Cochlear Implantation in a Patient with Kearns-Sayre Syndrome: Case Report and Literature Review
نویسندگان
چکیده
Introduction: The Kearns-Sayre syndrome (KSS) belongs to the group of mitochondrial diseases. Cells that require more energy intake, such as muscle, nerve, retinal and cochlear, are most commonly affected. The established diagnostic criteria are: 1) the age of onset before 20 years old (100%), 2) progressive external ophthalmoplegia (100%), 3) pigmentary retinopathy (100%), and 4) at least one of the following: heart block, cerebellar ataxia (84%) and protein> 100 mg/dL in the cerebrospinal fluid. Additional features are the presence of bilateral sensorineural hearing loss and myopathy. Genetic testing of mitochondrial DNA can confirm the diagnosis. The aspect of muscle biopsy is typical, showing ragged red fibers (RRF). Objectives: Present a literature review and report the cochlear implantation in a
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