Search Results (20)

Background/Objectives: This study evaluates the diagnostic efficacy of the apparent diffusion coefficient (ADC) and T1-weighted contrast-enhanced (T1W + C) and T2-weighted (T2W) imaging modalities in differentiating vestibular schwannomas (VSs) and cerebellopontine angle meningiomas (CPAMs), aiming to optimize clinical imaging protocols for these tumors. Methods: A retrospective analysis was conducted on 97 surgically and pathologically confirmed cases (65 VS, 32 CPAM) from Nanjing Drum Tower Hospital. Imaging features from ADC, T1W + C, and T2W sequences were extracted using medical imaging software. A support vector machine (SVM) model was trained to classify tumors based on these features, focusing on first-, second-, and third-order radiomic characteristics. Results: The ADC images demonstrated the highest classification efficiency, particularly with third-order features (AUC = 0.9817). The T2W images achieved the best accuracy (87.63%) using second-order features. Multimodal analysis revealed that ADC alone outperformed combinations with T1W + C or T2W sequences, suggesting limited added value from multi-sequence integration. Conclusions: Diffusion-weighted imaging (DWI) sequences, particularly ADC maps, exhibit superior diagnostic utility compared to T1W + C and T2W sequences in distinguishing VS and CPAM. The findings advocate prioritizing DWI in clinical imaging workflows to enhance diagnostic accuracy and streamline protocols. Full article

Background: Vestibular symptoms can severely affect patients with vestibular schwannomas (VSs). Studies assessing vestibular symptoms beyond clinical routine assessment in patients with VS treated by stereotactic radiosurgery (SRS) are scarce. Therefore, we employed the standardized questionnaire Dizziness Handicap Inventory (DHI) to systematically evaluate vestibular symptoms prior to and after SRS. Methods: For this retrospective single center study, we included patients who received Cyberknife^® SRS for newly diagnosed unilateral VS between 2012 and 2022, and who had a minimum of two follow-up (FU) visits. Besides clinical assessment, the presence and severeness of vestibular symptoms before and after treatment was recorded by using the DHI. Overall DHI symptom scores (1–100) were classified into four grades (0 = “none”, 1 = “mild”, 2 = “moderate” and 3 = “severe”). The results were correlated with tumor-, patient-, and treatment-related characteristics. Results: We analyzed 128 patients with a median age of 60 years (range: 20–82) and a median FU of 36 months (range: 11–106 months). The median tumor volume was 0.99 cm³ (range: 0.04–7.1 cm³). A median marginal dose of 13 Gy (range: 12–14 Gy) was administered. The crude rate of local tumor control was 99.2%. The mean DHI total score at last follow-up (LFU, 25.5 ± 24.7; range 0–92) was significantly lower than before SRS (29.4 ± 25.3; range:0–92, p = 0.026), which was reflected in a higher proportion of patients with DHI grade “none” and a lower proportion of patients with DHI grade “severe” at LFU. Chi-square tests showed a significant correlation of the DHI grades (DHI 0–1 vs. DHI 2–3) with the absence or presence of vestibular symptoms both before SRS (p < 0.001, CI 95%) and at LFU (p = 0.038). Conclusions: The DHI is a feasible and valid instrument for measuring vestibular symptoms after SRS. In addition, the DHI enables the quantification of symptoms and can therefore serve as an important tool for outcome assessment after SRS of VS. In the present cohort, DHI scores improved significantly during FU. Full article

