SLOWLY PROGRESSIVE PURE WORD DEAFNESS

Among the reports of primary progressive aphasia, there are few about patients who exhibited progressive pure word deafness with detailed au ditory and radiological examination as well as neuropsychological asse ssment. We describe a 67-year-old right-handed man who exhibited slowl y progressive pure word deafness over a period of 9 years without exhi biting any other cognitive or mental deterioration. Magnetic resonance imaging of his brain revealed generalized cortical atrophy, particula rly in the left superior temporal region. Auditory examination reveale d severe disability in discriminating each syllable or mora of Japanes e words despite adequate auditory acuity. He also showed impairment in temporal auditory discrimination assessed by the click fusion test an d the click counting test. His ability to discriminate meaningful envi ronmental sounds was mildly impaired. We discuss the pathophysiology o f slowly progressive pure word deafness over a period of many years wh ich was not complicated by other language or cognitive dysfunctions.