Progressive loss of hearing and balance in superficial siderosis due to occult spinal dural defects.

PURPOSE: Superficial siderosis, a progressive, debilitating, neurological disease, often presents with bilateral impairment of auditory and vestibular function. We highlight that superficial siderosis is often due to a repairable spinal dural defect of the type that can also cause spontaneous intracranial hypotension. METHODS: Retrospective chart review of five patients presenting with moderate to severe, progressive bilateral sensorineural hearing loss as well as vestibular loss. All patients had developed superficial siderosis from spinal dural defects: three after trauma, one after spinal surgery and one from a thoracic discogenic microspur. RESULTS: The diagnosis was made late in all five patients; despite surgical repair in four, hearing and vestibular loss failed to improve. CONCLUSIONS: In patients presenting with progressive bilateral sensorineural hearing loss, superficial siderosis should be considered as a possible cause. If these patients also have bilateral vestibular loss, cerebellar impairment and anosmia, then the diagnosis is likely and the inevitable disease progress might be halted by finding and repairing the spinal dural defect.

Transverse Sinus Stenting Reverses Medically Refractory Idiopathic Intracranial Hypertension.

Aim: To characterise the ophthalmic indications for, and ophthalmic efficacy of, transverse sinus stenting in adults with medically refractory idiopathic intracranial hypertension. Methods: A retrospective cohort study was undertaken on a single-author database of 226 successive patients with confirmed idiopathic intracranial hypertension (IIH). A total of 32 patients were identified who received a transverse sinus stent for medically refractory disease. This which was defined as visual threat and/or intolerance of maximal medical therapy. Patients with medically refractory disease proceeded to stenting, if found to have a significant transverse sinus stenosis gradient at catheter venography. Visual threat was quantified via the degree of papilledema on optical coherence tomography of the retinal nerve fibre layer, and via the visual field mean deviation. CSF opening pressure at lumbar puncture and cerebral venous sinus pressure measurements from catheter venography were correlated with the ophthalmic data, noting also intolerance of maximal medical therapy. Complications of stenting were fully assessed. Results: Medically refractory IIH was found in 18% of the total cohort of IIH patients. 90% of those with medically refractory disease had a significant transverse sinus stenosis pressure gradient, and 80% proceeded to stenting. The intervention eliminated papilledema in 96% of stented patients, and allowed 81% to cease acetazolamide. The need for a further procedure was low at 6%, and the safety profile was favourable. Conclusions: Medically refractory disease in IIH is common (18%), and nearly always associated with a significant transverse sinus stenosis pressure gradient (90%). Endovascular stenting of the stenosis deserves wider uptake as a highly effective, safe, and usually definitive treatment. It safeguards vision by eliminating papilledema (96%), and allows most patients to cease acetazolamide (81%). By analogy with glaucoma, if acetazolamide is the prostaglandin of IIH and CSF diversion the emergency glaucoma filter, stenting is the minimally invasive glaucoma surgery.

Fatal reversible cerebral vasoconstriction syndrome.

We report four fatal cases of fulminant reversible cerebral vasoconstriction syndrome, all initially diagnosed as primary central nervous system vasculitis and treated with corticosteroids. Although reversible cerebral vasoconstriction syndrome is usually self-limiting without permanent neurologic deficits, rarely it can be fatal and worse outcomes have been associated with corticosteroid treatment.

Venous hypertension as the cause of intracranial hypertension in patients with transverse sinus dural arteriovenous fistula.

We describe 2 patients with transverse sinus dural arteriovenous fistulas (DAVFs) who presented with headache and papilledema due to intracranial hypertension. It has been proposed, but never proven, that venous hypertension causes the intracranial hypertension in DAVF. The data from our patients support this hypothesis. An additional factor leading to intracranial hypertension could be stenosis of the fellow transverse sinus.

Contrast-induced transient cortical blindness.

We present a case of transient cortical blindness secondary to contrast medium toxicity. A 58-year-old man had successful endovascular coiling of a right posterior inferior cerebellar artery aneurysm but became confused and unable to see after the procedure. His visual acuity was no light perception bilaterally. Clinically, there was no new intra-ocular pathology. An urgent non-contrast computed tomography scan of the brain showed cortical hyperdensity in both parieto-occipital cortices, consistent with contrast medium leakage through the blood-brain barrier from the coiling procedure. The man remained completely blind for 72 hours, after which his visual acuity improved gradually back to his baseline level.

Imaging of endolymphatic hydrops in Meniere's disease at 1.5 T using phase-sensitive inversion recovery: (1) demonstration of feasibility and (2) overcoming the limitations of variable gadolinium absorption.

Endolymphatic hydrops is the primary histopathological finding in Meniere's disease. We demonstrate the feasibility of imaging endolymphatic hydrops at 1.5 T using phase-sensitive inversion recovery (PS-IR) MRI following intratympanic injection of gadolinium (Gd). PS-IR data were imaged using real reconstruction to enable visualization of the phase of the signal permitting clear definition between bone, unopacified endolymph and perilymph. Data were obtained 24h following injection in 2 control subjects and in 13 successive patients with Meniere's disease. In 11 out of 13 patients, dilated endolymphatic structures were clearly identified as filling defects within the opacified perilymph allowing identification of endolymphatic hydrops. There was a large range in the degree of perilymphatic signal enhancement due to variability in absorption of Gd from the middle ear into the perilymph. The use of multiple TI values allowed confident identification of endolymphatic hydrops in Meniere's patients even when perilymph opacification was suboptimal at one TI value. This is the first time endolymphatic hydrops has been demonstrated at 1.5 T in humans. The methods presented are of significant practical importance and will permit broader application of endolymphatic imaging and may also act to reduce the frequency of failed exams due to inadequate Gd uptake.

Interventional treatment of carotid cavernous fistula.

We reviewed retrospectively the outcomes of interventional endovascular treatment of direct or dural (indirect) carotid cavernous fistulas in 24 consecutive patients requiring endovascular treatment at Royal Prince Alfred Hospital between 1994 and 2009. Data was collected from each patient's neurological, ophthalmological and radiological reports. Of the 12 patients with direct fistulas all had signs of orbital and ocular venous congestion and ophthalmoplegia; nine also had reduced vision ranging from 6/9 to nil perception of light, two had normal vision and one was unconscious. Nine of the 12 direct fistulas were embolized transarterially, two transvenously, one by a combination of both approaches and all were successfully closed. After treatment, seven of the nine patients with reduced vision had complete or nearly complete restoration of vision,while two who presented with nil perception of light (one in both eyes) had no recovery of vision. In contrast, seven of the 12 patients with dural fistulas had ophthalmoplegia, three had reduced vision, down to 6/24 and one did not have any sign of venous congestion. Vision recovered in all three patients after embolization of the dural fistula. Dural fistulas were embolized transvenously in 11 and transarterially in one patient. Apart from ophthalmoplegia, all other ocular signs and symptoms rapidly resolved after closure of the fistula in each of the 24 patients. The diagnosis was delayed by being missed either during the first admission or at the first specialist consultation in three of the 12 patients with direct fistulas, and in seven of the 12 patients with dural fistulas. One patient with a direct and another with a dural fistula had limited cerebral infarctions during embolization. In this series, endovascular interventional treatment of carotid cavenous fistulas restored visual loss in 10 of 12 patients with visual loss. The two who did not recover had presented with nil perception of light, one after a delay in diagnosis of 6 weeks. Some degree of ophthalmoplegia tended to remain. This emphasizes the need for early diagnosis and treatment before visual loss or ophthalmoplegia becomes severe.