Background/Objectives: Vestibular schwannomas (VSs), also called acoustic neuromas, are benign tumors affecting the vestibulocochlear nerve, often leading to hearing loss and balance issues. This condition is particularly challenging in patients with neurofibromatosis type 2 (NF2), where VSs tend to develop bilaterally. Conventional treatments, such as surgery and radiotherapy, although effective, carry risks like hearing loss and nerve damage. Bevacizumab, a VEGF-targeting monoclonal antibody, has emerged as a less invasive treatment option, showing potential for tumor volume reduction and hearing preservation. This systematic review aims to assess the efficacy of bevacizumab in controlling tumor volume, preserving hearing, and identifying associated adverse events. Methods: A comprehensive systematic review was performed using PRISMA guidelines. PubMed and Cochrane Library databases were searched for studies evaluating the effects of bevacizumab on VS, focusing on key outcomes like tumor volume reduction, hearing preservation, and adverse events. Data extraction and quality assessment were independently conducted by two reviewers using the Newcastle-Ottawa Scale. Results: Nine studies involving 176 patients were included. Bevacizumab showed a partial tumor volume reduction (≥20%) in 40% of cases and disease stabilization in 50%, while 10% experienced tumor progression. Hearing outcomes revealed improvement in 36% of patients, stabilization in 46%, and deterioration in 18%. Severe adverse effects, including hypertension and thromboembolic events, occurred in 13% of patients, while 18% reported no side effects. Tumor regrowth was observed in some patients after treatment discontinuation, emphasizing the need for long-term monitoring. Conclusions: Bevacizumab demonstrates effectiveness in managing VS, particularly in NF2 patients, by reducing tumor size and preserving hearing in a substantial proportion of cases. However, the variability in patient response and the risk of adverse events underscore the need for individualized treatment approaches and further research, including randomized controlled trials, to optimize its clinical application. Full article

Background: Ruptured pseudoaneurysm of the distal anterior inferior cerebellar artery (AICA) in patients with a history of gamma knife surgery (GKS) for vestibular schwannoma (VS) is rare. Several previous reports have described treatment strategies for radiation-induced pseudoaneurysm in the AICA: either surgical trapping or endovascular parent artery occlusion of the AICA. Methods: We present the first case of endosaccular coil embolization for a ruptured pseudoaneurysm in a large-diameter AICA after GKS for VS, successfully preserving the parent AICA. Results: Major recanalization of the coiled pseudoaneurysm was observed on follow-up imaging 3 months after the initial endovascular treatment. The patient subsequently underwent additional endosaccular coil embolizations for regrowth of the treated pseudoaneurysm buried in the VS. Two years later, another major recanalization was detected, prompting further retreatment. Subsequently, the patient has remained in a stable condition for 4 years. Conclusions: We suggest that endosaccular coil embolization of the distal AICA aneurysm with parent artery preservation might be a safe and feasible treatment option for radiation-induced saccular pseudoaneurysm arising from a large parent artery. However, close and regular follow-up imaging and preparation for potential retreatment are necessary, as pseudoaneurysms coiled within VSs are prone to recanalization. Full article

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. Full article

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. Full article

Background: A vestibular schwannoma (VS) is a benign tumor, causing audiological and vestibular symptoms. This study aimed to propose a comprehensive audio-vestibular test battery protocol for diagnosis and follow-up monitoring in patients with unilateral VSs undergoing surgical removal. Methods: The detailed interpretation of audiological and vestibular findings was presented in two example cases. The surgery was performed through the middle cranial fossa (#1) and translabyrinthine approach (#2). The participants were evaluated with tonal, speech, and impedance audiometry, ABR, caloric test, vHIT, cVEMP, oVEMP, SOT, and DHI. Patient and tumor characteristics were retrieved from the patient’s history. Results: In the postoperative period, the reduction in gain of the lateral semicircular canal was observed in the vHITs of both patients. The DHI in case #1 increased after surgery, while it decreased in case #2. The improvement in postural performances compared to the preoperative SOT (CON 5, CON 6, composite score) and immediately after the procedure was observed. Conclusions: A specific diagnostic protocol is necessary to compare the results of different surgical techniques and approaches. Diagnostic tests performed before the surgery should be repeated within a specific time frame during postoperative follow-up to enable the comparison of results. The proposed protocol can help us better understand the processes ongoing during tumor growth and postoperative vestibular compensation. Full article

