Relationship between Signal Intensity of the Labyrinth and Cochleovestibular Testing and Morphologic Features of Vestibular Schwannoma
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ABSTRACT:
SUBMITTER: Constanzo F
PROVIDER: S-EPMC9272303 | biostudies-literature | 2021 Mar
REPOSITORIES: biostudies-literature
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Project description:Vestibular schwannoma (VS) is commonly accompanied by hearing loss, tinnitus, and dizziness and tends to be chronically progressive in nature. We report a case of VS presenting as left vestibular neuritis (VN) in a previously healthy 57-year-old patient. Right-beating horizontal-torsional spontaneous nystagmus was observed, and the bedside head impulse test revealed a left catch-up saccade. The bithermal caloric test showed left canal paresis, and pure-tone audiometry revealed an average threshold of 22.5 dB bilaterally. Brain magnetic resonance imaging (MRI) demonstrated a 0.7-cm enhancing mass in the left internal auditory canal, consistent with VS. The patient was administered with high-dose systemic corticosteroids and vestibular suppressants with antiemetic, which relieved acute vertigo. Although dizziness in VS is chronically progressive in nature, VS may present as an acute vestibular syndrome that mimics VN. VS should be considered a potential cause of acute vestibular syndrome, and thorough neurological examination with MRI may be helpful for accurate diagnosis.
| S-EPMC9830296 | biostudies-literature
Project description:ObjectiveThe Koos grading scale is a frequently used classification system for vestibular schwannoma (VS) that accounts for extrameatal tumor dimension and compression of the brain stem. We propose an artificial intelligence (AI) pipeline to fully automate the segmentation and Koos classification of VS from MRI to improve clinical workflow and facilitate patient management.MethodsWe propose a method for Koos classification that does not only rely on available images but also on automatically generated segmentations. Artificial neural networks were trained and tested based on manual tumor segmentations and ground truth Koos grades of contrast-enhanced T1-weighted (ceT1) and high-resolution T2-weighted (hrT2) MR images from subjects with a single sporadic VS, acquired on a single scanner and with a standardized protocol. The first stage of the pipeline comprises a convolutional neural network (CNN) which can segment the VS and 7 adjacent structures. For the second stage, we propose two complementary approaches that are combined in an ensemble. The first approach applies a second CNN to the segmentation output to predict the Koos grade, the other approach extracts handcrafted features which are passed to a Random Forest classifier. The pipeline results were compared to those achieved by two neurosurgeons.ResultsEligible patients (n = 308) were pseudo-randomly split into 5 groups to evaluate the model performance with 5-fold cross-validation. The weighted macro-averaged mean absolute error (MA-MAE), weighted macro-averaged F1 score (F1), and accuracy score of the ensemble model were assessed on the testing sets as follows: MA-MAE = 0.11 ± 0.05, F1 = 89.3 ± 3.0%, accuracy = 89.3 ± 2.9%, which was comparable to the average performance of two neurosurgeons: MA-MAE = 0.11 ± 0.08, F1 = 89.1 ± 5.2, accuracy = 88.6 ± 5.8%. Inter-rater reliability was assessed by calculating Fleiss' generalized kappa (k = 0.68) based on all 308 cases, and intra-rater reliabilities of annotator 1 (k = 0.95) and annotator 2 (k = 0.82) were calculated according to the weighted kappa metric with quadratic (Fleiss-Cohen) weights based on 15 randomly selected cases.ConclusionsWe developed the first AI framework to automatically classify VS according to the Koos scale. The excellent results show that the accuracy of the framework is comparable to that of neurosurgeons and may therefore facilitate management of patients with VS. The models, code, and ground truth Koos grades for a subset of publicly available images (n = 188) will be released upon publication.
| S-EPMC10365083 | biostudies-literature
Project description:Vestibular schwannoma is a common benign tumour that may cause local complications. However, vestibular schwannoma has a known association with communicating hydrocephalus presenting with symptoms of normal pressure hydrocephalus and requiring treatment by ventricular shunting or tumour resection. We report a 79-year-old woman who presented with subacute gait apraxia, cognitive impairment and urinary incontinence. CT and MR imaging identified a 20 mm vestibular schwannoma and communicating hydrocephalus; her cerebrospinal fluid (CSF) protein was elevated. Her symptoms improved following ventriculoperitoneal shunt insertion. The mechanism by which non-obstructing vestibular schwannoma causes hydrocephalus is unclear, but hyperproteinorrachia is probably important, likely by impeding CSF resorption.
