Auditory brainstem responses (ABR) in patients with Wolfram syndrome

: Auditory brainstem responses (ABR) and other evoked potentials were studied in four patients with the Wolfram syndrome. In three cases ABR was abnormal in the early stage of the disease. There was no responses or only the wave V with prolonged latency at a stimulation level of 80 dBnHL. A prolongation of the I-V interpeak latency (IPL) was also revealed at a stimulation level of 105 dBnHL. The remaining patient showed shortening of the I-V IPL. The visual evoked potentials showed prolonged peak latency in three cases, and the median nerve short latency somatosensory evoked potentials were normal in two cases. These ABR findings indicated not only sensory neuronal hearing loss but also a degenerative change in the brain stem in the Wolfram syndrome.