Background/Objectives: Surgical resection of medium to large vestibular schwannomas (VSs, Koos grade 3 and 4) is a widely used approach, although stereotactic radiosurgery (SRS) is increasingly proposed as initial treatment. The quality of life-centered approach is challenged in cases where tumor growth control cannot be achieved with SRS, thus necessitating salvage surgery. We present a series of eight consecutive patients who required surgery due to continued tumor growth after SRS. Methods: Of the 146 patients with VS grades 3 and 4 initially treated with SRS, only eight patients (mean age, 54 ± 7.2 years; range, 42–63 years) required subsequent surgery. Their mean tumor volume was 9.9 ± 3.2 cm³. The mean time from SRS to first tumor progression and planned subtotal resection was 23 ± 5.9 months and 45 ± 17.5 months, respectively. SRS was not performed after the surgery in favor of a “wait and rescan” approach. Tumor residue was monitored on follow-up magnetic resonance imaging. In all patients, tumor growth control after planned subtotal resection was maintained at 63 ± 19.8 months. Results: None of the 146 patients had serious complications after SRS. In the eight patients who required surgery, tumor growth between 22% and 212% (mean, 4 cm³) was reported within 26 to 84 months after SRS. Before salvage surgery, they scored 1 point on the House–Brackmann scale. Subtotal excision was performed, and VIIth nerve function was preserved in all patients. At 63 ± 19.8 months, 3 patients had a House–Brackmann score of 1, four patients had a score of 2, and one patient had a score of 3. Conclusions: Surgical excision of medium to large VS after SRS can be relatively safe, provided that a quality of life-centered approach of subtotal resection is used. Full article

NF2-related Schwannomatosis (NF2-SWN) is a disease that needs new solutions. The hallmark of NF2-SWN, a dominantly inherited neoplasia syndrome, is bilateral vestibular schwannomas (VSs), which progressively enlarge, leading to sensorineural hearing loss, tinnitus, facial weakness, and pain that translates to social impairment and clinical depression. Standard treatments for growing VSs include surgery and radiation therapy (RT); however, both carry the risk of further nerve damage that can result in deafness and facial palsy. The resultant suffering and debility, in combination with the paucity of therapeutic options, make the effective treatment of NF2-SWN a major unmet medical need. A better understanding of these mechanisms is essential to developing novel therapeutic targets to control tumor growth and improve patients’ quality of life. Previously, we developed the first orthotopic cerebellopontine angle mouse model of VSs, which faithfully mimics tumor-induced hearing loss. In this model, we observed that mice exhibit symptoms of ataxia and vestibular dysfunction. Therefore, we further developed a panel of five tests suitable for the mouse VS model and investigated how tumor growth and treatment affect gait, coordination, and motor function. Using this panel of ataxia tests, we demonstrated that both ataxia and motor function deteriorated concomitantly with tumor progression. We further demonstrated that (i) treatment with anti-VEGF resulted in tumor size reduction, mitigated ataxia, and improved rotarod performance; (ii) treatment with crizotinib stabilized tumor growth and led to improvements in both ataxia and rotarod performance; and (iii) treatment with losartan did not impact tumor growth nor ameliorate ataxia or motor function. Our studies demonstrated that these methods, paired with hearing tests, enable a comprehensive evaluation of tumor-induced neurological deficits and facilitate the assessment of the effectiveness of novel therapeutics to improve NF2 treatments. Full article

