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Keywords = facial nerve schwannomas

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21 pages, 2885 KB  
Review
The Facial Nerve in Contemporary Surgery: Anatomical Variability, Pathology-Induced Distortion, and Functional Preservation
by Piotr Łabętowicz, Nina Szczerba, Łukasz Olewnik, Nazar Włodarczyk, Kuba Borowski and Ingrid C. Landfald
J. Clin. Med. 2026, 15(14), 5622; https://doi.org/10.3390/jcm15145622 - 17 Jul 2026
Viewed by 525
Abstract
Objectives: The facial nerve (FN) possesses one of the most intricate anatomical courses in the head and neck, traversing the brainstem, temporal bone, and parotid gland before terminating within the muscles of facial expression. Owing to its complex anatomy, marked anatomical variability, and [...] Read more.
Objectives: The facial nerve (FN) possesses one of the most intricate anatomical courses in the head and neck, traversing the brainstem, temporal bone, and parotid gland before terminating within the muscles of facial expression. Owing to its complex anatomy, marked anatomical variability, and frequent distortion by adjacent pathology, preservation of FN integrity remains a fundamental challenge in skull base, otologic, and head and neck surgery. This review aims to provide a comprehensive synthesis of the contemporary literature regarding the clinical anatomy of the FN and to examine how anatomical variation, pathology-induced distortion, surgical strategy, and emerging technologies influence nerve preservation and functional outcomes. Methods: A comprehensive narrative review of the literature was conducted using PubMed, Scopus, and Google Scholar. Publications from 1983 through 2026 were searched using combinations of keywords, including “facial nerve,” “facial nerve anatomy,” “anatomical variation,” “vestibular schwannoma,” “hemifacial spasm,” “parotid surgery,” “facial nerve injury,” “facial nerve reconstruction,” “facial reanimation,” “diffusion tensor imaging,” “intraoperative neurophysiological monitoring,” and “artificial intelligence.” Peer-reviewed anatomical, radiological, clinical, and review articles published in English were included, while conference abstracts and studies lacking direct anatomical or surgical relevance were excluded. Particular emphasis was placed on surgically relevant anatomical variations, pathology-related anatomical distortion, advanced imaging modalities, intraoperative neurophysiological monitoring, reconstructive techniques, and predictors of postoperative facial nerve function. Results: Facial nerve preservation was found to depend on the interplay between individual anatomical variability, disease-related anatomical distortion, and operative strategy. In vestibular schwannoma surgery, nerve displacement, capsular adhesion, and cystic tumor degeneration were consistently associated with increased surgical complexity and less favorable postoperative facial function. In hemifacial spasm, successful microvascular decompression relied on precise identification of neurovascular conflict at the root exit zone. Within the parotid gland, substantial variability in branching architecture and surgical landmarks contributed to an increased risk of iatrogenic injury. Advanced imaging techniques, particularly diffusion tensor imaging tractography, improved preoperative prediction of FN location, while intraoperative neurophysiological monitoring enabled real-time assessment of neural integrity and functional preservation. Emerging artificial intelligence-based predictive models demonstrated potential to enhance patient-specific surgical planning and prognostication. Conclusions: Contemporary facial nerve surgery has evolved toward an individualized, anatomy-driven, and function-preserving paradigm supported by advanced imaging, intraoperative monitoring, and reconstructive strategies. Detailed understanding of both normal FN anatomy and pathology-induced anatomical distortion remains essential for optimizing surgical decision-making, maximizing nerve preservation, and improving long-term functional outcomes. Future developments integrating multimodal imaging, predictive analytics, and artificial intelligence may further refine patient-specific management and enhance postoperative facial function. Full article
(This article belongs to the Section General Surgery)
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8 pages, 1566 KB  
Case Report
Structural Mimics of Recurrent Bell’s Palsy: A Case Report Highlighting Diagnostic Red Flags in Facial Nerve Schwannoma and Metastasis
by Sohyeon Kim, Hyesoo Kwon and Hung Youl Seok
Reports 2026, 9(2), 178; https://doi.org/10.3390/reports9020178 - 7 Jun 2026
Viewed by 455
Abstract
Background and Clinical Significance: Recurrent peripheral facial palsy is most often attributed to idiopathic Bell’s palsy (BP), yet a minority of patients harbor underlying structural lesions. Early recognition of such lesions is essential for timely and appropriate management. Case Presentation: We [...] Read more.
