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Published on: January 9, 2019
Cochlear implantation in patients with MELAS syndrome
P D Karkos1, S Anari, I J Johnson
1Department of Otolaryngology, The Freeman Hospital, Newcastle Upon Tyne, UK. pkarkos@aol.com
Summary
MELAS syndrome, a mitochondrial DNA disorder, can cause genetic sensorineural hearing loss (SNHL). Cochlear implantation shows promise for treating SNHL in MELAS patients, offering improved outcomes.
Area of Science:
- Genetics
- Neurology
- Otolaryngology
Background:
- MELAS syndrome is a rare mitochondrial DNA (mt-DNA) disorder.
- Mitochondrial cytopathies can manifest with sensorineural hearing loss (SNHL), sometimes as the initial symptom.
Observation:
- Presents two cases of MELAS patients with SNHL who underwent successful cochlear implantation.
- Reviews literature on diagnosis, anesthetic considerations, and management of MELAS patients.
Findings:
- Cochlear implantation can be an effective treatment for sensorineural hearing loss in MELAS syndrome.
- Successful outcomes were observed in the presented cases following cochlear implantation.
Implications:
- Highlights the importance of considering genetic hearing loss in MELAS syndrome diagnosis.
- Suggests cochlear implantation as a viable therapeutic option for hearing restoration in affected individuals.
- Emphasizes the need for multidisciplinary management, including otolaryngological and anesthetic considerations.
