Abstract
MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) is a rare congenital disorder of mitochondrial DNA (mt-DNA). Patients with this syndrome may present to the otolaryngologist with sensorineural hearing loss (SNHL) that is genetic in origin. Mitochondrial cytopathies can present with a variety of symptoms, but they occasionally present with SNHL as their first manifestation. Two cases of MELAS patients who responded well to cochlear implantation are presented. A review of the literature is also carried out focusing mainly on diagnosis, anesthetic considerations and management of these patients.
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Karkos, P.D., Anari, S. & Johnson, I.J. Cochlear implantation in patients with MELAS syndrome. Eur Arch Otorhinolaryngol 262, 322–324 (2005). https://doi.org/10.1007/s00405-004-0817-x
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DOI: https://doi.org/10.1007/s00405-004-0817-x