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Vestibular schwannoma

Christine T Dinh, Olena Bracho, Christine Mei, Esperanza Bas, Cristina Fernandez-Valle, Fred Telischi, Xue-Zhong Liu
HYPOTHESIS: Microsurgical implantation of mouse merlin-deficient Schwann cells (MD-SC) into the cerebellopontine angle of immunodeficient rats will initiate tumor formation, hearing loss, and vestibular dysfunction. BACKGROUND: The progress in identifying effective drug therapies for treatment of Neurofibromatosis type II (NF2) is limited by the availability of animal models of VS that develop hearing loss and imbalance. METHODS: A microsurgical technique for implanting MD-SCs onto the cochleovestibular nerve of rats was developed...
March 19, 2018: Otology & Neurotology
Michael J Link, Morten Lund-Johansen, Christine M Lohse, Colin L W Driscoll, Ehrling Myrseth, Oystein Vesterli Tveiten, Matthew L Carlson
BACKGROUND: The goal of microsurgical removal of a vestibular schwannoma is to completely remove the tumor, to provide long-term durable cure. In many cases, less than gross total resection (GTR) is performed to preserve neurological, and especially facial nerve function. OBJECTIVE: To analyze long-term quality of life (QoL) in a cohort of patients who received either GTR or less than GTR. METHODS: Patients operated for vestibular schwannoma less than 3...
April 1, 2018: Neurosurgery
Alejandra M Petrilli, Cristina Fernández-Valle
Schwannomas are benign nerve tumors that occur sporadically in the general population and in those with neurofibromatosis type 2 (NF2), a tumor predisposition genetic disorder. NF2-associated schwannomas and most sporadic schwannomas are caused by inactivating mutations in Schwann cells in the neurofibromatosis type 2 gene (NF2) that encodes the merlin tumor suppressor. Despite their benign nature, schwannomas and especially vestibular schwannomas cause considerable morbidity. The primary available therapies are surgery or radiosurgery which usually lead to loss of function of the compromised nerve...
2018: Methods in Molecular Biology
Geetha Anand, Grace Vasallo, Maria Spanou, Saumya Thomas, Michael Pike, Didu Sanduni Kariyawasam, Sanjay Mehta, Allyson Parry, Juliette Durie-Gair, James Nicholson, Karine Lascelles, Vanessa Everett, Frances Mary Gibbon, Nicola Jarvis, John Elston, Dafydd Gareth Evans, Dorothy Halliday
OBJECTIVE: Onset of symptoms in severe sporadic neurofibromatosis type 2 (NF2) is typically within childhood; however, there is poor awareness of presenting features in young children, potentially resulting in delayed diagnosis and poorer outcome. We have reviewed presentation of sporadic paediatric NF2 to raise awareness of early features, highlighting those requiring further investigation. DESIGN: Patients diagnosed with NF2 at age ≤16 and seen between 2012 and 2015 were notified via the British Paediatric Neurology Surveillance Unit or identified through the English NF2 service...
March 13, 2018: Archives of Disease in Childhood
Daniela Aneta Starosta, Birgit Lorenz
BACKGROUND: Neurofibromatosis type 2 (NF2) is a genetic condition with an autosomal dominant pattern of inheritance and incomplete penetrance. It is characterized by multiple benign tumors of the central and peripheral nervous system including astrocytomas, ependymomas, meningeomas, and schwannomas, among which bilateral vestibular schwannomas are the most frequent. Among ocular manifestations of NF2, juvenile subcapsular cataract is the most common followed by epiretinal membranes and combined hamartomas of the retina and retinal pigment epithelium...
March 2018: Klinische Monatsblätter Für Augenheilkunde
Magdalena Lachowska, Paulina Glinka, Kazimierz Niemczyk
BACKGROUND: Air-conducted and skull-tap cervical vestibular evoked myogenic potentials (AC-cVEMP and Tap-cVEMP) have been shown to be very promising tools in clinical practice. They are noninvasive, easy to obtain and - importantly - they require little time and the cost of the instruments is low. OBJECTIVES: The aim of this study was to evaluate the usefulness of the combined use of ACand Tap-cVEMPs as a diagnostic tool for advanced assessment of vestibular schwannoma in determining tumor origin, and to investigate whether the results are helpful for a surgeon as an additional source of information about the tumor before surgery...
March 13, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Felix Ehret, Alexander Muacevic
Vestibular schwannomas are mostly sporadic, neuroectodermal, benign tumors of the myelin-forming cells of the vestibulocochlear nerve. Typical initial symptoms of vestibular schwannomas often include unilateral hearing loss, tinnitus, vertigo, and headaches. As schwannomas characteristically show a slow growth rate and various symptoms, different therapeutic approaches are possible, ranging from a watchful waiting strategy to radiation therapy and neurosurgical tumor removal. In addition, these treatment options should be evaluated carefully and assigned individually to the patients' needs and symptoms while respecting the feasibility and possible outcome of the chosen treatment...
