O'Brien WT , Sr et al: Nonsyndromic congenital causes of sensorineural hearing loss in children: an illustrative review. AJR Am J Roentgenol. 1-8, 2021
D'Arco F et al: Temporal bone and intracranial abnormalities in syndromic causes of hearing loss: an updated guide. Eur J Radiol. 123:108803, 2020
Verheij E et al: Anatomic malformations of the middle and inner ear in 22q11.2 deletion syndrome: case series and literature review. AJNR Am J Neuroradiol. 39(5):928-34, 2018
Green GE et al: CHD7 mutations and CHARGE syndrome in semicircular canal dysplasia. Otol Neurotol. 35(8):1466-70, 2014
Saliba I et al: Down syndrome: an electrophysiological and radiological profile. Laryngoscope. 124(4):E141-7, 2014
Breheret R et al: Bilateral semicircular canal aplasia. Eur Ann Otorhinolaryngol Head Neck Dis. 130(4):225-8, 2013
Elmaleh-Bergès M et al: Spectrum of temporal bone abnormalities in patients with Waardenburg syndrome and SOX10 mutations. AJNR Am J Neuroradiol. 34(6):1257-63, 2013
Intrapiromkul J et al: Inner ear anomalies seen on CT images in people with Down syndrome. Pediatr Radiol. 42(12):1449-55, 2012
Joshi VM et al: CT and MR imaging of the inner ear and brain in children with congenital sensorineural hearing loss. Radiographics. 32(3):683-98, 2012
Blaser S et al: Inner ear dysplasia is common in children with Down syndrome (trisomy 21). Laryngoscope. 116(12):2113-9, 2006
Jongmans MC et al: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet. 43(4):306-14, 2006
Koch B et al: Partial absence of the posterior semicircular canal in Alagille syndrome: CT findings. Pediatr Radiol. 36(9):977-9, 2006
Morimoto AK et al: Absent semicircular canals in CHARGE syndrome: radiologic spectrum of findings. AJNR Am J Neuroradiol. 27(8):1663-71, 2006
Purcell D et al: Establishment of normative cochlear and vestibular measurements to aid in the diagnosis of inner ear malformations. Otolaryngol Head Neck Surg. 128(1):78-87, 2003
Satar B et al: Congenital aplasia of the semicircular canals. Otol Neurotol. 24(3):437-46, 2003
Yu KK et al: Molecular genetic advances in semicircular canal abnormalities and sensorineural hearing loss: a report of 16 cases. Otolaryngol Head Neck Surg. 129(6):637-46, 2003
Lemmerling MM et al: Unilateral semicircular canal aplasia in Goldenhar's syndrome. AJNR Am J Neuroradiol. 21(7):1334-6, 2000
Mizuno M et al: Labyrinthine anomalies with normal cochlear function. ORL J Otorhinolaryngol Relat Spec. 54(5):278-81, 1992
Parnes LS et al: Bilateral semicircular canal aplasia with near-normal cochlear development. Two case reports. Ann Otol Rhinol Laryngol. 99(12):957-9, 1990
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Diagnostic Checklist
TERMINOLOGY
Synonyms
Semicircular canal (SCC)-vestibule malformation
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
O'Brien WT , Sr et al: Nonsyndromic congenital causes of sensorineural hearing loss in children: an illustrative review. AJR Am J Roentgenol. 1-8, 2021
D'Arco F et al: Temporal bone and intracranial abnormalities in syndromic causes of hearing loss: an updated guide. Eur J Radiol. 123:108803, 2020
Verheij E et al: Anatomic malformations of the middle and inner ear in 22q11.2 deletion syndrome: case series and literature review. AJNR Am J Neuroradiol. 39(5):928-34, 2018
Green GE et al: CHD7 mutations and CHARGE syndrome in semicircular canal dysplasia. Otol Neurotol. 35(8):1466-70, 2014
Saliba I et al: Down syndrome: an electrophysiological and radiological profile. Laryngoscope. 124(4):E141-7, 2014
Breheret R et al: Bilateral semicircular canal aplasia. Eur Ann Otorhinolaryngol Head Neck Dis. 130(4):225-8, 2013
Elmaleh-Bergès M et al: Spectrum of temporal bone abnormalities in patients with Waardenburg syndrome and SOX10 mutations. AJNR Am J Neuroradiol. 34(6):1257-63, 2013
Intrapiromkul J et al: Inner ear anomalies seen on CT images in people with Down syndrome. Pediatr Radiol. 42(12):1449-55, 2012
Joshi VM et al: CT and MR imaging of the inner ear and brain in children with congenital sensorineural hearing loss. Radiographics. 32(3):683-98, 2012
Blaser S et al: Inner ear dysplasia is common in children with Down syndrome (trisomy 21). Laryngoscope. 116(12):2113-9, 2006
Jongmans MC et al: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet. 43(4):306-14, 2006
Koch B et al: Partial absence of the posterior semicircular canal in Alagille syndrome: CT findings. Pediatr Radiol. 36(9):977-9, 2006
Morimoto AK et al: Absent semicircular canals in CHARGE syndrome: radiologic spectrum of findings. AJNR Am J Neuroradiol. 27(8):1663-71, 2006
Purcell D et al: Establishment of normative cochlear and vestibular measurements to aid in the diagnosis of inner ear malformations. Otolaryngol Head Neck Surg. 128(1):78-87, 2003
Satar B et al: Congenital aplasia of the semicircular canals. Otol Neurotol. 24(3):437-46, 2003
Yu KK et al: Molecular genetic advances in semicircular canal abnormalities and sensorineural hearing loss: a report of 16 cases. Otolaryngol Head Neck Surg. 129(6):637-46, 2003
Lemmerling MM et al: Unilateral semicircular canal aplasia in Goldenhar's syndrome. AJNR Am J Neuroradiol. 21(7):1334-6, 2000
Mizuno M et al: Labyrinthine anomalies with normal cochlear function. ORL J Otorhinolaryngol Relat Spec. 54(5):278-81, 1992
Parnes LS et al: Bilateral semicircular canal aplasia with near-normal cochlear development. Two case reports. Ann Otol Rhinol Laryngol. 99(12):957-9, 1990
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