; the remaining patients underwent FSRT using other modalities. Postoperative cerebrospinal fluid leak was seen in 15 of 126 patients (12%), hydrocephalus in 8 (6%), symptomatic stroke in 7 (6%), and meningitis in 2 (2%). Each 1-mm increase in cerebellopontine angle tumor size was associated with a 13% increased likelihood of foregoing GTR (64 of 102 patients [63%]) or long-term postoperative House-Brackmann grade higher than I (48 of 102 patients [47%]) (odds ratio, 1.13; 95% CI, 1.04-1.23). Following salvage microsurgery, tumor growth-free survival rates at 1, 3, and 5 years were 97% (95% CI, 94%-100%), 93% (95% CI, 87%-99%), and 91% (95% CI, 84%-98%), respectively. Conclusions: In this cohort study, more than half of patients who received salvage microsurgery following primary SRS/FSRT underwent less than GTR or experienced some degree of facial paresis long term. These data suggest that the cumulative risk of developing facial paresis following primary SRS/FSRT by the end of the patient's journey with treatment approximates 2.5% to 7.5% when using published primary SRS/FSRT long-term tumor control rates.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

Upfront Radiosurgery vs a Wait-and-Scan Approach for Small- or Medium-Sized Vestibular Schwannoma: The V-REX Randomized Clinical Trial.

Importance: Current guidelines for treating small- to medium-sized vestibular schwannoma recommend either upfront radiosurgery or waiting to treat until tumor growth has been detected radiographically. Objective: To determine whether upfront radiosurgery provides superior tumor volume reduction to a wait-and-scan approach for small- to medium-sized vestibular schwannoma. Design, Setting, and Participants: Randomized clinical trial of 100 patients with a newly diagnosed (<6 months) unilateral vestibular schwannoma and a maximal tumor diameter of less than 2 cm in the cerebellopontine angle as measured on magnetic resonance imaging. Participants were enrolled at the Norwegian National Unit for Vestibular Schwannoma from October 28, 2014, through October 3, 2017; 4-year follow-up ended on October 20, 2021. Interventions: Participants were randomized to receive either upfront radiosurgery (n = 50) or to undergo a wait-and-scan protocol, for which treatment was given only upon radiographically documented tumor growth (n = 50). Participants underwent 5 annual study visits consisting of clinical assessment, radiological examination, audiovestibular tests, and questionnaires. Main Outcomes and Measures: The primary outcome was the ratio between tumor volume at the trial end at 4 years and baseline (V4:V0). There were 26 prespecified secondary outcomes, including patient-reported symptoms, clinical examinations, audiovestibular tests, and quality-of-life outcomes. Safety outcomes were the risk of salvage microsurgery and radiation-associated complications. Results: Of the 100 randomized patients, 98 completed the trial and were included in the primary analysis (mean age, 54 years; 42% female). In the upfront radiosurgery group, 1 participant (2%) received repeated radiosurgery upon tumor growth, 2 (4%) needed salvage microsurgery, and 45 (94%) had no additional treatment. In the wait-and-scan group, 21 patients (42%) received radiosurgery upon tumor growth, 1 (2%) underwent salvage microsurgery, and 28 (56%) remained untreated. For the primary outcome of the ratio of tumor volume at the trial end to baseline, the geometric mean V4:V0 was 0.87 (95% CI, 0.66-1.15) in the upfront radiosurgery group and 1.51 (95% CI, 1.23-1.84) in the wait-and-scan group, showing a significantly greater tumor volume reduction in patients treated with upfront radiosurgery (wait-and-scan to upfront radiosurgery ratio, 1.73; 95% CI, 1.23-2.44; P = .002). Of 26 secondary outcomes, 25 showed no significant difference. No radiation-associated complications were observed. Conclusion and relevance: Among patients with newly diagnosed small- and medium-sized vestibular schwannoma, upfront radiosurgery demonstrated a significantly greater tumor volume reduction at 4 years than a wait-and-scan approach with treatment upon tumor growth. These findings may help inform treatment decisions for patients with vestibular schwannoma, and further investigation of long-term clinical outcomes is needed. Trial Registration: ClinicalTrials.gov Identifier: NCT02249572.

Choice of Implantable Pulse Generators for Deep Brain Stimulation: An Overview of Clinical Practice.

