Cochlear implantation in profoundly deaf patients with MELAS syndrome

Abstract

Mitochondrial myopathy encephalopathy, lactic acidosis and stroke-like episodes syndrome (MELAS) is a type of mitochondrial disease characterized by recurrently stroke-like episodes. Approximately 40-60% of patients with MELAS syndrome have sensorineural hearing loss cannot obtain hearing aids. These patients sometimes fall into severe hearing loss. Because the onset of MELAS syndrome is rare, there are limited reports of cochlear implantation in profoundly deaf patients with MELAS syndrome. We herein report cochlear implantation for three mitochondrial encephalomyopathy patients ; two of the patients noticed a significant improvement in communication due to the cochlear implant. Inner ear deafness is often the cause of sensorineural hearing loss associated with mitochondrial encephalomyopathy. However, retrocochlear deafness may also be a cause. We should therefore consider cochlear implant adaptation when retrocochlear deafness is suspected to be the cause.