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Aplasia of the vestibule

MedGen UID:
868975
•Concept ID:
C4023389
•
Anatomical Abnormality
Synonym: Absent vestibule
 
HPO: HP:0011377

Definition

Complete absence of the vestibule of the inner ear. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAplasia of the vestibule

Professional guidelines

PubMed

The vestibular implant: Opinion statement on implantation criteria for research.
van de Berg R, Ramos A, van Rompaey V, Bisdorff A, Perez-Fornos A, Rubinstein JT, Phillips JO, Strupp M, Della Santina CC, Guinand N
J Vestib Res 2020;30(3):213-223. doi: 10.3233/VES-200701. PMID: 32651339Free PMC Article
Advances in the diagnosis and treatment of vestibular disorders: psychophysics and prosthetics.
Lewis RF
J Neurosci 2015 Apr 1;35(13):5089-96. doi: 10.1523/JNEUROSCI.3922-14.2015. PMID: 25834036Free PMC Article
SLC26A4 genotypes and phenotypes associated with enlargement of the vestibular aqueduct.
Ito T, Choi BY, King KA, Zalewski CK, Muskett J, Chattaraj P, Shawker T, Reynolds JC, Butman JA, Brewer CC, Wangemann P, Alper SL, Griffith AJ
Cell Physiol Biochem 2011;28(3):545-52. Epub 2011 Nov 18 doi: 10.1159/000335119. PMID: 22116369Free PMC Article

Recent clinical studies

Etiology

MRI can help differentiate Ménière's disease from other menieriform diseases.
Li J, Li L, Jin X, Hu N, Kong X, Wang L, Li X, Dou W, Sun L, Li C, Gong R
Sci Rep 2023 Dec 6;13(1):21527. doi: 10.1038/s41598-023-49066-5. PMID: 38057393Free PMC Article
Biomechanical analysis of the clinical characteristics of enlarged vestibular aqueduct syndrome with Mondini malformation.
Han JW, Wang L, Zhao H, Yang SM
Acta Otolaryngol 2020 Oct;140(10):813-817. Epub 2020 Jul 14 doi: 10.1080/00016489.2020.1780312. PMID: 32662706
New Imaging Findings of Incomplete Partition Type III Inner Ear Malformation and Literature Review.
Hong R, Du Q, Pan Y
AJNR Am J Neuroradiol 2020 Jun;41(6):1076-1080. Epub 2020 May 28 doi: 10.3174/ajnr.A6576. PMID: 32467182Free PMC Article
Anatomic Malformations of the Middle and Inner Ear in 22q11.2 Deletion Syndrome: Case Series and Literature Review.
Verheij E, Elden L, Crowley TB, Pameijer FA, Zackai EH, McDonald-McGinn DM, Thomeer HGXM
AJNR Am J Neuroradiol 2018 May;39(5):928-934. Epub 2018 Mar 15 doi: 10.3174/ajnr.A5588. PMID: 29545254Free PMC Article
Molecular epidemiology and functional assessment of novel allelic variants of SLC26A4 in non-syndromic hearing loss patients with enlarged vestibular aqueduct in China.
Yuan Y, Guo W, Tang J, Zhang G, Wang G, Han M, Zhang X, Yang S, He DZ, Dai P
PLoS One 2012;7(11):e49984. Epub 2012 Nov 21 doi: 10.1371/journal.pone.0049984. PMID: 23185506Free PMC Article

Diagnosis

Meniere's disease.
Nakashima T, Pyykkö I, Arroll MA, Casselbrant ML, Foster CA, Manzoor NF, Megerian CA, Naganawa S, Young YH
Nat Rev Dis Primers 2016 May 12;2:16028. doi: 10.1038/nrdp.2016.28. PMID: 27170253
Advances in the diagnosis and treatment of vestibular disorders: psychophysics and prosthetics.
Lewis RF
J Neurosci 2015 Apr 1;35(13):5089-96. doi: 10.1523/JNEUROSCI.3922-14.2015. PMID: 25834036Free PMC Article
KCNJ10 may not be a contributor to nonsyndromic enlargement of vestibular aqueduct (NSEVA) in Chinese subjects.
Zhao J, Yuan Y, Huang S, Huang B, Cheng J, Kang D, Wang G, Han D, Dai P
PLoS One 2014;9(11):e108134. Epub 2014 Nov 5 doi: 10.1371/journal.pone.0108134. PMID: 25372295Free PMC Article
Molecular epidemiology and functional assessment of novel allelic variants of SLC26A4 in non-syndromic hearing loss patients with enlarged vestibular aqueduct in China.
Yuan Y, Guo W, Tang J, Zhang G, Wang G, Han M, Zhang X, Yang S, He DZ, Dai P
PLoS One 2012;7(11):e49984. Epub 2012 Nov 21 doi: 10.1371/journal.pone.0049984. PMID: 23185506Free PMC Article
Testing otolith function.
Gresty MA, Bronstein AM
Br J Audiol 1992 Apr;26(2):125-36. doi: 10.3109/03005369209077880. PMID: 1628117