INTRODUCTION: The success of deep brain stimulation (DBS) treatment depends on several factors, including proper patient selection, accurate electrode placement, and adequate stimulation settings. Another factor that may impact long-term satisfaction and therapy outcomes is the type of implantable pulse generator (IPG) used: rechargeable or non-rechargeable. However, there are currently no guidelines on the choice of IPG type. The present study investigates the current practices, opinions, and factors DBS clinicians consider when choosing an IPG for their patients. METHODS: Between December 2021 and June 2022, we sent a structured questionnaire with 42 questions to DBS experts of two international, functional neurosurgery societies. The questionnaire included a rating scale where participants could rate the factors influencing their choice of IPG type and their satisfaction with certain IPG aspects. Additionally, we presented four clinical case scenarios to assess preference of choice of IPG-type in each case. RESULTS: Eighty-seven participants from 30 different countries completed the questionnaire. The three most relevant factors for IPG choice were "existing social support," "cognitive status," and "patient age." Most participants believed that patients valued avoiding repetitive replacement surgeries more than the burden of regularly recharging the IPG. Participants reported that they implanted the same amount of rechargeable as non-rechargeable IPGs for primary DBS insertions and 20% converted non-rechargeable to rechargeable IPGs during IPG replacements. Most participants estimated that rechargeable was the more cost-effective option. CONCLUSION: This present study shows that the decision-making of the choice of IPG is very individualized. We identified the key factors influencing the physician's choice of IPG. Compared to patient-centric studies, clinicians may value different aspects. Therefore, clinicians should rely not only on their opinion but also counsel patients on different types of IPGs and consider the patient's preferences. Uniform global guidelines on IPG choice may not represent regional or national differences in the healthcare systems.

Salvage radiosurgery following subtotal resection of vestibular schwannomas: does timing influence tumor control?

OBJECTIVE: The goal of microsurgical resection of vestibular schwannoma (VS) is gross-total resection (GTR) to provide oncological cure. However, a popular strategy is to halt the resection if the surgical team feels the risk of cranial nerve injury is imminent, achieving a maximally safe subtotal resection (STR) instead. The tumor remnant can then be treated with stereotactic radiosurgery (SRS) once the patient has recovered from the immediate postoperative period, or it can be followed with serial imaging and treated with SRS in a delayed fashion if residual tumor growth is seen. In this study, the authors evaluated the efficacy of this multimodality approach, particularly the influence of timing and dose of SRS on radiological tumor control, need for salvage treatment, and cranial nerve function. METHODS: VS patients treated with initial microsurgery and subsequent radiosurgery were retrospectively included from two tertiary treatment centers and dichotomized depending on whether SRS was given upfront (defined as before 12 months) or later. Radiological tumor control was defined as less than 20% tumor volume expansion and oncological tumor control as an absence of salvage treatment. Facial and cochlear nerve functions were assessed after surgery, at the time of SRS, and at last follow-up. Finally, a systematic literature review was conducted according to PRISMA guidelines. RESULTS: A total of 110 VS patients underwent SRS following microsurgical resection, with a mean preradiosurgical tumor volume of 2.2 cm3 (SD 2.5 cm3) and mean post-SRS follow-up time of 5.8 years (SD 4.1 years). The overall radiological tumor control and oncological tumor control were 77.3% and 90.9%, respectively. Thirty-five patients (31.8%) received upfront SRS, while 75 patients (68.2%) were observed for a minimum of 12 months prior to SRS. The timing of SRS did not influence the radiological tumor control (p = 0.869), the oncological tumor control (p = 0.560), or facial nerve (p = 0.413) or cochlear nerve (p = 0.954) function. An escalated marginal dose (> 12 Gy) was associated with greater tumor shrinkage (p = 0.020) and superior radiological tumor control (p = 0.020), but it did not influence the risk of salvage treatment (p = 0.904) or facial (p = 0.351) or cochlear (p = 0.601) nerve deterioration. CONCLUSIONS: Delayed SRS after close observation of residuals following STR is a safe alternative to upfront SRS regarding tumor control and cranial nerve preservation in selected patients.

Variations in the management of diffuse low-grade gliomas-A Scandinavian multicenter study.

