Cockayne's syndrome (CS) is a rare autosomal recessive premature-aging disorder which is clinically characterized by physical and mental retardation, retinal pigmentation, sensorineural deafness and other neurological abnormalities. Auditory brainstem responses (ABRs) and behavioral audiometry were studied in 4 cases of confirmed CS chronologically. In one case, ABRs were normal at first but became abnormal. Initially, ABRs revealed only wave I with prolonged latency and thereafter no response. In another case, ABRs revealed the absence of all waves beyond wave III and then revealed the disappearance of all waves. In 2 other cases, ABRs were absent from the first testing. Behavioral levels were elevated in all 4 cases. In 2 cases, behavioral levels did not change in spite of partial disappearance of ABR waves, but they had elevated severely since all waves including wave I disappeared. Our findings suggest that the disease spreads from the upper brainstem to the cochlear nerve and that the site of the lesion causing hearing loss in CS is in the brainstem lesion as well as the peripheral one.