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Acute cortical deafness in a child with MELAS syndrome

Publié dansJournal of Inherited Metabolic Disease, vol. 39, no. 3, p. 465-466
Date de publication2016
Résumé

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 (format ISO)
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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ISSN du journal0141-8955
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Création19.09.2017 15:58:00
Première validation19.09.2017 15:58:00
Heure de mise à jour15.03.2023 02:01:44
Changement de statut15.03.2023 02:01:43
Dernière indexation17.01.2024 00:42:02
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