BACKGROUND: Early extensive surgery is a cornerstone in treatment of diffuse low-grade gliomas (DLGGs), and an additional survival benefit has been demonstrated from early radiochemotherapy in selected "high-risk" patients. Still, there are a number of controversies related to DLGG management. The objective of this multicenter population-based cohort study was to explore potential variations in diagnostic work-up and treatment between treating centers in 2 Scandinavian countries with similar public health care systems. METHODS: Patients screened for inclusion underwent primary surgery of a histopathologically verified diffuse WHO grade II glioma in the time period 2012 through 2017. Clinical and radiological data were collected from medical records and locally conducted research projects, whereupon differences between countries and inter-hospital variations were explored. RESULTS: A total of 642 patients were included (male:female ratio 1:4), and annual age-standardized incidence rates were 0.9 and 0.8 per 100 000 in Norway and Sweden, respectively. Considerable inter-hospital variations were observed in preoperative work-up, tumor diagnostics, surgical strategies, techniques for intraoperative guidance, as well as choice and timing of adjuvant therapy. CONCLUSIONS: Despite geographical population-based case selection, similar health care organizations, and existing guidelines, there were considerable variations in DLGG management. While some can be attributed to differences in clinical implementation of current scientific knowledge, some of the observed inter-hospital variations reflect controversies related to diagnostics and treatment. Quantification of these disparities renders possible identification of treatment patterns associated with better or worse outcomes and may thus represent a step toward more uniform evidence-based care.

Comparing the impact of upfront radiosurgery versus expectation in vestibular schwannoma (the V-REX study): protocol for a randomised, observer-blinded, 4-year, parallel-group, single-centre, superiority study.

INTRODUCTION: The optimal management of small-sized to medium-sized vestibular schwannoma (VS) is a matter of controversy. Clinical results of the prevailing treatment modalities (microsurgery, stereotactic radiosurgery (SRS), and conservative management (CM)) are documented, but comparative studies are few, and none are randomised or blinded. Upfront radiosurgery, or a careful follow-up by MRI with subsequent treatment on growth, are two strategies used at many centres. The present study aims at comparing these strategies by randomising individuals with newly diagnosed tumours to either upfront SRS or initial CM. METHODS AND ANALYSIS: The Vestibular Schwannoma: Radiosurgery or Expectation study is designed as a randomised, controlled, observer-blinded, single-centre superiority trial with two parallel groups. Eligible patients will be randomised using sequentially numbered opaque sealed envelopes, and the radiosurgery group will undergo standard Gamma Knife Radiosurgery (GKRS) within 2 months following randomisation. The primary endpoint is tumour growth measured as volume ratio V4years/Vbaseline and volume doubling time, evaluated by annual T1 contrast MRI volumetric analysis. Secondary endpoints include symptom and sign development measured by clinical examination, audiovestibular tests, and by patient's responses to standardised validated questionnaires. In addition, the patient's working status, and the health economics involved with both strategies will be evaluated and compared. All outcome assessments will be performed by blinded observers. Power analysis indicates that 100 patients is sufficient to demonstrate the effect of GKRS on tumour volume. ETHICS AND DISSEMINATION: The trial has ethical approval from the Regional Ethical Committee (23503) and funding from The Western Norway Regional Health Authority. Trial methods and results will be reported according to the Consolidated Standards of Reporting Trials 2010 guidelines in a peer-reviewed journal. TRIAL REGISTRATION NUMBER: Clinical trials: NCT02249572. Haukeland University Hospital record: 2014/314. Regional Ethical Committee (REC West): 23 503. The Western Norway Regional Health Authority: 912 281.

Clinical Efficacy of Combined Surgical Patient Safety System and the World Health Organization's Checklists in Surgery: A Nonrandomized Clinical Trial.

