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Topic: Acoustic neurofibromatosis


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  Acoustic neuroma - Wikipedia, the free encyclopedia
Acoustic neuroma (or Vestibular Schwannoma) is a benign primary intracranial tumor of the myelin forming cells called "Schwann cells" (Schwannoma) of the 8th cranial nerve --- also known as the acoustic nerve, (or more properly the vestibulocochlear nerve).
Acoustic neuromas may occur sporadically, or in some cases occur as part of von Recklinhausen neurofibromatosis, in which case it may take on one of two forms.
Earliest symptoms of acoustic neuromas include unilateral sensorineural hearing loss/deafness, disturbed sense of balance and altered gait, vertigo with associated nausea and vomiting, and pressure in the ear, all of which can be attributed to the disruption of normal vestibulocochlear nerve function.
en.wikipedia.org /wiki/Acoustic_neuroma   (1490 words)

  
 AANS.org | Education and Meetings | AANS Scientific Journals | Neurosurgical Focus
The tumor was a typical acoustic neuroma with fibrous capsule and a relatively vascular interior displacing the fifth cranial nerve upward, the seventh and eighth cranial nerves forward, and the ninth, 10th and 11th cranial nerves downward.
The tumor was a 4-cm fibrous, vascular, yellow-tan typical acoustic neuroma arising from the superior vestibular nerve, displacing the fifth cranial nerve anteriorly, the seventh and eighth cranial nerves anteriorly and inferiorly, and the ninth, 10th, and 11th cranial nerves downward.
Attention was again turned back to the acoustic neuroma, and as the coring continued upward a plane was found in which a second tumor, obviously arising from a different nerve or a different fascicle, had its capsule in dense contact with the inferior tumor.
www.aans.org /education/journal/neurosurgical/mar98/4-3-1.asp?ShowMenu=false&ShowPrint=false   (21172 words)

  
 Neurofibromatosis - Wikipedia, the free encyclopedia
Neurofibromatosis type I (was known as Von Recklinghausen disease after Friedrich Daniel von Recklinghausen).
Neurofibromatosis type 2 is due to mutation on chromosome 22q, the gene product is Merlin, a cytoskeletal protein.
Neurofibromatosis is considered a member of the neurocutaneous syndromes (phakomatoses).
en.wikipedia.org /wiki/Neurofibromatosis   (707 words)

  
 Neurofibromatosis 2 (bilateral acoustic schwannomas). What is it? A review of literature and an update. Desouza CE, ...
Ojemann and Martuza[36] have noted that in unilateral acoustic schwannomas the 7th cranial nerve lies on the periphery of the tumour and can be preserved, whereas in bilateral acoustic schwannomas the 7th and 8th cranial nerves are surrounded by multilobulated tumor masses between the multiple lobules.
Gene for von Recklinghausen neurofibromatosis is in the pericentromeric region of chromosome 17.
Contralateral auditory brainstem response abnormalities in acoustic neuroma.
www.jpgmonline.com /article.asp?issn=0022-3859;year=1992;volume=38;issue=1;spage=27;epage=31;aulast=Desouza   (2975 words)

  
 Acoustic Neuroma Association of New Jersey :: Information about Acoustic Neuroma   (Site not responding. Last check: 2007-10-20)
An acoustic neuroma, also called vestibular schwannoma is a benign tumor that originates on the balance/hearing nerve that passes from the inner ear to the brain.
Acoustic neuromas are actually quite rare, accounting for less than 5% of all primary brain tumors, and their exact cause is unknown.
If an acoustic neuroma is small and acute symptoms are not present, a first option is to “wait-and-watch” by monitoring the growth characteristics of the tumor with periodic MRIs (Magnetic Resonance Imaging).
www.ananj.org /info.shtm   (557 words)

  
 Neurofibromatosis   (Site not responding. Last check: 2007-10-20)
Neurofibromatosis (NF) is a genetic disorder that is inherited in an autosomal dominant pattern.
In 1987, at an international neurofibromatosis symposium, a group of investigators pooled the results of their genetic analysis studies, and determined that the NF1 gene was located near the long arm of chromosome 17.
Neurofibromatosis is a single gene disorder meaning that the disorder results from a mutation in a single gene (Bullock, 1996).
www.missouristate.edu /nursing/geneticsinnursing/neurofibromatosis.htm   (12175 words)