January 5, 2018: Curēus
John P Sheppard, Carlito Lagman, Giyarpuram N Prashant, Yasmine Alkhalid, Thien Nguyen, Courtney Duong, Methma Udawatta, Bilwaj Gaonkar, Stephen E Tenn, Orin Bloch, Isaac Yang
OBJECTIVE: To retrospectively compare ideal radiosurgical target volumes defined by a manual method (surgeon) to those determined by Adaptive Hybrid Surgery (AHS) operative planning software in 7 vestibular schwannoma (VS) cases. METHODS: Four attending surgeons (3 neurosurgeons and 1 ear, nose, and throat surgeon) manually contoured planned residual tumors volumes for 7 consecutive VS patients. Next, the AHS software determined the ideal radiosurgical target volumes based on a specified radiotherapy plan...
March 9, 2018: World Neurosurgery
Rabih Aboukaïs, Nicolas-Xavier Bonne, Gustavo Touzet, Christophe Vincent, Nicolas Reyns, Jean-Paul Lejeune
OBJECTIVE: We aimed to evaluate the outcome of patients who underwent salvage microsurgery for vestibular schwannoma (VS) that failed primary Gammaknife radiosurgery (GKS). PATIENTS AND METHODS: Among the 1098 patients who received GKS for the treatment of VS in our center between January 2004 and December 2012, the follow-up was organized in our institution for 290 patients who lived in our recruitment area. Tumor progression was noted in 23 patients. A salvage microsurgical resection was performed in 11 patients, who were included in our study...
March 5, 2018: Clinical Neurology and Neurosurgery
Jonathan D Breshears, Joseph Chang, Annette M Molinaro, Penny K Sneed, Michael W McDermott, Aaron Tward, Philip V Theodosopoulos
BACKGROUND: The optimal observation interval after the radiosurgical treatment of a sporadic vestibular schwannoma, prior to salvage intervention, is unknown. OBJECTIVE: To determine an optimal postradiosurgical treatment interval for differentiating between pseudoprogression and true tumor growth by analyzing serial volumetric data. METHODS: This single-institution retrospective study included all sporadic vestibular schwannomas treated with Gamma Knife radiosurgery (Eketa AB, Stockholm, Sweden; 12-13 Gy) from 2002 to 2014...
March 5, 2018: Neurosurgery
Maria Breun, Alexandra Schwerdtfeger, Donato Daniel Martellotta, Almuth F Kessler, Jose M Perez, Camelia M Monoranu, Ralf-Ingo Ernestus, Cordula Matthies, Mario Löhr, Carsten Hagemann
Background: CXCR4 is a chemokine receptor that recruits blood stem cells and increases tumor cell growth and invasiveness. We examined CXCR4 expression in vestibular schwannomas (VS) from patients with and without neurofibromatosis type 2 (NF2) and correlated the levels with the patients' clinical characteristics. The aim was to determine whether CXCR4 can be used as a prognostic marker and as a target for systemic therapy. Results: Overall, CXCR4 mRNA levels were 4...
February 9, 2018: Oncotarget
Ali Kouhi, Varasteh Vakili Zarch, Ali Pouyan
The rate of hearing preservation after vestibular schwannoma surgery is variable and is not as high as expected, possibly due to injuries to the posterior semicircular canal while exposing the tumor. The aim of this study was to estimate the risk of posterior semicircular canal injuries using temporal bone computed tomography (CT) scan findings. Temporal bone CT scans of 30 patients selected between 2013 and 2015 were studied. The median age of the patients was 40 years. Two planes were studied: (1) the axial plane that shows the common crus of the posterior semicircular canal and (2) the coronal plane that shows the two crura of the posterior semicircular canal...
January 2018: Ear, Nose, & Throat Journal
J Shapey, K Barkas, S Connor, A Hitchings, H Cheetham, S Thomson, J M U-King-Im, R Beaney, D Jiang, S Barazi, R Obholzer, Nwm Thomas
Introduction Conservative management of patients with a stable vestibular schwannoma (VS) places a significant burden on National Health Service (NHS) resources and yet patients' surveillance management is often inconsistent. Our unit has developed a standardised pathway to guide surveillance imaging of patients with stable VS. In this article, we provide the basis for our imaging protocol by reviewing the measurement, natural history and growth patterns of VS, and we present a cost analysis of implementing the pathway both regionally and nationally...
March 2018: Annals of the Royal College of Surgeons of England
Fu Zhao, Bo Wang, Zhijun Yang, Qiangyi Zhou, Peng Li, Xingchao Wang, Jing Zhang, Junting Zhang, Pinan Liu
Surgical treatment of vestibular schwannoma (VS) in patients with neurofibromatosis type 2 (NF2) along with functional preservation of cranial nerves is challenging. The aim of this study was to analyze the outcomes of hearing and facial nerve function in patients with NF2 who underwent large-size VS (> 2 cm) surgery. From 2006 to 2016, one hundred and forty NF2 patients were included with 149 large-size VS resections using retrosigmoid approach. Hearing function was classified according to the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria...