Background: Our primary objective was to monitor nonprogressive unilateral vestibular schwannomas (VSs) to assess the efficiency of rapid bedside examinations, such as the video head impulse test (vHIT) and skull vibration-induced nystagmus test (SVINT), in identifying vestibular damage. Methods: An observational study was conducted from March 2021 to March 2022 on all adult patients (>18 years old) with a confirmed nonprogressive VS (no active treatment). The SVINT (using a 100 Hz vibrator with two (SVINT2) or three (SVINT3) stimulation locations) and vHIT (for the six semicircular canals (SCCs)) were performed on all patients. The asymmetry of function between the vestibules was considered significant when the gain asymmetry was greater than 0.1. Rapid and repeatable assessment of VSs using two- and three-stimulation SVINT plus vHIT was performed to quantify intervestibular asymmetry. Results: SVINT3 and SVINT2 triggered VIN in 40% (24/60) and 65% (39/60) of patients, respectively. There was significant asymmetry in the vestibulo-ocular reflex (VOR), as shown by a VS-side gain < healthy-side gain in 58% (35/60) of the patients. Among the patients with significant gain asymmetry between the two vestibules according to the vHIT (VS-side gain < healthy-side gain), the proportion of patients expressing vestibular symptomatology was significantly greater than that of patients without any symptoms [67% (29/43) vs. 35% (6/17), respectively; p = 0.047]. Conclusions: The SVINT2 can be combined with the vHIT to form an interesting screening tool for revealing vestibular asymmetry. This work revealed the superiority of mastoid stimulation over vertex stimulation for SVINT in patients with unilateral vestibular loss. Full article

(1) Background: Incomplete excision of vestibular schwannomas (VSs) is sometimes preferable for facial nerve preservation. On the other hand, subtotal resection may be associated with higher tumor recurrence. We evaluated the correlation between intra-operative assessment of residual tumor and early and follow-up imaging. (2) Methods: The charts of all patients undergoing primary surgery for sporadic vestibular schwannoma during the study period were retrospectively reviewed. Data regarding surgeons’ assessments of the extent of resection, and the residual size of the tumor on post-operative day (POD) one and follow-up MRI were extracted. (3) Results: Of 109 vestibular schwannomas meeting inclusion criteria, gross-total resection (GTR) was achieved in eighty-four, near-total (NTR) and sub-total resection (STR) in twenty-two and three patients, respectively. On follow up imaging, volumetric analysis revealed that of twenty-two NTRs, eight were radiographic GTR and nine were radiographic STR (mean volume ratio 11.9%), while five remained NTR (mean volume ratio 1.8%). Of the three STRs, two were radiographic GTR while one remained STR. Therefore, of eighteen patients with available later follow up MRIs, radiographic classification of the degree of resection changed in six. (4) Conclusions: An early MRI (POD#1) establishes a baseline for the residual tumor that may be more accurate than the surgeon’s intraoperative assessment and may provide a beneficial point of comparison for long-term surveillance. Full article

Although diagnosis and treatment of vestibular schwannomas (VSs) improved in recent years, no factors have yet been identified as being capable of predicting tumor growth. Molecular rearrangements occur in neoplasms before any macroscopic morphological changes become visible, and the former are the underlying cause of disease behavior. Tumor microenvironment (TME) encompasses cellular and non-cellular elements interacting together, resulting in a complex and dynamic key of tumorigenesis, drug response, and treatment outcome. The aim of this systematic, narrative review was to assess the level of knowledge on TME implicated in the biology, behavior, and prognosis of sporadic VSs. A search (updated to November 2022) was run in Scopus, PubMed, and Web of Science electronic databases according to the PRISMA guidelines, retrieving 624 titles. After full-text evaluation and application of inclusion/exclusion criteria, 37 articles were included. VS microenvironment is determined by the interplay of a dynamic ecosystem of stromal and immune cells which produce and remodel extracellular matrix, vascular networks, and promote tumor growth. However, evidence is still conflicting. Further studies will enhance our understanding of VS biology by investigating TME-related biomarkers able to predict tumor growth and recognize immunological and molecular factors that could be potential therapeutic targets for medical treatment. Full article

