We performed a retrospective study on 7 children in whom the auditory brainstem responses (ABR) showed absence or significantly poor formation of waves II-V. Two patients had degenerative diseases that affected the brains diffusely. In the remaining 5 patients, the symptoms consisted of action tremor (3 patients) or pyramidal signs (2 patients). There were neither clinical signs nor neuroimaging findings suggesting brainstem involvements. The evolutional changes of the ABRs had a good correlation with the clinical course. Absence or significantly poor formation of waves II-V of ABR occurs in various diseases and may not necessarily reflect severe organic brain lesions.