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Items 1 to 37 of about 37
1. Mautner VF, Nguyen R, Kutta H, Fuensterer C, Bokemeyer C, Hagel C, Friedrich RE, Panse J: Bevacizumab induces regression of vestibular schwannomas in patients with neurofibromatosis type 2. Neuro Oncol; 2010 Jan;12(1):14-8
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  • [Title] Bevacizumab induces regression of vestibular schwannomas in patients with neurofibromatosis type 2.
  • Bilateral vestibular schwannomas are the hallmark of neurofibromatosis type 2 (NF2), and these tumors impair hearing and frequently lead to deafness.
  • We report 2 cases in which treatment with bevacizumab (for 3 months in one case and 6 months in the other) induced regression of progressive vestibular schwannomas by more than 40% and substantially improved hearing in the patient treated for 6 months.
  • Bevacizumab therapy may thus provide an effective treatment for progressive vestibular schwannomas in patients with NF2.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antineoplastic Agents / therapeutic use. Neurofibromatosis 2 / drug therapy. Neuroma, Acoustic / drug therapy

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  • [Cites] J Neurosurg. 2002 Jun;96(6):1063-71 [12066908.001]
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  • (PMID = 20150363.001).
  • [ISSN] 1523-5866
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antineoplastic Agents; 2S9ZZM9Q9V / Bevacizumab
  • [Other-IDs] NLM/ PMC2940556
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2. Dohi O, Hatori M, Ichinohasama R, Hosaka M, Hashimoto S, Kokubun S: Diffuse large B-cell lymphoma arising in a patient with neurofibromatosis type I and in a patient with neurofibromatosis type II. Tohoku J Exp Med; 2006 Feb;208(2):169-76
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  • Neurofibromatosis II typically consists of bilateral schwannomas of the acoustic nerve.

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  • (PMID = 16434841.001).
  • [ISSN] 0040-8727
  • [Journal-full-title] The Tohoku journal of experimental medicine
  • [ISO-abbreviation] Tohoku J. Exp. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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3. Lassaletta L, Gavilán J: [An update on the treatment of vestibular schwannoma]. Acta Otorrinolaringol Esp; 2009 Mar-Apr;60(2):131-40
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  • [Title] [An update on the treatment of vestibular schwannoma].
  • [Transliterated title] Actualización en el tratamiento del schwannoma vestibular.
  • The increase in the diagnosis of ever smaller vestibular schwannomas (VS), the fact that many tumours can be observed with serial MRI, and the development of radiosurgery as an alternative to microsurgery have led the neurotologic surgeon to a new global approach to patients with vs. On the other hand, the spread of internet-based information sources, often with biased or incomplete information, makes counselling patients with VS a challenging task.
  • Due to the completely different approach and peculiar features of bilateral VS in patients with type II neurofibromatosis, these are excluded.
  • [MeSH-major] Neuroma, Acoustic / therapy

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  • (PMID = 19401081.001).
  • [ISSN] 0001-6519
  • [Journal-full-title] Acta otorrinolaringológica española
  • [ISO-abbreviation] Acta Otorrinolaringol Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 50
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4. Sabol Z, Kipke-Sabol L, Miklić P, Hajnsek-Propadalo S, Sabol F: [Neurofibromatosis type 2 (central neurofibromatosis or bilateral acoustic neuromas, vestibular schwannomas): from phenotype to gene]. Lijec Vjesn; 2006 Sep-Oct;128(9-10):309-16
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  • [Title] [Neurofibromatosis type 2 (central neurofibromatosis or bilateral acoustic neuromas, vestibular schwannomas): from phenotype to gene].
  • Neurofibromatosis type 2 (NF2) is an autosomal dominant disease that predisposes to bilateral vestibular schwannomas (neurinomas), other central and peripheral nervous system tumours (multiple meningeomas and neurofibromas) and ocular abnormalities (cataract).
  • [MeSH-major] Neurofibromatosis 2 / genetics. Neuroma, Acoustic / genetics


5. Vachhani JA, Friedman WA: Radiosurgery in patients with bilateral vestibular schwannomas. Stereotact Funct Neurosurg; 2007;85(6):273-8
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  • [Title] Radiosurgery in patients with bilateral vestibular schwannomas.
  • BACKGROUND: Patients with bilateral vestibular schwannomas offer a unique opportunity to determine the effectiveness of radiosurgery.
  • This study shows that radiosurgery alters the natural history of vestibular schwannomas.
  • [MeSH-major] Neurofibromatosis 2 / surgery. Neuroma, Acoustic / surgery. Radiosurgery. Vestibular Nerve / surgery

