Congenital X-linked Stapes Gusher Syndrome in an Infant. A Case Report.

Congenital X-linked deafness with stapes gusher is a rare, but well-known clinical entity with characteristic radiological features. Recognition of these findings is important as it changes the treatment course and precludes stapedectomy, which if done can lead to stapes gusher in these patients. We present the case of a nine-month-old male infant with hearing loss. CT scan showed typical radiological features of X-linked stapes gusher syndrome. In addition, the superior semicircular canals appeared dehiscent which could be due to immature ossification or less likely due to defective development as part of the disease. To the best of our knowledge this is the youngest patient prospectively identified by imaging in the English medical literature.

Persistent trigeminal artery and associated vascular variations.

Persistent trigeminal artery (PTA) is the most frequent type of persistent primitive carotid-basilar artery anastomoses. Persistent trigeminal artery is usually associated with small or absent posterior communicating artery (Pcom). It may also be associated with hypoplastic vertebral and basilar arteries. Patients may present with symptoms related to hypo-perfusion of posterior circulation or microembolization from carotid artery to posterior circulation. We are presenting a 60-year-old who had recurrent episodes of vertigo and parasthesia, in whom MR angiography revealed a medial, Saltzman type 1 PTA on the left with absent ipsilateral Pcom, duplicated ipsilateral superior cerebellar artery, hypoplastic A1 segment of contralateral anterior cerebral artery (ACA), azygous ACA and hypoplastic ipsilateral vertebral artery.

Perirolandic sparing and pituitary apoplexy in adult brain with global hypoxia. A case report.

Global hypoxia in adult brain usually manifests as ischemic lesions in watershed territories. Acute profound hypoxia involves the cortex especially the perirolandic area, white matter and deep grey matter. Perirolandic sparing in adult global hypoxia is not described in literature. Few cases of perirolandic sparing are described in conditions like anoxia in term infants in the post-neonatal period and hepatic encephalopathy. We report a case of global hypoxia in adult brain with perirolandic sparing and unique association with pituitary apoplexy. It is well known that the "diving reflex" redistribution of cerebral blood flow to the high metabolically active regions occurs when the hypoxic insult is prolonged and partial. The perirolandic sparing in our case could be explained by this phenomenon, wherein the patient acutely developed hypoxia, which was profound and prolonged but not prolonged enough for deep grey matter sparing.