BACKGROUND: The medical community has shown a growing interest in developing methods for measuring and comparing objective patient outcomes coupled with subjective patient assessments. Questionnaires enable healthcare professionals to obtain the patient\'s perspective about their experienced vestibular schwannomas (VS) symptoms quickly. To date, in Poland, a cross-cultural adapted version of a disease-specific questionnaire for the measurement of quality of life (QoL) in patients with VS has not been produced.
OBJECTIVE: This study aimed to adapt the questionnaire evaluating disease-specific QoL in patients with VS (Penn Acoustic Neuroma Quality-of-Life Scale; PANQOL) to Polish and evaluate its psychometric properties.
METHODS: One-hundred twenty-four patients aged between 24 and 85 years (mean (M) = 60.17 ±standard deviation (SD) = 13.27) diagnosed with VS and treated with Gamma Knife were included in the study. We used a questionnaire translated from English into Polish by a bilingual professional, verified through a back-translation. The final version consisted of 26 items. The internal consistency of the Polish version of the PANQOL scale domains was measured using the Cronbach\'s alpha (α). To verify the validity of PANQOL subscales, a correlation analysis was conducted between the domains of PANQOL and other questionnaires, including the Assessment of Quality of Life (AQoL-8D), the Glasgow Benefit Inventory (GBI), the 5 Well-Being Index (WHO-5), the Skarzynski Tinnitus Scale (STS) for the presence of dizziness, and the Gardner-Robertson classes.
RESULTS: The majority of PANQOL domains showed excellent or good internal consistency (for a PANQOL total of 0.934; for subscales in the range of 0.916-0.424). Our analysis showed strong correlations between the total PANQOL score and AQoL-8D utility score, as well as between the subscales. We observed weak to moderately significant relationships between GBI and PANQOL domains (r = 0.18-0.43), the WHO-5 (r = 0.18-0.56) and the STS scale (r = -0.40- -0.19).
CONCLUSIONS: The results demonstrated that the POL-PANQOL is a reliable and valid questionnaire for measuring QoL.

Vestibular schwannomas (VS) are benign intracranial tumors posing significant management challenges. This study aims to compare the outcomes of stereotactic radiosurgery (SRS) and watchful waiting (WW) in the management of newly diagnosed VS, integrating findings from both retrospective and the pioneering V-REX prospective trial. Adhering to PRISMA guidelines, a systematic review was conducted using MEDLINE, Embase, and Cochrane databases. Studies directly comparing SRS with WW for newly diagnosed VS were included. Primary outcomes focused on hearing preservation assessed through the AAO-HNS or Gardner-Robertson hearing classification scales and tumor progression, with secondary outcomes focusing on neurological symptoms, and the need for further treatment. Thirteen studies encompassing 1,635 patients (WW: 891; SRS: 744) were included.While no significant difference was found in serviceable hearing loss at last follow-up (RR = 1.51, [95%CI: 0.98, 2.32], p = 0.06), significant differences favoring WW were observed in pure tone audiometry (PTA) (MD = -13.51 [95%CI: -22.66, -4.37], p = 0.004) and word recognition score (WRS) (MD = 20.48 [95%CI: 9.72, 31.25], p = 0.0002). Analysis of tumor progression indicated no overall significant difference in risk between SRS and WW (RR = 0.40, [95%CI 0.07, 2.40], p = 0.32), but subgroup analysis suggested a lower risk with SRS in certain contexts. The need for further treatments favored SRS (RR = 0.24, [95%CI: 0.07, 0.74], p = 0.007). No significant differences were found in tinnitus and imbalance between the two groups. This comprehensive analysis suggests no marked difference in functional hearing preservation between SRS and WW in managing VS. However, untreated tumors commonly necessitate additional interventions. These findings highlight the need for individualized treatment decisions and underscore the importance of continued monitoring. The study advocates for further prospective trials to refine management strategies for VS.

