Pure word deafness in a patient with systemic lupus erythematosus.

Pure word deafness is characterized by an impainnent of auditory comprehension and repetition of speech while a preservation of reading, writing, spontaneous speech and ability to comprehend non-verbal sounds. It is caused by trauma, infection, tumor or infarction in the temporal lobe of brain. In most cases, the lesions are bilateral, in the middle third of superior temporal gyri, but in a few cases unilateral lesions of dominant temporal lobe are localized. We experienced a case of 45 year-old right handed woman with systemic lupus erythematosus(SLE) who presented pure word deafness after left temporal lobe infarction including primary auditory center. Her auditory comprehension was impaired, while reading and visual comprehension were normal. Audiometric tests and auditory evoked potentials disclosed no hearing defect. Brain MRI showed a left temporal lobe infarction without abnonnal vascular finding in MRA. She was diagnosed as pure word deafness due to SLE vasculitis and managed with high dose steroid and cyclophosphamide therapies.