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  • [Copyright] (c) 2007 S. Karger AG, Basel.
  • (PMID = 17709979.001).
  • [ISSN] 1423-0372
  • [Journal-full-title] Stereotactic and functional neurosurgery
  • [ISO-abbreviation] Stereotact Funct Neurosurg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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6. Sahu RN, Mehrotra N, Tyagi I, Banerji D, Jain VK, Behari S: Management strategies for bilateral vestibular schwannomas. J Clin Neurosci; 2007 Aug;14(8):715-22
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  • [Title] Management strategies for bilateral vestibular schwannomas.
  • BACKGROUND: Bilateral vestibular schwannomas (VS) are rare.
  • AIMS: To highlight the differences in clinical presentations and surgical results of bilateral VS compared to unilateral VS; and, to propose a management strategy for these tumors with reference to tumor size, extent of growth and the presence or absence of hearing impairment.
  • METHOD: This is a retrospective study of 16 patients with bilateral VS treated over 10 years in a tertiary referral hospital.
  • CONCLUSIONS: Patients with bilateral schwannomas are younger, have larger lesions, poorer preoperative hearing and are more likely to lose either auditory and/or facial nerve function during attempted total resection of the tumor.
  • [MeSH-major] Cranial Nerve Neoplasms / therapy. Functional Laterality. Neuroma, Acoustic / therapy

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  • (PMID = 17577524.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Scotland
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7. Plotkin SR, Stemmer-Rachamimov AO, Barker FG 2nd, Halpin C, Padera TP, Tyrrell A, Sorensen AG, Jain RK, di Tomaso E: Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med; 2009 Jul 23;361(4):358-67
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  • BACKGROUND: Profound hearing loss is a serious complication of neurofibromatosis type 2, a genetic condition associated with bilateral vestibular schwannomas, benign tumors that arise from the eighth cranial nerve.
  • METHODS: We determined the expression pattern of vascular endothelial growth factor (VEGF) and three of its receptors, VEGFR-2, neuropilin-1, and neuropilin-2, in paraffin-embedded samples from 21 vestibular schwannomas associated with neurofibromatosis type 2 and from 22 sporadic schwannomas.
  • Ten consecutive patients with neurofibromatosis type 2 and progressive vestibular schwannomas who were not candidates for standard treatment were treated with bevacizumab, an anti-VEGF monoclonal antibody.
  • RESULTS: VEGF was expressed in 100% of vestibular schwannomas and VEGFR-2 in 32% of tumor vessels on immunohistochemical analysis.
  • CONCLUSIONS: VEGF blockade with bevacizumab improved hearing in some, but not all, patients with neurofibromatosis type 2 and was associated with a reduction in the volume of most growing vestibular schwannomas.

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  • [Copyright] 2009 Massachusetts Medical Society
  • [CommentIn] N Engl J Med. 2009 Oct 29;361(18):1810; author reply 1810-1 [19877310.001]
  • [CommentIn] N Engl J Med. 2009 Oct 29;361(18):1809-10; author reply 1810-1 [19864683.001]
  • (PMID = 19587327.001).
  • [ISSN] 1533-4406
  • [Journal-full-title] The New England journal of medicine
  • [ISO-abbreviation] N. Engl. J. Med.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA080124; United States / NINDS NIH HHS / NS / P01 NS024279; United States / NCI NIH HHS / CA / P01CA80124; United States / NINDS NIH HHS / NS / P01NS024279
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Neuropilin-2; 0 / Vascular Endothelial Growth Factor A; 144713-63-3 / Neuropilin-1; 2S9ZZM9Q9V / Bevacizumab; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-2
  • [Other-IDs] NLM/ NIHMS765691; NLM/ PMC4816642
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8. White JB, Scheithauer BW, Amrami KK, Babovic-Vuksanovic D, Spinner RJ: Contiguous conventional and plexiform schwannomas. Report of two cases. J Neurosurg; 2006 Feb;104(2):319-24
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  • [Title] Contiguous conventional and plexiform schwannomas. Report of two cases.
  • The authors present clinical, imaging, and histological features of two adult patients found to harbor a conventional schwannoma contiguous with a deep plexiform schwannoma.
  • One patient had neurofibromatosis (NF) Type 2 (NF2), and both intracranial (bilateral oculomotor, trigeminal, acoustic, and hypoglossal schwannomas as well as meningiomas) and intraspinal (schwannomas and meningiomas) lesions.
  • The proximal forearm lesions consisted of a conventional schwannoma and an underlying plexiform component.
  • Plexiform schwannomas are rare lesions that occur sporadically or, on occasion, in association with NF2 or meningiomas with or without multiple schwannomas.
  • The authors believe that a more careful examination of patients with NF2 may show that these people have a higher incidence of plexiform schwannoma than previously thought.
  • Pathological confirmation is crucial in distinguishing plexiform schwannomas from plexiform neurofibromas, because the latter are directly associated with NF1 and have a significant tendency to undergo malignant transformation.
  • To the best of the authors' knowledge, this is the first report of a conventional schwannoma contiguous with a deep plexiform schwannoma.

