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Acute cortical deafness in a child with MELAS syndrome
Author(s) -
Pittet Marie P.,
Idan Roni B.,
Kern Ilse,
Guinand Nils,
Van Hélène Cao,
Toso Seema,
Fluss Joël
Publication year - 2016
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-016-9929-x
Subject(s) - melas syndrome , medicine , hearing loss , sensorineural hearing loss , temporal lobe , mitochondrial disease , audiology , auditory cortex , cortex (anatomy) , neuroimaging , pediatrics , neuroscience , psychology , mitochondrial myopathy , epilepsy , mitochondrial dna , psychiatry , biochemistry , gene , chemistry
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.