Auditory brain stem response abnormalities in severely and profoundly retarded adults.

The auditory brain stem evoked responses (ABRs) of two groups of retarded adults (Down's syndrome and retarded of unknown etiology) were compared with those of a group of non-retarded control subjects as a function of changes in stimulus repetition rate and stimulus intensity. The absence of ABRs at the highest stimulus intensity suggested profound hearing deficits in one or both ears of 4 Down's syndrome and 2 unknown-etiology subjects. Other abnormalities were noted in particular individuals of each group of retarded. In addition, the ABRs of the Down's group as a whole showed a significant pattern of abnormalities. The intervals between peaks I and II and III and IV were shorter than normal white the IV--V interval was prolonged. Wave V also showed abnormally small latency increases at fast click rates. The ABRs of the unknown-etiology group showed a shortened III--IV interval and a prolongation of the IV--V interval, but were otherwise not significantly different from the normal controls. These data suggest a high incidence of hearing loss in both diagnostic categories studies, and abnormal functioning of the auditory brain stem pathway in the Down's syndrome group as a whole.