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  • (PMID = 16509508.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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9. Sen A, Khan MI, Ramsden RT, Gillespie JE: Stenosis of the internal auditory meatus masquerading as bilateral vestibular schwannomas: a cautionary tale. J Laryngol Otol; 2005 Dec;119(12):995-7
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  • [Title] Stenosis of the internal auditory meatus masquerading as bilateral vestibular schwannomas: a cautionary tale.
  • They are known to symptomatically mimic vestibular schwannomas leading to potential diagnostic error.
  • We present a case (along with literature review) where a stenotic IAM was clinically and radiologically misdiagnosed as a vestibular schwannoma.
  • [MeSH-major] Cranial Nerve Neoplasms / diagnosis. Ear, Inner. Labyrinth Diseases / diagnosis. Neuroma, Acoustic / diagnosis. Vestibulocochlear Nerve Diseases / diagnosis

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  • (PMID = 16354367.001).
  • [ISSN] 0022-2151
  • [Journal-full-title] The Journal of laryngology and otology
  • [ISO-abbreviation] J Laryngol Otol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 8
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10. Meijer OW, Vandertop WP, Lagerwaard FJ, Slotman BJ: Linear accelerator-based stereotactic radiosurgery for bilateral vestibular schwannomas in patients with neurofibromatosis type 2. Neurosurgery; 2008 May;62(5 Suppl):A37-42; discussion A42-3
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  • [Title] Linear accelerator-based stereotactic radiosurgery for bilateral vestibular schwannomas in patients with neurofibromatosis type 2.
  • OBJECTIVE: Patients with neurofibromatosis Type 2 (NF2) patients typically have bilateral vestibular schwannomas (VS) and are at risk for developing bilateral deafness, bilateral trigeminal, and bilateral facial nerve function loss.
  • We studied our treatment results in patients with NF2 with bilateral VS, treated with linear accelerator-based RS and SRT.
  • METHODS: In 204 patients with VS treated with RS or SRT in Amsterdam starting from 1992, we identified 25 patients with NF2 who had bilateral tumors.
  • [MeSH-major] Neurofibromatosis 2 / complications. Neurofibromatosis 2 / surgery. Neuroma, Acoustic / complications. Neuroma, Acoustic / surgery. Radiosurgery / methods

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  • (PMID = 18580779.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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11. Akard W, Tubbs RS, Seymour ZA, Hitselberger WE, Cohen-Gadol AA: Evolution of techniques for the resection of vestibular schwannomas: from saving life to saving function. J Neurosurg; 2009 Apr;110(4):642-7
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  • [Title] Evolution of techniques for the resection of vestibular schwannomas: from saving life to saving function.
  • The current state of surgery for vestibular schwannomas (VSs) is the result of a century of step-by-step technical progress by groundbreaking surgeons who transformed the procedure from its hazardous infancy and high mortality rate to its current state of safety and low morbidity rate.
  • Harvey Cushing advocated bilateral suboccipital decompression and developed the method of intracapsular tumor enucleation.
  • [MeSH-major] Neuroma, Acoustic / history. Neurosurgical Procedures / history

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  • (PMID = 18991500.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Historical Article; Journal Article
  • [Publication-country] United States
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12. Miyakawa T, Kamada N, Kobayashi T, Hirano K, Fujii K, Sasahara Y, Nagai Y, Shinkai H: Neurofibromatosis type 2 in an infant with multiple plexiform schwannomas as first symptom. J Dermatol; 2007 Jan;34(1):60-4
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  • [Title] Neurofibromatosis type 2 in an infant with multiple plexiform schwannomas as first symptom.
  • Bilateral vestibular schwannomas are considered to be the hallmark of this disease, with hearing loss and tinnitus which are caused by these tumors, usually presenting as the initial symptoms.
  • He had multiple cutaneous tumors but did not have any symptoms related to vestibular schwannomas.
  • A histopathological examination revealed these tumors to be plexiform schwannomas; we therefore suspected NF2.
  • As a result of magnetic resonance imaging with gadolinium enhancement, bilateral vestibular schwannomas were detected and a final diagnosis of NF2 was thus made.
  • The association between NF2 and multiple depigmented spots is unknown, we therefore consider that multiple cutaneous plexiform schwannomas may strongly suggest an association with NF2.
  • [MeSH-minor] Child, Preschool. Humans. Male. Neuroma, Acoustic / pathology

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  • (PMID = 17204104.001).
  • [ISSN] 0385-2407
  • [Journal-full-title] The Journal of dermatology
  • [ISO-abbreviation] J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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13. Siddiqui MA, Leslie T, Scott C, Mackenzie J: Eyelid schwannoma in a male adult. Clin Exp Ophthalmol; 2005 Aug;33(4):412-3
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  • [Title] Eyelid schwannoma in a male adult.
  • Schwannomas (neurilemmomas) are benign tumours of peripheral nerves arising from Schwann cells.
  • Solitary lesions can occur sporadically in the general population but multiple neurofibromas are distinctive feature of neurofibromatosis type 1 and bilateral acoustic schwannomas are a feature of neurofibromatosis type 2.
  • Schwannomas of ophthalmic interest are rare although they have been reported in relation to the orbit, and infrequently uveal tract and conjunctiva.
  • Isolated eyelid schwannomas appear infrequent; only four cases in adults and one in a child in the published literature have been found.
  • Herein, a case of eyelid schwannoma in a 53-year-old man is described.

