Erschienen in:
01.04.2005 | Otology
Cochlear implantation in patients with MELAS syndrome
verfasst von:
P. D. Karkos, S. Anari, I. J. Johnson
Erschienen in:
European Archives of Oto-Rhino-Laryngology
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Ausgabe 4/2005
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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.