Importance: Checklists have been shown to improve patient outcomes in surgery. The intraoperatively used World Health Organization surgical safety checklist (WHO SSC) is now mandatory in many countries. The only evidenced checklist to address preoperative and postoperative care is the Surgical Patient Safety System (SURPASS), which has been found to be effective in improving patient outcomes. To date, the WHO SSC and SURPASS have not been studied jointly within the perioperative pathway. Objective: To investigate the association of combined use of the preoperative and postoperative SURPASS and the WHO SSC in perioperative care with morbidity, mortality, and length of hospital stay. Design, Setting, and Participants: In a stepped-wedge cluster nonrandomized clinical trial, the preoperative and postoperative SURPASS checklists were implemented in 3 surgical departments (neurosurgery, orthopedics, and gynecology) in a Norwegian tertiary hospital, serving as their own controls. Three surgical units offered additional parallel controls. Data were collected from November 1, 2012, to March 31, 2015, including surgical procedures without any restrictions to patient age. Data were analyzed from September 25, 2018, to March 29, 2019. Interventions: Individualized preoperative and postoperative SURPASS checklists were added to the intraoperative WHO SSC. Main Outcomes and Measures: Primary outcomes were in-hospital complications, emergency reoperations, unplanned 30-day readmissions, and 30-day mortality. The secondary outcome was length of hospital stay (LOS). Results: In total, 9009 procedures (5601 women [62.2%]; mean [SD] patient age, 51.7 [22.2] years) were included, with 5117 intervention procedures (mean [SD] patient age, 51.8 [22.4] years; 2913 women [56.9%]) compared with 3892 controls (mean [SD] patient age, 51.5 [21.8] years; 2688 women [69.1%]). Parallel control units included 9678 procedures (mean [SD] patient age, 57.4 [22.2] years; 4124 women [42.6%]). In addition to the WHO SSC, adjusted analyses showed that adherence to the preoperative SURPASS checklists was associated with reduced complications (odds ratio [OR], 0.70; 95% CI, 0.50-0.98; P = .04) and reoperations (OR, 0.42; 95% CI, 0.23-0.76; P = .004). Adherence to the postoperative SURPASS checklists was associated with decreased readmissions (OR, 0.32; 95% CI, 0.16-0.64; P = .001). No changes were observed in mortality or LOS. In parallel control units, complications increased (OR, 1.09; 95% CI, 1.01-1.17; P = .04), whereas reoperations, readmissions, and mortality remained unchanged. Conclusions and Relevance: In this nonrandomized clinical trial, adding preoperative and postoperative SURPASS to the WHO SSC was associated with a reduction in the rate of complications, reoperations, and readmissions. Trial Registration: ClinicalTrials.gov Identifier: NCT01872195.

Fatigue in patients with vestibular schwannoma.

BACKGROUND: Patients with vestibular schwannoma (VS) often complain about tiredness, exhaustion, lack of energy, and strength, but such symptoms of fatigue have scarcely been objectified and analyzed in a VS population. We aimed to characterize fatigue in a cohort of patients with VS and compare such symptoms with a control group. METHODS: All patients who attended an educational course for patients with VS were surveyed with validated tools for assessment of fatigue (fatigue severity scale), anxiety and depression (hospital anxiety and depression scale), sleepiness (Epworth sleepiness scale), and apathy (Starkstein apathy scale). Quality of Life was assessed with the disease-specific Penn Acoustic Neuroma Quality of Life (PANQOL). Symptom severity was estimated with a visual analog scale (VAS). The results have been compared to a control group consisting of patient companions. RESULTS: Data from 88 VS patients and 49 controls were analyzed. The controls had similar age and sex distribution as patients. Fifty-seven percent of VS patients had significant fatigue, compared to 25% in the control group. The mean fatigue score was 4.1 for the patients, and 2.8 for controls. Patients with fatigue were more likely to have depression, anxiety, sleepiness, and apathy. No correlation of fatigue was found with age, gender, or treatment modality. Regression analyses revealed depression, apathy, and vertigo to be predictors of fatigue. Fatigue was strongly correlated to QoL. CONCLUSION: Almost six out of ten VS patients had fatigue, significantly higher than the control group. Interest and focus on fatigue in VS patients can improve the patient's QoL.

Patient Motivation and Long-Term Satisfaction with Treatment Choice in Vestibular Schwannoma.

OBJECTIVES: To ascertain primary motivation and long-term satisfaction with treatment selection in patients with vestibular schwannoma. METHODS: A multicenter, cross-sectional survey was performed. Patients with small- to medium-sized sporadic vestibular schwannoma who underwent stereotactic radiosurgery (SRS; n = 247), microsurgery (n = 144), or observation (n = 148) between 1998 and 2008 were surveyed regarding their motivation behind treatment selection and hindsight satisfaction with their choice of management. RESULTS: "Physician recommendation" was the most commonly stated reason for modality selection in all 3 groups. The second and third most common reasons for selecting SRS included "less invasive option than surgery" in 80 patients (32%) and "less recovery time than surgery" in 16 patients (6%). The second and third most common reasons for selecting observation included "to avoid side-effects of treatment" in 25 patients (17%) and "symptoms not severe enough to warrant intervention" in 22 patients (15%). The second and third most common reasons for selecting microsurgery included "do not want tumor in head" in 35 patients (24%) and "most definitive treatment" in 15 patients (10%). Overall, 232 patients (96%) treated with SRS, 141 observed patients (97%), and 121 patients (85%) who underwent microsurgical treatment were satisfied with their original decision (P < 0.001). CONCLUSIONS: Motivation behind treatment selection varies between individuals. Those who select observation and SRS commonly reference less invasiveness and lower risk, whereas those who select microsurgery are commonly motivated by having their tumor physically removed and the more definitive nature of treatment. Posttreatment satisfaction is highest in patients who undergo SRS and observation, although all 3 groups report high levels of satisfaction.

