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Bilateral vestibular schwannoma(ACN)

MedGen UID:
209678
Concept ID:
C1136042
Neoplastic Process
Synonyms: Acoustic Neurinoma, Bilateral; Acoustic Neurinomas, Bilateral; Bilateral Acoustic Neurinoma; Bilateral Acoustic Neurinomas; Neurinoma, Bilateral Acoustic; Neurinomas, Bilateral Acoustic; Neuroma, Acoustic, Bilateral
 
HPO: HP:0009589
OMIM®: 101000

Definition

A bilateral vestibular Schwannoma (acoustic neurinoma). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBilateral vestibular schwannoma

Conditions with this feature

Neurofibromatosis, type 2
MedGen UID:
18014
Concept ID:
C0027832
Neoplastic Process
Neurofibromatosis 2 (NF2) is characterized by bilateral vestibular schwannomas with associated symptoms of tinnitus, hearing loss, and balance dysfunction. The average age of onset is 18 to 24 years. Almost all affected individuals develop bilateral vestibular schwannomas by age 30 years. Affected individuals may also develop schwannomas of other cranial and peripheral nerves, meningiomas, ependymomas, and, very rarely, astrocytomas. Because NF2 is considered an adult-onset disease, it may be underrecognized in children, in whom skin tumors and ocular findings (retinal hamartoma, thickened optic nerves, cortical wedge cataracts, third cranial nerve palsy) may be the first manifestations. Mononeuropathy that occurs in childhood is an increasingly recognized finding; it frequently presents as a persistent facial palsy or hand/foot drop.
Neurofibromatosis, type III, mixed central and peripheral
MedGen UID:
419422
Concept ID:
C2931480
Disease or Syndrome

Professional guidelines

PubMed

Evans DG, King AT, Bowers NL, Tobi S, Wallace AJ, Perry M, Anup R, Lloyd SKL, Rutherford SA, Hammerbeck-Ward C, Pathmanaban ON, Stapleton E, Freeman SR, Kellett M, Halliday D, Parry A, Gair JJ, Axon P, Laitt R, Thomas O, Afridi S, Ferner RE, Harkness EF, Smith MJ; English Specialist NF2 Research Group
Genet Med 2019 Jul;21(7):1525-1533. Epub 2018 Dec 7 doi: 10.1038/s41436-018-0384-y. PMID: 30523344
Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, Della Santina CC, Kingma H
J Vestib Res 2017;27(4):177-189. doi: 10.3233/VES-170619. PMID: 29081426Free PMC Article

Recent clinical studies

Etiology

Abeshi A, Ferri GG
J Int Adv Otol 2023 Jun;19(3):263-265. doi: 10.5152/iao.2023.22922. PMID: 37272647Free PMC Article
Li P, Li S, Wang Y, Zhao F, Wang Z, Wang X, Wang B, Yang Z, Liu P
Clin Neurol Neurosurg 2021 Jan;200:106365. Epub 2020 Nov 11 doi: 10.1016/j.clineuro.2020.106365. PMID: 33243701
Sebök M, van Niftrik CHB, Bozinov O
World Neurosurg 2018 May;113:195-197. Epub 2018 Feb 21 doi: 10.1016/j.wneu.2018.02.075. PMID: 29477000
Evans DGR, Salvador H, Chang VY, Erez A, Voss SD, Druker H, Scott HS, Tabori U
Clin Cancer Res 2017 Jun 15;23(12):e54-e61. doi: 10.1158/1078-0432.CCR-17-0590. PMID: 28620005
Fisher LM, Doherty JK, Lev MH, Slattery WH
Otol Neurotol 2009 Sep;30(6):835-41. doi: 10.1097/MAO.0b013e3181b2364c. PMID: 19704365

