Cochlear implantation in patients with MELAS syndrome.
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.
Karkos
P D
PD
Department of Otolaryngology, The Freeman Hospital, Newcastle Upon Tyne, UK. pkarkos@aol.com
Anari
S
S
Johnson
I J
IJ
D000328:Adult; D019929:Cochlear Implantation; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D017241:MELAS Syndrome / Q000150:complications*; D008875:Middle Aged; D013069:Speech Reception Threshold Test; D016896:Treatment Outcome