Quality of Life in Patients with Vestibular Schwannomas Following Gross Total or Less than Gross Total Microsurgical Resection: Should We be Taking the Entire Tumor Out?

BACKGROUND: The goal of microsurgical removal of a vestibular schwannoma is to completely remove the tumor, to provide long-term durable cure. In many cases, less than gross total resection (GTR) is performed to preserve neurological, and especially facial nerve function. OBJECTIVE: To analyze long-term quality of life (QoL) in a cohort of patients who received either GTR or less than GTR. METHODS: Patients operated for vestibular schwannoma less than 3.0 cm in posterior fossa diameter at 1 of 2 international tertiary care centers were surveyed using generic and disease-specific QoL instruments. RESULTS: A total of 143 patients were analyzed. GTR was performed in 122, and 21 underwent less than GTR. QoL was assessed at a mean of 7.7 yr after surgery (interquartile range: 5.7-9.6). Patients who underwent GTR had smaller tumors; otherwise, there were no baseline differences between groups. Patients who underwent GTR, after multivariable adjustment for baseline features and facial nerve and hearing outcomes, reported statistically significantly better Short Form Health Survey-36 (SF-36) physical and mental scores, Patient-Reported Outcomes Measurement Information System (PROMIS-10) physical and mental scores, and Penn Acoustic Neuroma Quality of Life (PANQOL) facial, energy, general health, and total scores compared to patients receiving less than GTR. CONCLUSION: GTR is associated with better QoL using the general QoL measures SF-36 and PROMIS-10 and the disease-specific PANQOL, even after controlling for baseline and outcome differences. This is especially significant in the assessment of mental health, indicating there may indeed be a psychological advantage to the patient that translates to overall well-being to have the entire tumor removed if microsurgical resection is undertaken.

Audiovestibular Handicap and Quality of Life in Patients With Vestibular Schwannoma and "Excellent" Hearing.

BACKGROUND: Studies examining patient-reported outcomes in subjects with vestibular schwannoma (VS) and "excellent" hearing are lacking. OBJECTIVE: To assess patient-reported audiovestibular handicap and overall quality of life (QoL) in VS patients with class A hearing in both ears. METHODS: Among 539 VS patients treated during 1998 to 2008, we identified 296 patients with either bilateral class A (AA) hearing or 1 good ear and 1 deaf ear (AD) according to the American Academy of Otolaryngology-Head and Neck Surgery classification. Patients responded to validated hearing, tinnitus, and dizziness handicap inventories and 2 QoL questionnaires, and the 2 groups were compared. A reference group of 103 adults filled out the same questionnaires. RESULTS: Forty-nine patients (16.6%) had class AA and 247 patients (83.4%) had class AD hearing. AA patients scored poorer than control subjects without tumor on all handicap questionnaires ( P < .001) and a VS-specific QoL instrument ( P = .006). Con-versely, AA patients scored significantly better than patients with AD on the hearing inventory and the disease-specific QoL instrument ( P < .001), but no difference was found between these groups with regard to tinnitus and dizziness. The hearing disability score was approximately 3 times poorer for AA patients compared with control subjects without tumor; a third of AA patients reported a hearing handicap. CONCLUSION: Patients with VS and bilateral class A hearing report significantly poorer hearing handicap than control subjects without tumor but better hearing than those with unilateral deafness. When patients with bilateral class A hearing are counseled, it should be noted that one-third of patients experience self-perceived hearing handicap.

Patient- versus physician-reported facial disability in vestibular schwannoma: an international cross-sectional study.

