TY - JOUR
T1 - Acute auditory agnosia as the presenting hearing disorder in MELAS
AU - Miceli, Gabriele
AU - Conti, Guido
AU - Cianfoni, Alessandro
AU - Di Giacopo, Raffaella
AU - Zampetti, Patrizia
AU - Servidei, Serenella
PY - 2008
Y1 - 2008
N2 - MELAS is commonly associated with peripheral hearing loss. Auditory agnosia is a rare cortical auditory impairment, usually due to bilateral temporal damage. We document, for the first time, auditory agnosia as the presenting hearing disorder in MELAS. A young woman with MELAS (A3243G mtDNA mutation) suffered from acute cortical hearing damage following a single stroke-like episode, in the absence of previous hearing deficits. Audiometric testing showed marked central hearing impairment and very mild sensorineural hearing loss. MRI documented bilateral, acute lesions to superior temporal regions. Neuropsychological tests demonstrated auditory agnosia without aphasia. Our data and a review of published reports show that cortical auditory disorders are relatively frequent in MELAS, probably due to the strikingly high incidence of bilateral and symmetric damage following stroke-like episodes. Acute auditory agnosia can be the presenting hearing deficit in MELAS and, conversely, MELAS should be suspected in young adults with sudden hearing loss.
AB - MELAS is commonly associated with peripheral hearing loss. Auditory agnosia is a rare cortical auditory impairment, usually due to bilateral temporal damage. We document, for the first time, auditory agnosia as the presenting hearing disorder in MELAS. A young woman with MELAS (A3243G mtDNA mutation) suffered from acute cortical hearing damage following a single stroke-like episode, in the absence of previous hearing deficits. Audiometric testing showed marked central hearing impairment and very mild sensorineural hearing loss. MRI documented bilateral, acute lesions to superior temporal regions. Neuropsychological tests demonstrated auditory agnosia without aphasia. Our data and a review of published reports show that cortical auditory disorders are relatively frequent in MELAS, probably due to the strikingly high incidence of bilateral and symmetric damage following stroke-like episodes. Acute auditory agnosia can be the presenting hearing deficit in MELAS and, conversely, MELAS should be suspected in young adults with sudden hearing loss.
KW - Auditory agnosia
KW - Bilateral and symmetric stroke-like episodes
KW - Hearing loss
KW - MELAS syndrome
KW - Temporal lobes
UR - http://www.scopus.com/inward/record.url?scp=58149092011&partnerID=8YFLogxK
U2 - 10.1007/s10072-008-1028-9
DO - 10.1007/s10072-008-1028-9
M3 - Article
C2 - 19011735
AN - SCOPUS:58149092011
VL - 29
SP - 459
EP - 462
JO - Neurological Sciences
JF - Neurological Sciences
SN - 1590-1874
IS - 6
ER -