Sporadic Vestibular Schwannoma Research Articles

Associations of Microvascular Risk Factors with Sporadic Vestibular Schwannoma Outcomes Following Stereotactic Radiosurgery.

Stereotactic radiosurgery (SRS) is increasingly used for small-to-medium-sized sporadic vestibular schwannoma (VS) and is associated with good tumor control and low-risk of adverse radiation-associated events. The exact mechanism of VS tumor control is unknown but may relate to microvascular hyalinization and resultant tumoral ischemia. This study examined associations of microvascular risk factors with outcomes following SRS. Historical cohort of patients who underwent SRS for sporadic VS from 2000 to 2022. Tertiary academic center. Associations of microvascular risk factors with tumor control and complications were evaluated using Cox proportional hazards regression. In total 749 patients were studied, 31% with a history of smoking, 38% obesity, 19% hypertension, 8% diabetes, 3% peripheral vascular disease, and 2% history of coronary bypass. Regarding tumor control, no factor was associated with salvage treatment following SRS (n = 42). Hypertension (hazard ratio [HR] 2.81; P = .02) and coronary bypass (HR 6.91; P = .002) were significantly associated with developing facial nerve paresis (n = 22). No significant associations with new facial spasms (n = 53) were identified. Lastly, 191 of 294 patients with serviceable hearing at SRS progressed to nonserviceable hearing at a median 2.0 years (interquartile range: 1.0-5.0). After multivariable adjustment for age and ipsilateral hearing status, the HR for the association of smoking history with time to nonserviceable hearing was 1.46 (95% confidence interval 1.04-2.04; P = .03). We demonstrate that hypertension and history of coronary bypass may be associated with development of facial nerve weakness, while smoking may be associated with accelerated hearing loss in patients undergoing SRS for sporadic VS. These data may help guide patient counseling and inform decision-making regarding treatment.

Quantitative Assessment of Collagen Architecture to Determine Role of Tumor Stroma During Vestibular Schwannoma Progression.

The primary objective was to characterize the abundance and architecture of collagen in the extracellular matrix in vestibular schwannoma (VS). The secondary objective was to investigate the association between collagen architecture and tumor size. Retrospective cohort study. Academic referral center. Tumor samples were obtained from patients with sporadic VS undergoing microsurgical resection. Histological analyses were performed including picrosirius red (PSR) staining under polarized light. Collagen architecture was quantified using an automated fiber detection software. Second Harmonic Generation (SHG) microscopy and immunofluorescence (IF) were utilized to characterize collagen architecture. Eleven tumor specimens were included (mean tumor diameter = 2.80 cm, range 1.5-4.0 cm), and were divided into large (mean diameter = 3.5 ± 0.4 cm) and small (mean tumor diameter = 2.0 ± 0.4 cm) cohorts based on size. The large VS cohort showed significantly higher collagen density (27.65% vs 12.73%, P = .0043), with more thick fibers (mature Type I, 24.54% vs 12.97%, P = .0022) and thin fibers (immature Type I or mature Type III, 23.55% vs 12.27%, P = .026). Tumor volume correlated with greater degree of collagen fiber disorganization (P = .0413, r2 = 0.298). Specifically, collagen type I intensity was significantly higher in large VS compared to small tumors (P < .001) and peripheral nerve (P = .028). Larger VS exhibit increased collagen abundance in the tumor stroma, and a more disorganized collagen architecture compared to smaller VS and normal peripheral nerve tissue. This finding indicates that collagen organization may play a significant role in extracellular matrix remodeling and the progression of VS.

Minimum cochlear dose impacts hearing after radiosurgery for sporadic vestibular schwannoma.