Background and Clinical Significance: Recurrent peripheral facial palsy is most often attributed to idiopathic Bell’s palsy (BP), yet a minority of patients harbor underlying structural lesions. Early recognition of such lesions is essential for timely and appropriate management. Case Presentation: We retrospectively reviewed recurrent unilateral facial palsy at a single tertiary center and selected two illustrative patients in whom serial clinical, electrodiagnostic, and magnetic resonance imaging (MRI) findings ultimately indicated structural pathology. The first patient experienced three strictly right-sided episodes over 3.5 years, with worsening House–Brackmann grades (II → III → V) and progressive axonal loss on serial facial nerve conduction studies, culminating in a mass-like enhancing lesion at the geniculate ganglion consistent with schwannoma. The second patient had two left-sided recurrences within seven months. MRI evolved from a subtle intrameatal nodular suspicion without enhancement to a clearly enhancing intrameatal nodule with dural thickening. Additional intracranial nodular enhancements were discovered later and the patient was diagnosed with leptomeningeal metastasis. Conclusions: These cases illustrate that strictly ipsilateral recurrences, progressive electrophysiologic deterioration, and atypical or evolving MRI enhancement patterns should prompt thorough re-evaluation for structural causes rather than repeated labeling as idiopathic BP. Full article
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22 pages, 338 KB  
Review
Multidisciplinary Management of Cerebellopontine Angle Tumors with Brainstem Involvement
by Concheri Stefano, Vito Pontillo, Alberto D’Amico, Stefano Di Girolamo, Francesco Signorelli, Elisabetta Zanoletti and Nicola Antonio Adolfo Quaranta
Audiol. Res. 2025, 15(6), 168; https://doi.org/10.3390/audiolres15060168 - 4 Dec 2025
Viewed by 5816
Abstract
Background/Objectives: Tumors of the cerebellopontine angle (CPA) encompass a limited range of histologies, predominantly vestibular schwannomas (VSs), meningiomas, and paragangliomas (PGLs). Their growth region threatens the cranial nerves (V–XII), brainstem, and cerebellum, possibly causing functional deficits. This review aims to synthesize clinical features [...] Read more.
Background/Objectives: Tumors of the cerebellopontine angle (CPA) encompass a limited range of histologies, predominantly vestibular schwannomas (VSs), meningiomas, and paragangliomas (PGLs). Their growth region threatens the cranial nerves (V–XII), brainstem, and cerebellum, possibly causing functional deficits. This review aims to synthesize clinical features and multidisciplinary treatment strategies for CPA tumors with brainstem involvement, emphasizing functional preservation alongside tumor control. Methods: A systematic PubMed search identified studies on VSs, CPA meningiomas, and intradural PGLs. Eligibility criteria included studies reporting tumor management and cranial nerve outcomes. Data extraction focused on tumor size, neurological presentation, surgical approach, adjunctive therapies, and postoperative cranial nerve function. Multidisciplinary involvement and rehabilitation strategies were noted. Results: Twenty studies (3311 patients) analyzed large VSs, showing facial nerve dysfunction in 8–53%, trigeminal neuropathy in 20–77%, and cerebellar signs in up to 79%. Microsurgery (MS) achieved variable gross total resection, while stereotactic radiosurgery (SRS) preserved facial nerve function but carried trigeminal and hydrocephalus risks. CPA meningiomas demonstrated cranial nerve displacement patterns critical for surgical planning, with transient deficits common and recovery linked to baseline function. In 388 intradural PGL cases, staged surgery combined with preoperative embolization was standard; functional preservation of lower cranial nerves was often limited. Across all histologies, multidisciplinary management and targeted rehabilitation were essential. Conclusions: Optimal CPA tumor management balances tumor control with functional preservation. VSs benefit from individualized MS or SRS based on size and mass effect. Meningioma surgery prioritizes cranial nerve preservation over radical resection. Intradural PGLs require staged vascular-conscious approaches. Multidisciplinary care and structured rehabilitation are pivotal to improving outcomes and quality of life. Full article
20 pages, 5431 KB  
Article
Predicting the Consistency of Vestibular Schwannoma and Its Implication in the Retrosigmoid Approach: A Single-Center Analysis
by Raffaele De Marco, Giovanni Morana, Silvia Sgambetterra, Federica Penner, Antonio Melcarne, Diego Garbossa, Michele Lanotte, Roberto Albera and Francesco Zenga
Curr. Oncol. 2025, 32(11), 647; https://doi.org/10.3390/curroncol32110647 - 19 Nov 2025
Cited by 1 | Viewed by 1066
Abstract
To explore the relationship between magnetic resonance imaging (MRI) parameters, including T2-weighted intensity and apparent diffusion coefficient (ADC), and intraoperative tumor characteristics, particularly consistency, in vestibular schwannomas (VSs). The association between tumor consistency, facial nerve (FN) function, and postoperative outcomes was analyzed. A [...] Read more.
