티아민 보충으로 치료한 감각신경성 난청 1예

Wernicke encephalopathy is a severe neurological disorder caused by thiamine (vitamin Bi) deficiency. This disease occurs mostly in alcoholics due to malnutrition or malabsorption of vitamin B1 but also occurs in hyperemesis gravidarum, carcinoma, anorexia nervosa. Auditory neuropathy is characterized by abnormal auditory brainstem and preserved cochlear outer hair cell function. This is a case of Auditory neuropathy caused by thiamine deficiency. Brain MRI scan showed typical Wernicke encephalopathy finding. To evaluate hearing impairment, Pure tone audiogram, Speech audiogram, Otoacoustic emision and Auditory brainstem evoked response were measured and the result were compatible with auditory neuropathy. After thiamine replacement therapy, patient’s hearing imparment and serial speech recognition score showed improvement. From our study, we can assume that thiamine deficiency cause selective destruction of inner ear cell, this will eventually helps us to reveal pathophysi- olology of auditory neuropathy. (J Clinical Otolaryngol 2013;24:225–232)