Mondini anomaly (historic terminology): IP-II + large vestibular aqueduct (LVA)
Definitions
IP-II: Incomplete partition due to deficient interscalar septum (ISS) between middle & apical cochlear turns
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
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: The link between inner ear malformations and the rest of the body: what we know so far about genetic, imaging and histology. Neuroradiology. 62(5):539-44, 2020
Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
Leung KJ et al: Correlation of CT, MR, and histopathology in incomplete partition-II cochlear anomaly. Otol Neurotol. 37(5):434-7, 2016
Emmrich JV et al: Dilated dysplastic vestibule: a new computed tomographic finding in patients with large vestibular aqueduct syndrome. J Comput Assist Tomogr. 35(6):674-8, 2011
Griffith AJ et al: Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, and mouse models. Hear Res. 281(1-2):11-7, 2011
King KA et al: SLC26A4 genotype, but not cochlear radiologic structure, is correlated with hearing loss in ears with an enlarged vestibular aqueduct. Laryngoscope. 120(2):384-9, 2010
Fitoz S et al: SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 71(3):479-86, 2007
Sennaroglu L et al: Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 25(4):520-9; discussion 529, 2004
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References
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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
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TERMINOLOGY
Abbreviations
Cochlear incomplete partition type II (IP-II)
Synonyms
Mondini anomaly (historic terminology): IP-II + large vestibular aqueduct (LVA)
Definitions
IP-II: Incomplete partition due to deficient interscalar septum (ISS) between middle & apical cochlear turns
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
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: The link between inner ear malformations and the rest of the body: what we know so far about genetic, imaging and histology. Neuroradiology. 62(5):539-44, 2020
Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
Leung KJ et al: Correlation of CT, MR, and histopathology in incomplete partition-II cochlear anomaly. Otol Neurotol. 37(5):434-7, 2016
Emmrich JV et al: Dilated dysplastic vestibule: a new computed tomographic finding in patients with large vestibular aqueduct syndrome. J Comput Assist Tomogr. 35(6):674-8, 2011
Griffith AJ et al: Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, and mouse models. Hear Res. 281(1-2):11-7, 2011
King KA et al: SLC26A4 genotype, but not cochlear radiologic structure, is correlated with hearing loss in ears with an enlarged vestibular aqueduct. Laryngoscope. 120(2):384-9, 2010
Fitoz S et al: SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 71(3):479-86, 2007
Sennaroglu L et al: Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 25(4):520-9; discussion 529, 2004
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