It is uncertain which cochlear dose parameters significantly impact hearing after stereotactic radiosurgery (SRS) for sporadic vestibular schwannoma (VS). The objective of this study was to determine the impact of cochlear dose parameters on hearing outcomes for patients with serviceable hearing (SH). This was a historical cohort study performed at a single tertiary center that included patients with sporadic VS and SH who underwent single-session Gamma Knife radiosurgery treatment from 2007 to 2022. Associations of cochlear dose parameters with time to non-SH and rates of change in pure-tone average (PTA) and word recognition score (WRS) following SRS were assessed. A total of 205 patients with SH underwent SRS for a sporadic VS. At SRS, 54 (26%) tumors were confined to the internal auditory canal and 151 (74%) extended into the cerebellopontine angle. At 2, 5, and 10 years following SRS, 62%, 37%, and 15% of patients maintained SH, respectively. The median time to non-SH was 1.8 years. The median rates of change in PTA and WRS were 6.0 dB of hearing loss per year and -6.5% per year, respectively. In a multivariable analysis, each 1-Gy increase in minimum cochlear dose was significantly associated with time to non-SH (HR 1.5, 95% CI 1.2-1.9), rate of change in PTA decibel hearing loss per year (parameter estimate [PE] 3.4, 95% CI 0.6-6.2), and rate of change in WRS percentage per year (PE -6.4, 95% CI -11.2 to -1.5). The associations of each 1-Gy increase in mean cochlear dose with hearing outcomes were only significant among patients with class B hearing (time to non-SH HR 1.3, 95% CI 1.1-1.6; rate of change in PTA PE 3.6, 95% CI 1.2-5.9; and rate of change in WRS PE -5.7, 95% CI -9.7 to -1.7). The minimum cochlear dose impacts hearing outcomes after SRS for VS and should be considered in radiosurgical treatment planning. In this cohort, the mean cochlear dose was only associated with hearing outcomes in the subgroup of patients with class B hearing at SRS.

Functional Outcomes and Self-Reported Quality of Life in Patients with Facial Nerve Impairment Following Vestibular Schwannoma Surgery.

The aim of this observational retrospective study was to report quality of life (QoL) in patients with postoperative facial nerve (FN) palsy after vestibular schwannoma (VS) surgery, investigating clinical factors related to functional outcomes. Forty-eight consecutive patients (M:F 25:23; median age: 52.5 years) with facial palsy following surgery for sporadic VS were considered retrospectively. FN palsy was graded by using the Sunnybrook facial grading system (SBFGS), while postoperative QoL and subjective functional aspects were assessed by using the Penn Acoustic Neuroma Quality of Life (PANQOL) Scale, the Synkinesis Assessment Questionnaire, and questions on eating and drinking. A significant correlation emerged between all Sunnybrook scores and median PANQOL domain regarding facial function. Increasing overall SBFGS scores were associated with reduced risk of slow chewing on the affected side (p = 0.004), lack of masticatory strength (p = 0.025), masticatory fatigue (p < 0.001), accumulation of food in the oral vestibule (p < 0.001), difficulty in drinking from a glass (p = 0.019), and fluid spillage while drinking (p = 0.016). This study suggests that the clinical evaluation of patients with FN palsy after VS surgery should be integrated with patient reports about functional outcomes and perceived QoL to help clinicians guide rehabilitation choices.

Stereotactic Radiosurgery in Primary Treatment of Sporadic Small to Medium (

To analyze the current literature regarding use of SRS as primary treatment of VS to further evaluate efficacy and treatment-related neurologic deficits. Online databases were queried to identify relevant publications from January 2001-December 2020. Full text, English articles for sporadic VS treated primarily with radiosurgery and documented hearing preservation data were reviewed. Papers that had a minimum follow-up period of less than 36 months, did not utilize radiosurgery for primary treatment, or included patients with Neurofibromatosis II were excluded. A total of 33 studies involving 4286 patients with an average follow-up of 62.5 months were included in the final analysis. All 33 studies included eligible hearing data; overall preservation of serviceable hearing was found to be 58.27%. 27 studies with 3822 eligible patients were analyzed for tumor control rates; overall, tumor control was reported in 92.98% of cases. 27 studies were analyzed for post-treatment facial nerve dysfunction which was reported in 1.53% of cases. SRS is a safe and effective primary treatment modality for sporadic vestibular schwannoma as evidenced by the present analysis. Radiosurgery is effective with regard to tumor control and hearing preservation while offering a low rate of post-treatment facial nerve dysfunction.

Scoring System Assessing Risks of Growth in Sporadic Vestibular Schwannoma.