  
 Neurofibromatosis
Neurofibromatosis (NF) is a condition that causes tumors to grow on nerve tissue, producing skin and bone abnormalities.
Neurofibromatosis is a neurocutaneous syndrome, it's passed down through the parents' genes, and it affects the brain, spinal cord, nerves, and skin.
Neurofibromatosis can also be the result of a spontaneous change (mutation) in the genetic material of the sperm or egg at conception in families with no previous history of the disease.
kidshealth.org /parent/system/ill/nf.html   (1395 words)

  
 Statements of Principles — Acoustic neuroma — balance of probability
"Acoustic neuroma" means a sporadic, progressively enlarging, benign tumour, that arises within the internal auditory canal from Schwann cells of the vestibular division of the eighth cranial nerve and is also known as acoustic Schwannoma or vestibular Schwannoma, attracting ICD code 225.1.
The diagnosis of acoustic neuroma can be accepted by the Repatriation Commission when it is confirmed by a general medical practitioner or specialist as the final diagnosis or on advice by a Departmental Medical Officer.
The acoustic neuroma is also known as an acoustic Schwannoma, and arises from the myelin-forming Schwann cells which form a sheath or coating for the acoustic nerve fibres.
www.dva.gov.au /pensions/statemnt/b057.htm   (1175 words)

  
 Acoustic neuroma - MayoClinic.com   (Site not responding. Last check: 2007-10-20)
An acoustic neuroma is a noncancerous (benign) tumor of the eighth cranial nerve.
But these tumors often are associated with neurofibromatosis 2, an inherited disorder characterized by tumors of the cranial and spinal nerves.
Acoustic neuromas are usually diagnosed after age 30.
www.mayoclinic.com /health/acoustic-neuroma/HQ00182   (240 words)

  
 AANS.org | Education and Meetings | AANS Scientific Journals | Neurosurgical Focus
Neurofibromatosis type 2: growth stimulation of mixed acoustic schwannoma by concurrent adjacent meningioma: possible role of growth factors
During surgery, the acoustic schwannoma was firmly adherent to both meningioma and tentorium.
The association of meningioma tissue with and within acoustic schwannomas has been known since the writings of Harvey Cushing.[10] Cushing first drew attention to the fact that, in some instances, the microscopic structure of an acoustic schwannoma displays a conspicuously whorled pattern strongly resembling that of certain meningiomas.
www.aans.org /education/journal/neurosurgical/apr98/4-4-cp.asp?ShowMenu=false&ShowPrint=false   (3159 words)

  
 Zebra Card FA-003: family history of drowning
Central neurofibromatosis with bilateral acoustic neuroma: genetic, clinical and biochemical distinctions from peripheral neurofibromatosis.
Neurofibromatosis 2 (NF2): clinical characteristics of 63 affected individuals and clinical evidence for heterogeneity.
Neurofibromatosis type 2 appears to be a genetically homogeneous disease.
www.acponline.org /zebra/references/fa/FA-003.html   (251 words)

  
 Neurofibromatosis in Children - Keep Kids Healthy
Neurofibromatosis type 1 (also called von Recklinghaus disease) is a condition that affects the skin, eyes, kidneys, and many other parts of the body.
Neurofibromatosis I is a hereditary condition usually passed from parent to child (autosomal dominant), but in about half the people with Neurofibromatosis 1, there is no history of the disease in the family.
Neurofibromatosis Type 2 is a rare, hereditary disorder that is similar to Neurofibromatosis I in that it involves tumors along the nerves.
www.keepkidshealthy.com /welcome/conditions/neurofibromatosis.html   (1381 words)