OBJECTIVE: To examine patient characteristics that impact serial observation adherence among vestibular schwannoma (VS) patients.
METHODS: Retrospective chart review.
METHODS: Single tertiary care center.
METHODS: We selected for VS patients from 201 to 2020 who elected for serial observation as initial management. Patients under 18, with previous management, bilateral or intralabyrinthine VS, and neurofibromatosis type 2 were excluded. Demographics, tumor characteristics, and follow-up status were extracted. Single and multiple logistic regression was used to identify patient characteristics impacting follow-up.
RESULTS: We identified 507 VS patients who chose serial observation as initial management. Most were female (56.0%), white (73.0%), and married (72.8%). The mean age was 59.3 and most had private insurance (56.4%). Median Charlson Comorbidity Index was 2.00. Mean pure tone audiometry (PTA) average was 41.7 Hz. Average tumor size was 9.04 mm. Of 507 patients, 358 (70.6%) returned for at least one follow-up. On multiple logistic regression analysis, patients with private insurance (odds ratio [OR]: 0.39, confidence interval [CI]: 0.22-0.68; P = .001), racial minority background (OR: 0.54, CI: 0.35-0.83; P = .005), worse PTA averages (OR: 0.99, CI: 0.98-1.00; P = .044), and older age at diagnosis (OR: 0.97, CI: 0.95-1.00; P = .038) were less likely to follow-up.
CONCLUSIONS: Private health insurance, racial minority background, worse PTA average, and older age were associated with decreased follow-up among adult VS patients electing serial observation. Patients with these characteristics may require additional support to ensure serial observation adherence.

Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disease (frequency 1 in 25-90 000) characterized by the formation of tumors of the central nervous system due to a mutation in the NF2 gene on chromosome 22q12. Bilateral vestibular schwannomas are recognized as absolute diagnostic criteria of NF2 and occur in 95% of patients, are accompanied by hearing impairment, manifest at the age of 18-24 years. Skin manifestations can precede vestibular schwannomas for several years and predict the course of the disease: neurofibromas, cafe-au-lait macules, hypopigmented spots, recently described mesh capillary malformations. Despite the benign nature of schwannomas, they can lead to hearing loss, vestibular dysfunction, facial nerve paralysis, gait disorders, pain and convulsions, there is a risk of early death from compression of the brain stem. The probability of progressive hearing loss is partly determined by the type of mutation. We described a clinical case of NF2 in a 21-year-old patient with bilateral vestibular schwannomas without hearing loss, whose skin examination by ENT specialist revealed this disease. The importance of the presented observation is that the doctor should assume neurofibromatosis type 2 in a young patient with bilateral vestibular schwannomas. It is necessary to undertake a further examination of this patient, including: skin examination for the identification of characteristic neurofibromas and cafe-au-lait macules, consultation with an ophthalmologist, neurologist, MRI of the brain and spinal cord with contrast, genetic analysis - for timely initiation of therapy that prevents hearing loss and vestibular disorders.
Нейрофиброматоз 2-го типа (НФ2) — редкое аутосомно-доминантное заболевание (частота 1 случай на 25—90 000 населения), характеризующееся образованием опухолей центральной нервной системы вследствие мутации в гене NF2 на хромосоме 22q12. Двусторонние вестибулярные шванномы признаны абсолютным диагностическим критерием НФ2, они возникают у 95% больных НФ2, сопровождаются нарушением слуха, манифестируют в возрасте 18—24 лет. Кожные проявления могут предшествовать вестибулярным шванномам на несколько лет и прогнозировать течение заболевания: нейрофибромы, пятна цвета кофе с молоком, гипопигментированные пятна, описанные недавно сетчатые капиллярные мальформации. Несмотря на доброкачественный характер опухолей, они могут привести к потере слуха, вестибулярной дисфункции, параличу лицевого нерва, нарушению походки, болям и судорогам, существует риск ранней смерти от сдавления ствола головного мозга. Вероятность прогрессирования потери слуха частично определяется типом мутации. Описан клинический случай НФ2 у девушки 21 года с двусторонними вестибулярными шванномами без нарушений слуха, осмотр кожи которой оториноларингологом позволил выявить заболевание. Важность представленного наблюдения в том, что врач, встретившийся с наличием двусторонних неврином слуховых нервов у молодого пациента без нарушения слуха, должен предположить нейрофиброматоз 2-го типа и назначить дальнейшее тщательное обследование данного пациента, включающее: осмотр кожи на предмет выявления характерных нейрофибром и пятен, консультацию офтальмолога, невролога, магнитно-резонансную томографию головного и спинного мозга с контрастом, генетический анализ — для своевременного выбора и начала терапии, предотвращающей снижение слуха и вестибулярные нарушения.

