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Cochleovestibular nerve involvement in multifocal fibrosclerosis

Published online by Cambridge University Press:  05 July 2012

R K Tsuji ,
A G Bittencourt ,
M H Arai  and
E M Santiago Gebrim
R K Tsuji
Affiliation:
Department of Otolaryngology, University of São Paulo School of Medicine, Brazil
A G Bittencourt*
Affiliation:
Department of Otolaryngology, University of São Paulo School of Medicine, Brazil
M H Arai
Affiliation:
Department of Internal Medicine, University of São Paulo School of Medicine, Brazil
E M Santiago Gebrim
Affiliation:
Department of Radiology, University of São Paulo School of Medicine, Brazil
*
Address for correspondence: Dr Aline Gomes Bittencourt, Department of Otolaryngology, Hospital das Clínicas, Universidade de São Paulo, Av Dr Enéas de Carvalho Aguiar 255, 6° Andar/sala 6167, CEP 05403-000, São Paulo, SP, Brazil Fax: + 55 11 3088 0299 E-mail: [email protected]
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Abstract

Objectives:

To report a case of multifocal fibrosclerosis with a nine-year follow up, and to discuss this disease's radiological appearance and management. The disease is a rare systemic disorder of unknown cause characterised by fibrous proliferation involving multiple anatomical sites.

Case report:

A 50-year-old woman presented with histological findings characterised by similar inflammatory processes involving the meninges, pituitary gland, peritoneum, retroperitoneum and orbits, prompting a search for a common pathophysiology. A diagnosis of multifocal fibrosclerosis was postulated. Symptom improvement was noted after treatment with prednisone and azathioprine.

Conclusion:

This is the first documented case of involvement of the cochleovestibular nerve in a patient with multifocal fibrosclerosis. The rare association between fibrotic diseases and masses showing various clinical patterns should be kept in mind by otolaryngologists, and imaging performed to investigate for multifocal fibrosclerosis. However, diagnosis can only be confirmed with tissue biopsy and histopathological examination.

Keywords

Multifocal FibrosclerosisCochleovestibular NerveOrbital PseudotumorRetroperitoneal FibrosisSensorineural Hearing LossPachymeningitisMagnetic Resonance Imaging
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 126 , Issue 9 , September 2012 , pp. 935 - 938
Copyright
Copyright © JLO (1984) Limited 2012

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References

1Orabi, AA, Negam, A, Bulman, C. Subglottic and tracheal stenosis associated with multifocal fibrosclerosis. Acta Otolaryngol 2007;127:662–6CrossRefGoogle ScholarPubMed
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