Cortical hearing impairment is a rare condition affecting the bilateral temporal and It is mostly associated with cardiovascular disease. We report. a 35-year-old woman with a history of arrhythmia. Her chief complaint was sudden bilateral deafness for 3 weeks. Initial pure tone audiomertry showed bilateral severe sensorineural hearing loss; speech discrimination could not be tested. The acoustic reflex and auditory brainstem response were normal. Her pure tone test dramatically improved to nearmal normal a few days after admission, but she began to complain of loss of comprehension of spoken words. Brain MIR showed encephalomalacia of the right temporal lobe and abnormal enhancement of the left temporparietal cortex. Electroencephalography (EEG) revealed an abnormal slow wave. All the above were compatible with cortical hearing impairment with cortical deafness progressing to word deafness. Owing to sick sinus syndrome, a pacemaker was implanted. She also received speech rehabilitation and progressively regained the ability to communicate.