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Abnormal semicircular canal morphology

MedGen UID:: 868972
•Concept ID:: C4023386
•: Anatomical Abnormality

Synonym:	Morphological abnormality of the semicircular canal

HPO:	HP:0011380

Definition

An abnormality of the morphology of the semicircular canal. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

Abnormal semicircular canal morphology

Phenotypic abnormality
- Ear malformation
  - Abnormal ear morphology
    - Abnormality of the inner ear
      - Abnormal inner ear morphology
        Abnormal morphology of the vestibule of the inner ear
        Abnormal semicircular canal morphology
        Abnormal anterior semicircular canal morphology
        Abnormal lateral semicircular canal morphology
        Abnormal posterior semicircular canal morphology
        Abnormal saccule morphology
        Abnormal utricle morphology
        Aplasia of the semicircular canal
        Enlarged semicircular canal
        Hypoplasia of the semicircular canal

Conditions with this feature

Hearing loss, autosomal recessive 109

MedGen UID:: 1633308
•Concept ID:: C4693935
•: Disease or Syndrome

DFNB109 is characterized by bilateral congenital severe to profound sensorineural hearing loss. In addition, affected individuals exhibit vestibular dysplasia on CT scan, although they do not manifest problems with balance or movement (Rohacek et al., 2017).

See: Condition Record

Hearing loss, autosomal dominant 80

MedGen UID:: 1779667
•Concept ID:: C5543289
•: Disease or Syndrome

DFNA80 is characterized by nonsyndromic congenital deafness associated with absent or malformed cochleae and eighth cranial nerves (Schrauwen et al., 2018; Schrauwen et al., 2020).

See: Condition Record

Hearing loss, autosomal dominant 80

Hearing loss, autosomal recessive 109

Professional guidelines

PubMed

Cerebrospinal fluid otorrhea secondary to congenital inner ear dysplasia: diagnosis and management of 18 cases.

Wang B, Dai WJ, Cheng XT, Liuyang WY, Yuan YS, Dai CF, Shu YL, Chen B
J Zhejiang Univ Sci B 2019 Feb.;20(2):156-163. doi: 10.1631/jzus.B1800224. PMID: 30666849 Free PMC Article

Three-dimensional study of 31 cases of synostotic anterior plagiocephaly before and after surgical management the Lille protocol.

Simon A, Bocquet E, Pellerin P, Vinchon M, Dhellemmes P, Martinot V, Wolber A, Calibre C, Charlier P, Guerreschi P
J Craniomaxillofac Surg 2018 Jun;46(6):958-966. Epub 2018 Mar 28 doi: 10.1016/j.jcms.2018.03.014. PMID: 29661661

Endolymphatic hydrops imaging: Differential diagnosis in patients with Meniere disease symptoms.

Attyé A, Eliezer M, Galloux A, Pietras J, Tropres I, Schmerber S, Dumas G, Krainik A
Diagn Interv Imaging 2017 Oct;98(10):699-706. Epub 2017 Jun 20 doi: 10.1016/j.diii.2017.06.002. PMID: 28645678

See all (6)

Recent clinical studies

Etiology

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: 32467182 Free PMC Article

Disorders of the inner-ear balance organs and their pathways.

Young AS, Rosengren SM, Welgampola MS
Handb Clin Neurol 2018;159:385-401. doi: 10.1016/B978-0-444-63916-5.00025-2. PMID: 30482329

Pediatric superior semicircular canal dehiscence: illustrative case and systematic review.

Lagman C, Ong V, Chung LK, Elhajjmoussa L, Fong C, Wang AC, Gopen Q, Yang I
J Neurosurg Pediatr 2017 Aug;20(2):196-203. Epub 2017 May 26 doi: 10.3171/2017.3.PEDS1734. PMID: 28548616

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

Inferior vestibular neuritis.

Kim JS, Kim HJ
J Neurol 2012 Aug;259(8):1553-60. Epub 2012 Jan 4 doi: 10.1007/s00415-011-6375-4. PMID: 22215238

See all (171)

Diagnosis

Disorders of the inner-ear balance organs and their pathways.

Young AS, Rosengren SM, Welgampola MS
Handb Clin Neurol 2018;159:385-401. doi: 10.1016/B978-0-444-63916-5.00025-2. PMID: 30482329

Pediatric superior semicircular canal dehiscence: illustrative case and systematic review.

Lagman C, Ong V, Chung LK, Elhajjmoussa L, Fong C, Wang AC, Gopen Q, Yang I
J Neurosurg Pediatr 2017 Aug;20(2):196-203. Epub 2017 May 26 doi: 10.3171/2017.3.PEDS1734. PMID: 28548616

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

CHD7 mutations and CHARGE syndrome in semicircular canal dysplasia.