To explore the relationship between magnetic resonance imaging (MRI) parameters, including T2-weighted intensity and apparent diffusion coefficient (ADC), and intraoperative tumor characteristics, particularly consistency, in vestibular schwannomas (VSs). The association between tumor consistency, facial nerve (FN) function, and postoperative outcomes was analyzed. A single-center retrospective analysis included newly diagnosed VS cases (2020–2023) with cisternal involvement (Samii T3a; volume ≥ 0.7 cm3). T2 and ADC maps from the perimetral region of interest were normalized, and tumors were categorized into 3 classes by combining qualitative consistency (soft, fibrous, or fibrous/hard), ultrasonic aspirator power, and adherence to neurovascular structures. FN function was assessed using the House–Brackmann scale at the immediate postoperative period and 12-month follow-up. MRIs of 33 VSs (18 solid and 15 cystic) were analyzed. Normalized values of both T2 (N-T2mean) and ADC (N-ADCmin) maps predicted the classical radiological differentiation. N-ADCmin may have some role in predicting consistency (value 1.361, p = 0.017, accuracy 0.48) and demonstrated a significant association (p = 0.04) with the FN outcome in the immediate postoperative period. An augmented consistency could impair FN function by increasing the intrameatal pressure related to greater transmission of shocks derived from the dissection maneuvers of the cisternal component of the tumor. The possibility of non-invasively exploring VS consistency with a parameter easily calculable on MRI might be beneficial in surgical planning, modifying the timing of the opening of the meatus with respect to what could be the surgical routine in some centers. Full article
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17 pages, 12912 KB  
Article
Optical Coherence Tomography Imaging and Angiography of Skull Base Tumors Presenting as a Middle Ear Mass in Clinic
by Dorothy W. Pan, Marcela A. Morán, Wihan Kim, Zihan Yang, Brian E. Applegate and John S. Oghalai
Diagnostics 2025, 15(6), 732; https://doi.org/10.3390/diagnostics15060732 - 14 Mar 2025
Cited by 4 | Viewed by 2395
Abstract
Background: Skull base tumors can extend into the temporal bone and occasionally even be visible through the tympanic membrane (TM) if they grow into the middle ear cavity. The differential diagnosis of a skull base mass is extensive and ranges from non-tumorous [...] Read more.
Background: Skull base tumors can extend into the temporal bone and occasionally even be visible through the tympanic membrane (TM) if they grow into the middle ear cavity. The differential diagnosis of a skull base mass is extensive and ranges from non-tumorous lesions like cholesteatoma to benign tumors like schwannoma and to malignant lesions like metastatic cancer. Optical coherence tomography (OCT) is a noninvasive imaging technique that can image tissue with high resolution in three dimensions, including through structures such as the TM and bone. OCT angiography is also able to assess tissue vascularity. We hypothesized that OCT could help shrink the differential diagnosis in clinic on the day of initial presentation. Specifically, we thought that OCT angiography could help distinguish between highly vascular skull base tumors such as glomus jugulare and other less vascular tumors and middle ear pathologies such as cholesteatoma and schwannoma. Objectives: We sought to determine whether OCT can image through the TM in clinic to distinguish a normal ear from an ear with a mass behind the tympanic membrane. Furthermore, we sought to assess whether OCT angiography can detect vascularity in these masses to help inform the diagnosis. Methods: We designed and built a custom handheld OCT system that can be used like an otoscope in clinic. It is based off a 200 kHz swept-source laser with a center wavelength of 1310 nm and a bandwidth of 39 nm. It provides a 33.4 μm axial and 38 μm lateral resolution. Cross-sectional images of the middle ear space, including OCT angiography, were captured in an academic neurotology clinic. Patients with normal ear exams, glomus tumors, cholesteatomas, and facial nerve schwannoma were imaged. Results: OCT images revealed key structures within the middle ear space, including the TM, ossicles (malleus and incudostapedial joint), chorda tympani, and cochlear promontory. OCT also identified middle ear pathology (using pixel intensity ratio in the