Abstract
Mitochondrial encephalomyopathy is a multisystem disease, affecting predominantly the CNS and skeletal muscles, and is caused by defects in mitochondrial function. There are three distinctive syndromes: KSS (Kearns-Sayre syndrome), MELAS (mitochondrial myopathy, encephalomyopathy, lactic acidosis and stroke-like episodes), and MERRF (myoclonus epilepsy with ragged-red fibers).
Hearing impairment is one of the symptoms of mitochondrial encephalomyopathy. There have been several reports of progressive sensorineural hearing loss in children and adults with this disease. Acute hearing loss has not however, been previously reported. Two patients with KSS and one patient with MELAS, who showed sudden onset hearing loss, are described in this paper.
