Absent cochlea, but vestibule, semicircular canals (SCCs), & internal auditory canal (IAC) present in some form
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
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
Sennaroğlu L et al: Long-term results of ABI in children with severe inner ear malformations. Otol Neurotol.37(7):865-72, 2016
Kontorinis G et al: Aplasia of the cochlea: radiologic assessment and options for hearing rehabilitation. Otol Neurotol. 34(7):1253-60, 2013
Jeong SW et al: Cochlear implantation in children with cochlear aplasia. Acta Otolaryngol. 132(9):910-5, 2012
Sennaroglu L et al: Preliminary results of auditory brainstem implantation in prelingually deaf children with inner ear malformations including severe stenosis of the cochlear aperture and aplasia of the cochlear nerve. Otol Neurotol. 30(6):708-15, 2009
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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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Absent cochlea
Definitions
Absent cochlea, but vestibule, semicircular canals (SCCs), & internal auditory canal (IAC) present in some form
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
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
Sennaroğlu L et al: Long-term results of ABI in children with severe inner ear malformations. Otol Neurotol.37(7):865-72, 2016
Kontorinis G et al: Aplasia of the cochlea: radiologic assessment and options for hearing rehabilitation. Otol Neurotol. 34(7):1253-60, 2013
Jeong SW et al: Cochlear implantation in children with cochlear aplasia. Acta Otolaryngol. 132(9):910-5, 2012
Sennaroglu L et al: Preliminary results of auditory brainstem implantation in prelingually deaf children with inner ear malformations including severe stenosis of the cochlear aperture and aplasia of the cochlear nerve. Otol Neurotol. 30(6):708-15, 2009
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
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