Serial auditory evoked responses were investigated in 3 children with Krabbe disease. Auditory brainstem responses revealed prolongation of each wave component and interpeak latency with decreased amplitudes in later components which finally disappeared except for wave I. Long-latency auditory responses (LLRs) persisted in the advanced stage when all wave components of middle-latency auditory responses (MLRs) had disappeared. The results of auditory brainstem responses and MLRs are compatible with magnetic resonance imaging findings and a review of pathologic findings in Krabbe disease, including extensive involvement of brainstem and subcortical structures. It is suggested that the source of LLR waves is different from that of MLR because of the persistent existence of LLR waves. It is speculated that the cerebral cortex and/or subcortical U fibers, which are spared in Krabbe disease, have an important role in generating LLR wave components.