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  • (PMID = 16033357.001).
  • [ISSN] 1442-6404
  • [Journal-full-title] Clinical & experimental ophthalmology
  • [ISO-abbreviation] Clin. Experiment. Ophthalmol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
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14. von Eckardstein KL, Beatty CW, Driscoll CL, Link MJ: Spontaneous regression of vestibular schwannomas after resection of contralateral tumor in neurofibromatosis Type 2. J Neurosurg; 2010 Jan;112(1):158-62
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  • [Title] Spontaneous regression of vestibular schwannomas after resection of contralateral tumor in neurofibromatosis Type 2.
  • The authors report on 2 patients with bilateral vestibular schwannomas (VSs) who underwent unilateral surgical tumor removal.
  • They underline the importance of careful observation of VS involving the only hearing ear in the management of bilateral VS to determine the natural growth pattern of the tumors.
  • [MeSH-major] Functional Laterality. Neurofibromatosis 2 / physiopathology. Neurofibromatosis 2 / surgery. Neuroma, Acoustic / physiopathology. Neuroma, Acoustic / surgery


15. Stieglitz LH, Wrede KH, Gharabaghi A, Gerganov VM, Samii A, Samii M, Luedemann WO: Factors affecting postoperative cerebrospinal fluid leaks after retrosigmoidal craniotomy for vestibular schwannomas. J Neurosurg; 2009 Oct;111(4):874-83
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  • [Title] Factors affecting postoperative cerebrospinal fluid leaks after retrosigmoidal craniotomy for vestibular schwannomas.
  • OBJECT: The aim of this study was to identify patients likely to develop CSF leaks after vestibular schwannoma surgery using a retrospective analysis for the identification of risk factors.
  • Twenty-six patients had bilateral tumors due to neurofibromatosis Type 2, and 4 patients had previously undergone radiosurgical treatment.
  • [MeSH-major] Craniotomy / adverse effects. Craniotomy / methods. Neuroma, Acoustic / surgery. Subdural Effusion / etiology

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  • (PMID = 19326990.001).
  • [ISSN] 1933-0693
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 42
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16. Roche PH, Khalil M, Soumare O, Régis J: Hydrocephalus and vestibular schwannomas: considerations about the impact of gamma knife radiosurgery. Prog Neurol Surg; 2008;21:200-6
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  • [Title] Hydrocephalus and vestibular schwannomas: considerations about the impact of gamma knife radiosurgery.
  • Hydrocephalus may occur at various stages of the natural course of vestibular schwannoma and can also be diagnosed after the therapeutic procedure.
  • Among the first 1,000 vestibular schwannoma patients treated by GKR in our institution, 30 patients (3%) belonged to group A and 1% to group B.
  • In both groups, hydrocephalus was more often associated with the following data: Elderly, large tumor, previous MS, NF2 disease and bilateral tumors.
  • [MeSH-major] Hydrocephalus / epidemiology. Neuroma, Acoustic / surgery. Radiosurgery

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  • (PMID = 18810220.001).
  • [ISSN] 0079-6492
  • [Journal-full-title] Progress in neurological surgery
  • [ISO-abbreviation] Prog Neurol Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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17. Evans DG: Neurofibromatosis 2 [Bilateral acoustic neurofibromatosis, central neurofibromatosis, NF2, neurofibromatosis type II]. Genet Med; 2009 Sep;11(9):599-610
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  • [Title] Neurofibromatosis 2 [Bilateral acoustic neurofibromatosis, central neurofibromatosis, NF2, neurofibromatosis type II].
  • Affected individuals inevitably develop schwannomas typically affecting both vestibular nerves leading to deafness.
  • Schwannomas also occur on other cranial nerves, on spinal nerve roots, and on peripheral nerves.

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  • (PMID = 19652604.001).
  • [ISSN] 1530-0366
  • [Journal-full-title] Genetics in medicine : official journal of the American College of Medical Genetics
  • [ISO-abbreviation] Genet. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 85
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18. Shih C, Tseng FY, Yeh TH, Hsu CJ, Chen YS: Ipsilateral and contralateral acoustic brainstem response abnormalities in patients with vestibular schwannoma. Otolaryngol Head Neck Surg; 2009 Dec;141(6):695-700
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  • [Title] Ipsilateral and contralateral acoustic brainstem response abnormalities in patients with vestibular schwannoma.
  • OBJECTIVE: To analyze auditory brainstem response (ABR) data in patients with vestibular schwannomas (VS) in an effort to identify correlations between abnormal ABR parameters and tumor size.
  • SUBJECTS AND METHODS: Retrospective chart review and analysis of bilateral ABR records in patients with vs. Interaural latency differences of interpeak I-V (ILD-I-V) and ILD-V longer than 0.2 ms were used as abnormal criteria.
  • Tumor size was positively correlated with pure-tone average (P = 0.0106) and with the total number of bilateral abnormal ABR parameters (P = 0.004).
  • [MeSH-major] Evoked Potentials, Auditory, Brain Stem / physiology. Neuroma, Acoustic / physiopathology

