U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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
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
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

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
Cochlear and Vestibular Volumes in Inner Ear Malformations.
Breitsprecher TM, Pscheidl A, Bächinger D, Volkenstein S, Dhanasingh A, Van Rompaey V, Mlynski R, Dazert S, Van de Heyning P, Langner S, Roland P, Weiss NM
Otol Neurotol 2022 Sep 1;43(8):e814-e819. doi: 10.1097/MAO.0000000000003615. PMID: 35970155
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
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

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
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
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
Neuro-otological emergencies.
Seemungal BM
Curr Opin Neurol 2007 Feb;20(1):32-9. doi: 10.1097/WCO.0b013e3280122514. PMID: 17215686

Therapy

Radiological feature heterogeneity supports etiological diversity among patient groups in Meniere's disease.
Bächinger D, Filidoro N, Naville M, Juchler N, Kurtcuoglu V, Nadol JB Jr, Schuknecht B, Kleinjung T, Veraguth D, Eckhard AH
Sci Rep 2023 Jun 26;13(1):10303. doi: 10.1038/s41598-023-36479-5. PMID: 37365255Free 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
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
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

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
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
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
Cochlear Implantation in Pediatric Patients With Enlarged Vestibular Aqueduct: A Systematic Review.
Benchetrit L, Jabbour N, Appachi S, Liu YC, Cohen MS, Anne S
Laryngoscope 2022 Jul;132(7):1459-1472. Epub 2021 Jul 7 doi: 10.1002/lary.29742. PMID: 34233033
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
Natural history of hearing loss in children with enlarged vestibular aqueduct syndrome.
Mori T, Westerberg BD, Atashband S, Kozak FK
J Otolaryngol Head Neck Surg 2008 Feb;37(1):112-8. PMID: 18479637

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...