middle ear normalized to the TM) when compared with patients with normal ear exams (mean 0.082, n = 6), in all patients with a glomus tumor (mean 0.620, n = 6, p < 0.001), cholesteatoma (mean 0.153, n = 4, p < 0.01), and facial nerve schwannoma (0.573, n = 1). OCT angiography revealed significant vascularity within glomus tumors (mean 1.881, n = 3), but minimal vascularity was found in normal ears (mean 0.615, n = 3, p < 0.05) and ears with cholesteatoma (mean 0.709, n = 3, p < 0.01), as expected. Conclusions: OCT is able to image through the TM and detect middle ear masses. OCT angiography correctly assesses the vascularity within these masses. Thus, OCT permits the clinician to have additional point-of-care data that can help make the correct diagnosis. Full article
(This article belongs to the Special Issue Diagnosis and Management in Otology and Neurotology)
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15 pages, 3417 KB  
Article
Clinical Presentation, Treatment Outcomes, and Demographic Trends in Vestibular Schwannomas: A 135-Case Retrospective Study
by Corneliu Toader, Petrinel Mugurel Rădoi, Milena-Monica Ilie, Razvan-Adrian Covache-Busuioc, Vlad Buica, Luca-Andrei Glavan, Matei Serban, Antonio Daniel Corlatescu, Carla Crivoi and Radu Mircea Gorgan
J. Clin. Med. 2025, 14(2), 482; https://doi.org/10.3390/jcm14020482 - 14 Jan 2025
Cited by 3 | Viewed by 3208
Abstract
Background: This study presents a comprehensive analysis of 135 cases of vestibular schwannoma (VS) treated between 2006 and 2022 at the National Institute of Neurology and Neurovascular Diseases in Bucharest, Romania. The investigation focuses on the clinical presentation, treatment outcomes, and demographic [...] Read more.
Background: This study presents a comprehensive analysis of 135 cases of vestibular schwannoma (VS) treated between 2006 and 2022 at the National Institute of Neurology and Neurovascular Diseases in Bucharest, Romania. The investigation focuses on the clinical presentation, treatment outcomes, and demographic trends of VS patients, highlighting region-specific insights that fill critical gaps in Eastern European data. Methods: Patients were treated with either open surgery (93.3%) or gamma knife radiosurgery (6.6%). The study identifies predominant symptoms, including hearing impairment, facial palsy, and balance disorders, with variations observed across age and gender subgroups. Comorbidities such as hypertension and obesity were prevalent, and they influenced perioperative risks. Results: Post-treatment outcomes showed a significant correlation between clinical symptoms and treatment modalities, with a majority achieving favorable results. The findings emphasize the need for tailored approaches in VS management and underscore the importance of region-specific factors in influencing clinical outcomes. Conclusions: This study contributes to refining treatment strategies and improving healthcare delivery for VS patients in Romania and beyond. Full article
(This article belongs to the Special Issue Current Trends in the Management of Vestibular Schwannoma)
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13 pages, 1341 KB  
Article
Functional Outcomes and Self-Reported Quality of Life in Patients with Facial Nerve Impairment Following Vestibular Schwannoma Surgery
by Leonardo Franz, Silvia Montino, Anna Agostinelli, Giulia Tealdo, Diego Cazzador, Elisabetta Zanoletti and Gino Marioni
Diagnostics 2024, 14(21), 2387; https://doi.org/10.3390/diagnostics14212387 - 26 Oct 2024
Cited by 9 | Viewed by 2461
Abstract
Objective: 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. Methods: Forty-eight consecutive patients (M:F 25:23; median age: [...] Read more.
Objective: 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. Methods: 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. Results: 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). Conclusions: 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. Full article
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12 pages, 1723 KB  
Article
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
by Franziska Glieme, Lisa Haddad, Felix Arlt, Martin Vychopen, Clemens Seidel, Alonso Barrantes-Freer, Erdem Güresir and Johannes Wach
J. Clin. Med. 2024, 13(17), 5319; https://doi.org/10.3390/jcm13175319 - 8 Sep 2024
Cited by 1 | Viewed by 2058
Abstract
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 [...] Read more.
