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.
Key Points
• 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
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Abbreviations
- CC:
-
Common cavity
- CPA:
-
cerebello pontine angle cistern
- IEM:
-
Inner ear malformation
- IAC:
-
Internal auditory canal
- IPT1:
-
Incomplete partition type 1
- IPT2:
-
Incomplete partition type 2
- SCC:
-
Semicircular canal
- VCN:
-
Vestibulocochlear nerve
- VCOMM:
-
common vestibular nerve
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Acknowledgements
The study was presented in parts at the Annual Congress of the German Society for Neuroradiology (DGNR) in Köln, October 6th–8th 2011.
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Giesemann, A.M., Kontorinis, G., Jan, Z. et al. The vestibulocochlear nerve: aplasia and hypoplasia in combination with inner ear malformations. Eur Radiol 22, 519–524 (2012). https://doi.org/10.1007/s00330-011-2287-z
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DOI: https://doi.org/10.1007/s00330-011-2287-z