Mitochondrial Encephalopathy with Progressive Hearing Impairment; A Case Report.

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An 11-year-old female had complained of headache, eye pain, fever and vomiting at the age of 5 years. MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke like episodes) was diagnosed at the age of 7 years by muscle biopsy. She was referred to our department because of hearing impairment at 8 years of age.Our first examination disclosed weakness of the extremities, mental disturbance, and hearing impairment. The pure tone audiogram showed a hearing level of 46dB on the right and of 39dB on the left. Two year later, the hearing level was about 20dB higher bilateraly. Auditory brainstem responses showed no brainstem damage and hearing levels similar to the pure tone audiogram.Electrocochleograms and evoked otoacoustic emission tests suggested that hearing impairment was due to a cochlear disturbance.However, speech audiograms suggested that there might be an associated retrocochlear disturbance too.Vestibular function was not disturbed. MELAS is known to include degeneration of small arteries of the brain (mitochondrial angiopathy).In our patient, cochlear and retrocochlear disturbances seem to have been caused partially by disturbances secondary to degeneration of small arteries.
耳鼻咽喉科臨床学会の論文