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  • (PMID = 19932840.001).
  • [ISSN] 1097-6817
  • [Journal-full-title] Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
  • [ISO-abbreviation] Otolaryngol Head Neck Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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19. Fisher LM, Doherty JK, Lev MH, Slattery WH: Concordance of bilateral vestibular schwannoma growth and hearing changes in neurofibromatosis 2: neurofibromatosis 2 natural history consortium. Otol Neurotol; 2009 Sep;30(6):835-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Concordance of bilateral vestibular schwannoma growth and hearing changes in neurofibromatosis 2: neurofibromatosis 2 natural history consortium.
  • OBJECTIVE: To examine the relationship between the amount of change in size and associated hearing in bilateral vestibular schwannomas (VSs) in persons with neurofibromatosis 2 (NF2).
  • Vestibular schwannoma size changes were not associated with the corresponding hearing changes (Kendall's tau, p = not significant [n.s.]).
  • [MeSH-major] Cranial Nerve Neoplasms / pathology. Hearing Loss / etiology. Neurofibromatosis 2 / complications. Neuroma, Acoustic / pathology

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  • (PMID = 19704365.001).
  • [ISSN] 1537-4505
  • [Journal-full-title] Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
  • [ISO-abbreviation] Otol. Neurotol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Neurofibromin 2
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20. Samii A, Lenarz M, Majdani O, Lim HH, Samii M, Lenarz T: Auditory midbrain implant: a combined approach for vestibular schwannoma surgery and device implantation. Otol Neurotol; 2007 Jan;28(1):31-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Auditory midbrain implant: a combined approach for vestibular schwannoma surgery and device implantation.
  • HYPOTHESIS: The lateral suboccipital approach is a well-established route for safe removal of vestibular schwannomas in neurofibromatosis Type 2 (NF2) patients.
  • The initial candidates are NF2 patients who, because of the growth and/or surgical removal of bilateral acoustic neuromas, develop neural deafness and are unable to benefit from cochlear implants.
  • The ideal surgical approach in NF2 patients must first enable safe removal of vestibular schwannomas and then provide sufficient exposure of the midbrain for AMI implantation.
  • CONCLUSION: This modified lateral suboccipital approach ensures safe removal of large vestibular schwannomas and provides sufficient exposure of the inferior colliculus for ideal AMI implantation.
  • [MeSH-major] Auditory Brain Stem Implants. Deafness / etiology. Deafness / surgery. Neuroma, Acoustic / complications. Neuroma, Acoustic / surgery. Otologic Surgical Procedures / methods

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  • (PMID = 17195743.001).
  • [ISSN] 1531-7129
  • [Journal-full-title] Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
  • [ISO-abbreviation] Otol. Neurotol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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21. Ito E, Saito K, Yatsuya H, Nagatani T, Otsuka G: Factors predicting growth of vestibular schwannoma in neurofibromatosis type 2. Neurosurg Rev; 2009 Oct;32(4):425-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Factors predicting growth of vestibular schwannoma in neurofibromatosis type 2.
  • We retrospectively reviewed characteristics of patients with neurofibromatosis type 2 to identify factors predicting further growth of bilateral vestibular schwannomas.
  • Subjects comprised 27 neurofibromatosis type 2 patients with 54 vestibular schwannomas, followed for 24-204 months (mean, 86 months).
  • This study investigated factors predictive of vestibular schwannoma growth in neurofibromatosis type 2.
  • Features distinguishing actively growing from quiescent VS were determined for untreated course (28 vestibular schwannomas) and posttreatment course (including either resection or radiosurgery; 33 vestibular schwannomas).
  • During the untreated course, 19 vestibular schwannomas showed growth and 9 vestibular schwannomas were stable.
  • [MeSH-major] Cranial Nerve Neoplasms / pathology. Neurofibromatosis 2 / pathology. Neuroma, Acoustic / pathology. Trigeminal Nerve / pathology


22. Lu-Emerson C, Plotkin SR: The neurofibromatoses. Part 2: NF2 and schwannomatosis. Rev Neurol Dis; 2009;6(3):E81-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • NF2 is characterized by bilateral vestibular schwannomas, meningiomas, ependymomas, cataracts, and epiretinal membranes.
  • The combination of complete hearing loss from vestibular schwannomas and blindness from bifacial weakness is a devastating potential outcome of NF2.
  • Schwannomatosis is characterized by multiple nonvestibular, nonintradermal schwannomas and chronic pain.
  • [MeSH-minor] Blindness / etiology. Blindness / physiopathology. Genetic Predisposition to Disease / genetics. Humans. Meningeal Neoplasms / genetics. Meningeal Neoplasms / pathology. Meningeal Neoplasms / physiopathology. Neuroma, Acoustic / genetics. Neuroma, Acoustic / pathology. Neuroma, Acoustic / physiopathology. Spinal Cord Neoplasms / genetics. Spinal Cord Neoplasms / pathology. Spinal Cord Neoplasms / physiopathology

