A young adult male presented with a 3-week history of tinnitus and vertigo and a 3-day history of ataxia and bilateral deafness. An ataxic gait, staccato speech and bilateral sensorineural deafness were noted on examination, together with massive splenomegaly and extensive bilateral retinal haemorrhages. The blood leucocyte count was 605 × 109/l, with karyotype and blood film morphological features consistent with chronic phase chronic myeloid leukaemia. He was not significantly thrombocytopaenic. A magnetic resonance imaging scan demonstrated leucostasis-induced bilateral intracerebral haemorrhages involving the primary auditory cortex (known as Heschl's gyri), with a further haemorrhage in the right lobe of the cerebellum (indicated by arrows). Haematological remission was induced with leucapheresis, hydroxycarbamide and then imatinib. The deafness has not resolved. Cortical deafness is rare and usually occurs following damage to the bilateral temporal or temporoparietal lobes, including the primary auditory cortex. This results in the interruption of the projection fibres from the medial geniculate bodies to the auditory-related areas, resulting in severe, persistent hearing loss (Tanaka, Y., Kamo, T., Yoshida, M. & Yamadori, A., 1991, So called cortical deafness: clinical, neurophysiological and radiological obervations. Brain, 114, 2385-2401).
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