Auditory brainstem response findings in Rett syndrome: stability over time.

OBJECTIVE

To determine whether changes consistent with the presence of white matter neurodegenerative disease would be observed in the auditory brainstem response (ABR) in Rett syndrome in conjunction with age advancement.

STUDY DESIGN

Initial and follow-up ABR findings were analyzed in a sample of 27 female patients with Rett syndrome. The interval between ABR tests ranged from 1 to 9 years.

RESULTS

No significant group differences consistent with neurodegenerative disease were observed in the wave I-III, III-V, or I-V interpeak latency intervals between the initial and follow-up test sessions.

CONCLUSIONS

Rett syndrome is not a disorder characterized by progressive white matter degeneration affecting the integrity of the brainstem central auditory pathways.