Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Apr 2004
Case ReportsProgressive auditory neuropathy in patients with Leber's hereditary optic neuropathy.
To investigate auditory neural involvement in patients with Leber's hereditary optic neuropathy (LHON). ⋯ The findings are consistent with auditory neuropathy-a lesion of the cochlear nerve presenting with abnormal auditory brain stem responses and with normal inner hair cells and the cochlear nucleus (lower brain stem). The association of auditory neuropathy, or any other auditory dysfunction, with LHON has not been recognised previously. Further studies are necessary to establish whether this is a consistent finding.