OBJECTIVE Patient-reported outcomes are increasingly used in studies of vestibular schwannoma (VS); however, few studies have examined self-evaluated facial nerve function and its relation to physician-reported outcomes. The primary objective of this study was to compare patient self-evaluations of facial disability with physician-evaluated facial nerve status and with self-evaluations of a healthy control group. The second objective was to provide insight into the controversial subject of the optimal initial management of small- and medium-sized VSs; consequently, the authors compared patient-reported facial nerve disability following treatment via observation (OBS), Gamma Knife surgery (GKS), or microsurgery (MS). Lastly, the authors sought to identify risk factors for facial nerve dysfunction following treatment for small- and medium-sized VSs. METHODS All patients with a VS 3 cm or smaller that was singly treated with OBS, GKS, or MS at either of 2 independent treatment centers between 1998 and 2008 were retrospectively identified. Longitudinal facial nerve measures and clinical data, including facial nerve evaluation according to the House-Brackmann (HB) grading system, were extracted from existing VS databases. Supplementing the objective data were Facial Disability Index (FDI) scores, which were obtained via survey of patients a mean of 7.7 years after initial treatment. RESULTS The response rate among the 682 eligible patients was 79%; thus, data from a total of 539 patients were analyzed. One hundred forty-eight patients had been managed by OBS, 247 with GKS, and 144 with MS. Patients who underwent microsurgery had larger tumors and were younger than those who underwent OBS or GKS. Overall, facial nerve outcomes were satisfactory following treatment, with more than 90% of patients having HB Grade I function at the last clinical follow-up. Treatment was the major risk factor for facial nerve dysfunction. Almost one-fifth of the patients treated with MS had an objective decline in facial nerve function, whereas only 2% in the GKS group and 0% in the OBS cohort had a decline. The physical subscale of the FDI in the VS patients was highly associated with HB grade; however, the social/well-being subscale of the FDI was not. Thus, any social disability caused by facial palsy was not detectable by use of this questionnaire. CONCLUSIONS The majority of patients with small- and medium-sized VSs attain excellent long-term facial nerve function and low facial nerve disability regardless of treatment modality. Tumor size and microsurgical treatment are risk factors for facial nerve dysfunction and self-reported disability. The FDI questionnaire is sensitive to the physical but not the social impairment associated with facial dysfunction.

Surgical salvage of recurrent vestibular schwannoma following prior stereotactic radiosurgery.

OBJECTIVES/HYPOTHESIS: To evaluate outcomes of salvage surgery for vestibular schwannoma (VS) that failed primary stereotactic radiosurgery (SRS). METHODS: Case-control study of 37 patients who underwent surgical resection of sporadic VS following prior SRS at two tertiary academic referral centers between 2003 and 2015. A cohort of nonirradiated control subjects, matched according to tumor size, age, and treatment center, were used as comparison. RESULTS: Thirty-seven patients were included. The median time from radiation to surgical salvage was 36 months (range 9.6-153 months). Following tumor progression after SRS, 18 (49%) patients underwent gross total resection, 10 (27%) underwent near-total resection, and nine (24%) underwent subtotal resection. Postoperative complications following salvage surgery included one (3%) case of stroke, four (11%) cases of cerebrospinal fluid leak, and two (5%) cases of meningitis. Twenty-seven (73%) patients had good postoperative facial nerve outcome (House-Brackmann Score I-II) at long-term follow-up. There were no cases of tumor recurrence or regrowth after a median length of 26 months following microsurgical salvage (range 3-114 months). The rate of satisfactory postoperative facial nerve function was not different between study and control subjects (73% vs. 76%; P = 0.8); however, less-than-complete resection was utilized more frequently among previously radiated patients (P = 0.01). CONCLUSION: Microsurgical salvage of VS following primary radiation therapy is challenging. Less-than-complete resection is required in a greater percentage of patients to preserve facial nerve integrity and prevent neurological complications. Long-term follow-up is needed to determine the risk of delayed progression following incomplete tumor removal. LEVEL OF EVIDENCE: 3b. Laryngoscope, 126:2580-2586, 2016.

Risk factors and analysis of long-term headache in sporadic vestibular schwannoma: a multicenter cross-sectional study.

