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Vestibulocochlear dysfunction, progressive

MedGen UID:
419730
•Concept ID:
C2931176
•
Disease or Syndrome
Synonym: Familial progressive vestibulocochlear dysfunction
 
Monarch Initiative: MONDO:0008655
OMIM®: 193005

Clinical features

From HPO
Tinnitus
MedGen UID:
52760
•Concept ID:
C0040264
•
Disease or Syndrome
Tinnitus is an auditory perception that can be described as the experience of sound, in the ear or in the head, in the absence of external acoustic stimulation.
See: Feature record | Search on this feature
Progressive hearing impairment
MedGen UID:
331224
•Concept ID:
C1842138
•
Finding
A progressive form of hearing impairment.
See: Feature record | Search on this feature
Vestibular areflexia
MedGen UID:
863489
•Concept ID:
C4015052
•
Finding
Vestibular areflexia can be measured as the absence of the caloric nystagmus response in electronystagmography.
See: Feature record | Search on this feature
Abnormality of the nervous system
MedGen UID:
105425
•Concept ID:
C0497552
•
Congenital Abnormality
An abnormality of the nervous system.
See: Feature record | Search on this feature
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Professional guidelines

PubMed

Management of vestibular schwannomas (acoustic neuromas): auditory and facial nerve function after resection of 120 vestibular schwannomas in patients with neurofibromatosis 2.
Samii M, Matthies C, Tatagiba M
Neurosurgery 1997 Apr;40(4):696-705; discussion 705-6. doi: 10.1097/00006123-199704000-00007. PMID: 9092842
The surgical treatment of vertigo. The essentials of patient selection and long-term results.
Smyth GD, Hassard TH, Kerr AG
Am J Otol 1981 Jan;2(3):179-87. PMID: 7282887

Recent clinical studies

Etiology

Paraneoplastic cochleovestibulopathy: clinical presentations, oncological and serological associations.
Hammami MB, Eggers SDZ, Madhavan A, Montalvo MJ, Pittock SJ, Dubey D
J Neurol Neurosurg Psychiatry 2021 Nov;92(11):1181-1185. Epub 2021 Jul 20 doi: 10.1136/jnnp-2021-326775. PMID: 34285066
Long-term outcomes of patients with Streptococcus suis infection in Viet Nam: A case-control study.
Huong VTL, Long HB, Kinh NV, Ngan TTD, Dung VTV, Nadjm B, van Doorn HR, Hoa NT, Horby P, Wertheim HFL
J Infect 2018 Feb;76(2):159-167. Epub 2017 Sep 29 doi: 10.1016/j.jinf.2017.09.019. PMID: 28970042Free PMC Article
Long-term tumor control and cranial nerve outcomes following γ knife surgery for larger-volume vestibular schwannomas.
Milligan BD, Pollock BE, Foote RL, Link MJ
J Neurosurg 2012 Mar;116(3):598-604. Epub 2011 Dec 16 doi: 10.3171/2011.11.JNS11811. PMID: 22175724
Autoimmune vestibulo-cochlear disorders.
Rahman MU, Poe DS, Choi HK
Curr Opin Rheumatol 2001 May;13(3):184-9. doi: 10.1097/00002281-200105000-00006. PMID: 11333346
Microvascular decompression for cochlear symptoms.
Okamura T, Kurokawa Y, Ikeda N, Abiko S, Ideguchi M, Watanabe K, Kido T
J Neurosurg 2000 Sep;93(3):421-6. doi: 10.3171/jns.2000.93.3.0421. PMID: 10969939

Diagnosis

Paediatric Cavernous Malformation of the Trigeminal Nerve: Case Report and Review of the Literature.
Thompson D, Zammit A, Yuen J, Hand C, Likeman M, Singleton W, Nelson R, Fellows G
Pediatr Neurosurg 2022;57(3):207-212. Epub 2022 Apr 8 doi: 10.1159/000524522. PMID: 35398849
Paraneoplastic cochleovestibulopathy: clinical presentations, oncological and serological associations.
Hammami MB, Eggers SDZ, Madhavan A, Montalvo MJ, Pittock SJ, Dubey D
J Neurol Neurosurg Psychiatry 2021 Nov;92(11):1181-1185. Epub 2021 Jul 20 doi: 10.1136/jnnp-2021-326775. PMID: 34285066
Progressive cranial nerve involvement and grading of facial paralysis in gelsolin amyloidosis.
Pihlamaa T, Salmi T, Suominen S, Kiuru-Enari S
Muscle Nerve 2016 May;53(5):762-9. Epub 2016 Feb 26 doi: 10.1002/mus.24922. PMID: 26422119
Audiovestibular loss in anterior inferior cerebellar artery territory infarction: a window to early detection?
Lee H
J Neurol Sci 2012 Feb 15;313(1-2):153-9. Epub 2011 Oct 13 doi: 10.1016/j.jns.2011.08.039. PMID: 21996273
Vascular compression syndrome of the eighth nerve. Clinical correlations and surgical findings.
Møller MB, Møller AR
Neurol Clin 1990 May;8(2):421-39. PMID: 2193220

