|Year : 2011 | Volume
| Issue : 4 | Page : 167-169
Urmila Gurung1, Anurag2, KS Ullah3, RP Shrivastav1, H Bhattarai1
1 Ganesh Man Singh Memorial Academy of ENT-HN Studies, Tribhuvan University Teaching Hospital, Institute of Medicine, Kathmandu, Nepal
2 Department of ENT, All India Institute of Medical Sciences, New Delhi, India
3 Department of ENT, Sri Devraj Medical College, Kolar, Karnataka, India
|Date of Web Publication||29-Mar-2012|
Ganesh Man Singh Memorial Academy of ENT-HN Studies, Tribhuvan University Teaching Hospital, Institute of Medicine, Kathmandu
Source of Support: None, Conflict of Interest: None
Otitic hydrocephalus is a rare intracranial complication of middle ear infection. It presents with features of raised intracranial pressure without any neurological deficits. Its exact pathogenesis is still not known but most associate it with lateral sinus thrombosis. Magnetic resonance imaging and Magnetic resonance venography are useful in diagnosis. We present a case of otitic hydrocephalus and discuss its clinical findings, pathogenesis, diagnostic tools, and treatment modalities.
Keywords: Intracranial, Otitic hydrocephalus, Cerebrospinal fluid (CSF), MRV
|How to cite this article:|
Gurung U, Anurag, Ullah K S, Shrivastav R P, Bhattarai H. Otitic hydrocephalus. Indian J Otol 2011;17:167-9
| Introduction|| |
Otitic hydrocephalus is a rare intracranial complication of middle ear infection. It has been described as a syndrome of raised intracranial pressure during or following middle ear infection with normal cerebrospinal fluid (CSF) findings, spontaneous recovery, and no abscess. , Taylor was the first to describe it in 1880, but it was only in 1897 when it became a recognized condition in literature after Quincke published a detailed report of it. ,, He had termed it as serous meningitis then. Several other terms like pseudotumor cerebri, toxic hydrocephalus, meningeal hypertension, benign raised intracranial tension, and otitic hydrocephalus have been used over the years. , The latter term was coined by Symonds in 1931. He reported three cases of increased intracranial pressure following chronic and acute otitis media with no clinical or serological evidence of meningeal inflammation.  It remains the accepted term in the literature , in spite of being a misnomer because it can occur in the absence of otitis and dilated ventricles.
We report a case of otitic hydrocephalus treated at our institute and present literature review of the same.
| Case Report|| |
A 24-year-old male patient presented with bilateral scanty, foul-smelling, purulent, blood-tinged ear discharge for 10 years, deep-seated headache for 10 days, and blurring of vision for 4 days. On examination, the patient had right lateral rectus palsy [Figure 1] and bilateral papilledema. There was aural polyp bilaterally. Following aural polypectomy, the pars tensa was seen to be grossly retracted bilaterally. In addition, in the right side, there was posterosuperior retraction pocket with discharge and destruction of the posterosuperior part of bony meatus adjacent to the posterior attic, while in the left side there was anterior attic destruction. Rinnie was negative bilaterally while weber was central. Lumbar puncture was done. Though the opening pressure was not measured, the microscopic findings were normal. Contrast enhanced computerized tomography (CECT) scan on head and temporal bone showed poorly aerated mastoid air cells with no focal lesions in the brain and normal ventricular system and cisterns [Figure 2]. The patient underwent right modified radical mastoidectomy with grafting. Cholesteatoma and granulation tissue was removed from the mesotympanum, attic, aditus, antrum, periantral cells, and sinodural angle. The sinus plate and dural plate were intact. The patient's headache and blurring of vision persisted even after the surgery, so Magnetic resonance imaging (MRI) with magnetic resonance venography (MRV) was carried out [Figure 3]. It showed thrombosis extending from the lateral sinus to the superior sagittal sinus. The patient was treated with IV antibiotics, steroids, mannitol, acetazolamide, and anticoagulants. The patient was discharged after 44 days. He had no diplopia and his vision was normal at the time of discharge.
|Figure 2: CECT scan of temporal bone showing poorly aerated mastoid air cells|
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| Discussion|| |
Intracranial complications have declined remarkably since the advent of antibiotics. , Though these complications are less common these days than in the preantibiotic era, they still occur and may still be fatal.  Any patient with prior otologic symptoms presenting with headache, diplopia, photophobia, fever, nausea, vomiting and altered sensorium is suggestive of intracranial complications. Such patients need further evaluation. ,,
Otitic hydrocephalus is a rare complication of middle ear infection following acute or chronic otitis media. , The onset may be many weeks after an acute otitis media or many years after chronic otitis media. 
