Scientific article
Open access

Acute cortical deafness in a child with MELAS syndrome

Published inJournal of Inherited Metabolic Disease, vol. 39, no. 3, p. 465-466
Publication date2016

Auditory impairment in mitochondrial disorders are usually due to peripheral sensorineural dysfunction. Central deafness is only rarely reported. We report here an 11-year-old boy with MELAS syndrome who presented with subacute deafness after waking up from sleep. Peripheral hearing loss was rapidly excluded. A brain MRI documented bilateral stroke-like lesions predominantly affecting the superior temporal lobe, including the primary auditory cortex, confirming the central nature of deafness. Slow recovery was observed in the following weeks. This case serves to illustrate the numerous challenges caused by MELAS and the unusual occurrence of acute cortical deafness, that to our knowledge has not be described so far in a child in this setting.

Citation (ISO format)
PITTET, Marie-Pascale et al. Acute cortical deafness in a child with MELAS syndrome. In: Journal of Inherited Metabolic Disease, 2016, vol. 39, n° 3, p. 465–466. doi: 10.1007/s10545-016-9929-x
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Article (Published version)
ISSN of the journal0141-8955

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