  
 Neurofibromatosis Type 1 (NF-1)
Neurofibromatosis Type 1 (NF-1), also called von Recklinghausen's Disease, is a rare genetic disorder characterized by the development of multiple noncancerous (benign) tumors of nerves and skin (neurofibromas) and areas of abnormally decreased or increased coloration (hypo- or hyperpigmentation) of the skin.
The first sign of Neurofibromatosis Type 1 is usually the appearance of multiple brown spots on the skin (cafe-au-lait) and freckling under the arms (axillary) or in the groin area (inguinal).
Neurofibromatosis Type 1 is estimated to occur in 1 in 4,000 births.
hw.healthdialog.com /kbase/nord/nord3.htm   (3012 words)

  
 Medical Dictionary: Neurofibromatosis type 2 - WrongDiagnosis.com
Neurofibromatosis type 2: An autosomal dominant disorder characterized by a high incidence of bilateral acoustic neuromas as well as schwannomas (NEURILEMMOMA) of other cranial and peripheral nerves, and other benign intracranial tumors including meningiomas, ependymomas, spinal neurofibromas, and gliomas.
Neurofibromatosis type 2 is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH).
This means that Neurofibromatosis type 2, or a subtype of Neurofibromatosis type 2, affects less than 200,000 people in the US population.
www.wrongdiagnosis.com /medical/neurofibromatosis_type_2.htm   (361 words)

  
 Bone Cancer Resources and Information | OncoLink Ewing's Sarcoma   (Site not responding. Last check: 2007-10-20)
Neurofibromatosis (NF) is one of the most frequent and clinically important Mendelian disorders in man, with an incidence of 1 in 3,000.
Neurofibromatosis is an important disorder for the study of the psychodynamic processes that families experience in dealing with uncertainty.
Neurofibromatosis has an extremely variable expressivity, making the identification of affected individuals difficult and allowing for some reduction in the risk figures; although there is a 50 percent risk of a child inheriting the mutant gene, only one-fourth to one-third of these will experience serious consequences.
www.oncolink.upenn.edu /types/article.cfm?c=1&s=2&ss=11&id=7931   (6341 words)

  
 The National Institutes of Health (NIH) Consensus Development Program: Neurofibromatosis
Neurofibromatosis 2 or NF-2 (previously known as bilateral acoustic neurofibromatosis or central neurofibromatosis) is an autosomal dominant disorder occurring in about 1 in 50,000 individuals.
Other forms of neurofibromatosis may exist, but their precise classification is not possible at the present time.
This NIH Consensus Development Conference on Neurofibromatosis was convened by the National Institute of Neurological and Communicative Disorders and Stroke, the National Cancer Institute, and the National Institutes of Health Office of Medical Applications of Research, on July 13-15, 1987, to address issues concerning the diagnosis and management of the neurofibromatoses.
consensus.nih.gov /1987/1987Neurofibramatosis064html.htm   (3459 words)

  
 Neurofibromatosis Symptoms
neu·ro·fi·bro·ma·to·sis: a disorder inherited as an autosomal dominant trait and characterized by brown spots on the skin, neurofibromas of peripheral nerves, and deformities of subcutaneous tissues and bone
Neurofibromatosis (NF), also called Recklinghausen's disease and von Recklinghausen's disease is a genetic neurological disorder that can affect the brain, spinal cord, nerves and skin.
Neurofibromatosis has been classified into two distinct types: neurofibromatosis type 1 (NF1) and NF2.
www.symptoms-4u.com /neurofibromatosis.htm   (598 words)

  
 Neurofibromatosis Type 2 (NF-2) - SJMMC, Ann Arbor, Michigan MI   (Site not responding. Last check: 2007-10-20)
Neurofibromatosis Type 2 (NF-2) is a rare genetic disorder that is primarily characterized by benign (noncancerous) tumors of the nerves that transmit sound impulses from the inner ears to the brain (bilateral acoustic neuromas vestibular schwannomas).
Depending on the exact location and size of the acoustic neuromas/vestibular schwannomas, such findings may include disturbances of balance and walking (gait); dizziness; headache; facial weakness, numbness, or pain; ringing in the ears (tinnitus); and/or progressive hearing loss.
The term "Neurofibromatosis" is sometimes also used to describe a second, distinct form of NF known as Neurofibromatosis Type I (NF-1).
www.sjmercyhealth.org /14766.cfm   (676 words)

