Deaf and Dizzy. Have We Been Susacked?

The diagnostic procedure performed in this case was a cerebral biopsy, which established the diagnosis of intravascular lymphoma. Like syphilis and sarcoidosis, intravascular lymphoma has been called the oncologist's ‘great imitator', due to the variety of CNS symptoms which can develop. In our patient, further work up included a normal bone marrow biopsy, normal chest and abdomen CT yielding no evidence of systemic lymphoma. Treatment with Methotrexate, Rituximab, and CHOP for an advanced lymphoma is currently in progress. The clinical phenotype in our case is best approached as a "cochleo-encephalopathy syndrome". The association of acute, unilateral cochleo-vestibular loss, acute sensori-neural hearing impairment in the fellow ear, visual blurring and hallucinations, dysphasia, confusion and incontinence were the essential manifestations. MRI showed multiple, acute "microinfarcts" involving several vascular territories. Angiographic sparing of large and mid-size arteries, suggests a microangiopathy, targeting pre-capillary arterioles with a lumen of <100 mM. Both Susac's and Sjogren's syndrome were considered prior to tissue diagnosis, however there was no ancillary evidence for these diagnoses. It is unclear if the spinal cord syndrome 17 months earlier was related to his current diagnosis.