Korean J Otorhinolaryngol-Head Neck Surg.
2023 Jun;66(6):400-403. 10.3342/kjorl-hns.2023.00017.
A Case of MELAS Syndrome Involving Diabetes Mellitus, Cerebellar Ataxia and Progressive Sensorineural Hearing Loss
- Affiliations
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- 1Department of Otorhinolaryngology-Head and Neck Surgery, Nowon Eulji Medical Center, Eulji University School of Medicine, Seoul, Korea
- 2Department of Neurology, Nowon Eulji Medical Center, Eulji University School of Medicine, Seoul, Korea
- KMID: 2543073
- DOI: http://doi.org/10.3342/kjorl-hns.2023.00017
Abstract
- Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a maternally inherited mitochondrial disorder. MELAS syndrome manifests different clinical symptoms due to mitochondrial heteroplasmy and individual susceptibility and tends to be aggravated by metabolic events. It presents various clinical manifestations including hearing impairment, diabetes mellitus, and stroke like episodes. However, to date, only a few cases of MELAS syndrome accompanied with cerebellar ataxia have been reported in Korea. We hereby present an unusual case of a patient diagnosed with MELAS syndrome involving diabetes mellitus, cerebellar ataxia and sensorineural hearing loss, which were aggravated by metabolic stresses such as pregnancy and hypoglycemic shock.
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