February 28, 2018: Journal of Neuro-oncology
Pinar Eser Ocak, Ihsan Dogan, Umut Ocak, Cem Dinc, Mustafa K Başkaya
OBJECTIVE Cystic vestibular schwannomas (CVSs) are a subgroup of vestibular schwannomas (VSs) that are reported to be associated with unpredictable clinical behavior and unfavorable postoperative outcomes. The authors aimed to review their experience with microsurgical treatment of CVSs in terms of extent of resection and postoperative facial nerve (FN) function and compare these outcomes with those of their solid counterparts. METHODS Two hundred-eleven VS patients were treated surgically between 2006 and 2017...
March 2018: Neurosurgical Focus
Daniele Starnoni, Roy Thomas Daniel, Constantin Tuleasca, Mercy George, Marc Levivier, Mahmoud Messerer
OBJECTIVE During the last decade, the primary objective for large vestibular schwannoma (VS) management has progressively shifted, from tumor excision to nerve preservation by using a combined microsurgical and radiosurgical approach. The aim of this study was to provide a systematic review and meta-analysis of the available literature regarding the combined strategy of subtotal resection (STR) followed by stereotactic radiosurgery (SRS) for large VSs. METHODS The authors performed a systematic review and meta-analysis in compliance with the PRISMA guidelines for article identification and inclusion using the PubMed, Embase, and Cochrane databases...
March 2018: Neurosurgical Focus
Brandon Lucke-Wold, Erik C Brown, Justin S Cetas, Aclan Dogan, Sachin Gupta, Timothy E Hullar, Timothy L Smith, Jeremy N Ciporen
Cerebrospinal fluid (CSF) leaks occur in approximately 10% of patients undergoing a translabyrinthine, retrosigmoid, or middle fossa approach for vestibular schwannoma resection. Cerebrospinal fluid rhinorrhea also results from trauma, neoplasms, and congenital defects. A high degree of difficulty in repair sometimes requires repetitive microsurgical revisions-a rate of 10% of cases is often cited. This can not only lead to morbidity but is also costly and burdensome to the health care system. In this case-based theoretical analysis, the authors summarize the literature regarding endoscopic endonasal techniques to obliterate the eustachian tube (ET) as well as compare endoscopic endonasal versus open approaches for repair...
March 2018: Neurosurgical Focus
Adam N Master, Daniel S Roberts, Eric P Wilkinson, William H Slattery, Gregory P Lekovic
OBJECTIVE The authors describe their results using an endoscope as an adjunct to microsurgical resection of inferior vestibular schwannomas (VSs) with extension into the fundus of the internal auditory canal below the transverse crest. METHODS All patients who had undergone middle fossa craniotomy for VSs performed by the senior author between September 2014 and August 2016 were prospectively enrolled in accordance with IRB policies, and the charts of patients undergoing surgery for inferior vestibular nerve tumors, as determined either on preoperative imaging or as intraoperative findings, were retrospectively reviewed...
March 2018: Neurosurgical Focus
Pedro Helo Dos Santos Neto, Johnni Oswaldo Zamponi, Rogério Hamerschmidt, Gislaine Richter Minhoto Wiemes, Marcio S Rassi, Luis A B Borba
Hearing loss is the most common symptom of vestibular schwannomas (VSs). The management of these lesions includes observation, radiosurgery, and microsurgical resection. Hearing preservation and rehabilitation are the major challenges after the tumor treatment. A 43-year-old man with previous left-sided profound hearing loss and tinnitus presented with a 2-mm left-sided intracanalicular VS. The decision was made to perform a simultaneous cochlear implantation (CI) and microsurgical resection of the tumor. The patient did well postoperatively, with significant improvement of tinnitus, sound localization, and speech recognition in noise...
March 2018: Neurosurgical Focus
Amey R Savardekar, Devi P Patra, Jai D Thakur, Vinayak Narayan, Nasser Mohammed, Papireddy Bollam, Anil Nanda
OBJECTIVE Total tumor excision with the preservation of neurological function and quality of life is the goal of modern-day vestibular schwannoma (VS) surgery. Postoperative facial nerve (FN) paralysis is a devastating complication of VS surgery. Determining the course of the FN in relation to a VS preoperatively is invaluable to the neurosurgeon and is likely to enhance surgical safety with respect to FN function. Diffusion tensor imaging-fiber tracking (DTI-FT) technology is slowly gaining traction as a viable tool for preoperative FN visualization in patients with VS...
March 2018: Neurosurgical Focus
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