title: Acute cortical deafness in a child with MELAS syndrome.
abstract: 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.
authors.affiliation: Pediatric Neurology Unit, Pediatric Subspecialties Service, Children's Hospital, Geneva University Hospitals, Geneva, Switzerland. marie-pascale.pittet@hcuge.ch.
authors.forename: Marie P
authors.initials: MP
authors.lastname: Pittet
authors.affiliation: Child and Adolescent Medicine Department, Children's Hospital, Geneva University Hospitals, Geneva, Switzerland.
authors.forename: Roni B
authors.initials: RB
authors.lastname: Idan
authors.affiliation: Pediatric Nephrology and Metabolism Unit, Pediatric Subspecialties Service, Children's Hospital, Geneva University Hospitals, Geneva, Switzerland.
authors.forename: Ilse
authors.initials: I
authors.lastname: Kern
authors.affiliation: Division of Otorhinolaryngology, Geneva University Hospitals, Geneva, Switzerland.
authors.forename: Nils
authors.initials: N
authors.lastname: Guinand
authors.affiliation: Division of Otorhinolaryngology, Geneva University Hospitals, Geneva, Switzerland.
authors.forename: Hélène Cao
authors.initials: HC
authors.lastname: Van
authors.affiliation: Pediatric Radiology Unit, Children's Hospital, Geneva University Hospitals, Geneva, Switzerland.
authors.forename: Seema
authors.initials: S
authors.lastname: Toso
authors.affiliation: Pediatric Neurology Unit, Pediatric Subspecialties Service, Children's Hospital, Geneva University Hospitals, Geneva, Switzerland.
authors.forename: Joël
authors.initials: J
authors.lastname: Fluss
mesh_terms: D002648:Child; D003638:Deafness / Q000209:etiology*; D006313:Hearing Loss, Central / Q000209:etiology; D006801:Humans; D017241:MELAS Syndrome / Q000150:complications*; D008297:Male; D020521:Stroke / Q000150:complications