Abstract
Superficial siderosis is an uncommon condition occurring in central nervous system (CNS) due to deposition of hemosiderin in the subpial meningeal layer causing progressive neurological dysfunction. The classical clinical manifestation is a triad of chronic ataxia, bipyramidal signs and progressive bilateral symmetrical sensorineural hearing loss (SNHL). It has rarely been reported in Indian literature. We report an unusual case of superficial siderosis in a 60-year-old farmer who presented with the above triad along with involvement of olfactory nerve. We present this case to highlight the fact that progressive SNHL can be an important sign for the early awareness of this rare disorder. The literature on superficial siderosis is reviewed and the pathogenesis is discussed.
This is a preview of subscription content, log in via an institution to check access.
References
Levy M, Turtzo C, Llinas RH (2007) Superficial siderosis: a case report and review of the literature. Nat Clin Pract Neurol 3(1):54–58
Cammermeyer J (1947) Deposition of iron in paraventricular areas of the human brain in hemochromatosis. J Neuropathol Exp Neurol 6:111–127
Fearnley JM, Stevens JM, Rudge P (1995) Superficial siderosis of the central nervous system. Brain 118:1051–1066
Sahin S, Agilkaya S, Karsidag S (2006) SS of CNS: an unusual cause for headache and hearing loss. Neurol Asia 11:145–149
Hsu WC, Loevner LA, Forman MS, Thaler ER (1999) Superficial siderosis of the CNS associated with multiple cavernous malformations. Am J Neuroradiol 20:1245–1248
Bracchi M, Savoiardo M, Triulzi F et al (1993) Superficial siderosis of the CNS. MR diagnosis and clinical findings. AJNR Am J Neuroradiol 14:227–236
Kumar N et al (2007) Superficial siderosis—associations and therapeutic implications. Arch Neurol 64(4):491–496
Koeppen AH, Borke RC (1991) Experimental superficial siderosis of the central nervous system. I: morphological observations. J Neuropathol Exp Neurol 50:579–594
Koeppen AH et al (1992) Experimental superficial siderosis of the central nervous system: biochemical correlates. J Neurol Sci 112:38–45
Koeppen AH, Dickson AC, Chu RC, Thach RE (1993) The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol 34:646–653
Fearnley J (2002) Superficial siderosis of the central nervous system. Pract Neurol 2:50–54
Koeppen AH, Dentinger MP (1988) Brain hemosiderin and superficial siderosis of the central nervous system. J Neuropathol Exp Neurol 47:249–270
Offenbacher H, Fazekas F, Schmidt R, Kapellar P, Fazekas G (1996) Superficial siderosis of the central nervous system. MRI findings and clinical significance. Neuroradiology 38:S51–S56
Kumar N et al (2006) Superficial siderosis. Neurology 66:1144–1152
River Y et al (1994) Superficial hemosiderosis of the central nervous system. Mov Disord 9:559–562
Angstwurm K et al (2002) Superficial siderosis of the central nervous system: response to steroid therapy. J Neurol 249:1223–1225
Conflicts of interest
None declared.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ramadass, T., Narayanan, N., Dhanraj, M. et al. Superficial Siderosis of the CNS and Otoneurological Manifestations. Indian J Otolaryngol Head Neck Surg 63, 380–383 (2011). https://doi.org/10.1007/s12070-011-0152-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12070-011-0152-7