  
 Neurofibromatosis - CancerIndex
Neurofibromatosis 2 (NF2) is much rarer, this is characterised by multiple tumours on the nerves of the brain and spine, and can affect hearing.
International Neurofibromatosis Association The Association aims to promote awareness of neurofibromatosis, encourage collaborations among scientists and NF organisations throughout the world.
Neurofibromatosis, type 2 and the NF2 gene (National Center for Biotechnology Information) an overview of neurofibromatosis and the NF2 oncogene with links to other gene databases.
www.cancerindex.org /clinks6c.htm   (323 words)

  
 NIH Guide: NEUROFIBROMATOSIS
This program announcement, Neurofibromatosis, is related to the priority areas of chronic disabling conditions and cancer.
It requires familial markers (not available for all families or for sporadic cases), and it cannot predict the rate or degree of progression of the disorder in a given diagnosed individual.
Neurofibromatosis 2 (NF2), or bilateral acoustic neurofibromatosis, is an autosomal dominant disorder associated with vestibular schwannomas and other schwannomas, meningiomas, ependymomas, gliomas, and posterior subcapsular cataracts.
grants.nih.gov /grants/guide/pa-files/PA-92-047.html   (1295 words)

  
 MedPix™ 1451: Bilateral acoustic schwannomas, Neurofibromatosis type 2 - Medical Image Database and Atlas   (Site not responding. Last check: 2007-10-20)
The presence of bilateral acoustic schwannomas establishes the diagnosis of neurofibromatosis-2, the central form of neurofibromatosis.
By far the most common of these is the acoustic schwannoma, which arises from the vestibular division of the 8th nerve.
One fourth of intracranial meningiomas in the pediatric population occur in patients with neurofibromatosis.
rad.usuhs.mil /medpix/radpix.html?mode=single&comebackto=mode=geo_browse&recnum=1451   (570 words)

  
 Neurofibromatosis type 2 - Genetics Home Reference
Neurofibromatosis type 2 is a disorder in which noncancerous tumors called vestibular schwannomas or acoustic neuromas develop along nerves in the inner ear.
Neurofibromatosis type 2 is considered to have an autosomal dominant pattern of inheritance.
The remaining cases are the result of new mutations in the NF2 gene, and occur in people with no history of the disorder in their family.
ghr.nlm.nih.gov /condition=neurofibromatosis2   (821 words)

  
 Neurofibromatosis
Neurofibromatosis is a genetic disorder (can be passed from parent to child).
Neurofibromatosis (NF) affects development and growth of nerve cell (neural) tissues.
Also known as bilateral acoustic neurofibromatosis, NF2 mostly affects the central nervous system, causing tumors of the brain and spinal cord.
orthoinfo.aaos.org /fact/thr_report.cfm?Thread_ID=402&topcategory=Pediatrics   (1199 words)

  
 ASM 15(4); Case Report: Cranio-Orbital Anomalies
Zain Alabedeen Jamjoom, MD; Willem Selen, MD Neurofibromatosis is a hereditary autosomal dominant disorder which is classified into two main types, referred to as von Recklinghausen neurofibromatosis (VRN) and bilateral acoustic schwannoma.
In neurofibromatosis, the sella turcica may be enlarged and deepened, suggesting an intrasellar expanding lesion.
It is recognized that in neurofibromatosis patients, the sellar changes (and possibly the empty sella) can result from excavation and enlargement of the chiasmatic sulcus by neurofibromatosis tissue.
www.kfshrc.edu.sa /annals/154/94232/94232.html   (826 words)

  
 Neurofibromatosis 2
Neurofibromatosis 2 (NF2) is characterized by bilateral vestibular schwannomas with associated symptoms of tinnitus, hearing loss, and balance dysfunction.
The term "neurofibromatosis" is a misnomer as the primary tumor types in NF2 are schwannoma and meningioma.
neurofibromatosis type 1, leading to multiple instances in which individuals with NF2 were included in series of individuals with NF1.
www.geneclinics.org /profiles/nf2/details.html   (5363 words)

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