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Semicircular canal dehiscence syndrome

MedGen UID:
1642474
Concept ID:
C4708600
Disease or Syndrome
Synonyms: Dehiscence of the Semicircular Canal; Semicircular Canal Dehiscence; Semicircular Canal Dehiscence Syndrome
SNOMED CT: Semicircular canal dehiscence syndrome (768933005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018484
Orphanet: ORPHA420402

Definition

A rare otorhinolaryngologic disease with characteristics of the unilateral or bilateral dehiscence of the bone(s) overlying the superior (most common), lateral or posterior semicircular canal(s). Patients present audiological (autophony, aural fullness, conductive hearing loss, pulsatile tinnitus) and/or vestibular symptoms (sound or pressure-evoked oscillopsia or vertigo, characteristic vertical-torsional eye movements), depending on which semicircular canal is affected. Posterior SCD syndrome is associated with high-riding jugular bulb and fibrous dysplasia, while lateral SCD syndrome is associated with chronic otitis media and cholesteatoma, with or without audiological and vestibular symptoms. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSemicircular canal dehiscence syndrome

Professional guidelines

PubMed

Zhang L, Creighton FX Jr, Carey JP
Otol Neurotol 2021 Oct 1;42(9):1429-1433. doi: 10.1097/MAO.0000000000003274. PMID: 34528926
Ward BK, van de Berg R, van Rompaey V, Bisdorff A, Hullar TE, Welgampola MS, Carey JP
J Vestib Res 2021;31(3):131-141. doi: 10.3233/VES-200004. PMID: 33522990Free PMC Article
Minor LB
Curr Opin Otolaryngol Head Neck Surg 2003 Oct;11(5):340-6. doi: 10.1097/00020840-200310000-00006. PMID: 14502064

Recent clinical studies

Etiology

Bunne M, Andersson H, Myhrum M
Otol Neurotol 2022 Jul 1;43(6):709-716. doi: 10.1097/MAO.0000000000003561. PMID: 35761466
Formeister EJ, Zhang L, Dent J, Aygun N, Carey JP
Otol Neurotol 2022 Apr 1;43(4):494-499. doi: 10.1097/MAO.0000000000003481. PMID: 35213476
McCrary HC, Babajanian E, Patel N, Yang S, Kircher M, Carlson ML, Gurgel RK
Laryngoscope 2021 Nov;131(11):E2810-E2818. Epub 2021 Jul 17 doi: 10.1002/lary.29751. PMID: 34272884
Banakis Hartl RM, Cass SP
Otolaryngol Head Neck Surg 2018 Mar;158(3):534-540. Epub 2018 Jan 9 doi: 10.1177/0194599817751092. PMID: 29313443Free PMC Article
Stevens SM, O'Connell BP, Meyer TA
Curr Opin Otolaryngol Head Neck Surg 2015 Oct;23(5):341-7. doi: 10.1097/MOO.0000000000000194. PMID: 26339964

Diagnosis

Ward BK, van de Berg R, van Rompaey V, Bisdorff A, Hullar TE, Welgampola MS, Carey JP
J Vestib Res 2021;31(3):131-141. doi: 10.3233/VES-200004. PMID: 33522990Free PMC Article
Steenerson KK, Crane BT, Minor LB
Semin Neurol 2020 Feb;40(1):151-159. Epub 2020 Jan 27 doi: 10.1055/s-0039-3402738. PMID: 31986544
Kobayashi T, Morita M, Yoshioka S, Mizuta K, Ohta S, Kikuchi T, Hayashi T, Kaneko A, Yamaguchi N, Hashimoto S, Kojima H, Murakami S, Takahashi H
Auris Nasus Larynx 2018 Feb;45(1):1-5. Epub 2017 Nov 15 doi: 10.1016/j.anl.2017.09.017. PMID: 29153260
Bi WL, Brewster R, Poe D, Vernick D, Lee DJ, Eduardo Corrales C, Dunn IF
J Neurosurg 2017 Dec;127(6):1268-1276. Epub 2017 Jan 13 doi: 10.3171/2016.9.JNS16503. PMID: 28084916
Lempert T, von Brevern M
Curr Opin Neurol 2005 Feb;18(1):5-9. doi: 10.1097/00019052-200502000-00003. PMID: 15655395

