Cochlear aperture (CA) (a.k.a. cochlear fossette or bony CNC) stenosis or atresia
Isolated cochlea: CNC (CA) absent & filled with bone
Definitions
CNC hypoplasia-aplasia: Narrowing or absence of CNC
Cochlear nerve (CN) deficiency (CND): Hypoplasia or aplasia
IMAGING
General Features
Imaging Recommendations
CT Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
Dewyer NA et al: Pediatric single-sided deafness: a review of prevalence, radiologic findings, and cochlear implant candidacy. Ann Otol Rhinol Laryngol. Epub, 2021
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
Yousef M et al: Cochlear implantation versus auditory brainstem implantation in children with auditory nerve deficiencies. Eur Arch Otorhinolaryngol. ePub, 2021
Freeman SR et al: Management of cochlear nerve hypoplasia and aplasia. Adv Otorhinolaryngol. 81:81-92, 2018
Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
Birman CS et al: Cochlear implant outcomes in cochlea nerve aplasia and hypoplasia. Otol Neurotol. 37(5):438-45, 2016
Nixon JN et al: Temporal bone and cranial nerve findings in pontine tegmental cap dysplasia. Neuroradiology. 58(2):179-87, 2016
Wilkins A et al: Frequent association of cochlear nerve canal stenosis with pediatric sensorineural hearing loss. Arch Otolaryngol Head Neck Surg. 138(4):383-8, 2012
Miyasaka M et al: CT and MR imaging for pediatric cochlear implantation: emphasis on the relationship between the cochlear nerve canal and the cochlear nerve. Pediatr Radiol. 40(9):1509-16, 2010
Laury AM et al: Etiology of unilateral neural hearing loss in children. Int J Pediatr Otorhinolaryngol. 73(3):417-27, 2009
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
Kono T: Computed tomographic features of the bony canal of the cochlear nerve in pediatric patients with unilateral sensorineural hearing loss. Radiat Med. 26(3):115-9, 2008
Adunka OF et al: Value of computed tomography in the evaluation of children with cochlear nerve deficiency. Otol Neurotol. 28(5):597-604, 2007
Ito K et al: Isolated cochlear nerve hypoplasia with various internal auditory meatus deformities in children. Ann Otol Rhinol Laryngol. 116(7):520-4, 2007
Komatsubara S et al: Evaluation of cochlear nerve imaging in severe congenital sensorineural hearing loss. ORL J Otorhinolaryngol Relat Spec. 69(3):198-202, 2007
Colletti V et al: Auditory brainstem implant (ABI): new frontiers in adults and children. Otolaryngol Head Neck Surg. 133(1):126-38, 2005
Glastonbury CM et al: Imaging findings of cochlear nerve deficiency. AJNR Am J Neuroradiol. 23(4):635-43, 2002
Stjernholm C et al: Dimensions of the cochlear nerve canal: a radioanatomic investigation. Acta Otolaryngol. 122(1):43-8, 2002
Fatterpekar GM et al: Hypoplasia of the bony canal for the cochlear nerve in patients with congenital sensorineural hearing loss: initial observations. Radiology. 215(1):243-6, 2000
Casselman JW et al: Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging. Radiology. 202(3):773-81, 1997
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References
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Cochlear nerve canal (CNC)
Synonyms
Cochlear aperture (CA) (a.k.a. cochlear fossette or bony CNC) stenosis or atresia
Isolated cochlea: CNC (CA) absent & filled with bone
Definitions
CNC hypoplasia-aplasia: Narrowing or absence of CNC
Cochlear nerve (CN) deficiency (CND): Hypoplasia or aplasia
IMAGING
General Features
Imaging Recommendations
CT Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
Dewyer NA et al: Pediatric single-sided deafness: a review of prevalence, radiologic findings, and cochlear implant candidacy. Ann Otol Rhinol Laryngol. Epub, 2021
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
Yousef M et al: Cochlear implantation versus auditory brainstem implantation in children with auditory nerve deficiencies. Eur Arch Otorhinolaryngol. ePub, 2021
Freeman SR et al: Management of cochlear nerve hypoplasia and aplasia. Adv Otorhinolaryngol. 81:81-92, 2018
Sennaroğlu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
Birman CS et al: Cochlear implant outcomes in cochlea nerve aplasia and hypoplasia. Otol Neurotol. 37(5):438-45, 2016
Nixon JN et al: Temporal bone and cranial nerve findings in pontine tegmental cap dysplasia. Neuroradiology. 58(2):179-87, 2016
Wilkins A et al: Frequent association of cochlear nerve canal stenosis with pediatric sensorineural hearing loss. Arch Otolaryngol Head Neck Surg. 138(4):383-8, 2012
Miyasaka M et al: CT and MR imaging for pediatric cochlear implantation: emphasis on the relationship between the cochlear nerve canal and the cochlear nerve. Pediatr Radiol. 40(9):1509-16, 2010
Laury AM et al: Etiology of unilateral neural hearing loss in children. Int J Pediatr Otorhinolaryngol. 73(3):417-27, 2009
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
Kono T: Computed tomographic features of the bony canal of the cochlear nerve in pediatric patients with unilateral sensorineural hearing loss. Radiat Med. 26(3):115-9, 2008
Adunka OF et al: Value of computed tomography in the evaluation of children with cochlear nerve deficiency. Otol Neurotol. 28(5):597-604, 2007
Ito K et al: Isolated cochlear nerve hypoplasia with various internal auditory meatus deformities in children. Ann Otol Rhinol Laryngol. 116(7):520-4, 2007
Komatsubara S et al: Evaluation of cochlear nerve imaging in severe congenital sensorineural hearing loss. ORL J Otorhinolaryngol Relat Spec. 69(3):198-202, 2007
Colletti V et al: Auditory brainstem implant (ABI): new frontiers in adults and children. Otolaryngol Head Neck Surg. 133(1):126-38, 2005
Glastonbury CM et al: Imaging findings of cochlear nerve deficiency. AJNR Am J Neuroradiol. 23(4):635-43, 2002
Stjernholm C et al: Dimensions of the cochlear nerve canal: a radioanatomic investigation. Acta Otolaryngol. 122(1):43-8, 2002
Fatterpekar GM et al: Hypoplasia of the bony canal for the cochlear nerve in patients with congenital sensorineural hearing loss: initial observations. Radiology. 215(1):243-6, 2000
Casselman JW et al: Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging. Radiology. 202(3):773-81, 1997
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