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
(This article belongs to the Special Issue Current Trends in the Management of Vestibular Schwannoma)
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10 pages, 1333 KB  
Article
Planned Subtotal Resection following Stereotactic Radiosurgery of Koos 3 and 4 Vestibular Schwannomas
by Grzegorz Turek, Sebastian Dzierzęcki, Paweł Obierzyński, Adrian Drożdż, Zenon Mariak, Justyna Zielińska-Turek, Wojciech Czyżewski, Karolina Dżaman and Mirosław Ząbek
J. Clin. Med. 2024, 13(14), 4107; https://doi.org/10.3390/jcm13144107 - 14 Jul 2024
Cited by 3 | Viewed by 2980
Abstract
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 [...] Read more.
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
(This article belongs to the Section Clinical Neurology)
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11 pages, 3775 KB  
Article
Comparative Analysis on Vestibular Schwannoma Surgery with and without Intraoperative Fluorescein Sodium Enhancement
by Amer A. Alomari, Sadeen Sameer Eid, Flavia Fraschetti, Silvia Michelini and Luciano Mastronardi
Brain Sci. 2024, 14(6), 571; https://doi.org/10.3390/brainsci14060571 - 3 Jun 2024
Cited by 3 | Viewed by 2400
Abstract
Background: Vestibular schwannoma (VS), also known as acoustic neuroma, is a benign, well-encapsulated, and slow-growing tumor that originates from Schwann cells, which form the myelin sheath around the vestibulocochlear nerve (VIII cranial nerve). The surgical treatment of this condition presents a challenging task [...] Read more.
Background: Vestibular schwannoma (VS), also known as acoustic neuroma, is a benign, well-encapsulated, and slow-growing tumor that originates from Schwann cells, which form the myelin sheath around the vestibulocochlear nerve (VIII cranial nerve). The surgical treatment of this condition presents a challenging task for surgeons, as the tumor’s location and size make it difficult to remove without causing damage to the surrounding structures. In recent years, fluorescein sodium (FS) has been proposed as a tool to enhance surgical outcomes in VS surgery. This essay will provide an analytical comparison of the use of FS in VS surgery, evaluating its benefits and limitations and comparing surgical outcomes with and without FS-assisted surgery. Methods: In a retrospective study conducted at San Filippo Neri Hospital, we examined VS cases that were operated on between January 2017 and December 2023. The patients were divided into two groups: group A, which consisted of patients who underwent surgery without the use of FS until January 2020 (102 cases), and group B, which included patients who underwent surgery with FS after January 2020 (55 cases). All operations were performed using the retrosigmoid approach, and tumor size was classified according to the Koos, et al. classification system. The extent of surgical removal was evaluated using both the intraoperative surgeon’s opinion and postoperative MRI imaging. Preoperatively and postoperatively, facial nerve function and hearing were assessed. In group B, FS was used to assist the surgical procedures, which were performed using a surgical microscope equipped with an integrated fluorescein filter. Postoperative clinical and MRI controls were performed at six months and annually, with no patients lost to follow-up. Results: This study investigated the impact of intraoperative fluorescein exposure on tumor resection and clinical outcomes in patients with VS. The study found a statistically significant difference in the tumor resection rates between patients who received fluorescein intraoperatively (p = 0.037). Further analyses using the Koos classification system revealed a significant effect of fluorescein exposure, particularly in the Koos 3 subgroup (p = 0.001). Notably, no significant differences were observed in hearing loss or facial nerve function between the two groups. A Spearman correlation analysis revealed a positive correlation between tumor size and Koos, age, and size, but no significant correlation was found between facial nerve function tests. Conclusions: FS-assisted surgery for VS may potentially enhance tumor resection, allowing for more comprehensive tumor removal. Full article
(This article belongs to the Special Issue Advanced Clinical Technologies in Treating Neurosurgical Diseases)
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11 pages, 873 KB  
Article
Integrating Ataxia Evaluation into Tumor-Induced Hearing Loss Model to Comprehensively Study NF2-Related Schwannomatosis
by Simeng Lu, Zhenzhen Yin, Jie Chen, Limeng Wu, Yao Sun, Xing Gao, Peigen Huang, Justin T. Jordan, Scott R. Plotkin and Lei Xu
Cancers 2024, 16(11), 1961; https://doi.org/10.3390/cancers16111961 - 22 May 2024
Cited by 5 | Viewed by 2312
Abstract
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 [...] Read more.