BACKGROUND: Our goal was to develop a 3D tumor slice model, replicating the individual tumor microenvironment and for individual pharmaceutical testing in vestibular schwannomas with and without relation to NF2.
METHODS: Tissue samples from 16 VS patients (14 sporadic, 2 NF2-related) were prospectively analyzed. Slices of 350 µm thickness were cultured in vitro, and the 3D tumor slice model underwent thorough evaluation for culturing time, microenvironment characteristics, morphology, apoptosis, and proliferation rates. Common drugs - Lapatinib (10 µM), Nilotinib (20 µM), and Bevacizumab (10 µg/ml) - known for their responses in VS were used for treatment. Treatment responses were assessed using CC3 as an apoptosis marker and Ki67 as a proliferation marker. Standard 2D cell culture models of the same tumors served as controls.
RESULTS: The 3D tumor slice model accurately mimicked VS ex vivo, maintaining stability for three months. Cell count within the model was approximately tenfold higher than in standard cell culture, and the tumor microenvironment remained stable for 46 days. Pharmacological testing was feasible for up to three weeks, revealing interindividual differences in treatment response to Lapatinib and intraindividual variability in response to Lapatinib and Nilotinib. The observed effects were less pronounced in tumor slices than in standard cell culture, indicating the model\'s proximity to in vivo tumor biology and enhanced realism. Bevacizumab had limited impact in both models.
CONCLUSIONS: This study introduces a 3D tumor slice model for sporadic and NF2-related VS, demonstrating stability for up to 3 months, replication of the schwannoma microenvironment, and utility for individualized pharmacological testing.

The objective of this study is to preliminarily investigate the surgical safety, efficacy, techniques, and clinical value of fully neuroendoscopic surgery for the resection of cerebellopontine angle (CPA) tumors via a retrosigmoid approach. The clinical data of 47 cerebellopontine angle area (CPA) tumors that were treated by full neuroendoscopic surgery from June 2014 to June 2023 were retrospectively analyzed. The efficacy and advantages of the surgical techniques were evaluated based on indicators such as duration of the surgery, neuroendoscopic techniques, intraoperative integrity of nerves and blood vessels, extent of tumor resection, outcomes or postoperative symptoms, and incidence of complications. The 47 cases of cerebellopontine angle tumors include 34 cases of epidermoid cysts, 7 cases of vestibular schwannomas, and 6 cases of meningiomas. All patients underwent fully neuroendoscopic surgery. Twenty tumors were removed using the one-surgeon two-hands technique, and 27 tumors were removed using the two-surgeons four-hands technique. The anatomical integrity of the affected cranial nerves was preserved in all 47 cases. None of the patients suffered a postoperative hemorrhage, cerebrospinal fluid leak, and aseptic or septic meningitis, or died. The rate of total tumor resection was 72.3% (34/47), and the symptom improvement rate was 89.4% (42/47). All patients were followed up for 2 to 12 months, and none died nor showed any signs of tumor recurrence. By analyzing 47 fully neuroendoscopic resections of CPA tumors using the posterior sigmoid sinus approach in our center, we believe that such method allows complete, safe, and effective resection of CPA tumors and is thereby worthy of clinical promotion.

Vestibular schwannoma is the most common benign neoplasm of the cerebellopontine angle. Its first symptoms include hearing loss, tinnitus, and vestibular symptoms, followed by cerebellar and brainstem symptoms, along with palsy of the adjacent cranial nerves. However, the clinical picture has unpredictable dynamics and currently, there are no reliable predictors of tumor behavior. Hence, it is desirable to have a fast routine method for analysis of vestibular schwannoma tissues at the molecular level. The major objective of this study was to verify whether a technique using in-sample specific protein digestion with trypsin would have the potential to provide a proteomic characterization of these pathological tissues. The achieved results showed that the use of this approach with subsequent liquid chromatography-tandem mass spectrometry (LC-MS/MS) analysis of released peptides allowed a fast identification of a considerable number of proteins in two differential parts of vestibular schwannoma tissue as well as in tissues of control healthy samples. Furthermore, mathematical analysis of MS data was able to discriminate between pathological vestibular schwannoma tissues and healthy tissues. Thus, in-sample protein digestion combined with LC-MS/MS separation and identification of released specific peptides followed by mathematical analysis appears to have the potential for routine characterization of vestibular schwannomas at the molecular level. Data are available via ProteomeXchange with identifier PXD045261.

