link
Bookmarks
Cochlear Nerve and Cochlear Nerve Canal Aplasia-Hypoplasia
Surjith Vattoth, MD, FRCR; Caroline D. Robson, MBChB
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
4
5
6

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Cochlear nerve canal (CNC)
              • Synonyms

                • Cochlear aperture (CA) (also called 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): CN hypoplasia or absence (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

                                        1. Freeman SR et al: Management of cochlear nerve hypoplasia and aplasia. Adv Otorhinolaryngol. 81:81-92, 2018
                                        2. Sennaro─člu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                        3. Birman CS et al: Cochlear implant outcomes in cochlea nerve aplasia and hypoplasia. Otol Neurotol. 37(5):438-45, 2016
                                        4. Nixon JN et al: Temporal bone and cranial nerve findings in pontine tegmental cap dysplasia. Neuroradiology. 58(2):179-87, 2016
                                        5. 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
                                        6. 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
                                        7. Laury AM et al: Etiology of unilateral neural hearing loss in children. Int J Pediatr Otorhinolaryngol. 73(3):417-27, 2009
                                        8. 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
                                        9. 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
                                        10. Adunka OF et al: Value of computed tomography in the evaluation of children with cochlear nerve deficiency. Otol Neurotol. 28(5):597-604, 2007
                                        11. 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
                                        12. 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
                                        13. Colletti V et al: Auditory brainstem implant (ABI): new frontiers in adults and children. Otolaryngol Head Neck Surg. 133(1):126-38, 2005
                                        14. Glastonbury CM et al: Imaging findings of cochlear nerve deficiency. AJNR Am J Neuroradiol. 23(4):635-43, 2002
                                        15. Stjernholm C et al: Dimensions of the cochlear nerve canal: a radioanatomic investigation. Acta Otolaryngol. 122(1):43-8, 2002
                                        16. 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
                                        17. Casselman JW et al: Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging. Radiology. 202(3):773-81, 1997
                                        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 nerve canal (CNC)
                                                    • Synonyms

                                                      • Cochlear aperture (CA) (also called 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): CN hypoplasia or absence (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

                                                                              1. Freeman SR et al: Management of cochlear nerve hypoplasia and aplasia. Adv Otorhinolaryngol. 81:81-92, 2018
                                                                              2. Sennaro─člu L et al: Classification and current management of inner ear malformations. Balkan Med J. 34(5):397-411, 2017
                                                                              3. Birman CS et al: Cochlear implant outcomes in cochlea nerve aplasia and hypoplasia. Otol Neurotol. 37(5):438-45, 2016
                                                                              4. Nixon JN et al: Temporal bone and cranial nerve findings in pontine tegmental cap dysplasia. Neuroradiology. 58(2):179-87, 2016
                                                                              5. 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
                                                                              6. 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
                                                                              7. Laury AM et al: Etiology of unilateral neural hearing loss in children. Int J Pediatr Otorhinolaryngol. 73(3):417-27, 2009
                                                                              8. 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
                                                                              9. 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
                                                                              10. Adunka OF et al: Value of computed tomography in the evaluation of children with cochlear nerve deficiency. Otol Neurotol. 28(5):597-604, 2007
                                                                              11. 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
                                                                              12. 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
                                                                              13. Colletti V et al: Auditory brainstem implant (ABI): new frontiers in adults and children. Otolaryngol Head Neck Surg. 133(1):126-38, 2005
                                                                              14. Glastonbury CM et al: Imaging findings of cochlear nerve deficiency. AJNR Am J Neuroradiol. 23(4):635-43, 2002
                                                                              15. Stjernholm C et al: Dimensions of the cochlear nerve canal: a radioanatomic investigation. Acta Otolaryngol. 122(1):43-8, 2002
                                                                              16. 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
                                                                              17. Casselman JW et al: Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging. Radiology. 202(3):773-81, 1997