title: The vestibulocochlear nerve: aplasia and hypoplasia in combination with inner ear malformations.
abstract: OBJECTIVE
  To determine features of hypoplasia and aplasia of the vestibulocochlear nerve (VCN) in combination with inner ear malformations (IEMs).
  
  
  METHODS
  Index cases were retrospectively selected from all IEMs collected since 1995. CT and MRI data were reviewed by two neuroradiologists. The number and thickness of visible nerves in the cerebellopontine angle cistern and inside the internal auditory canal (IAC) were analysed.
  
  
  RESULTS
  MR images for 176 patients with IEMs were analysed. Labyrinthine aplasia and otocyst deformity showed 100% correlation, and IAC malformations exhibited 92% correlation with VCN aplasia. Cochlear aplasia, complete aplasia of the semicircular canals, severe cochlear hypoplasia, common cavity, incomplete partition type 1 and mild cochlear hypoplasia showed decreasing degrees of correlation with hypoplasia of the VCN. The remaining types of IEM did not demonstrate VCN hypoplasia.
  
  
  CONCLUSIONS
  Certain forms of IEM show 100% correlation with hypoplasia or aplasia of the VCN, while others correlate less strongly and some do not usually exhibit VCN hypoplasia. MRI should always be carried out for those forms often correlated with VCN hypoplasia.
  
  
  CONCLUSIONS
  Vestibulocochlear nerve deficiency can be strongly suspected in certain inner ear malformations • Bony cochlear aplasia and cochlear nerve aplasia are strongly correlated • In semicircular canal aplasia, hypoplasia of the vestibular nerve can be found • Before cochlear implantation, the type of any IEM should be fully understood.
authors.affiliation: Department of Diagnostic and Interventional Neuroradiology, Hannover Medical School, Carl-Neuberg-Str. 1, 30625 Hannover, Germany. Giesemann.Anja@mh-hannover.de
authors.forename: Anja M
authors.initials: AM
authors.lastname: Giesemann
authors.forename: Georgios
authors.initials: G
authors.lastname: Kontorinis
authors.forename: Zajaczek
authors.initials: Z
authors.lastname: Jan
authors.forename: Thomas
authors.initials: T
authors.lastname: Lenarz
authors.forename: Heinrich
authors.initials: H
authors.lastname: Lanfermann
authors.forename: Friedrich
authors.initials: F
authors.lastname: Goetz
mesh_terms: D002648:Child; D003051:Cochlea / Q000002:abnormalities; D003056:Cochlear Nerve / Q000002:abnormalities; D007758:Ear, Inner / Q000002:abnormalities*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D012665:Semicircular Canals / Q000002:abnormalities; D036542:Tomography, Spiral Computed; D014725:Vestibular Nerve / Q000002:abnormalities; D000159:Vestibulocochlear Nerve / Q000002:abnormalities*