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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]

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: 30666849Free PMC Article
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
Diagnosis and management of the patulous eustachian tube.
Poe DS
Otol Neurotol 2007 Aug;28(5):668-77. doi: 10.1097/mao.0b013e31804d4998. PMID: 17534202

Recent clinical studies

Etiology

Superior semicircular canal dehiscence in relation with the superior petrosal sinus: our experience, surgical management and systematic review of literature.
Rueda Vega M, López Granados C, Arístegui Torrano I, Martín Sanz E, Arístegui Ruiz M
Eur Arch Otorhinolaryngol 2024 Sep;281(9):4665-4675. Epub 2024 May 2 doi: 10.1007/s00405-024-08682-1. PMID: 38698161
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
Congenital inner ear malformations without sensorineural hearing loss in children.
Ozeki M, Kato Z, Sasai H, Kubota K, Funato M, Orii K, Kaneko H, Fukao T, Kondo N
Int J Pediatr Otorhinolaryngol 2009 Oct;73(10):1484-7. Epub 2009 Aug 19 doi: 10.1016/j.ijporl.2009.07.013. PMID: 19695719
Superior semicircular canal dehiscence: a series of 13 cases.
Modugno G, Brandolini C, Savastio G, Ceroni AR, Pirodda A
ORL J Otorhinolaryngol Relat Spec 2005;67(3):180-4. doi: 10.1159/000086573. PMID: 15990466

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
Imaging Review of the Temporal Bone: Part II. Traumatic, Postoperative, and Noninflammatory Nonneoplastic Conditions.
Juliano AF, Ginat DT, Moonis G
Radiology 2015 Sep;276(3):655-72. doi: 10.1148/radiol.2015140800. PMID: 26302389
How to prevent a stapes gusher.
Cremers CWRJ
Adv Otorhinolaryngol 2007;65:278-284. doi: 10.1159/000098843. PMID: 17245059
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: 17119781Free PMC Article
Rebound caloric nystagmus.
Kumar A, Pieri A, Krol G
Laryngoscope 1993 Nov;103(11 Pt 1):1205-13. doi: 10.1288/00005537-199311000-00001. PMID: 8231572

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
How to prevent a stapes gusher.
Cremers CWRJ
Adv Otorhinolaryngol 2007;65:278-284. doi: 10.1159/000098843. PMID: 17245059
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: 17119781Free PMC Article
Radiology of the ear in children.
Fitz CR, Harwood-Nash DC
Radiol Clin North Am 1974 Dec;12(3):553-70. PMID: 4548198

Prognosis

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
Could genetic determinants of inner ear anomalies be a factor for the development of idiopathic scoliosis?
Patten SA, Moldovan F
Med Hypotheses 2011 Mar;76(3):438-40. Epub 2010 Dec 10 doi: 10.1016/j.mehy.2010.11.015. PMID: 21146321
Congenital inner ear malformations without sensorineural hearing loss in children.
Ozeki M, Kato Z, Sasai H, Kubota K, Funato M, Orii K, Kaneko H, Fukao T, Kondo N
Int J Pediatr Otorhinolaryngol 2009 Oct;73(10):1484-7. Epub 2009 Aug 19 doi: 10.1016/j.ijporl.2009.07.013. PMID: 19695719
A clinical evaluation of head impulse testing.
Beynon GJ, Jani P, Baguley DM
Clin Otolaryngol Allied Sci 1998 Apr;23(2):117-22. doi: 10.1046/j.1365-2273.1998.00112.x. PMID: 9597280
Rebound caloric nystagmus.
Kumar A, Pieri A, Krol G
Laryngoscope 1993 Nov;103(11 Pt 1):1205-13. doi: 10.1288/00005537-199311000-00001. PMID: 8231572

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
Multiple Sclerosis Presenting as an Anterior Semicircular Canal Benign Paroxysmal Positional Vertigo: Case Report.
Musat GC, Musat AAM
Ear Nose Throat J 2021 Sep;100(5_suppl):636S-640S. Epub 2020 Jan 20 doi: 10.1177/0145561319897983. PMID: 31955602
Could genetic determinants of inner ear anomalies be a factor for the development of idiopathic scoliosis?
Patten SA, Moldovan F
Med Hypotheses 2011 Mar;76(3):438-40. Epub 2010 Dec 10 doi: 10.1016/j.mehy.2010.11.015. PMID: 21146321
The large vestibular aqueduct syndrome.
Jackler RK, De La Cruz A
Laryngoscope 1989 Dec;99(12):1238-42; discussion 1242-3. doi: 10.1288/00005537-198912000-00006. PMID: 2601537
The large vestibular aqueduct syndrome.
Valvassori GE, Clemis JD
Laryngoscope 1978 May;88(5):723-8. doi: 10.1002/lary.1978.88.5.723. PMID: 306012

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

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