Download CASE REPORT: A RARE CASE OF CEREBRAL VENOUS SINUS

CASE REPORT: A RARE CASE OF CEREBRAL VENOUS SINUS THROMBOSIS
PRESENTING WITH UNILATERAL SENSORINEURAL HEARING LOSS
INTRODUCTION
Thrombosis of the cerebral veins and sinuses is a distinct cerebrovascular
disorder that, unlike arterial stroke, most often affects young adults and children mainly
females (1). Hearing loss is an unusual presentation of central venous sinus
thrombosis. The symptoms and clinical course can be highly variable. Recently, few
studies and case reports have highlighted the incidence of cerebral sinus venous
thrombosis (CSVT) in association with otological symptoms such as hearing loss and
tinnitus; and few even suggest these features as being prodrome of cerebral sinus
venous thrombosis (2). CSVT in a adult male is a very rare entity and even rarer is the
otological symptoms being the presenting feature. We present such a case of cerebral
sinus venous thrombosis who presented with sudden onset unilateral sensorineural
hearing loss and tinnitus.
Case Report:
A 31 years old non diabetic, normotensive male, occasional smoker and tobacco
chewer, presented with history of tinnitus (L) & sudden onset hearing loss (L) since 25
days. Patient denied any history of otorrhoea, otalgia, aural fullness and vertigo. He
gave no history of recent head trauma, acoustic trauma or exposure to ototoxic drugs.
There was no history of fever, headache, seizures and loss of consciousness,
altered sensorium and weakness of any part of body, projectile vomiting, and diminution
of vision. No history of use of any medication for long term.
On examination, patient was fully conscious and oriented in time, place and
person with Glasgow coma scale 15/15. He was afebrile, with normal heart rate and
blood pressure. There were no signs of meningitis.
Neurological and other systemic examination revealed no abnormality. Otoscopic
examination revealed normal tympanic membrane bilaterally. Free field hearing and
tuning fork tests revealed normal hearing in right ear and severe sensorineural hearing
loss in left ear. The otoneurological examination was essentially normal.
Pure tone audiometry was done which revealed showed severe sensorineural
hearing loss in left ear and normal hearing sensitivity in right ear.
Pure Tone Audiometry of patient
MRI & MRA Brain and inner ear showed Left transverse sinus, sigmoid sinus and
Internal Jugular Vein thrombosis. The cochlea, semicircular canals, auditory nerves,
internal acoustic meatus posterior cranial fossa and cerebellopontine angle were normal
on both sides.
MRI Brain
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 (3). 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 (1, 4).
The clinical manifestations of CSVT are different from that of arterial stroke.
Presenting symptoms may show great diversity depending on its pathophysiology (1)
.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 (5). 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 (6). 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 (2, 4, 7).
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.