Surveillance studies offer sparse knowledge of predictors of future growth in sporadic vestibular schwannomas (VS).Our aim was identification of these risk factors. We propose a scoring system to estimate the risk of growth in sporadic vestibular schwannoma. This retrospective study is based on the demographic and radiological data of 615 adult patients under the surveillance for single VS in our center. Univariate analysis, multivariate regression, and Kaplan-Meier analysis were used when appropriate. The regression coefficient-based "VS score" was calculated based on Cox proportional-hazards regression. During surveillance, 285 tumors (46%) remained stable, 314 tumors (51%) grew, and 16 tumors (3%) shrank. The significant risks factors for future growth identified both in univariate and multivariate analyses were younger age at onset, cystic morphology, larger tumor volume, and cisternal location (as per Hannover grade). The proportion of growing tumors was 40%, 75%, and 96% among the homogeneous VS, primary cystic, and VS transformed to cystic, respectively. Moreover, tumor growth during the 1st year was significant predictor of continuous growth. Our "VS score" includes variables such as age, sex, morphology, and Hannover grade. The score extends between -3 and 6 points. Kaplan-Meier, confusion matrix, and receiver operating characteristic analysis proved high accuracy of our scoring model. Our retrospective study revealed that younger age, cystic morphology, cisternal extent, larger volume, and growth during 1st year were strong predictors of future growth. Moreover, we propose a scoring system that accurately estimates the risks of future tumor growth.

Long-Term Prospective Quality-of-Life Outcomes in 445 Patients with Sporadic Vestibular Schwannoma.

To evaluate the long-term changes in sporadic vestibular schwannoma (VS) disease-specific quality-of-life (QOL) outcomes. Prospective longitudinal study using the Penn Acoustic Neuroma Quality of Life (PANQOL) Scale. National survey. Patients with sporadic VS who completed a baseline survey before treatment and at least one follow-up survey recruited through the authors' center and through the Acoustic Neuroma Association. Observation, microsurgery, radiosurgery. Changes in PANQOL scores from baseline to most recent survey. Among 445 eligible patients the mean duration of follow-up was 4.4 (SD, 2.3) years, including 122, 218, and 105 in the observation, microsurgery, and radiosurgery groups, respectively. Patients managed with observation (p = 0.03) or microsurgery (p < 0.001) demonstrated improvement in anxiety scores. Changes in facial function scores differed significantly by management group (p = 0.01), with patients undergoing microsurgery demonstrating a mean decline of 10 points in facial function scores compared with mean declines of 3 for those managed with observation or radiosurgery. Hearing loss scores decreased similarly over time for all three groups (p = 0.3). There were minimal changes in total PANQOL scores over time across all management groups (p = 0.5). Long-term changes in total QOL among VS management groups are not significantly different. Microsurgery may continue to confer an advantage regarding improvement in anxiety postoperatively, but with a greater decline in facial function when compared to observation or radiosurgery. Long-term declines in hearing loss scores were not statistically significantly different among groups.

Rates and predictors of loss to follow-up for sporadic vestibular schwannomas undergoing imaging surveillance.

Imaging surveillance with serial MRI, or a "wait-and-scan" approach, is a management option for patients with small or medium-sized vestibular schwannomas (VSs). Prior publications have indicated no distinct quality of life advantage to upfront treatment compared with initial wait-and-scan management. However, imaging surveillance is dependent on patient adherence to follow-up. In this study, the authors aimed to identify rates and predictors of patient loss to follow-up during wait-and-scan management of sporadic VS. A single-center study was conducted including all patients from 2013 to 2018 who had undergone upfront imaging surveillance of sporadic VS. Patient data were retrospectively obtained from the electronic medical record. Outcomes of interest included loss to follow-up unrelated to death and inconsistent adherence to imaging surveillance recommendations. Logistic regression analyses were conducted to evaluate factors associated with loss to follow-up. Over a 6-year study period, 270 patients underwent initial imaging surveillance of a sporadic VS. The median tumor diameter was 8.6 mm (range 1-28.9 mm). At the time of censoring, 106 patients (39.3%) had received treatment, 157 (58.1%) had been advised to continue follow-up, and 7 (2.6%) had died of non-VS-related causes. In total, 73 patients (27.0%) were completely lost to follow-up prior to the first treatment or death. Additionally, 60 patients (22.2%) missed at least 1 MRI follow-up or imaging follow-up was delayed by more than 1 year. Multivariable logistic regression identified an out-of-state residence (OR 3.05, 95% CI 1.58-5.89, p = 0.0009) and a smaller tumor size (unit OR per 1-mm increase in size, OR 0.88, 95% CI 0.83-0.95, p = 0.0006) to be associated with loss to follow-up. Patients living ≥ 350 miles from the hospital or with tumors ≤ 3 mm at the time of initial clinic evaluation were most likely to be lost to follow-up. Only a smaller tumor size was associated with an increased risk of inconsistent imaging follow-up (unit OR per 1-mm increase in size, OR 0.92, 95% CI 0.87-0.98, p = 0.007). Patients undergoing imaging surveillance of VS are at risk for loss to follow-up and inconsistent imaging surveillance. Patients with smaller tumors or those living farther away from the treating institution are at highest risk for being lost to follow-up.

