Case Report Right auditory dysfunction during acute Leber’s hereditary optic neuropathy harboring the 14484 mtDNA mutation: a case report

Puspose: We report a case of a 17-year-old man who developed right auditory dysfunction, one month after the onset of visual loss caused by Leber’s hereditary optic neuropathy (LHON). Methods: A full examination including a blood Mitochondrial DNA examination, auditory assessment and cranial magnetic resonance imaging were performed during the acute stage of the Leber’s hereditary optic neuropathy. Results: A blood examination coexisted m.11969G>A, m.10398A>G and m.14484T>C mitochondrial DNA mutations. The right auditory assessment indicated auditory dysfunction. Cranial magnetic resonance imaging demonstrated abnormal signal in the bilateral prechiasmal optic nerve. Conclusions: The association between LHON and auditory dysfunction had been suggested more than case reports, however, to our knowledge, the unilateral auditory dysfunction has not been previously reported during the acute stage of visual loss in patients with LHON harboring T14484C.