| S-EPMC11877107 | biostudies-literature
Project description:Vestibular schwannomas are tumors arising from the vestibulocochlear nerve at the cerebellopontine angle. Their proximity to eloquent brainstem structures means that the pathology itself and the treatment thereof can be associated with significant morbidity. The vast majority of these tumors are sporadic, with the remainder arising as a result of the genetic syndrome Neurofibromatosis Type 2 or, more rarely, LZTR1-related schwannomatosis. The natural history of these tumors is extremely variable, with some tumors not displaying any evidence of growth, others demonstrating early, persistent growth and a small number growing following an extended period of indolence. Emerging evidence now suggests that far from representing Schwann cell proliferation only, the tumor microenvironment is complex, with inflammation proposed to play a key role in their growth. In this review, we provide an overview of this new evidence, including the role played by immune cell infiltration, the underlying molecular pathways involved, and biomarkers for detecting this inflammation in vivo. Given the limitations of current treatments, there is a pressing need for novel therapies to aid in the management of this condition, and we conclude by proposing areas for future research that could lead to the development of therapies targeted toward inflammation in vestibular schwannoma.
| S-EPMC7212860 | biostudies-literature
Project description:This SuperSeries is composed of the SubSeries listed below.
2014-11-30 | GSE56598 | GEO
Project description:We designed a prospective study to evaluate changes in tinnitus after vestibular schwannoma (VS) surgery. Subjects included 41 patients who were diagnosed with a VS and underwent translabyrinthine microsurgery (TLM) between January 2015 and May 2016. All patients underwent related examinations and were asked to answer the Tinnitus Handicap Inventory (THI) scale and a visual analog scale (VAS) of tinnitus severity both pre- and postoperatively. Of the 41 patients, 31 (75.6%) suffered from tinnitus before surgery. Microsurgery was associated with an overall decrease in tinnitus (p < 0.001). There was a significant improvement in THI and VAS scores after surgery (p = 0.001 and p = 0.005, respectively). The decrease in THI scores in the low-frequency group was significantly larger than that of the mid- and high-frequency groups after surgery (p = 0.034 and p = 0.001, respectively). The loudness of tinnitus decreased significantly after surgery (p = 0.031). Tinnitus in patients with VS improved after TLM. Patients with mid-/high-frequency tinnitus and louder tinnitus preoperatively seemed to have a worse prognosis than those with low-frequency and quieter tinnitus.
| S-EPMC6370768 | biostudies-literature
Project description:Vestibular schwannoma (VS) is intracranial tumor arising from neoplastic Schwann cells, causing hearing loss in about 95% of patients. The traditional belief that hearing deficit is caused by physical expansion of the VS, compressing the auditory nerve, does not explain the common clinical finding that patients with small tumors can have profound hearing loss, suggesting that tumor-secreted factors could influence hearing ability in VS patients. Here, we conducted profiling of patients' plasma for 67 immune-related factors on a large cohort of VS patients (N>120) and identified candidate biomarkers associated with tumor growth (IL-16 and S100B) and hearing (MDC). We identified the 7-biomarker panel composed of MCP-3, BLC, S100B, FGF-2, MMP-14, eotaxin, and TWEAK that showed outstanding discriminatory ability for VS. These findings revealed possible therapeutic targets for VS-induced hearing loss and provided a unique diagnostic tool that may predict hearing change and tumor growth in VS patients and may help inform the ideal timing of tumor resection to preserve hearing.
