SUMMARY Cortical deafness in a patient with multiple sclerosis is reported. Complete recovery from total deafness was seen following stages of auditory agnosia and pure word deafness. The otological and neurophysiological studies suggested lesions in subcortical white matter. This report stresses the rarity of the condition, its subcortical origin and good prognosis.Cortical deafness is a rare condition, seen in patients with bilateral temporal lobe lesions.' In multiple sclerosis (MS) deafness due to lesions at the lower levels of the auditory pathway is seen in a few per cent2 3 and audiometric examination increases the incidence.4 5 Cortical deafness, however, is rarely observed.3 This report concerns a case of MS with transient cortical deafness and describes the results of otological and neurophysiological studies and its natural course. A preliminary report was published elsewhere.6Case report A 29-year-old right handed man had an acute onset in March, 1977, of numbness on the right side of the body below the neck without any previous symptoms, followed by quadriplegia with some diplopia in April. He was admitted first to Kyushu University Hospital, when neurological examination revealed horizontal nystagmus on lateral gaze, positive Lhermitte's sign, marked quadriplegia with increased knee and ankle jerks, Babinski signs, and some sensory impairment below the level of C5. Cerebrospinal fluid (CSF) contained 0635 g/l total protein (normal range 0-1A4 g/l), 0 108 g/l IgG (0O005-0045 g/l), IgG ratio 1-6 (less than 1-5), and 11 lymphocytes (less than 5) mm-3. Other laboratory data, EEG and CT scan were normal. A diagnosis of acute disseminated encephalomyelitis was made, and corticosteroids were given; the patient recovered.