BAEP changes in Leber's hereditary optic atrophy: further confirmation of multisystem involvement

A neurophysiological study of 11 patients belonging to 5 families affected by Leber's hereditary optic atrophy is reported. Electromyography, nerve conduction velocities and somatosensory evoked potentials were normal. Visual evoked potentials were absent or delayed, desynchronized and reduced in amplitude. Brainstem auditory evoked potentials were anomalous in 64% of subjects all without hearing defects. These changes which have never before been reported, confirm multisystem involvement in this disease.