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.