Green GE, Huq FS, Emery SB, Mukherji SK, Martin DM
Otol Neurotol 2014 Sep;35(8):1466-70. doi: 10.1097/MAO.0000000000000260. PMID: 24979395 Free PMC Article

Inferior vestibular neuritis.

Kim JS, Kim HJ
J Neurol 2012 Aug;259(8):1553-60. Epub 2012 Jan 4 doi: 10.1007/s00415-011-6375-4. PMID: 22215238

See all (226)

Therapy

Vestibular hydrops in patients with semicircular canal malformation.

Niu Y, Chen W, Lin M, Sha Y
Auris Nasus Larynx 2023 Oct;50(5):727-732. Epub 2023 Feb 4 doi: 10.1016/j.anl.2023.01.009. PMID: 36740469

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: 29545254 Free 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

Superior Canal Dehiscence Syndrome.

da Cunha Ferreira S, de Melo Tavares de Lima MA
Braz J Otorhinolaryngol 2006 May-Jun;72(3):414-8. doi: 10.1016/s1808-8694(15)30978-2. PMID: 17119781 Free PMC Article

See all (33)

Prognosis

Surgical management of endolymphatic sac tumor: classification, outcomes and strategy. A single institution's experience.

Wu N, Ma X, Shen W, Hou Z, Han W, Dai P, Zhao H, Huang D, Han D, Yang S
Eur Arch Otorhinolaryngol 2023 Jan;280(1):69-76. Epub 2022 Jun 23 doi: 10.1007/s00405-022-07447-y. PMID: 35739425

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

Vestibulo-ocular physiology underlying vestibular hypofunction.

Schubert MC, Minor LB
Phys Ther 2004 Apr;84(4):373-85. PMID: 15049730

Molecular genetic advances in semicircular canal abnormalities and sensorineural hearing loss: a report of 16 cases.

Yu KK, Mukherji S, Carrasco V, Pillsbury HC, Shores CG
Otolaryngol Head Neck Surg 2003 Dec;129(6):637-46. doi: 10.1016/S0194-59980301593-6. PMID: 14663429

Anatomic variations and anomalies involving the facial canal.

Nager GT, Proctor B
Otolaryngol Clin North Am 1991 Jun;24(3):531-53. PMID: 1762775

See all (71)

Clinical prediction guides

Vestibular hydrops in patients with semicircular canal malformation.

Niu Y, Chen W, Lin M, Sha Y
Auris Nasus Larynx 2023 Oct;50(5):727-732. Epub 2023 Feb 4 doi: 10.1016/j.anl.2023.01.009. PMID: 36740469

Nystagmus in patients with lateral semicircular canal dysplasia.

Choi B, Lee DH, Shin JE, Kim CH
Acta Otolaryngol 2020 Dec;140(12):971-976. Epub 2020 Aug 18 doi: 10.1080/00016489.2020.1804615. PMID: 32808842

Pediatric superior semicircular canal dehiscence: illustrative case and systematic review.

Lagman C, Ong V, Chung LK, Elhajjmoussa L, Fong C, Wang AC, Gopen Q, Yang I
J Neurosurg Pediatr 2017 Aug;20(2):196-203. Epub 2017 May 26 doi: 10.3171/2017.3.PEDS1734. PMID: 28548616

Molecular genetic advances in semicircular canal abnormalities and sensorineural hearing loss: a report of 16 cases.

Yu KK, Mukherji S, Carrasco V, Pillsbury HC, Shores CG
Otolaryngol Head Neck Surg 2003 Dec;129(6):637-46. doi: 10.1016/S0194-59980301593-6. PMID: 14663429

Anatomic variations and anomalies involving the facial canal.

Nager GT, Proctor B
Otolaryngol Clin North Am 1991 Jun;24(3):531-53. PMID: 1762775

See all (107)

Recent systematic reviews

The influence of the subarcuate artery in the superior semicircular canal dehiscence and its frequency on stillbirths: illustrative cases and systematic review.

Bom Braga GP, Noble JH, Gebrim EMMS, Labadie RF, Bento RF
Acta Otolaryngol 2018 May;138(5):437-442. Epub 2017 Dec 22 doi: 10.1080/00016489.2017.1416169. PMID: 29272988

Pediatric superior semicircular canal dehiscence: illustrative case and systematic review.

Lagman C, Ong V, Chung LK, Elhajjmoussa L, Fong C, Wang AC, Gopen Q, Yang I
J Neurosurg Pediatr 2017 Aug;20(2):196-203. Epub 2017 May 26 doi: 10.3171/2017.3.PEDS1734. PMID: 28548616

See all (2)

MedGen

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Abnormal semicircular canal morphology

Definition

Term Hierarchy

Conditions with this feature

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

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