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  • (PMID = 19898272.001).
  • [ISSN] 1949-4378
  • [Journal-full-title] Reviews in neurological diseases
  • [ISO-abbreviation] Rev Neurol Dis
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 34
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23. Clarke L: Neurofibromatosis 2--a family journey. Can J Neurosci Nurs; 2009;31(4):7-14
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • NF2's clinical manifestations of bilateral vestibular schwannomas and multiple other nervous system tumours have far-reaching implications for patients and families diagnosed with this challenging disorder.
  • The Atlantic Lateral Skull Base Clinic in Halifax follows approximately 400 patients with unilateral vestibular schwannomas and other skull base lesions.
  • [MeSH-minor] Adaptation, Psychological. Adolescent. Adult. Aged. Attitude to Health. Female. Genetic Testing. Humans. Male. Meningioma / genetics. Middle Aged. Neuroma, Acoustic / genetics. Nova Scotia. Patient Care Team / organization & administration. Referral and Consultation

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  • (PMID = 20085115.001).
  • [ISSN] 1913-7176
  • [Journal-full-title] Canadian journal of neuroscience nursing
  • [ISO-abbreviation] Can J Neurosci Nurs
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Canada
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24. Bian LG, Sun QF, Tirakotai W, Zhao WG, Shen JK, Luo QZ, Bertalanffy H: Loss of heterozygosity on chromosome 22 in sporadic schwannoma and its relation to the proliferation of tumor cells. Chin Med J (Engl); 2005 Sep 20;118(18):1517-24
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Loss of heterozygosity on chromosome 22 in sporadic schwannoma and its relation to the proliferation of tumor cells.
  • BACKGROUND: Schwannoma is the tumor arising mainly from the cranial and spinal nerves.
  • Bilateral vestibular schwannoma is the hallmark of neurofibromatosis type 2 (NF2).
  • The NF2 gene has been cloned with comprehensive analysis of its mutations in schwannoma.
  • However, most studies focused on vestibular schwannoma.
  • There are differences in proliferation of tumor cell and ultrastructure between vestibular and spinal schwannomas.
  • It is unknown whether genetic alterations in vestibular schwannoma are different from those in non-vestibular schwannoma.
  • We analyzed the loss of heterozygosity (LOH) on chromosome 22 in patients with sporadic schwannoma including vestibular and spinal schwannomas and correlated this genetic alteration with tumor proliferation.
  • METHODS: In 54 unrelated patients without clinical NF1 or NF2, 36 patients had sporadic vestibular schwannoma, and 18 dorsal spinal root schwannoma.
  • Student's t test was used to analyze the difference of the proliferative index between schwannoma with LOH and that without LOH.
  • The difference of the frequency of LOH in vestibular and spinal schwannomas was investigated by the chi-square test.
  • RESULTS: Twenty-three schwannomas (42.6%, 23/54) showed allele loss.
  • The frequency of LOH in vestibular schwannoma was significantly higher than that in spinal schwannoma (chi2 = 5.14, P < 0.05).
  • The proliferative index of schwannoma with LOH was significantly higher than that without LOH (tki-67 = 2.97, P = 0.0045; tPCNA = 2.93, P = 0.0051).
  • CONCLUSIONS: LOH on chromosome 22 is a frequent event in the tumorigenesis of sporadic schwannoma.
  • And, there is a correlation between LOH on chromosome 22 and proliferative activity in schwannoma.
  • The frequency of LOH in vestibular schwannoma is significantly different from that in spinal schwannoma.
  • [MeSH-major] Chromosomes, Human, Pair 22. Loss of Heterozygosity. Neurilemmoma / genetics. Neuroma, Acoustic / genetics. Spinal Cord Neoplasms / genetics. Spinal Nerve Roots


25. Neff BA, Wiet RM, Lasak JM, Cohen NL, Pillsbury HC, Ramsden RT, Welling DB: Cochlear implantation in the neurofibromatosis type 2 patient: long-term follow-up. Laryngoscope; 2007 Jun;117(6):1069-72
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • RESULTS: Patients with NF2-associated bilateral vestibular schwannomas frequently become profoundly deaf.
  • Frequently, tumor size or poor preoperative hearing status can require a surgical approach that leaves the patient with a profound, bilateral sensorineural hearing loss.
  • If the cochlear nerve is preserved anatomically after vestibular schwannoma surgery, and if promontory stimulation confirms the functionality of the cochlear nerve, then cochlear implantation is an excellent option to restore hearing.
  • CONCLUSION: Early surgical intervention for vestibular schwannomas in NF2 patients when the cochlear nerve can be spared is an important consideration to allow for possible cochlear implantation.
  • [MeSH-major] Cochlear Implantation. Hearing Loss, Sensorineural / etiology. Hearing Loss, Sensorineural / surgery. Neurofibromatosis 2 / complications. Neuroma, Acoustic / etiology


26. Sachdeva R, Rothner DA, Traboulsi EI, Hayden BC, Rychwalski PJ: Astrocytic hamartoma of the optic disc and multiple café-au-lait macules in a child with neurofibromatosis type 2. Ophthalmic Genet; 2010 Dec;31(4):209-14
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The characteristic and diagnostic finding of NF2 is bilateral vestibular nerve schwannomas (acoustic neuromas).
  • However, neuroimaging revealed bilateral acoustic neuromas, leading to a clinical diagnosis of NF2.