The Ratio of Baseline Ventricle Volume to Total Brain Volume Predicts Postoperative Ventriculo-Peritoneal Shunt Dependency after Sporadic Vestibular Schwannoma Surgery.

Background/Objectives: Obstructive hydrocephalus associated with vestibular schwannoma (VS) is the most common in giant VS. Despite tumor removal, some patients may require ongoing ventriculo-peritoneal (VP) surgery. This investigation explores the factors contributing to the requirement for VP surgery following VS surgery in instances of persistent hydrocephalus (HCP). Methods: Volumetric MRI analyses of pre- and postoperative tumor volumes, cerebellum, cerebrum, ventricle system, fourth ventricle, brainstem, and peritumoral edema were conducted using Brainlab Smartbrush and 3D Slicer. The total brain volume was defined as the sum of the cerebrum, cerebellum, and brainstem. ROC analyses were performed to identify the optimum cut-off values of the volumetric data. Results: Permanent cerebrospinal fluid (CSF) diversion after surgery was indicated in 12 patients (12/71; 16.9%). The ratio of baseline volume fraction of brain ventricles to total brain ventricle volume (VTB ratio) was found to predict postoperative VP shunt dependency. The AUC was 0.71 (95% CI: 0.51-0.91), and the optimum threshold value (</≥0.449) yielded a sensitivity and specificity of 67% and 81%, respectively. Multivariable logistic regression analyses of imaging data (pre- and postoperative VS volume, VTB ratio, and extent of resection (%) (EoR)) and patient-specific factors revealed that an increased VTB ratio (≥0.049, OR: 6.2, 95% CI: 1.0-38.0, p = 0.047) and an EoR < 96.4% (OR: 9.1, 95% CI: 1.2-69.3, p = 0.032) were independently associated with postoperative VP shunt dependency. Conclusions: Primary tumor removal remains the best treatment to reduce the risk of postoperative persistent hydrocephalus. However, patients with an increased preoperative VTB ratio are prone to needing postoperative VP shunt surgery and may benefit from perioperative EVD placement.

Vestibular schwannoma-related increased labyrinthine post-gadolinium 3D-FLAIR signal intensity and association with hearing impairment.

Vestibular schwannomas (VSs) are benign neurogenic tumors commonly associated with progressive unilateral hearing loss, tinnitus, and vestibular symptoms. Growing evidence links signal changes in the VS-adjacent labyrinth with sensorineural hearing loss. This study seeks to quantify the association of labyrinthine signal on post-gadolinium 3D-FLAIR imaging correlates with hearing loss and to evaluate potential longitudinal changes over time. Selected patients were identified from a prospectively maintained VS registry. Mean signal intensity ratios of the bilateral labyrinth and pons were measured on 3D-FLAIR post-gadolinium MRI. Correlations with paired audiometric data including pure tone average (PTA), word recognition score (WRS), and AAO-HNS hearing class within one year were evaluated. 125 studies obtained from 2015 to 2022 among 66 patients undergoing observational management for sporadic VS were analyzed. Increased signal intensity was noted of the VS-affected labyrinth/contralateral labyrinth (mean ratio 1.56, SD 0.58). Increased signal intensity was associated with increased PTA on both labyrinthine (correlation coefficient [CC] 0.20, p=0.03) and pontine comparisons (CC 0.24, p=0.006), and with decreased WRS on pontine comparisons (CC -0.18, p=0.04). Increased signal intensity was significantly associated with non-serviceable AAO-HNS C/D hearing when intensities were compared to the pons (p=0.01) but not the contralateral labyrinth (p=0.1). Among 44 patients with available follow-up, no statistically significant associations were identified between audiometric data and signal changes over the same interval. Increased 3D-FLAIR post-gadolinium labyrinthine signal is associated with sensorineural hearing loss; however, its relationship with hearing trajectory remains unclear. Overall findings suggest that while post-gadolinium 3D-FLAIR techniques are sensitive to inner ear involvement associated with VS, the driving mechanism and their temporal relationships with labyrinthine signal intensity and hearing impairment remain unknown. AAO-HNS =American Academy of Otolaryngology -Head and Neck Surgery. BLB =blood-labyrinth barrier. CPA = cerebellopontine angle. IAC = internal auditory canal. PTA = pure tone average; SIR = signal intensity ratio. VS = vestibular schwannoma. WRS = word recognition score.