Diagnosis

Abeshi A, Ferri GG
J Int Adv Otol 2023 Jun;19(3):263-265. doi: 10.5152/iao.2023.22922. PMID: 37272647Free PMC Article
Goshtasbi K, Abouzari M, Yasaka TM, Soltanzadeh-Zarandi S, Sarna B, Lin HW, Djalilian HR
Otol Neurotol 2021 Apr 1;42(4):592-597. doi: 10.1097/MAO.0000000000002984. PMID: 33351555Free PMC Article
Elsayed M, Hochet B, Torres R, Sterkers O, Nguyen Y, Lahlou G, Kalamarides M
Laryngoscope 2021 Jan;131(1):E250-E254. Epub 2020 Feb 17 doi: 10.1002/lary.28571. PMID: 32065398
Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, Della Santina CC, Kingma H
J Vestib Res 2017;27(4):177-189. doi: 10.3233/VES-170619. PMID: 29081426Free PMC Article
Strupp M, Feil K, Dieterich M, Brandt T
Handb Clin Neurol 2016;137:235-40. doi: 10.1016/B978-0-444-63437-5.00017-0. PMID: 27638075

Therapy

Shepard TH, Tucci DL, Grant GA, Kaylie DM
Otol Neurotol 2012 Aug;33(6):1066-70. doi: 10.1097/MAO.0b013e3182595325. PMID: 22710553
Lustig LR, Yeagle J, Driscoll CL, Blevins N, Francis H, Niparko JK
Otol Neurotol 2006 Jun;27(4):512-8. doi: 10.1097/01.mao.0000217351.86925.51. PMID: 16791043
Suh JH, Barnett GH, Sohn JW, Kupelian PA, Cohen BH
Int J Cancer 2000 Jun 20;90(3):145-51. doi: 10.1002/1097-0215(20000620)90:3<145::aid-ijc4>3.0.co;2-v. PMID: 10900426

Prognosis

Li P, Li S, Wang Y, Zhao F, Wang Z, Wang X, Wang B, Yang Z, Liu P
Clin Neurol Neurosurg 2021 Jan;200:106365. Epub 2020 Nov 11 doi: 10.1016/j.clineuro.2020.106365. PMID: 33243701
Sebök M, van Niftrik CHB, Bozinov O
World Neurosurg 2018 May;113:195-197. Epub 2018 Feb 21 doi: 10.1016/j.wneu.2018.02.075. PMID: 29477000
Evans DGR, Salvador H, Chang VY, Erez A, Voss SD, Druker H, Scott HS, Tabori U
Clin Cancer Res 2017 Jun 15;23(12):e54-e61. doi: 10.1158/1078-0432.CCR-17-0590. PMID: 28620005
Strupp M, Feil K, Dieterich M, Brandt T
Handb Clin Neurol 2016;137:235-40. doi: 10.1016/B978-0-444-63437-5.00017-0. PMID: 27638075
Fisher LM, Doherty JK, Lev MH, Slattery WH
Otol Neurotol 2009 Sep;30(6):835-41. doi: 10.1097/MAO.0b013e3181b2364c. PMID: 19704365

Clinical prediction guides

Goshtasbi K, Abouzari M, Yasaka TM, Soltanzadeh-Zarandi S, Sarna B, Lin HW, Djalilian HR
Otol Neurotol 2021 Apr 1;42(4):592-597. doi: 10.1097/MAO.0000000000002984. PMID: 33351555Free PMC Article
Li P, Li S, Wang Y, Zhao F, Wang Z, Wang X, Wang B, Yang Z, Liu P
Clin Neurol Neurosurg 2021 Jan;200:106365. Epub 2020 Nov 11 doi: 10.1016/j.clineuro.2020.106365. PMID: 33243701
Evans DGR, Salvador H, Chang VY, Erez A, Voss SD, Druker H, Scott HS, Tabori U
Clin Cancer Res 2017 Jun 15;23(12):e54-e61. doi: 10.1158/1078-0432.CCR-17-0590. PMID: 28620005
Fisher LM, Doherty JK, Lev MH, Slattery WH
Otol Neurotol 2009 Sep;30(6):835-41. doi: 10.1097/MAO.0b013e3181b2364c. PMID: 19704365
Colletti V
Adv Otorhinolaryngol 2006;64:167-185. doi: 10.1159/000094651. PMID: 16891842

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