OBJECT: The primary goals of this study were: 1) to examine the influence of disease and treatment on headache in patients with sporadic vestibular schwannoma (VS); and 2) to identify clinical predictors of long-term headache disability. METHODS: This was a cross-sectional observational study with international multicenter enrollment. Patients included those with primary sporadic <3-cm VS and a separate group of general population control subjects without tumors. Interventions included a postal survey incorporating the Headache Disability Inventory (HDI), the Hospital Anxiety and Depression Scale, and a VS symptom questionnaire. The main outcome measures were univariate and multivariable associations with HDI total score. RESULTS: The overall survey response rate was 79%. Data from 538 patients with VS were analyzed. The mean age at time of survey was 64 years, 56% of patients were female, and the average duration between treatment and survey was 7.7 years. Twenty-seven percent of patients received microsurgery, 46% stereotactic radiosurgery, and 28% observation. Patients with VS who were managed with observation were more than twice as likely to have severe headache disability compared with 103 control subjects without VS. When accounting for baseline differences, there was no statistically significant difference in HDI outcome between treatment modalities at time of survey. Similarly, among the microsurgery cohort, the long-term risk of severe headache disability was not different between surgical approaches. Multivariable regression demonstrated that younger age, greater anxiety and depression, and a preexisting diagnosis of headache were the primary predictors of severe long-term headache disability, while tumor size and treatment modality had little influence. CONCLUSIONS: At a mean of almost 8 years following treatment, approximately half of patients with VS experience headaches of varying frequency and severity. Patient-driven factors including age, sex, mental health, and preexisting headache syndrome are the strongest predictors of long-term severe headache disability. Tumor size and treatment modality have less impact. These data may assist with patient counseling regarding long-term expectations following diagnosis and treatment.

The Minimal Clinically Important Difference in Vestibular Schwannoma Quality-of-Life Assessment: An Important Step beyond P < .05.

OBJECTIVE: Several studies have demonstrated small but statistically significant differences in quality-of-life (QOL) scores among vestibular schwannoma (VS) treatment modalities. However, does a several-point difference on a 100-point scale really matter? The minimal clinically important difference (MCID)-defined as the smallest difference in scores that patients perceive as important and that could lead to a change in management-was developed to answer this important question. While the MCID has been determined for QOL measures used in other diseases, it remains undefined in the VS literature. STUDY DESIGN: Distribution- and anchor-based techniques were utilized to define the MCID for the Penn Acoustic Neuroma Quality of Life (PANQOL) and 36-Item Short Form Health Survey (SF-36). SETTING: Two academic referral centers. PATIENTS: Patients with VS (N = 538). INTERVENTION: Cross-sectional postal survey. MAIN OUTCOME MEASURES: MCID for PANQOL domains and total score and SF-36 Physical and Mental Health Component Summary scores. RESULTS: The MCID (median, interquartile range) for the PANQOL total score was 11 points (10-12); the MCIDs for individual domains were as follows: hearing, 6 (5-8); balance, 16 (14-19); facial, 10 (no interquartile range); pain, 11 (10-13); energy, 13 (10-17); anxiety, 11 (5-22); and general, 15 (11-19). The MCID was 7 points (6-11) for the SF-36 Mental Health Component Summary score and 8 points (6-10) for the Physical Health Component Summary score. CONCLUSIONS: The MCIDs determined in the current study generally exceed differences reported in previous prospective studies, in which conclusions about QOL benefit (or harm) among VS treatment modalities were based on statistical significance alone. Moving forward, these MCIDs should be considered when interpreting results of VS QOL studies.

Long-term Auditory Symptoms in Patients With Sporadic Vestibular Schwannoma: An International Cross-Sectional Study.

BACKGROUND: There are limited data on the long-term auditory symptoms in patients with sporadic small- and medium-sized vestibular schwannoma (VS). The initial treatment strategy for VS is controversial. OBJECTIVE: To characterize auditory symptoms in a large cohort of patients with VS. METHODS: Patients with ≤3 cm VS who underwent primary microsurgery, gamma knife surgery, or observation between 1998 and 2008 at 2 independent hospitals were identified. Clinical data were extracted from existing VS databases. At a mean time of 7.7 years after initial treatment, patients were surveyed via mail with the use of the Hearing Handicap Inventory for Adults (HHIA) and the Tinnitus Handicap Inventory. RESULTS: The response rate was 79%; a total of 539 respondents were analyzed. Overall, the hearing prognosis was poor, because more than 75% of all patients had nonserviceable hearing at the last clinical follow-up. Good baseline hearing proved to be a strong predictor for maintained serviceable hearing. Treatment modality was independently associated with both audiometric outcome and HHIA results. Active treatment with microsurgery or gamma knife surgery did not appear to be protective, because patients who were observed had the greatest probability of durable hearing. Patients in the surgical series had the greatest hearing loss. Tinnitus Handicap Inventory results were less predictable. The only predictors of tinnitus handicap were age and HHIA score. CONCLUSION: The overall prognosis for hearing in sporadic VS is poor regardless of treatment strategy. Treatment modality was an independent predictor of hearing status; observation was associated with the highest rate of hearing preservation. .

