Case report



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MRA Brain

Neurology consultation was done and patient was started with inj LMWH 60 mg subcutaneously q12h, and later switched to oral anticoagulants.



Patient showed significant improvement in hearing. BERA done after 6 days of anticoagulation showed wave V at 30 dB nHL in both ears indicating return of normal hearing acquity.





BERA

Discussion:

Cerebral sinus venous thrombosis accounts for approximately 0.5% of all cerebrovascular diseases . It is less prevalent than arterial stroke and usually affects young female patients. The risk factors for venous thrombosis are stasis of blood and changes in the blood composition that are best described by Virchow triad. The causative factors of arterial stoke include dehydration, infections, local trauma, rheumatologic diseases like SLE and Behecet’s disease, hypercoagulable states like pregnancy, anti-thrombin III deficiency, nephritic syndrome, cancer and use of oral contraceptives .

The clinical manifestations of CSVT are different from that of arterial stroke. Presenting symptoms may show great diversity depending on its pathophysiology .The symptoms may be related to elevated intracranial pressure such as headache or due to focal ischemia or hemorrhage such as hemiparesis and aphasia. The classical symptoms of CSVT are impaired consciousness, focal neurologic deficits, and generalized seizures . CSVT mainly occurs in young adults, whereas arterial stroke mainly affects elderly people. The cause of CSVT remains unknown in almost a quarter of cases even with all the available investigative modalities.

The incidence of Cerebral venous thrombosis is about 3-4 per million of people each year . It is 3 times more common in Women than in men. A majority of the patients gradually recover from acute episodes of cerebral venous thrombosis, but up to one in eight patients has either chronic disability or death. The early detection of Cerebral venous thrombosis has been enhanced by modern neuro imaging tools especially MRV and digital subtraction angiogram.

The neurological manifestations of CSVT may be vague, such as vomiting, headache, seizures, stroke like features or manifestations of chronic intracranial hypertension such as blurring of vision. Women with pregnancy may present with pregnancy related complications. Thus, physicians should have a fair idea about CSVT and their atypical presentations.

There may be 2 mechanisms causing SNHL in a case of CSVT. The cochlea is drained by the cochlear and the labyrinthine veins into the petrosal or directly the transverse sinus. Thus CSVT might increase inner ear pressure, induce anoxia and impede cochlear function. Also, elevated venous pressure could be transmitted to the endolymph, leading to increased inner ear fluid pressure .

The clinical scenario in this case seems to be the presentation of otologic symptoms secondary to CSVT.

References:

1. Stam J. Thrombosis of the cerebral veins and sinuses. New England Journal of Medicine. 2005;352(17):1791-8.

2. Gattringer T, Enzinger C, Birner A, Wünsch G, Niederkorn K, Walch C, et al. Acute unilateral hearing loss as an early symptom of lateral cerebral sinus venous thrombosis. Archives of neurology. 2012;69(11):1508-11.

3. Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. The Lancet Neurology. 2007;6(2):162-70.

4. Braun EM, Stanzenberger H, Nemetz U, Luxenberger W, Lackner A, Bachna-Rotter S, et al. Sudden unilateral hearing loss as first sign of cerebral sinus venous thrombosis? A 3-year retrospective analysis. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology. 2013;34(4):657-61.

5. Saposnik G, Barinagarrementeria F, Brown RD, Bushnell CD, Cucchiara B, Cushman M, et al. Diagnosis and management of cerebral venous thrombosis a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2011;42(4):1158-92.

6. de Bruijn SF, de Haan RJ, Stam J. Clinical features and prognostic factors of cerebral venous sinus thrombosis in a prospective series of 59 patients. For The Cerebral Venous Sinus Thrombosis Study Group. Journal of neurology, neurosurgery, and psychiatry. 2001;70(1):105-8.

7. Braun EM, Stanzenberger H, Nemetz U, Luxenberger W, Lackner A, Bachna-Rotter S, et al. Sudden unilateral hearing loss as first sign of cerebral sinus venous thrombosis? A 3-year retrospective analysis. Otology & Neurotology. 2013;34(4):657-61.




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