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
(This article belongs to the Special Issue Hot Topics in Neuro-Oncology)
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9 pages, 710 KB  
Article
Hearing Outcomes from Gamma Knife Treatment for Intracanalicular Vestibular Schwannomas with Good Initial Hearing
by Philippine Toulemonde, Nicolas Reyns, Michael Risoud, Pierre-Emmanuel Lemesre, Frédéric Gabanou, Marc Baroncini, Jean-Paul Lejeune, Rabih Aboukais and Christophe Vincent
J. Clin. Med. 2024, 13(6), 1685; https://doi.org/10.3390/jcm13061685 - 14 Mar 2024
Viewed by 4759
Abstract
Background: The objective of this study was to describe the long-term hearing outcomes of gamma knife treatment for unilateral progressing vestibular schwannomas (VS) presenting with good initial hearing using audiologic data. Methods: A retrospective review was performed between 2010 and 2020 to select [...] Read more.
Background: The objective of this study was to describe the long-term hearing outcomes of gamma knife treatment for unilateral progressing vestibular schwannomas (VS) presenting with good initial hearing using audiologic data. Methods: A retrospective review was performed between 2010 and 2020 to select patients with progressing unilateral VS and good hearing (AAO-HNS class A) treated with stereotactic gamma knife surgery (GKS). Their audiograms were analyzed along with treatment metrics and patient data. Results: Hearing outcomes with a median follow-up of 5 years post-treatment showed statistically significant loss of serviceable hearing: 34.1% of patients maintained good hearing (AAO-HNS class A), and 56.1% maintained serviceable hearing (AAO-HNS class A and B). Non-hearing outcomes are favorable with excellent tumor control and low facial nerve morbidity. Conclusions: Hearing declines over time in intracanalicular VS treated with GKS, with a significant loss of serviceable hearing after 5 years. The mean cochlear dose and the presence of cochlear aperture obliteration by the tumor are the main statistically significant factors involved in the hearing outcomes. Full article
(This article belongs to the Special Issue Current Trends in the Management of Vestibular Schwannoma)
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13 pages, 2938 KB  
Article
Facial Surface Electromyography: A Novel Approach to Facial Nerve Functional Evaluation after Vestibular Schwannoma Surgery
by Leonardo Franz, Gino Marioni, Antonio Daloiso, Elia Biancoli, Giulia Tealdo, Diego Cazzador, Piero Nicolai, Cosimo de Filippis and Elisabetta Zanoletti
J. Clin. Med. 2024, 13(2), 590; https://doi.org/10.3390/jcm13020590 - 19 Jan 2024
Cited by 5 | Viewed by 3794
Abstract
Background: Vestibular schwannoma (VS) surgery may cause facial nerve damage. However, a comprehensive evaluation of post-operative facial outcomes may be difficult to achieve. Surface electromyography (sEMG) is a promising non-invasive evaluation tool. However, its use in the follow-up after VS surgery has not [...] Read more.
Background: Vestibular schwannoma (VS) surgery may cause facial nerve damage. However, a comprehensive evaluation of post-operative facial outcomes may be difficult to achieve. Surface electromyography (sEMG) is a promising non-invasive evaluation tool. However, its use in the follow-up after VS surgery has not been reported yet. The main objective was to develop and validate a new sEMG application specifically for the post-VS surgery setting. Secondary goals were to provide a systematic description of facial muscle activity after VS surgery and assess the association between sEMG parameters and Sunnybrook scale scores. Methods: Thirty-three patients with facial palsy following VS surgery were included. The clinical outcomes (Sunnybrook symmetry, movement, and synkinesis scores) and sEMG parameters (signal amplitude normalized by the maximal voluntary contraction (NEMG) and sEMG synkinesis score (ESS, number of synkinesis per movement sequence)) were evaluated at the end of the follow-up. Results: In all tested muscles, NEMG variance was significantly higher on the affected side than the contralateral (variance ratio test, p < 0.00001 for each muscle). In total, 30 out of 33 patients (90.9%) showed an ESS ≥ 1 (median: 2.5, IQR: 1.5–3.0). On the affected side, NEMG values positively correlated with both dynamic and overall Sunnybrook scores (Spearman’s model, p < 0.05 for each muscle, except orbicularis oculi). ESS significantly correlated with the Sunnybrook synkinesis score (Spearman’s rho: 0.8268, p < 0.0001). Conclusions: We described and preliminarily validated a novel multiparametric sEMG approach based on both signal amplitude and synkinesis evaluation specifically for oto-neurosurgery. Large-scale studies are mandatory to further characterize the semiological and prognostic value of facial sEMG. Full article
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10 pages, 2237 KB  
Case Report
Two-Stage Surgical Management for Acutely Presented Large Vestibular Schwannomas: Report of Two Cases
by Abdullah Keles, Burak Ozaydin, Ufuk Erginoglu and Mustafa K. Baskaya
Brain Sci. 2023, 13(11), 1548; https://doi.org/10.3390/brainsci13111548 - 4 Nov 2023
Cited by 1 | Viewed by 3608
Abstract
The surgical management of vestibular schwannomas should be based on their presentation, neuro-imaging findings, surgeons’ expertise, and logistics. Multi-stage surgery can be beneficial for large-sized lesions with acute presentations. Herein, we highlighted the indications for two cases managed initially through the retrosigmoid and, [...] Read more.
