Bookmarks
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
- Complete labyrinthine aplasia (CLA)
- Old synonym: Michel anomaly
Definitions
- Absent cochlea, vestibule, and semicircular canals (SCCs)
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
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
- Siu JM et al: Efficacy of a selective imaging paradigm prior to pediatric cochlear implantation. Laryngoscope. 129(11):2627-33, 2019
- Eisenberg LS et al: Early communication development of children with auditory brainstem implants. J Deaf Stud Deaf Educ. 23(3):249-60, 2018
- Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
- Palomeque Vera JM et al: Effectiveness of cochlear implant in inner ear bone malformations with anterior labyrinth involvement. Int J Pediatr Otorhinolaryngol. 79(3):369-73, 2015
- Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int.17(1):3-20, 2015
- Marcus S et al: Computed tomography demonstrates abnormalities of contralateral ear in subjects with unilateral sensorineural hearing loss. Int J Pediatr Otorhinolaryngol. 78(2):268-71, 2014
- Young JY et al: Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation. Radiographics. 34(5):E133-49, 2014
- Clemmens CS et al: Unilateral cochlear nerve deficiency in children. Otolaryngol Head Neck Surg. 149(2):318-25, 2013
- Higley MJ et al: Bilateral complete labyrinthine aplasia with bilateral internal carotid artery aplasia, developmental delay, and gaze abnormalities: a presumptive case of a rare HOXA1 mutation syndrome. AJNR Am J Neuroradiol. 32(2):E23-5, 2011
- Ozgen B et al: Complete labyrinthine aplasia: clinical and radiologic findings with review of the literature. AJNR Am J Neuroradiol. 30(4):774-80, 2009
- Bosley TM et al: The clinical spectrum of homozygous HOXA1 mutations. Am J Med Genet A. 146A(10):1235-40, 2008
- Tekin M et al: Homozygous mutations in fibroblast growth factor 3 are associated with a new form of syndromic deafness characterized by inner ear agenesis, microtia, and microdontia. Am J Hum Genet. 80(2):338-44, 2007
- Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
- Hersh JH et al: Michel's anomaly, type I microtia and microdontia. Ear Nose Throat J. 70(3):155-7, 1991
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
- Complete labyrinthine aplasia (CLA)
- Old synonym: Michel anomaly
Definitions
- Absent cochlea, vestibule, and semicircular canals (SCCs)
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
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
- Siu JM et al: Efficacy of a selective imaging paradigm prior to pediatric cochlear implantation. Laryngoscope. 129(11):2627-33, 2019
- Eisenberg LS et al: Early communication development of children with auditory brainstem implants. J Deaf Stud Deaf Educ. 23(3):249-60, 2018
- Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
- Palomeque Vera JM et al: Effectiveness of cochlear implant in inner ear bone malformations with anterior labyrinth involvement. Int J Pediatr Otorhinolaryngol. 79(3):369-73, 2015
- Sennaroglu L: Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int.17(1):3-20, 2015
- Marcus S et al: Computed tomography demonstrates abnormalities of contralateral ear in subjects with unilateral sensorineural hearing loss. Int J Pediatr Otorhinolaryngol. 78(2):268-71, 2014
- Young JY et al: Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation. Radiographics. 34(5):E133-49, 2014
- Clemmens CS et al: Unilateral cochlear nerve deficiency in children. Otolaryngol Head Neck Surg. 149(2):318-25, 2013
- Higley MJ et al: Bilateral complete labyrinthine aplasia with bilateral internal carotid artery aplasia, developmental delay, and gaze abnormalities: a presumptive case of a rare HOXA1 mutation syndrome. AJNR Am J Neuroradiol. 32(2):E23-5, 2011
- Ozgen B et al: Complete labyrinthine aplasia: clinical and radiologic findings with review of the literature. AJNR Am J Neuroradiol. 30(4):774-80, 2009
- Bosley TM et al: The clinical spectrum of homozygous HOXA1 mutations. Am J Med Genet A. 146A(10):1235-40, 2008
- Tekin M et al: Homozygous mutations in fibroblast growth factor 3 are associated with a new form of syndromic deafness characterized by inner ear agenesis, microtia, and microdontia. Am J Hum Genet. 80(2):338-44, 2007
- Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112(12):2230-41, 2002
- Hersh JH et al: Michel's anomaly, type I microtia and microdontia. Ear Nose Throat J. 70(3):155-7, 1991
