Cochleovestibular nerve involvement in multifocal fibrosclerosis

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.

Palavras-chave

Multifocal Fibrosclerosis, Cochleovestibular Nerve, Orbital Pseudotumor, Retroperitoneal Fibrosis, Sensorineural Hearing Loss, Pachymeningitis, Magnetic Resonance Imaging

URI

https://observatorio.fm.usp.br/handle/OPI/1271

Referências

KITANO A, 1995, INTERNAL MED, V34, P267, DOI 10.2169/internalmedicine.34.267
Oguz KK, 2002, EUR RADIOL, V12, P1134, DOI 10.1007/s00330-001-1158-4
Orabi AA, 2007, ACTA OTO-LARYNGOL, V127, P662, DOI 10.1080/00016480600951467

Coleções

Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - HC/InRad

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