Cochlear underdevelopment: External dimensions < normal cochlea with internal architecture deformities
Term "cochlea with 1.5 turns" used to define CH (especially CH type III; cochlea with < 2 turns)
Difficult to count number of turns on CT/MR in CH
Avoid this term for cochlear incomplete partition type II (IP-II)
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Treatment
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
Pamuk G et al: Radiological measurement of cochlear dimensions in cochlear hypoplasia and its effect on cochlear implant selection. J Laryngol Otol. 1-7, 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
Collier TJ et al: Is alpha-synuclein loss-of-function a contributor to Parkinsonian Pathology? Evidence from Non-human Primates. Front Neurosci. 10:12, 2016
Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int. 17(1):3-2, 2016
Teissier N et al: Computed tomography measurements of the normal and the pathologic cochlea in children. Pediatr Radiol. 40(3):275-83, 2010
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Cochlear hypoplasia (CH)
Definitions
Cochlear underdevelopment: External dimensions < normal cochlea with internal architecture deformities
Term "cochlea with 1.5 turns" used to define CH (especially CH type III; cochlea with < 2 turns)
Difficult to count number of turns on CT/MR in CH
Avoid this term for cochlear incomplete partition type II (IP-II)
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Treatment
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
Pamuk G et al: Radiological measurement of cochlear dimensions in cochlear hypoplasia and its effect on cochlear implant selection. J Laryngol Otol. 1-7, 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
Collier TJ et al: Is alpha-synuclein loss-of-function a contributor to Parkinsonian Pathology? Evidence from Non-human Primates. Front Neurosci. 10:12, 2016
Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int. 17(1):3-2, 2016