Therapy

Anatomic Malformations of the Middle and Inner Ear in 22q11.2 Deletion Syndrome: Case Series and Literature Review.
Verheij E, Elden L, Crowley TB, Pameijer FA, Zackai EH, McDonald-McGinn DM, Thomeer HGXM
AJNR Am J Neuroradiol 2018 May;39(5):928-934. Epub 2018 Mar 15 doi: 10.3174/ajnr.A5588. PMID: 29545254Free PMC Article
Meniere's disease.
Nakashima T, Pyykkö I, Arroll MA, Casselbrant ML, Foster CA, Manzoor NF, Megerian CA, Naganawa S, Young YH
Nat Rev Dis Primers 2016 May 12;2:16028. doi: 10.1038/nrdp.2016.28. PMID: 27170253
Angina bullosa haemorrhagica.
Shoor H, Mutalik S, Pai KM
BMJ Case Rep 2013 Dec 11;2013 doi: 10.1136/bcr-2013-200352. PMID: 24334011Free PMC Article
Migraine-associated vertigo: diagnosis and treatment.
Cha YH
Semin Neurol 2010 Apr;30(2):167-74. Epub 2010 Mar 29 doi: 10.1055/s-0030-1249225. PMID: 20352586Free PMC Article
Perilymph fistulae.
Dawes JD, Watson RT
Clin Otolaryngol Allied Sci 1979 Aug;4(4):291-302. doi: 10.1111/j.1365-2273.1979.tb01902.x. PMID: 314364

Prognosis

Bilateral vestibulopathy: the causes, diagnosis, and treatments.
Kim JS, Kim HJ
Curr Opin Neurol 2022 Feb 1;35(1):98-106. doi: 10.1097/WCO.0000000000001014. PMID: 34879018
Otopathologic Abnormalities in CHARGE Syndrome.
da Costa Monsanto R, Knoll RM, de Oliveira Penido N, Song G, Santos F, Paparella MM, Cureoglu S
Otolaryngol Head Neck Surg 2022 Feb;166(2):363-372. Epub 2021 Apr 20 doi: 10.1177/01945998211008911. PMID: 33874787
Evaluation of the utricular and saccular function using oVEMPs and cVEMPs in BPPV patients.
Xu H, Liang FY, Chen L, Song XC, Tong MC, Thong JF, Zhang QQ, Sun Y
J Otolaryngol Head Neck Surg 2016 Feb 9;45:12. doi: 10.1186/s40463-016-0125-7. PMID: 26857819Free PMC Article
Long-Term Results of TORP-Vibroplasty.
Gostian AO, Hüttenbrink KB, Luers JC, Anagiotos A, Beutner D
Otol Neurotol 2015 Jul;36(6):1054-60. doi: 10.1097/MAO.0000000000000737. PMID: 25756460
Vestibulo-ocular physiology underlying vestibular hypofunction.
Schubert MC, Minor LB
Phys Ther 2004 Apr;84(4):373-85. PMID: 15049730

Clinical prediction guides

MRI can help differentiate Ménière's disease from other menieriform diseases.
Li J, Li L, Jin X, Hu N, Kong X, Wang L, Li X, Dou W, Sun L, Li C, Gong R
Sci Rep 2023 Dec 6;13(1):21527. doi: 10.1038/s41598-023-49066-5. PMID: 38057393Free PMC Article
The Genetic Background of Hearing Loss in Patients with EVA and Cochlear Malformation.
Bałdyga N, Oziębło D, Gan N, Furmanek M, Leja ML, Skarżyński H, Ołdak M
Genes (Basel) 2023 Jan 28;14(2) doi: 10.3390/genes14020335. PMID: 36833263Free PMC Article
The clinical utility of the cervical vestibular-evoked myogenic potential (cVEMP) in university-level athletes with concussion.
Felipe L, Shelton JA
Neurol Sci 2021 Jul;42(7):2803-2809. Epub 2020 Nov 8 doi: 10.1007/s10072-020-04849-w. PMID: 33161456
Enlarged vestibular aqueduct and Mondini Malformation: audiological, clinical, radiologic and genetic features.
Forli F, Lazzerini F, Auletta G, Bruschini L, Berrettini S
Eur Arch Otorhinolaryngol 2021 Jul;278(7):2305-2312. Epub 2020 Sep 10 doi: 10.1007/s00405-020-06333-9. PMID: 32910226Free PMC Article
Validation of the Normalized Laryngeal Constriction Ratio in Normal and Disordered Swallowing.
Miller M, Vose A, Rivet A, Smith-Sherry M, Humbert I
Laryngoscope 2020 Apr;130(4):E190-E198. Epub 2019 Aug 26 doi: 10.1002/lary.28161. PMID: 31448826

Recent systematic reviews

Airway anomalies in patients with 22q11.2 deletion syndrome: A scoping review.
Hankey PB, Ghulmiyyah J, Yeh HW, Tracy M, Arganbright J
Int J Pediatr Otorhinolaryngol 2022 Dec;163:111373. Epub 2022 Nov 2 doi: 10.1016/j.ijporl.2022.111373. PMID: 36335759
Systematic Review on Vestibular Symptoms in Patients With Enlarged Vestibular Aqueducts.
Stahl MC, Otteson T
Laryngoscope 2022 Apr;132(4):873-880. Epub 2021 Aug 16 doi: 10.1002/lary.29819. PMID: 34397103
Hearing Loss in Enlarged Vestibular Aqueduct: A Prognostic Factor Systematic Review of the Literature.
Saeed HS, Kenth J, Black G, Saeed SR, Stivaros S, Bruce IA
Otol Neurotol 2021 Jan;42(1):99-107. doi: 10.1097/MAO.0000000000002843. PMID: 33026783
Progressive Hearing Loss and Head Trauma in Enlarged Vestibular Aqueduct: A Systematic Review and Meta-analysis.
Alemi AS, Chan DK
Otolaryngol Head Neck Surg 2015 Oct;153(4):512-7. Epub 2015 Jul 23 doi: 10.1177/0194599815596343. PMID: 26204902
Auditory and vestibular dysfunctions in systemic sclerosis: literature review.
Rabelo MB, Corona AP
Codas 2014 Sep-Oct;26(5):337-42. doi: 10.1590/2317-1782/20140201475. PMID: 25388064

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