Auditory brainstem response findings in Rett syndrome.

The ABR findings for 11 subjects with Rett syndrome were compared with two normal control groups, one female and the other male. Measurements were made for ipsilateral and contralateral recorded responses. Significant findings were noted for the latency of Wave V and for the III-V interval between the Rett subjects and the normal female controls on ipsilateral recording and between the Rett group and both control groups for the difference obtained between ipsilateral and contralateral recorded III-V intervals. While the basis for these observations is not clear, the findings provide evidence for some degree of involvement within the auditory brainstem pathway of this group and further support for the rather diffuse nature of the CNS pathology within the syndrome. Suggestions are made for further ABR study of this clinical entity.