A case of locked-in syndrome complicated by central deafness

Abstract

Background: A 53-year-old male with a history of hypertension, diabetes mellitus, and factor V deficiency presented to an emergency room with progressively increasing headache, slurred speech, and left upper extremity weakness. Over the previous 3 months, he had been receiving warfarin for prophylaxis of deep venous thrombosis following knee surgery. After presentation and an initial period of coma, he became tetraplegic and anarthric, requiring intubation and ventilatory assistance. Investigations: Neurological examination, CT scan, electroencephalogram, brainstem auditory and visual evoked potential studies, neuropsychological assessment and functional MRI studies. Diagnosis: Locked-in syndrome following ventral pontine hemorrhage, complicated by central deafness secondary to extension of the lesion to the inferior colliculus. Management: Development of an augmentative communication system designed to exploit the patient's preserved cognitive and motor functions.