| S-EPMC9900840 | biostudies-literature
Project description:Introduction Vestibular schwannoma (also known as acoustic neuroma) is a benign tumor whose cells are derived from Schwann sheaths, which commonly occurs from the vestibular portion of the eighth cranial nerve. Furthermore, vestibular schwannomas account for ∼8% of intracranial tumors in adults and 80 to 90% of tumors of the cerebellopontine angle. Its symptoms are varied, but what stands out most is a unilateral sensorineural hearing loss, with a low index of speech recognition. Objective Describe an atypical manifestation of vestibular schwannoma. Case Report The 46-year-old woman had vertigo and binaural hearing loss and fullness, with ear, nose, and throat examination suggestive of cochlear injury. After 6 months, the patient developed worsening of symptoms and onset of right unilateral tinnitus. In further exams the signs of cochlear damage remained, except for the vestibular test (hyporeflexia). Magnetic resonance imaging showed an expansive lesion in the right cerebellopontine angle. Discussion This report warns about the atypical manifestations of vestibular schwannoma, which must always be remembered in investigating and diagnosing hearing loss.
| S-EPMC4399194 | biostudies-other
Project description:Previous research has shown that although NF2 gene mutation is the major cause of vestibular schwannoma (VS), it may not directly participate in cystic VS (CVS). To elucidate the underlying potential genetic mechanisms in the cystic formation of VS, we compared differences in gene expression between solid VS (SVS) and CVS via a bioinformatics analysis. The cDNA microarray method and miRNA sequencing were performed on 29 representative VSs (17 CVSs and 12 SVSs). A differential expression analysis was used to identify differentially expressed mRNAs (DEmRNAs) and miRNAs (DEmiRNAs). Then, miRNA-mRNA regulatory networks were constructed. Gene ontology (GO), a KEGG pathway enrichment analysis, and the protein-protein interaction (PPI) were used to analyze the co-differentially expressed DEmRNAs at the functional level. From the differential expression analyses, 1304 DEmRNAs, 55 DEmiRNAs, and hub genes including PTEN, FOXO1, FOXO3, VEGFA, and SIRT1 were identified. Histological evidence is presented to confirm the makeup of the hubs, which corresponded with the cDNA microarray. Our analysis revealed that the maps of apoptosis, cellular response to hypoxia, and the PI3K-Akt, AMPK, FOXO, and chemokine signaling pathways were significantly enriched. In addition, the TUNEL assay, immunoblotting analysis, and transmission electron microscope revealed increased degenerative changes in CVS. These findings could be the foundation for understanding the potential role of differential genes in the cystic formation of VS and be helpful in exploring the potential biomarkers for the differential diagnosis, prognosis, and development of drug targets for CVS.
| S-EPMC6570702 | biostudies-literature
Project description:A disintegrin and metalloproteinase 9 (ADAM9) is a member of the transmembrane ADAM family. It is expressed in different types of solid cancer and promotes tumor invasiveness. To the best of our knowledge, the present study was the first to examine ADAM9 expression in vestibular schwannomas (VS) from patients with and without neurofibromatosis type 2 (NF2) and to associate the data with clinical parameters of the patients. The aim of the present study was to evaluate if ADAM9 could be used as prognostic marker or therapeutic target. ADAM9 mRNA and protein levels were measured in VS samples (n=60). A total of 30 of them were from patients with neurofibromatosis. Healthy peripheral nerves from autopsies (n=10) served as controls. ADAM9 mRNA levels were measured by PCR, and protein levels were determined by immunohistochemistry (IHC) and western blotting (WB). The Hannover Classification was used to categorize tumor extension and hearing loss. ADAM9 mRNA levels were 8.8-fold higher in VS compared with in controls. The levels were 5.6-fold higher in patients with NF2 and 12-fold higher in patients with sporadic VS. WB revealed two mature isoforms of the protein, and according to IHC ADAM9 was mainly expressed by S100-positive Schwann cells. There was a strong correlation between ADAM9 mRNA expression and the level of functional impairment (r~1, p=0.01). Particularly, the secreted isoform of ADAM9 was expressed in patients with higher hearing impairment. ADAM9 mRNA was overexpressed in the tumor samples relative to healthy vestibular nerves, and there was an association between higher ADAM9 expression levels and greater hearing impairment. Therefore, ADAM9 may be a prognostic marker for VS, and ADAM9 inhibition might have the potential as a systemic approach for the treatment of VS.
| S-EPMC7039135 | biostudies-literature