Dynamics of tumor evolution after Gammaknife radiosurgery for sporadic vestibular schwannoma: defining volumetric patterns characterizing individual trajectory.

Definition of tumor control and treatment failure after Gammaknife radiosurgery (GKRS) for vestibular schwannoma (VS) is still debated. The lack of knowledge on the dynamics of tumor evolution can lead to misinterpretation and subsequent inappropriate second treatment. The aim of this study was to evaluate the post-GKRS dynamics of evolution of tumor volume, and characterize volumetric patterns. We included patients with sporadic VS treated by GKRS with an MRI follow-up of minimum 3 years. A clustering in 2 steps was performed: definition of the patterns of evolution based on a subset of patients with the most comprehensive follow-up, then assignment of the remaining patients on a best fit basis. The minimum length of follow-up was assessed by measuring the consistency of the clusters over time (Adjusted Rand Index and Normalized Mutual Information). An analysis of the discriminant variables was finally performed. 1,607 patients were included (median follow-up: 67 months). Five patterns were defined with one pattern gathering almost all cases of treatment failure. The clustering at 5 years afforded the highest consistency with long-term follow-up. Discriminant variables for clusters were: sex, initial symptoms, delay of diagnosis, Koos grading, fundus invasion, and number of isocenters. The definition of these robust distinct patterns is likely to help tremendously the physicians to distinguish tumor control from potential failure. We advocate for no retreatment decision before 5 years post-GKRS. Further investigations are required to decide if the dynamics of evolution can be predicted at GKRS on an individual basis.

The Impact of Tumor Elongation on Facial Nerve Outcome after Surgery for Koos Grade 3 and 4 Vestibular Schwannomas in the Semi-Sitting Position via the Retrosigmoid Approach.

Background: Facial nerve paralysis is a severe dysfunction after vestibular schwannoma (VS) surgery. Methods: This monocentric study analyzed 61 patients who underwent sporadic VS surgery in a standardized manner. The primary endpoint was the facial nerve outcome (FNO) at 3 months after VS surgery. FNO was dichotomized into "good" (House-Brackmann (HB) score ≤ 2) and "poor" (HB > 2). Results: Poor FNO was observed in 11 patients (18.0%) at 3 months after VS surgery. Radiomic tumor shape features were analyzed, and the AUC of elongation in the prediction of a poor HB at 3 months was 0.70 (95% CI: 0.56-0.85, p = 0.03) and the optimum threshold value (≤/>0.35) yielded a sensitivity and specificity of 64.0% and 75.4%, respectively. Multivariable logistic regression analyses considering the extent of resection (</≥93.4%), preoperative tumor volume (</≥2.6 cm3), age (</≥55), sex (female/male), and elongation (≤/>0.35) revealed that more elongated VSs (≤0.35; OR: 5.8; 95%CI: 1.2-28.2; p = 0.03) and those with an increased EoR (≥93.4%; OR: 6.5; 95%CI: 1.0-42.5; p = 0.05) are independently associated with poorer FNO at 3 months after surgery. Conclusions: Highly elongated VS shape seems to be a risk factor for worsened facial nerve outcome at 3 months after surgery for Koos grade 3 and 4 tumors.