The classical middle cranial fossa approach (MCFA) for vestibular schwannoma (VS) removal often requires a large incision and craniotomy, excessive temporal lobe manipulation, and a longer recovery. We describe a keyhole MCFA (KMCFA) with endoscopic assistance that allows for adequate access with minimal temporal lobe manipulation, resulting in a fast recovery and an invisible scar. Eight sides of four cadaveric heads were dissected through the endoscopic-assisted KMCFA to access the internal auditory canal (IAC). Furthermore, five patients with intracanalicular VS underwent tumor removal with the endoscopic-assisted KMCFA. During the endoscopic-assisted KMCFA with fine instruments, a 3-cm supra-auricular incision and a 2-cm diameter keyhole craniotomy achieved exposure of the entire length of the IAC in all cadaveric dissections without unintended violation of the cochlea, semicircular canal, and facial nerve. The gross tumor was totally removed in five patients with no major postoperative complications. The surgical time was reduced, the hearing outcomes were similar to those of the classical MCFA, and the scar was invisible 1 month after the surgery. The endoscopic-assisted KMCFA permits intracanalicular VS removal in a safe, efficient, and cosmetic way. For small intracanalicular VSs, this approach can replace the classical MCFA when indicated. Full article

In this study. we aimed to detect vestibular schwannomas (VSs) in individual magnetic resonance imaging (MRI) slices by using a 2D-CNN. A pretrained CNN (ResNet-34) was retrained and internally validated using contrast-enhanced T1-weighted (T1c) MRI slices from one institution. In a second step, the model was externally validated using T1c- and T1-weighted (T1) slices from a different institution. As a substitute, bisected slices were used with and without tumors originating from whole transversal slices that contained part of the unilateral VS. The model predictions were assessed based on the categorical accuracy and confusion matrices. A total of 539, 94, and 74 patients were included for training, internal validation, and external T1c validation, respectively. This resulted in an accuracy of 0.949 (95% CI 0.935–0.963) for the internal validation and 0.912 (95% CI 0.866–0.958) for the external T1c validation. We suggest that 2D-CNNs might be a promising alternative to 2.5-/3D-CNNs for certain tasks thanks to the decreased demand for computational power and the fact that there is no need for segmentations. However, further research is needed on the difference between 2D-CNNs and more complex architectures. Full article

Background: Vertigo and dizziness are common in community-dwelling people and can be treated in specialized multidisciplinary settings. To develop tailored interventions, however, we have to explore risk factors for favorable and unfavorable outcomes. Methods: We prospectively investigated patients with chronic vertigo and dizziness subjected to our 5-day multimodal and interdisciplinary day care treatment in the Center for Vertigo and Dizziness of Jena University Hospital, Germany. The Vertigo Severity Scale (VSS), the Body Sensations Questionnaire (BSQ), the Hospital Anxiety and Depression Scale (HADS), the Agoraphobic Cognitions Questionnaire (ACQ), the Mobility Inventory (MI), and the burden and intensity of dizziness (using a visual analogue scale) were assessed at baseline (n = 754) and after 6 months (n = 444). In addition, 14 Likert-scaled questions were used to quantify the change in personal attitude and behavior towards the complaints after 6 months. Results: Dizziness-related burden and intensity improved with a large effect size. The largest improvement was seen in the attitudes towards dizziness, the understanding of somatic causes, and the perceived ability to influence dizziness. However, the ability to work and to carry out professional activity was improved to a lesser extent. The overall improvement of dizziness was associated with the absence of a depressive mood, a short duration of vertigo, a lower VSS, a lower perceived intensity of vertigo, and distinct vertigo diagnoses, namely Meniere’s disease, vestibular migraine, vestibular neuritis, vestibular paroxysmia, and vestibular schwannoma. Worsening of dizziness/vertigo was associated with depressive symptoms, permanent vertigo, distinct vertigo diagnoses (central vertigo, multisensory deficit), and a higher perceived burden due to vertigo. Conclusion: The six-month outcome of patients with dizziness presented to a specialized outpatient clinic appears to be favorable. Nevertheless, people with the abovementioned risk factors at baseline have less benefit and probably need adapted and tailored vertigo interventions to improve long-term outcome. Full article