Hostname: page-component-586b7cd67f-g8jcs Total loading time: 0 Render date: 2024-11-24T19:21:57.748Z Has data issue: false hasContentIssue false
  • English
  • Français

Superficial siderosis of the central nervous system: an underestimated cause of hearing loss

Published online by Cambridge University Press:  29 June 2007

M. L. Castelli  and
A. Husband
M. L. Castelli*
Affiliation:
Department of Otorhinolaryngology, Queen Alexandra Hospital, Cosham, Portsmouth, UK.
A. Husband
Affiliation:
Department of Otorhinolaryngology, Queen Alexandra Hospital, Cosham, Portsmouth, UK.
*
Address for correspondence: Marco Castelli, F.R.C.S., Department of Otolaryngology, Queen Alexandra Hospital, Cosham, Portsmouth, Hants P06 3LY. Fax: 01705 286708
Get access Rights & Permissions [Opens in a new window]

Abstract

Superficial siderosis of the central nervous system (CNS) is a rare disease resulting in the accumulation of haemosiderin in the meninges, the brain surface, the spinal cord and the cranial nerves. The pigment is deposited as a result of chronic bleeding in the subarachnoid space. This produces a clinical picture of deafness, ataxia, cranial nerve deficits and in the latest stages dementia. In some cases the source of bleeding can be identified, whilst in others it can not. Despite its rarity the disease should be considered in the differential diagnosis of sensorineural deafness, particularly as it is a progressive and in some cases curable disease which is easily diagnosed by magnetic resonance imaging (MRI). In this case report the haemosiderin was derived from an ependymoma of the fourth ventricle with extension into the cerebello-pontine angle. The first symptom was a worsening sensorineural hearing loss.

Keywords

SiderosisCentral nervous systemHearing losssensorineuralEpendymoma
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 111 , Issue 1 , January 1997 , pp. 60 - 62
Copyright
Copyright © JLO (1984) Limited 1997

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

Bonito, V., Agostinis, C., Ferraresi, S., Defanti, C. A. (1994) Superficial siderosis of the central nervous system after brachial plexus injury. Case report. Journal of Neurosurgery 80 (5): 931–934.Google Scholar
Bracchi, M., Savoiardo, M., Triulzi, F., Daniele, D., Grisoli, M., Bradac, G. B., Agostinis, C., Pelucchetti, D., Scotti, G. (1993) Superficial siderosis of the CNS: MR diagnosis and clinical findings. American Journal of Neuroradiology 14 (1): 227–236.Google Scholar
Daniele, D., Bracchi, M., Riva, A., Duca, S., Stura, G., Bradac, G. B. (1992) Superficial siderosis of the central nervous system: neuroradiological evaluation of two cases. European Neurology 32 (5): 270–273.Google Scholar
deSouza, N. M., Cox, T. C., Hoare, R. D., Clarke, C. R. (1993) Cerebral siderosis: a complication of anticoagulant therapy? American Journal of Neuroradiology 14(3): 774–776.Google Scholar
Gomori, J., Grossmann, R., Bilaniuk, L., Zimmerman, R., Goldberg, H. (1985) High field MRI of superficial siderosis of central nervous system. Journal of Computer Assisted Tomography 9: 972–975.Google Scholar
Grunshaw, N. D., Blanshard, K. S., Hussain, S. S., Grace, A. R. (1993) Superficial siderosis of the central nervous system. Diagnosis by magnetic resonance imaging. Clinical Radiology 48(3): 186–188.Google Scholar
Ivanovski, K., Olzewski, J. (1960) The effect of subarachnoid injections of iron containing substances on the central nervous system. Journal of Neuropathology and Experimental Neurology 19: 433–448.Google Scholar
Janss, A. J., Galetta, S. L., Freese, A., Raps, E. C., Curtis, M. T., Grossman, R. I., Gomori, J. M., Duhaime, A. C. (1993) Superficial siderosis of the central nervous system: magnetic resonance imaging and pathological correlation. Case report. Journal of Neurosurgery 79: 756–760.Google Scholar
Koeppen, A. H., Dickson, A. C., Chu, R. C., Thach, R. E. (1993) The pathogenesis of superficial siderosis of the central nervous system. Annals of Neurology 34: 646–653.Google Scholar
Kwartler, J. A., De La Cruz, A., Lo, W. W. (1991) Superficial siderosis of the central nervous system. Annals of Otology, Rhinology and Laryngology 100: 249–250.Google Scholar
Lai, M. T., Ohmichi, I., Yuen, K., Egusa, K., Yorizane, S., Masuda, Y. (1995) Superficial siderosis of the central nervous system: a case with an unruptured intracranial aneurysm. Journal of Laryngology and Otology 10: 549–552.Google Scholar
Mamourian, A. C. (1993) MR of superficial siderosis. American Journal of Neuroradiology 14: 1445–1448.Google Scholar
Offenbacher, H., Fazekas, F., Reisecker, F., Schmidt, R., Payer, F., Lechner, H. (1991) Superficial siderosis of the spinal cord: a rare cause of myelopathy diagnosed by MRI. Neurology 41: 1987–1989.Google Scholar
Parnes, S. M., Weaver, S. A. (1992) Superficial siderosis of the central nervous system: a neglected cause of sensorineural hearing loss. Otolaryngology – Head and Neck Surgery 107: 69–77.Google Scholar
Pyhtinen, J., Paakko, E., Ilkko, E. (1995) Superficial siderosis in the central nervous system. Neuroradiology 37: 127–128.Google Scholar
Pribitkin, E. A., Rondinella, L., Rosenberg, S. I., Yousem, D. M. (1994) Superficial siderosis of the central nervous system: an underdiagnosed cause of sensorineural hearing loss and ataxia. American Journal of Otology 15: 415–418.Google Scholar
Shervin, I., Toll, K. (1972) Superficial siderosis of the central nervous system. Diseases of Central Nervous System 6: 413–417.Google Scholar
Spoto, G., Press, G., Hesselink, J., Solomon, M. (1990) Intracranial ependymoma and subependymoma: MR manifestations. American Journal of Neuroradiology 11: 83–91.Google Scholar
Tomlinson, B., Walton, J. (1964) Superficial haemosiderosis of the central nervous system. Journal of Neurology, Neurosurgery and Psychiatry 27: 332–339.Google Scholar
Willeit, J., Aichner, F., Felber, S., Berek, K., Deisenhammer, F., Kiechl, S. G., Gerstenbrand, F. (1992) Superficial siderosis of the central nervous system: report of three cases and review of the literature. Journal of the Neurological Sciences 111: 20–25.Google Scholar
Zimmermann, R., Hesselink, J., Bilaniuk, L. (1988) Bilateral pial siderosis and hearing loss: syndrome with negative CT and positive high field MR findings. Radiology 169: 312.Google Scholar