Hostname: page-component-76fb5796d-r6qrq Total loading time: 0 Render date: 2024-04-25T08:09:55.205Z Has data issue: false hasContentIssue false
  • English
  • Français

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: J.Waterval@gmail.com
Get access Rights & Permissions [Opens in a new window]

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Manni, JJ, Scaf, JJ, Huygen, PL, Cruysberg, JR, Verhagen, WI. Hyperostosis cranialis interna. A new hereditary syndrome with cranial-nerve entrapment. N Engl J Med 1990;322:450–4CrossRefGoogle ScholarPubMed
2Hamersma, H, Gardner, J, Beighton, P. The natural history of sclerosteosis. Clin Genet 2003 Mar;63(3):192–7 Erratum in. Clin Genet 2003 Aug;64(2):176CrossRefGoogle ScholarPubMed
3Hellier, WP, Brookes, GB. Vestibular nerve dysfunction and decompression in Engelmann's disease. J Laryngol Otol 1996;110:462–5CrossRefGoogle ScholarPubMed
4Hwang, JM, Kim, IO, Wang, KC. Complete visual recovery in osteopetrosis by early optic nerve decompression. Pediatr Neurosurg 2000;33:328–32CrossRefGoogle ScholarPubMed
5Vanier, V, Miller, NR, Carson, BS. Bilateral visual improvement after unilateral optic canal decompression and cranial vault expansion in a patient with osteopetrosis, narrowed optic canals, and increased intracranial pressure. J Neurol Neurosurg Psychiatry 2000;69:405–6CrossRefGoogle Scholar
6Hamersma, H. Total decompression of the facial nerve in osteopetrosis (marble bone disease-morbus Albers-Schonberg). ORL J Otorhinolaryngol Relat Spec 1974;36:21–32CrossRefGoogle ScholarPubMed
7Miyamoto, RT, House, WF, Brackmann, DE. Neurotologic manifestations of the osteopetroses. Arch Otolaryngol 1980;106:210–14CrossRefGoogle ScholarPubMed
8Benecke, JE Jr.Facial nerve dysfunction in osteopetrosis. Laryngoscope 1993;103:494–7CrossRefGoogle ScholarPubMed
9Tibesar, RJ, Brissett, AE, Shallop, JK, Driscoll, CL. Internal auditory canal decompression and cochlear implantation in Camurati-Engelmann disease. Otolaryngol Head Neck Surg 2004;131:1004–6CrossRefGoogle ScholarPubMed
10Hall, CM. International nosology and classification of constitutional disorders of bone. Am J Med Genet 2001;79:376–82Google Scholar