Correlation of Immunomodulatory Cytokines with Tumor Volume and Cerebrospinal Fluid in Vestibular Schwannoma Patients.

Sporadic vestibular schwannomas (VSs) often exhibit slow or negligible growth. Nevertheless, some VSs increase significantly in volume within a few months or grow continuously. Recent evidence indicates a role of inflammation in promoting VS growth. Therefore, our study aimed to identify cytokines, which are associated with larger VSs. The expression of different cytokines in VS tumor samples and VS primary cultures was investigated. Additionally, the concentration of cytokines in cell culture supernatants of VS primary cultures and cerebrospinal fluid (CSF) of VS patients and healthy controls were determined. Correlation analysis of cytokine levels with tumor volume, growth rate, Koos grade, age, and hearing was examined with Spearman's-rank test. The mRNA expression of CC-chemokine ligand (CCL) 18, growth differentiation factor (GDF) 15, and interferon regulatory factor 4 correlated positively with tumor volume. Moreover, the amount of GDF15 in the cell culture supernatant of primary cells correlated positively with tumor volume. The concentrations of the cytokines CCL2, CCL5, and CCL18 and transforming growth factor beta (TGFB) 1 in the CSF of the patients were significantly different from those in the CSF controls. Inhibition of immune cell infiltration could be a putative approach to prevent and control VS growth.

Long-Term Hearing Outcome For Vestibular Schwannomas After Microsurgery And Radiotherapy: A Systematic Review and Meta-Analysis.

Hearing loss is a common symptom associated with vestibular schwannoma (VS), either because of the tumor's effects on the cochlear nerve or due to active treatments such as surgery or stereotactic radiosurgery (SRS). Treatment decisions for VS are based on factors including tumor size, hearing status, patient symptoms, and institutional preference. The study aimed to investigate long-term auditory outcomes in VS patients undergoing active treatments with a hearing preservation intent. A systematic literature review was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, searching Scopus, Pubmed, and Web of Science databases from inception to January 2024. Studies meeting inclusion criteria, including a minimum 5-year follow-up and assessment of pre- and posttreatment hearing outcomes, were included. Pooled prevalence estimates for serviceable hearing after SRS and microsurgery were calculated using MetaXL software. Risk of bias assessment was performed with the Risk of Bias in Non-randomized Studies of Interventions tool. Nine studies met the inclusion criteria, with 356 patients included for analysis. The pooled prevalence of maintaining serviceable hearing after SRS at 10 years was 18.1% (95% confidence interval [CI]: 1.7%-43.3%), with wide prediction intervals indicating variability in outcomes. Microsurgery demonstrated a higher prevalence of maintaining long-term serviceable hearing, with a pooled estimate of 74.5% (95% CI: 63.5%-84.1%). This systematic review underscores the importance of long-term follow-up in evaluating auditory outcomes in VS treatment. Despite the biases inherent to pretreatment patients selection, hearing preservation microsurgery for sporadic VS removal demonstrated favorable and stable long-term serviceable hearing.

Losartan May Not Prevent Vestibular Schwannoma Growth or Related Hearing Loss During Observation.

To evaluate the impact of losartan on vestibular schwannoma (VS) growth and related hearing loss during observation. Retrospective cohort study. Tertiary referral center. Sporadic VS patients undergoing initial observation with at least two magnetic resonance imaging and audiologic examinations. Losartan. Endpoints included VS growth, quantitative audiologic changes, survival free of tumor growth, and survival free of nonserviceable hearing. Patient characteristics and endpoints were compared by losartan use. Seventy-nine patients were included, of which 33% were taking losartan. Tumor growth was observed in 50% of patients in the losartan group and 36% in the non-losartan group (p = 0.329). Survival analysis failed to show a significant difference in the hazard rate of VS growth between groups (hazard ratio, 1.38; 95% confidence interval, 0.70-2.70; p = 0.346). Throughout observation, mean decreases in normalized pure-tone average were 5.5 and 9.3 dB in the losartan and non-losartan groups, respectively (p = 0.908). Mean decreases in normalized word recognition score were 11.0 and 16.6% in the losartan and non-losartan groups, respectively (p = 0.757). Nonserviceable hearing developed in 19% of patients in the losartan group and 28% in the non-losartan group (p = 0.734). Survival analysis did not demonstrate a significant difference in the hazard rate of developing nonserviceable hearing between groups (hazard ratio, 1.71; 95% confidence interval, 0.56-5.21; p = 0.337). Losartan use may not reduce the risk of VS growth or hearing loss during observation. A randomized trial would be ideal to further identify the true effect on growth and hearing.