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  • (PMID = 21067482.001).
  • [ISSN] 1744-5094
  • [Journal-full-title] Ophthalmic genetics
  • [ISO-abbreviation] Ophthalmic Genet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Neurofibromin 2
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27. Walcott BP, Sivarajan G, Bashinskaya B, Anderson DE, Leonetti JP, Origitano TC: Sporadic unilateral vestibular schwannoma in the pediatric population. Clinical article. J Neurosurg Pediatr; 2009 Aug;4(2):125-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sporadic unilateral vestibular schwannoma in the pediatric population. Clinical article.
  • OBJECT: Vestibular schwannomas (VSs) are rare in the pediatric population.
  • Most often, these lesions manifest as a bilateral disease process in the setting of neurofibromatosis Type 2.
  • [MeSH-major] Facial Nerve / physiopathology. Neuroma, Acoustic / surgery

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  • (PMID = 19645545.001).
  • [ISSN] 1933-0707
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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28. Grayeli AB, Kalamarides M, Bouccara D, Ambert-Dahan E, Sterkers O: Auditory brainstem implant in neurofibromatosis type 2 and non-neurofibromatosis type 2 patients. Otol Neurotol; 2008 Dec;29(8):1140-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The population comprised 23 NF2, 3 postmeningitis bilateral ossified cochleas, 3 solitary vestibular schwannomas on the only hearing ear, 1 inner ear malformation, and 1 bilateral cochlear destruction by otosclerosis.
  • Auditory brainstem implant may also be indicated in patients with bilateral profound hearing loss and a predictable failure of cochlear implantation.
  • [MeSH-minor] Adolescent. Adult. Aged. Cochlea / pathology. Cohort Studies. Ear, Inner / abnormalities. Electric Stimulation. Evoked Potentials, Auditory / physiology. Humans. Meningitis / complications. Middle Aged. Neuroma, Acoustic / surgery. Ossification, Heterotopic / etiology. Otosclerosis / complications. Retrospective Studies. Young Adult

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  • (PMID = 18849886.001).
  • [ISSN] 1537-4505
  • [Journal-full-title] Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
  • [ISO-abbreviation] Otol. Neurotol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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29. Gong S, Chen G, Zhong G, Yan K, Zhang E, Chen P, Lin N, Nie X: [Neurofibromatosis type 2]. Lin Chuang Er Bi Yan Hou Ke Za Zhi; 2006 Aug;20(16):721-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • MRI scan indicated space-occupying lesions in the bilateral cerebellopontine angles, bilateral cavernous sinuses and bilateral cervical parts of the patient.
  • The patient was diagnosed as NF2 according to the National Institutes of Health (NIH) criteria, and received operation on the left acoustic tumor.
  • The tumor was proved to be schwannomas by pathological test.
  • CONCLUSION: NF2 is an autosomal dominant, highly penetrant disease which is characterized by bilateral vestibular schwannomas.

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  • (PMID = 17058913.001).
  • [Journal-full-title] Lin chuang er bi yan hou ke za zhi = Journal of clinical otorhinolaryngology
  • [ISO-abbreviation] Lin Chuang Er Bi Yan Hou Ke Za Zhi
  • [Language] chi
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] China
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30. Menon G, Nair S, Sudhir J, Rao BR, Mathew A, Bahuleyan B: Childhood and adolescent meningiomas: a report of 38 cases and review of literature. Acta Neurochir (Wien); 2009 Mar;151(3):239-44; discussion 244
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Eleven children (28.9%) had evidence of NF of whom three had NF2 with bilateral vestibular schwannomas.
  • [MeSH-minor] Adolescent. Age Factors. Child. Comorbidity. Dura Mater / pathology. Dura Mater / surgery. Female. Humans. Intracranial Hypertension / etiology. Male. Neoplasm Recurrence, Local / epidemiology. Neuroma, Acoustic / epidemiology. Neurosurgical Procedures. Optic Nerve / pathology. Retrospective Studies. Seizures / etiology. Skull Base Neoplasms / epidemiology. Skull Base Neoplasms / pathology. Skull Base Neoplasms / surgery. Spinal Neoplasms / epidemiology. Spinal Neoplasms / pathology. Spinal Neoplasms / surgery. Treatment Outcome

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  • (PMID = 19238320.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Austria
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31. Willekens BM, Rooker S, Dua G, Appel B, Martin JJ, Crols R, De Deyn PP: Central nervous system metastases of pulmonary adenocarcinoma mimicking neurofibromatosis type 2. Acta Neurol Belg; 2006 Jun;106(2):87-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Neuroimaging revealed bilateral VIIIth cranial nerve masses and multiple cerebral and spinal cord lesions that were interpreted as being acoustic schwannomas and multiple meningeomas.

