| Main clinical features
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neurologic disorder characterized by onset of visual and hearing impairment in the first or second decades
sensorineural neuropathies affecting vision and hearing
onset of hearing loss and visual impairment in the first or second decades; auditory neuropathy, based on severe impaired auditory brainstem responses (ABRs), discrepancies between tonal and vocal audiometry, and the presence of otoacoustic emissions in both ears; ophthalmologic work-up showed variable abnormalities, including optic atrophy, decreased visual evoked potentials, and papilledema with disc pallor |