Long-term quality of life in patients with vestibular schwannoma: an international multicenter cross-sectional study comparing microsurgery, stereotactic radiosurgery, observation, and nontumor controls.

OBJECT: The optimal treatment for sporadic vestibular schwannoma (VS) is highly controversial. To date, the majority of studies comparing treatment modalities have focused on a narrow scope of technical outcomes including facial function, hearing status, and tumor control. Very few publications have investigated health-related quality of life (HRQOL) differences between individual treatment groups, and none have used a disease-specific HRQOL instrument. METHODS: All patients with sporadic small- to medium-sized VSs who underwent primary microsurgery, stereotactic radiosurgery (SRS), or observation between 1998 and 2008 were identified. Subjects were surveyed via postal questionnaire using the 36-Item Short Form Health Survey (SF-36), the 10-item Patient-Reported Outcomes Measurement Information System short form (PROMIS-10), the Glasgow Benefit Inventory (GBI), and the Penn Acoustic Neuroma Quality-of-Life (PANQOL) scale. Additionally, a pool of general population adults was surveyed, providing a nontumor control group for comparison. RESULTS: A total of 642 respondents were analyzed. The overall response rate for patients with VS was 79%, and the mean time interval between treatment and survey was 7.7 years. Using multivariate regression, there were no statistically significant differences between management groups with respect to the PROMIS-10 physical or mental health dimensions, the SF-36 Physical or Mental Component Summary scores, or the PANQOL general, anxiety, hearing, or energy subdomains. Patients who underwent SRS or observation reported a better total PANQOL score and higher PANQOL facial, balance, and pain subdomain scores than the microsurgical cohort (p < 0.02). The differences in scores between the nontumor control group and patients with VS were greater than differences observed between individual treatment groups for the majority of measures. CONCLUSIONS: The differences in HRQOL outcomes following SRS, observation, and microsurgery for VS are small. Notably, the diagnosis of VS rather than treatment strategy most significantly impacts quality of life. Understanding that a large number of VSs do not grow following discovery, and that intervention does not confer a long-term HRQOL advantage, small- and medium-sized VS should be initially observed, while intervention should be reserved for patients with unequivocal tumor growth or intractable symptoms that are amenable to treatment. Future studies assessing HRQOL in VS patients should prioritize use of validated disease-specific measures, such as the PANQOL, given the significant limitations of generic instruments in distinguishing between treatment groups and tumor versus nontumor subjects.

What drives quality of life in patients with sporadic vestibular schwannoma?

OBJECTIVES/HYPOTHESIS: To investigate the influence of posttreatment audiovestibular symptoms, facial neuropathy, and headache on long-term quality-of-life outcomes in patients with sporadic vestibular schwannoma (VS) utilizing the Short Form 36 (SF-36) Health Survey and the Penn Acoustic Neuroma Quality of Life (PANQOL) scale. STUDY DESIGN: Cross-sectional observation study. METHODS: Patients with sporadic small- or medium-sized VS (< 3.0 cm) who were evaluated between 1998 and 2008 at two independent tertiary academic referral centers were surveyed. Multivariable associations with the PANQOL total score and the SF-36 physical and mental component scores evaluated using regression analysis. RESULTS: A total of 538 surveyed patients returned a completed questionnaire, providing a response rate of 79%. Two hundred forty-seven (46%) patients underwent stereotactic radiosurgery, 143 (27%) microsurgery, and 148 (28%) observation. Multivariable regression analysis revealed that ongoing dizziness was associated with the greatest reduction in PANQOL total score, followed by headache. After adjusting for all examined features, ongoing dizziness and ongoing headache were the only two variables that were associated with both the SF-36 physical and mental component scores. Patient sex and treatment modality did not significantly influence PANQOL or SF-36 scores. CONCLUSIONS: Ongoing dizziness and headache are the strongest predictors of long-term quality-of-life reduction in patients with sporadic VS, while the impact of hearing loss, facial nerve function, and tinnitus are less by comparison. This information may be valuable for patient counseling, refinement of VS quality-of-life assessment instruments, and determining high-yield targets for therapy in efforts to further improve patient outcomes.