Therapy

Yong M, Zaia E, Westerberg B, Lea J
Otol Neurotol 2017 Sep;38(8):1071-1075. doi: 10.1097/MAO.0000000000001490. PMID: 28657952
Stevens SM, O'Connell BP, Meyer TA
Curr Opin Otolaryngol Head Neck Surg 2015 Oct;23(5):341-7. doi: 10.1097/MOO.0000000000000194. PMID: 26339964
Chilvers G, McKay-Davies I
J Laryngol Otol 2015 Mar;129(3):217-25. Epub 2015 Feb 6 doi: 10.1017/S0022215115000183. PMID: 25655361
Goddard JC, Wilkinson EP
Otolaryngol Head Neck Surg 2014 Sep;151(3):478-83. Epub 2014 Jun 23 doi: 10.1177/0194599814538233. PMID: 24962178
Lempert T, von Brevern M
Curr Opin Neurol 2005 Feb;18(1):5-9. doi: 10.1097/00019052-200502000-00003. PMID: 15655395

Prognosis

Bunne M, Andersson H, Myhrum M
Otol Neurotol 2022 Jul 1;43(6):709-716. doi: 10.1097/MAO.0000000000003561. PMID: 35761466
Formeister EJ, Zhang L, Dent J, Aygun N, Carey JP
Otol Neurotol 2022 Apr 1;43(4):494-499. doi: 10.1097/MAO.0000000000003481. PMID: 35213476
Noij KS, Remenschneider AK, Herrmann BS, Guinan JJ Jr, Rauch SD
Ear Hear 2021 Sep/Oct;42(5):1295-1305. doi: 10.1097/AUD.0000000000001015. PMID: 33625057
Goddard JC, Wilkinson EP
Otolaryngol Head Neck Surg 2014 Sep;151(3):478-83. Epub 2014 Jun 23 doi: 10.1177/0194599814538233. PMID: 24962178
Castellucci A, Brandolini C, Piras G, Modugno GC
Acta Otorhinolaryngol Ital 2013 Apr;33(2):112-20. PMID: 23853402Free PMC Article

Clinical prediction guides

Bunne M, Andersson H, Myhrum M
Otol Neurotol 2022 Jul 1;43(6):709-716. doi: 10.1097/MAO.0000000000003561. PMID: 35761466
Formeister EJ, Zhang L, Dent J, Aygun N, Carey JP
Otol Neurotol 2022 Apr 1;43(4):494-499. doi: 10.1097/MAO.0000000000003481. PMID: 35213476
McCrary HC, Babajanian E, Patel N, Yang S, Kircher M, Carlson ML, Gurgel RK
Laryngoscope 2021 Nov;131(11):E2810-E2818. Epub 2021 Jul 17 doi: 10.1002/lary.29751. PMID: 34272884
Banakis Hartl RM, Cass SP
Otolaryngol Head Neck Surg 2018 Mar;158(3):534-540. Epub 2018 Jan 9 doi: 10.1177/0194599817751092. PMID: 29313443Free PMC Article
Chilvers G, McKay-Davies I
J Laryngol Otol 2015 Mar;129(3):217-25. Epub 2015 Feb 6 doi: 10.1017/S0022215115000183. PMID: 25655361

Recent systematic reviews

Naert L, Van de Berg R, Van de Heyning P, Bisdorff A, Sharon JD, Ward BK, Van Rompaey V
Laryngoscope 2018 Aug;128(8):1932-1938. Epub 2017 Dec 27 doi: 10.1002/lary.27062. PMID: 29280497

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