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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).
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).

Professional guidelines

PubMed

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
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
Poe DS
Otol Neurotol 2007 Aug;28(5):668-77. doi: 10.1097/mao.0b013e31804d4998. PMID: 17534202

Recent clinical studies

Etiology

Zainol Abidin Z, Mohd Zaki F, Kew TY, Goh BS, Abdullah A
J Laryngol Otol 2020 Jul;134(7):603-609. Epub 2020 Jul 27 doi: 10.1017/S0022215120001334. PMID: 32713375
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
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
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
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

Diagnosis

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

Therapy

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
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
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
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
Lim ZM, Friedland PL, Boeddinghaus R, Thompson A, Rodrigues SJ, Atlas M
Otol Neurotol 2012 Jun;33(4):610-2. doi: 10.1097/MAO.0b013e3182536de7. PMID: 22525216

Prognosis

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
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
Choo DI, Tawfik KO, Martin DM, Raphael Y
Am J Med Genet C Semin Med Genet 2017 Dec;175(4):439-449. Epub 2017 Oct 30 doi: 10.1002/ajmg.c.31587. PMID: 29082607Free PMC Article
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
Nager GT, Proctor B
Otolaryngol Clin North Am 1991 Jun;24(3):531-53. PMID: 1762775

Clinical prediction guides

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
Kimura Y, Kaga K
Int J Pediatr Otorhinolaryngol 2022 Nov;162:111303. Epub 2022 Sep 15 doi: 10.1016/j.ijporl.2022.111303. PMID: 36137473
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
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
Nager GT, Proctor B
Otolaryngol Clin North Am 1991 Jun;24(3):531-53. PMID: 1762775

Recent systematic reviews

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
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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