Cochleovestibular nerve involvement in multifocal fibrosclerosis

J Laryngol Otol. 2012 Sep;126(9):935-8. doi: 10.1017/S0022215112001387. Epub 2012 Jul 5.

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.

Publication types

  • Case Reports

MeSH terms

  • Azathioprine / therapeutic use
  • Biopsy
  • Diagnosis, Differential
  • Disease Progression
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Meningitis / complications
  • Meningitis / diagnosis
  • Meningitis / pathology*
  • Middle Aged
  • Orbital Diseases / complications
  • Orbital Diseases / diagnostic imaging
  • Orbital Diseases / pathology
  • Prednisone / therapeutic use
  • Radiography
  • Recurrence
  • Retroperitoneal Fibrosis / complications
  • Retroperitoneal Fibrosis / congenital*
  • Retroperitoneal Fibrosis / diagnosis
  • Retroperitoneal Fibrosis / pathology
  • Retroperitoneal Fibrosis / therapy
  • Seizures / complications
  • Sensation Disorders / complications*
  • Sensation Disorders / pathology
  • Sinusitis / complications
  • Vestibulocochlear Nerve Diseases / complications
  • Vestibulocochlear Nerve Diseases / pathology*

Substances

  • Glucocorticoids
  • Immunosuppressive Agents
  • Azathioprine
  • Prednisone

Supplementary concepts

  • Multifocal fibrosclerosis