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Hypoplasia of the vestibule of the inner ear

MedGen UID:
868974
•Concept ID:
C4023388
•
Anatomical Abnormality
HPO: HP:0011378

Definition

Underdevelopment of the vestibule of the inner ear. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHypoplasia of the vestibule of the inner ear

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

Congenital Inner Ear Abnormalities and COVID-19-Related Ear Infections.
Maharaj S, Hari K
Ear Nose Throat J 2022 Aug;101(7):NP308-NP310. Epub 2020 Oct 23 doi: 10.1177/0145561320968934. PMID: 33095653Free PMC Article
Should children with an enlarged vestibular aqueduct be restricted from playing contact sports?
Brodsky JR, Choi SS
Laryngoscope 2018 Oct;128(10):2219-2220. Epub 2018 Feb 26 doi: 10.1002/lary.27119. PMID: 29481706
Management of surgical difficulties during cochlear implant with inner ear anomalies.
Aldhafeeri AM, Alsanosi AA
Int J Pediatr Otorhinolaryngol 2017 Jan;92:45-49. Epub 2016 Nov 3 doi: 10.1016/j.ijporl.2016.11.001. PMID: 28012532
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
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

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
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
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

Management of surgical difficulties during cochlear implant with inner ear anomalies.
Aldhafeeri AM, Alsanosi AA
Int J Pediatr Otorhinolaryngol 2017 Jan;92:45-49. Epub 2016 Nov 3 doi: 10.1016/j.ijporl.2016.11.001. PMID: 28012532
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
Radiology of the ear in children.
Fitz CR, Harwood-Nash DC
Radiol Clin North Am 1974 Dec;12(3):553-70. PMID: 4548198
Tomography in inner and middle ear malformations: value, limits, results.
Reisner K
Radiology 1969 Jan;92(1):11-20. doi: 10.1148/92.1.11. PMID: 5303921
Ototoxicity of chloroquine.
Matz GJ, Naunton RF
Arch Otolaryngol 1968 Oct;88(4):370-2. doi: 10.1001/archotol.1968.00770010372008. PMID: 5302911

Prognosis

Method to estimate the basal turn length in inner ear malformation types.
Alshalan A, Almuhawas F, Alhabib S, Hamed N, Abdelsamad Y, Dhanasingh A
Sci Rep 2023 Jan 5;13(1):66. doi: 10.1038/s41598-022-23911-5. PMID: 36604454Free PMC Article
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
Posterior Semicircular Canal Dehiscence: Case Series and Systematic Review.
Lee JA, Liu YF, Nguyen SA, McRackan TR, Meyer TA, Rizk HG
Otol Neurotol 2020 Apr;41(4):511-521. doi: 10.1097/MAO.0000000000002576. PMID: 32176140
Current evidence of peripheral vestibular symptoms secondary to otitis media.
Monsanto RDC, Kasemodel ALP, Tomaz A, Paparella MM, Penido NO
Ann Med 2018 Aug;50(5):391-401. Epub 2018 May 6 doi: 10.1080/07853890.2018.1470665. PMID: 29699430

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
Method to estimate the basal turn length in inner ear malformation types.
Alshalan A, Almuhawas F, Alhabib S, Hamed N, Abdelsamad Y, Dhanasingh A
Sci Rep 2023 Jan 5;13(1):66. doi: 10.1038/s41598-022-23911-5. PMID: 36604454Free 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

Recent systematic reviews

A Systematic Review on the Association Between Vestibular Dysfunction and Balance Performance in Children With Hearing Loss.
Singh A, Heet H, Guggenheim DS, Lim M, Garg B, Bao M, Smith SL, Garrison D, Raynor EM, Lee JW, Wrigley J, Riska KM
Ear Hear 2022 May/Jun;43(3):712-721. doi: 10.1097/AUD.0000000000001131. PMID: 34611117Free PMC Article
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
Posterior Semicircular Canal Dehiscence: Case Series and Systematic Review.
Lee JA, Liu YF, Nguyen SA, McRackan TR, Meyer TA, Rizk HG
Otol Neurotol 2020 Apr;41(4):511-521. doi: 10.1097/MAO.0000000000002576. PMID: 32176140
Current evidence of peripheral vestibular symptoms secondary to otitis media.
Monsanto RDC, Kasemodel ALP, Tomaz A, Paparella MM, Penido NO
Ann Med 2018 Aug;50(5):391-401. Epub 2018 May 6 doi: 10.1080/07853890.2018.1470665. PMID: 29699430

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