Vestibular Schwannomas: implementation of PANQOL and Mayo VSQOL Index scales in Ukraine and justification of treatment strategy with preservation of quality of life (problem analysis, own experience, discussion points)

Objective: To evaluate the treatment outcomes of sporadic vestibular schwannomas (VS) to substantiate the choice of optimal treatment strategy considering quality of life indicators using the Penn Acoustic Neuroma Quality of Life Scale (PANQOL) and the Mayo Clinic Vestibular Schwannoma Quality of Life Index (Mayo VSQOL Index) scales in Ukraine. Materials and Methods: 1100 patients with VS were treated at the Subtentorial Neurooncology Department from 2001 to October 2023 and 373 were primarily considered for dynamic observation (“wait-and-scan”) at the Department of Ear Microsurgery and Otoneurosurgery of the Kolomiichenko Otolaryngology Institute. Data on 359 patients (some of whom underwent surgery in the mentioned departments) who received radiotherapy (RT) from 2010 to 2023 in the Radioneurosurgery Department are also provided. The PANQOL and Mayo VSQOL Index scales were implemented to assess the quality of life of patients with VS. Scientific publications from the PubMed database related to the study of the quality of life in patients with VS were analyzed. Results: The analysis was based on patient data from the database for the period 2017 - October 2023, 391 patients with VS were treated, including 359 who underwent surgical treatment (schwannomas resection), 4 – tarsorrhaphy after VS resection, and 28 who received combined treatment (surgery + radiotherapy). A separate group consisted of 359 patients who underwent radiotherapy (RT). Of 177 patients who underwent radiosurgical treatment, 65 (36.7%) had Stage III VS according to the classification of W.T. Koos, and 112 (63.3%) had stage IV VS. Primarily diagnosed tumors were treated in 110 (62.1%) and 67(37.9%) patients with residual postoperative. Follow-up data were obtained for 159 patients. At the Kolomiichenko Otolaryngology Institute, 373 patients were under observation with the primary "wait-and-scan" strategy, of whom 110 (29.5%) had their VS removed due to symptom progression or progressive course, and 24 (6.4%) underwent RT. Of the 27 patients in the Subtentorial Neurooncology Department study group, 7 (25.9%) underwent surgery, and 5 (18.5%) underwent RT. Conclusions: Global practice in assisting patients with VS indicates a shift towards dynamic follow-up and an increase in the frequency of using radiation treatment methods. Over the past years, strategy have been proposed that do not recommend the "wait-and-scan" mode due to the risk of life-threatening complications associated with tumor growth. However, there is very little data on long-term outcomes (more than 5 years) and quality of life. VS primarily detected by magnetic resonance imaging in Ukraine significantly larger (Koos III-IV) than those reported in the literature. The implementation of specialized scales for assessing the quality of life of VS patients in Ukraine will help improve outcomes by applying personalized criteria for treatment routes, increasing the level of awareness and responsibility of both patients and doctors.

Hearing loss and its association with the proteome of perilymph, cerebrospinal fluid, and tumor tissue in patients with vestibular schwannoma

This study examined the association between hearing loss in sporadic vestibular schwannoma patients and the proteome of perilymph (PL), cerebrospinal fluid (CSF), and vestibular schwannoma. Intraoperative sampling of PL and of CSF, and biopsy of vestibular schwannoma tissue, was performed in 32, 32, and 20 patients with vestibular schwannoma, respectively. Perilymph and CSF in three patients with meningioma and normal hearing were also sampled. The proteomes were identified by liquid chromatography coupled to high-resolution tandem mass spectrometry. Preoperative hearing function of the patients was evaluated with pure tone audiometry, with mean values at frequencies of 500, 1000, 2000, and 4000 Hz (PTA4) in the tumor-affected ear used to delineate three hearing groups. Analysis of the PL samples revealed significant upregulation of complement factor H-related protein 2 (CFHR2) in patients with severe to profound hearing loss after false discovery rate correction. Pathway analysis of biofunctions revealed higher activation scores in the severe/profound hearing loss group of leukocyte migration, viral infection, and migration of cells in PL. Upregulation of CFHR2 and activation of these pathways indicate chronic inflammation in the cochlea of vestibular schwannoma patients with severe to profound hearing loss compared with patients with normal hearing or mild hearing loss.