UNASSIGNED: One of the hallmarks of NF2-related Schwannomatosis (NF2-related SWN) is bilateral vestibular schwannomas (VS) that can cause progressive hearing impairment in patients. This systematic review was performed to investigate the efficacy and toxicity of tested targeted agents.
UNASSIGNED: The systematic search was conducted on PubMed and EMBASE Ovid databases from inception to October 2022, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The incidence of outcomes in studies involving bevacizumab and other targeted therapies was extracted. The bevacizumab results were pooled, and 95% confidence intervals (95% CI) were calculated.
UNASSIGNED: Sixteen studies (8 prospective and 8 retrospective) testing 6 drugs were selected out of 721 search results. There were 10 studies concerning bevacizumab, with a total of 200 patients. The pooled radiographic response rate (RR) was 38% (95% CI: 31 - 45%) and the pooled hearing response rate (HR) was 45% (95% CI: 36 - 54%). The most frequent bevacizumab-related toxicities were hypertension and menorrhagia. Of other targeted therapies showing activity, lapatinib had a RR of 6% and a HR of 31%. A VEGFR vaccine showed RR in 29% and HR in 40% of patients. Both agents had a manageable safety profile.
UNASSIGNED: Bevacizumab, in comparison to other targeted agents, showed the highest efficacy. Lower dosage of bevacizumab shows comparable efficacy and may reduce toxicity. Other targeted agents, administered alone or as combination therapy, have the potential to improve outcomes for VS in patients with NF2-related SWN, but future clinical studies are needed.

Schwannomas are benign tumors originating from the Schwann cells of cranial or spinal nerves. The most common cranial schwannomas originate from the eight cranial nervevestibular schwannomas (VS). VS account for 6-8% of all intracranial tumors, 25-33% of the tumors localized in the posterior cranial fossa, and 80-94% of the tumors in the cerebellopontine angle (CPA). Schwannomas of other cranial nerves/trigeminal, facial, and schwannomas of the lower cranial nerves/are much less frequent. According to the World Health Organization (WHO), intracranial and intraspinal schwannomas are classified as Grade I. Some VS are found incidentally, but most present with hearing loss (95%), tinnitus (63%), disequilibrium (61%), or headache (32%). The neurological symptoms of VSs are mainly due to compression on the surrounding structures, such as the cranial nerves and vessels, or the brainstem. The gold standard for the imaging diagnosis of VS is MRI scan. The optimal management of VSs remains controversial. There are three main management options-conservative treatment or \"watch-and-wait\" policy, surgical treatment, and radiotherapy in all its variations. Currently, surgery of VS is not merely a life-saving procedure. The functional outcome of surgery and the quality of life become issues of major importance. The most appropriate surgical approach for each patient should be considered according to some criteria including indications, risk-benefit ratio, and prognosis of each patient. The approaches to the CPA and VS removal are generally divided in posterior and lateral. The retrosigmoid suboccipital approach is a safe and simple approach, and it is favored for VS surgery in most neurosurgical centers. Radiosurgery is becoming more and more available nowadays and is established as one of the main treatment modalities in VS management. Radiosurgery (SRS) is performed with either Gamma knife, Cyber knife, or linear accelerator. Larger tumors are being increasingly frequently managed with combined surgery and radiosurgery. The main goal of VS management is preservation of neurological function - facial nerve function, hearing, etc. The reported recurrence rate after microsurgical tumor removal is 0.5-5%. Postoperative follow-up imaging is essential to diagnose any recurrence.

Objective: To investigate the technique and efficacy of fully endoscope resection of intralabyrinthine schwannomas (ILS) by transcanal transpromontorial endoscopic approach (TTEA). Study Design: Retrospective case review. Setting: Hospital. Patients: All patients who were affected by ILS, without extension to the internal auditory canal and underwent surgery with TTEA in our hospital in 2020. Intervention(s): Therapeutic. Main Outcome Measure(s): Recovery status, postoperative complications and remaining symptoms after surgery. Results: Three patients were included, all of which underwent gross total resections. The follow-up period was from 10 months to 2 years. No intraoperative and postoperative major complications were observed. There was no facial paralysis or cerebrospinal fluid leakage postoperatively. The hospitalization time of TTEA was 5 days. Three patients\' vertigo was relieved after 1 week without receiving vestibular therapy. Only 1 patient complained of transient episodes of vertigo when climbing or holding heavy objects. Conclusions: TTEA has the advantages of clear vision to identify the anatomical structure, enabling complete tumor resection, reduced operation time, and faster postoperative recovery.Level of Evidence: IV.