Clinically, otitic hydrocephalus presents with symptoms of increased intracranial pressure in the presence of acute or chronic otitis media.  On examination, papilledema and lateral rectus palsy are usually present. Focal neurological signs are absent except lateral rectus palsy, which actually is a false localizing sign of raised intracranial due to VI th nerve stretching. , Because it presents with features common to all causes of raised intracranial pressure, it is a diagnosis of exclusion. 
The exact mechanism of otitic hydrocephalus is still not clear though association with lateral venous sinus thrombosis is often encountered. ,,, Symonds (1937) suggested that the features seen in otitic hydrocephalus would be explained by the presence of a nonobstructing mural thrombosis extending from the lateral sinus into the sagittal sinus. This impedes the CSF resorption by pacchionian bodies thus increasing the CSF pressure. ,, The superior sagittal sinus is often in continuity with the right lateral venous sinus, with the ratio being 6:4 or 7:3, so it is expected to occur frequently in right-sided ear disease.  Our patient also could have developed otitic hydrocephalus due to his right ear disease. However, superior sagittal sinus thrombosis is associated with more neurological deficits than are found in otitic hydrocephalus, so their relation is still doubted. [1,9] Evidence of such a thrombus partially obstructing the sagittal sinus has been demonstrated, but it has not been proven whether it was a coincidental finding or the pathology behind otitic hydrocephalus.  In our case, the clinical findings were suggestive of otitic hydrocephalus and not as expected in superior sagittal sinus thrombosis in spite of the thrombosis involving the superior sagittal sinus.
Impeded venous drainage into the neck in the presence of lateral sinus thrombosis has been proposed as another mechanism, especially if the thrombus occurs in the dominant lateral sinus.  There is a wide anatomical variation in the venous drainage of the intracranial contents, , so it may be the reason why not all episodes of lateral sinus thrombosis lead to otitic hydrocephalus.  The fact that ligation of the internal jugular vein in the neck does not cause hydrocephalus weakens this proposed mechanism. 
Gower and McGuirt (1983) considered otitic hydrocephalus as an idiopathic benign intracranial hypertension associated with ear disease and categorized raised intracranial pressure following lateral sinus thrombosis as a different entity. 
There are other mechanisms described in literature. Sahs and Joynt postulated that the hydrocephalus is secondary to brain oedema. This was based on brain biopsies that revealed interstitial oedema.  Some authors considered it to be a vasomotor reflex phenomenon in response to the thrombosed epithelium of the vein. ,,
Radiological investigations like MRI and MRV help in diagnosis  and rule out other intracranial complications, specifically any space-occupying lesion, before carrying out lumbar puncture.  MRI is the best imaging modality since it gives a superior evaluation of the venous sinuses, has high sensitivity to blood flow rates, and excellent soft tissue resolution to detect intravascular thrombi. , MRV on the other hand facilitates detection of intrasinus thrombosis and assesses its extent. , Increased intracranial pressure with clinical symptoms and papilledema in the absence of ventricular dilatation or meningitis clinches the diagnosis. 
Treatment of otitic hydrocephalus involves eradication of ear disease and decreasing the intracranial pressure, and preventing potential devastating complications of optic atrophy.  Acute ear disease may recover with medical treatment. ,
Chronic ear disease requires surgical treatment, which includes complete mastoidectomy with eradication of middle ear and mastoid disease, drainage of perisinus abscess, and clot removal.  Ligation of the internal jugular vein is done only when sepsis continues even with adequate surgical procedure and antibiotic coverage.  Use of anticoagulants is limited and is considered only when the spreading thrombus has reached intracranial sinuses  as the risk of neurologic sequelae and death is very high. 
Decreasing the intracranial pressure is important so as to prevent visual impairment due to papilledema. Medical treatments include usage of steroids, diuretics, and hyperosmolar dehydrating agents like mannitol. If medical treatment fails to normalize the intracranial pressure, continuous or serial lumbar drainage can be performed, , but with the risk of cerebellar coning.  Other CSF drainage procedures like thecoperitoneal shunting may occasionally be needed.  Optic nerve sheath fenestration is an effective means to decompress the nerve by reducing the CSF pressure on the nerve and thus improve vision.  Our patient underwent right modified radical mastoidectomy under general anesthesia. He received high-dose intravenous antibiotics, steroids, mannitol, acetazolamide, and anticoagulants. Anticoagulants were used since the thrombus had reached the superior sagittal sinus. His condition gradually improved following this treatment with no neurological sequelae.
| Conclusion|| |
Otitic hydrocephalus is a rare intracranial complication of middle ear infection. Because it presents with features of raised intracranial pressure, other conditions causing raised intracranial pressure have to be ruled out before diagnosing it. MRI and MRV are helpful in its diagnosis. Treatment is focused on eradicating the middle ear disease and lowering the intracranial pressure.
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[Figure 1], [Figure 2], [Figure 3]