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Facial nerve decompression via middle fossa approach for hyperostosis cranialis interna: a feasible therapeutic approach

Published online by Cambridge University Press:  17 April 2009

J J Waterval ,
R J Stokroos ,
R B J De Bondt  and
J J Manni
J J Waterval*
Affiliation:
Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Maastricht, The Netherlands
R J Stokroos
Affiliation:
Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Maastricht, The Netherlands
R B J De Bondt
Affiliation:
Department of Radiology, University Hospital Maastricht, The Netherlands
J J Manni
Affiliation:
Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Maastricht, The Netherlands
*
Address for correspondence: Dr J J Waterval, Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Maastricht, PO 5800, 6202AZ Maastricht, The Netherlands. Fax: +31 43 3875580 E-mail: [email protected]
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Abstract

Hyperostosis cranialis interna is an autosomal dominant disorder characterised by endosteal hyperostosis and osteosclerosis of the skull base and calvaria, leading to compression and dysfunction of cranial nerves I, II, VII and VIII.

Case report:

We report the use of bilateral surgical decompression of the internal auditory canals to treat hyperostosis cranialis interna in an eight-year-old girl presenting with bilateral facial palsy due to hyperostosis cranialis interna.

Intervention and outcome:

Using a middle fossa craniotomy approach, both internal auditory canals were unroofed and cranial nerves VII and VIII were decompressed, with a one-year interval between sides. The mimic function recovered. One year post-operatively, the right and left facial sides had been restored to House–Brackmann grades I and II, respectively.

Conclusion:

This is the first report of the use of surgical decompression of the internal auditory canal in a case of hyperostosis cranialis interna. Surgical decompression of the internal auditory canal is recommended therapeutically, but may also be performed prophylactically in younger patients with hyperostosis cranialis interna.

Keywords

HyperostosisFacial NerveInternal Auditory CanalMiddle Cranial Fossa
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 10 , October 2009 , pp. 1177 - 1180
Copyright
Copyright © JLO (1984) Limited 2009

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