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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Cystic cochleovestibular malformation (CCVM)
Synonyms
- Cystic cochleovestibular anomaly
- Cochlear incomplete partition type I (IP-I)
Definitions
- Cochlea lacks interscalar septum & modiolus (IP-I) + dilated vestibule & lateral (horizontal) semicircular canal (SCC)
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
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
- Ohta S et al: Hearing crosstalk: the molecular conversation orchestrating inner ear dorsoventral patterning. Wiley Interdiscip Rev Dev Biol. 7(1), 2018
- Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
- Banumathy N et al: Communication outcomes following cochlear implantation in a child with cystic cochleovestibular anomaly. J Otol. 11(1):38-41, 2016
- Wani NA et al: Recurrent meningitis in an adult secondary to an inner ear malformation: imaging demonstration. Ear Nose Throat J. 91(4):E23-6, 2012
- Sennaroglu L et al: Surgical results of cochlear implantation in malformed cochlea. Otol Neurotol. 27(5):615-23, 2006
- Sennaroglu L et al: Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 25(4):520-9; discussion 529, 2004
- Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
- Jackler RK et al: Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope. 97(3 Pt 2 Suppl 40):2-14, 1987
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Cystic cochleovestibular malformation (CCVM)
Synonyms
- Cystic cochleovestibular anomaly
- Cochlear incomplete partition type I (IP-I)
Definitions
- Cochlea lacks interscalar septum & modiolus (IP-I) + dilated vestibule & lateral (horizontal) semicircular canal (SCC)
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
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
- Ohta S et al: Hearing crosstalk: the molecular conversation orchestrating inner ear dorsoventral patterning. Wiley Interdiscip Rev Dev Biol. 7(1), 2018
- Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
- Banumathy N et al: Communication outcomes following cochlear implantation in a child with cystic cochleovestibular anomaly. J Otol. 11(1):38-41, 2016
- Wani NA et al: Recurrent meningitis in an adult secondary to an inner ear malformation: imaging demonstration. Ear Nose Throat J. 91(4):E23-6, 2012
- Sennaroglu L et al: Surgical results of cochlear implantation in malformed cochlea. Otol Neurotol. 27(5):615-23, 2006
- Sennaroglu L et al: Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 25(4):520-9; discussion 529, 2004
- Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
- Jackler RK et al: Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope. 97(3 Pt 2 Suppl 40):2-14, 1987