Age, preoperative tumor volume and widening of the internal acoustic meatus are independent factors associated with poor preoperative hearing in vestibular schwannoma patients – results of a single-center retrospective analysis

Preoperative hearing function shows wide variations among patients diagnosed with vestibular schwannoma. Besides the preoperative tumor size there are other factors that influence the preoperative hearing function that are frequently discussed. A comprehensive analysis of a large cohort of vestibular schwannomas has the potential to describe new insights and influence the preoperative management. We analyzed clinical factors, imaging data and the expression of the proliferation marker MIB1 as potential influencing factors on the preoperative hearing function in a retrospective cohort of 523 primary sporadic vestibular schwannomas. The results of the preoperative audiometry were quantified using the Gardner-Robertson Score. Uni- and multivariate analyses were performed. Serviceable hearing (Gardner-Robertson class 1 or 2) was documented in 391 patients (74.8%). Factors associated with non-serviceable hearing (Gardner-Robertson class 3–5) were patients of older age (p < 0.0001), larger preoperative tumor volume (p = 0.0013) and widening of the internal acoustic meatus compared to the healthy side (p = 0.0353). Gender and differences in the expression of the proliferation marker MIB1 had no influence on preoperative hearing. In the multivariate nominal logistic regression older age (OR 27.60 (CI 9.17–87.18), p < 0.0001), larger preoperative tumor volume (OR 20.20 (CI 3.43–128.58), p = 0.0011) and widening of the internal acoustic canal (OR 7.86 (CI 1.77–35.46), p = 0.0079) remained independent factors associated with non-serviceable hearing. Widening of the internal acoustic canal is an independent factor for non-serviceable preoperative hearing in vestibular schwannoma patients together with older age and larger preoperative tumor volume.

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

To describe outcomes of patients with sporadic vestibular schwannoma (VS) who underwent repeat stereotactic radiosurgery (SRS) after primary SRS failure. Multi-institutional historical cohort study. Five tertiary care referral centers. Adults ≥18 years old with sporadic VS. Primary and repeat treatment with SRS. Microsurgery-free survival after repeat SRS. 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. Overall, repeat SRS for sporadic VS has comparable risk profile, but lower rates of tumor control, compared with primary SRS.

Vestibular Schwannoma Management: Patient Perspectives and Health Literacy

Objective: To assess medical decision-making, reasons for attrition, and health literacy among vestibular schwannoma (VS) patients last seen in clinic more than 2 years ago. Study Design: Survey. Setting: Tertiary skull base center. Patients: Adults with sporadic VS last evaluated more than 2 years ago. Methods: Survey including Brief Health Literacy Screen (BHLS), a validated 3-question measure of health literacy, and open-ended questions on care; retrospective chart review. Main Outcome Measures: BHLS score, patient reasons for attrition. Results: Of 1011 patients contacted, 205 (20.3%) patients responded and met all criteria for analysis. At initial evaluation, mean patient age was 50.6 ± 12.8 years, roughly half (51.4%) were female, and the majority (80.2%) identified as White. At the time of the survey, 48 (23%) continued to observe their tumor. The remaining 160 (76.9%) patients previously underwent treatment (surgery and/or radiation). Of those treated, 34 (21.3%) underwent intervention elsewhere. Symptoms since the last patient visit did not differ between observed and treated patients. About 94.7% of the cohort displayed high health literacy (BHLS &gt; 9), though rates of inadequate health literacy were higher among observed patients (12.5% versus 3.1% in those treated). The most common reasons for opting not to follow-up included counseling issues, personal decisions, and social/life stressors (including the COVID-19 pandemic). Conclusions: Despite being a highly health-literate cohort, patients cited multiple reasons for attrition. Regardless of whether they were observed or treated, patients described follow-up visits as burdensome and perceived them to be of minimal benefit.