Therapy

Phase II study of mTORC1 inhibition by everolimus in neurofibromatosis type 2 patients with growing vestibular schwannomas.
Goutagny S, Raymond E, Esposito-Farese M, Trunet S, Mawrin C, Bernardeschi D, Larroque B, Sterkers O, Giovannini M, Kalamarides M
J Neurooncol 2015 Apr;122(2):313-20. Epub 2015 Jan 8 doi: 10.1007/s11060-014-1710-0. PMID: 25567352
Autoimmune vestibulo-cochlear disorders.
Rahman MU, Poe DS, Choi HK
Curr Opin Rheumatol 2001 May;13(3):184-9. doi: 10.1097/00002281-200105000-00006. PMID: 11333346
Microvascular decompression for cochlear symptoms.
Okamura T, Kurokawa Y, Ikeda N, Abiko S, Ideguchi M, Watanabe K, Kido T
J Neurosurg 2000 Sep;93(3):421-6. doi: 10.3171/jns.2000.93.3.0421. PMID: 10969939
Physiopathology of peripheral non-Menière's vestibular disorders.
Tran Ba Huy P
Acta Otolaryngol Suppl 1994;513:5-10. doi: 10.3109/00016489409127320. PMID: 8191889
Neural correlates of sensorineural hearing loss.
Salvi RJ, Henderson D, Hamernik R, Ahroon WA
Ear Hear 1983 May-Jun;4(3):115-29. doi: 10.1097/00003446-198305000-00001. PMID: 6345246

Prognosis

Paediatric Cavernous Malformation of the Trigeminal Nerve: Case Report and Review of the Literature.
Thompson D, Zammit A, Yuen J, Hand C, Likeman M, Singleton W, Nelson R, Fellows G
Pediatr Neurosurg 2022;57(3):207-212. Epub 2022 Apr 8 doi: 10.1159/000524522. PMID: 35398849
Paraneoplastic cochleovestibulopathy: clinical presentations, oncological and serological associations.
Hammami MB, Eggers SDZ, Madhavan A, Montalvo MJ, Pittock SJ, Dubey D
J Neurol Neurosurg Psychiatry 2021 Nov;92(11):1181-1185. Epub 2021 Jul 20 doi: 10.1136/jnnp-2021-326775. PMID: 34285066
Neurophysiologic, audiometric and vestibular function tests in patients with hyperostosis cranialis interna.
Waterval JJ, Bischoff MP, Stokroos RJ, Anteunis LJ, Hilkman DM, Kingma H, Manni JJ
Clin Neurol Neurosurg 2013 Sep;115(9):1701-8. Epub 2013 Apr 23 doi: 10.1016/j.clineuro.2013.03.008. PMID: 23622937
Long-term tumor control and cranial nerve outcomes following γ knife surgery for larger-volume vestibular schwannomas.
Milligan BD, Pollock BE, Foote RL, Link MJ
J Neurosurg 2012 Mar;116(3):598-604. Epub 2011 Dec 16 doi: 10.3171/2011.11.JNS11811. PMID: 22175724
Management of seventh and eighth nerve involvement by cerebellopontine angle tumors.
Samii M, Turel KE, Penkert G
Clin Neurosurg 1985;32:242-72. PMID: 3933876

Clinical prediction guides

Predictors of volumetric growth and auditory deterioration in vestibular schwannomas followed in a wait and scan policy.
van de Langenberg R, de Bondt BJ, Nelemans PJ, Dohmen AJ, Baumert BG, Stokroos RJ
Otol Neurotol 2011 Feb;32(2):338-44. doi: 10.1097/MAO.0b013e3182040d9f. PMID: 21150682
Transient infantile auditory neuropathy and its clinical implications.
Psarommatis I, Riga M, Douros K, Koltsidopoulos P, Douniadakis D, Kapetanakis I, Apostolopoulos N
Int J Pediatr Otorhinolaryngol 2006 Sep;70(9):1629-37. doi: 10.1016/j.ijporl.2006.05.005. PMID: 16769129
Microvascular decompression for cochlear symptoms.
Okamura T, Kurokawa Y, Ikeda N, Abiko S, Ideguchi M, Watanabe K, Kido T
J Neurosurg 2000 Sep;93(3):421-6. doi: 10.3171/jns.2000.93.3.0421. PMID: 10969939
Physiopathology of peripheral non-Menière's vestibular disorders.
Tran Ba Huy P
Acta Otolaryngol Suppl 1994;513:5-10. doi: 10.3109/00016489409127320. PMID: 8191889
Management of seventh and eighth nerve involvement by cerebellopontine angle tumors.
Samii M, Turel KE, Penkert G
Clin Neurosurg 1985;32:242-72. PMID: 3933876

Recent systematic reviews

Different clinical presentation of intralabyrinthine schwannomas - a systematic review.
Elias TGA, Perez Neto A, Zica ATS, Antunes ML, Penido NO
Braz J Otorhinolaryngol 2019 Jan-Feb;85(1):111-120. Epub 2018 Jun 21 doi: 10.1016/j.bjorl.2018.05.007. PMID: 29980446Free PMC Article

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