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  • (PMID = 16898259.001).
  • [ISSN] 0300-9009
  • [Journal-full-title] Acta neurologica Belgica
  • [ISO-abbreviation] Acta Neurol Belg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Belgium
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32. Cotticelli L, Romano M, Russo S, Borrelli M: Neurofibromatosis type 2: a case of ptosis. Graefes Arch Clin Exp Ophthalmol; 2007 Sep;245(9):1393-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The hallmark is bilateral vestibular schwannomas, which may not appear until after the second decade.
  • Other cranial nerve schwannomas occur as well.
  • In addition, there may be schwannomas of spinal nerve roots or peripheral nerves, as well as meningiomas, gliomas, and ependymomas.
  • The patient showed positive familiar history for NF (the father was affected), bilateral involvement of the acoustic nerves (schwannoma), multiple neurofibromas, and bilateral posterior subcapsular lens opacity.
  • Magnetic resonance imaging (MRI) showed bilateral acoustic neuromas in the left temporal region close to the cavernous sinus; since neurological examination and ocular motility problems had remained stationary over time, surgical correction of ptosis and strabismus was suggested.

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  • (PMID = 17347811.001).
  • [ISSN] 0721-832X
  • [Journal-full-title] Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv für klinische und experimentelle Ophthalmologie
  • [ISO-abbreviation] Graefes Arch. Clin. Exp. Ophthalmol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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33. Harris GJ, Plotkin SR, Maccollin M, Bhat S, Urban T, Lev MH, Slattery WH: Three-dimensional volumetrics for tracking vestibular schwannoma growth in neurofibromatosis type II. Neurosurgery; 2008 Jun;62(6):1314-9; discussion 1319-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Three-dimensional volumetrics for tracking vestibular schwannoma growth in neurofibromatosis type II.
  • OBJECTIVE: Vestibular schwannomas (VS) are common, benign, VIIIth cranial nerve tumors.
  • Treatment in patients with the genetic disorder neurofibromatosis type II (NF2) is complicated by their development of bilateral VS and risk of complete deafness.
  • [MeSH-major] Imaging, Three-Dimensional. Magnetic Resonance Imaging. Neurofibromatosis 2 / pathology. Neuroma, Acoustic / pathology


34. Poptodorov G, Gabrovski S, Kamenova M, Nacheva M, Iordanov I, Shirov T, Petkov R, Dŭbov D: [Primary ectopic intraosseous meningioma associated with multiple meningioma in a patient with central neurofibromatosis]. Khirurgiia (Sofiia); 2006;(4-5):64-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • She was followed up and five years after the initial symptoms asymptotic bilateral vestibular schwannomas were found at CT and MRI.
  • [MeSH-minor] Adult. Chromosome Deletion. Diagnosis, Differential. Exophthalmos / diagnosis. Exophthalmos / etiology. Female. Humans. Neuroma, Acoustic / complications. Neuroma, Acoustic / diagnosis. Neuroma, Acoustic / surgery. Optic Nerve Diseases / diagnosis. Optic Nerve Diseases / etiology

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  • (PMID = 18843923.001).
  • [ISSN] 0450-2167
  • [Journal-full-title] Khirurgii︠a︡
  • [ISO-abbreviation] Khirurgiia (Sofiia)
  • [Language] bul
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Bulgaria
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35. Aghi M, Kluwe L, Webster MT, Jacoby LB, Barker FG 2nd, Ojemann RG, Mautner VF, MacCollin M: Unilateral vestibular schwannoma with other neurofibromatosis type 2-related tumors: clinical and molecular study of a unique phenotype. J Neurosurg; 2006 Feb;104(2):201-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Unilateral vestibular schwannoma with other neurofibromatosis type 2-related tumors: clinical and molecular study of a unique phenotype.
  • OBJECT: Although the manifestations of neurofibromatosis Type 2 (NF2) vary, the hallmark is bilateral vestibular schwannomas (VSs).
  • [MeSH-major] Neurofibromatosis 2 / complications. Neuroma, Acoustic / genetics. Neuroma, Acoustic / pathology


36. Jeblaoui Y, Neji B, Haddad S, Mnif D, Hchicha S: [Difficulties of the management of head and neck neurofibromatosis]. Ann Chir Plast Esthet; 2007 Feb;52(1):43-50
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Neurofibromatosis type 2 (NF2) is also an autosomal dominantly inherited disease, but is ten times less frequent than the NF1 and is characterized by bilateral vestibular schwannomas (former acoustic neurinomas).

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  • (PMID = 17137698.001).
  • [ISSN] 0294-1260
  • [Journal-full-title] Annales de chirurgie plastique et esthétique
  • [ISO-abbreviation] Ann Chir Plast Esthet
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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37. Takahashi H, Nakao M, Kaga K: Accessing ampli-tonotopic organization of rat auditory cortex by microstimulation of cochlear nucleus. IEEE Trans Biomed Eng; 2005 Jul;52(7):1333-44

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Auditory brainstem implants (ABI) that electrically stimulate the surface of cochlear nucleus have been clinically used for the rehabilitation of deaf patients with bilateral vestibular schwannomas.
  • Our experimental results demonstrated that microstimulation of both the dorsal and ventral cochlear nucleus (DCN and VCN) could access the cortical ampli-tonotopic organization as acoustic stimuli did.

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  • (PMID = 16041997.001).
  • [ISSN] 0018-9294
  • [Journal-full-title] IEEE transactions on bio-medical engineering
  • [ISO-abbreviation] IEEE Trans Biomed Eng
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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