The surgical management of vestibular schwannomas should be based on their presentation, neuro-imaging findings, surgeons’ expertise, and logistics. Multi-stage surgery can be beneficial for large-sized lesions with acute presentations. Herein, we highlighted the indications for two cases managed initially through the retrosigmoid and, subsequently, translabyrinthine approaches. The first case presented with acute balance and gait issues and a long history of hearing loss and blurred vision. Neuroimaging findings revealed a cerebellopontine angle lesion, resembling a vestibular schwannoma, with significant brainstem compression and hydrocephalus. Due to the rapidly deteriorating clinical status and large-sized tumor, we first proceeded with urgent decompression via a retrosigmoid approach, followed by gross total resection via a translabyrinthine approach two weeks later. The second case presented with gradually worsening dizziness and hemifacial numbness accompanied by acute onset severe headaches and hearing loss. Neuroimaging findings showed a large cerebellopontine angle lesion suggestive of a vestibular schwannoma with acute intratumoral hemorrhage. Given the acute clinical deterioration and large size of the tumor, we performed urgent decompression with a retrosigmoid approach followed by gross total resection through a translabyrinthine approach a week later. Post-surgery, both patients showed excellent recovery. When managing acutely presented large-sized vestibular schwannomas, immediate surgical decompression is vital to avoid permanent neurological deficits. Full article
(This article belongs to the Special Issue Advances in Skull Base Tumor Surgery: The Practical Pearls)
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12 pages, 2636 KB  
Article
Monitoring Cochlear Nerve Action Potential for Hearing Preservation in Medium/Large Vestibular Schwannoma Surgery: Tips and Pitfalls
by Baptiste Hochet, Hannah Daoudi, Etienne Lefevre, Yann Nguyen, Isabelle Bernat, Olivier Sterkers, Ghizlene Lahlou and Michel Kalamarides
J. Clin. Med. 2023, 12(21), 6906; https://doi.org/10.3390/jcm12216906 - 2 Nov 2023
Cited by 10 | Viewed by 2891
Abstract
The diagnosis of large vestibular schwannomas (VS) with retained useful hearing has become increasingly common. Preservation of facial nerve (FN) function has improved using intraoperative EMG monitoring, hearing preservation remains challenging, with the recent use of cochlear nerve action potential (CNAP) monitoring. This [...] Read more.
The diagnosis of large vestibular schwannomas (VS) with retained useful hearing has become increasingly common. Preservation of facial nerve (FN) function has improved using intraoperative EMG monitoring, hearing preservation remains challenging, with the recent use of cochlear nerve action potential (CNAP) monitoring. This prospective longitudinal series of VS with useful hearing operated on using a retrosigmoid approach included 37 patients with a mean largest extrameatal VS. diameter of 25 ± 8.7 mm (81% of Koos stage 4). CNAP was detected in 51% of patients, while auditory brainstem responses (ABR) were present in 22%. Patients were divided into two groups based on the initial intraoperative CNAP status, whether it was present or absent. FN function was preserved (grade I–II) in 95% of cases at 6 months. Serviceable hearing (class A + B) was preserved in 16% of the cases, while 27% retained hearing with intelligibility (class A–C). Hearing with intelligibility (class A–C) was preserved in 42% of cases when CNAP could be monitored in the early stages of VS resection versus 11% when it was initially absent. Changes in both the approach to the cochlear nerve and VS resection are mandatory in preserving CNAP and improve the rate of hearing preservation. Full article
(This article belongs to the Special Issue Current Trends in the Management of Vestibular Schwannoma)
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