학술논문
Auditory cortex hypoperfusion: a metabolic hallmark in Beta Thalassemia
Document Type
article
Author
Renzo Manara; Sara Ponticorvo; Silverio Perrotta; Maria Rosaria Barillari; Giuseppe Costa; Davide Brotto; Rosanna Di Concilio; Angela Ciancio; Elisa De Michele; Pasquale Alessandro Carafa; Antonietta Canna; Andrea Gerardo Russo; Donato Troisi; Martina Caiazza; Federica Ammendola; Domenico Roberti; Claudia Santoro; Stefania Picariello; Maria Sole Valentino; Emanuela Inserra; Roberta Carfora; Mario Cirillo; Simona Raimo; Gabriella Santangelo; Francesco di Salle; Fabrizio Esposito; Immacolata Tartaglione
Source
Orphanet Journal of Rare Diseases, Vol 16, Iss 1, Pp 1-11 (2021)
Subject
Language
English
ISSN
1750-1172
Abstract
Abstract Background Sensorineural hearing loss in beta-thalassemia is common and it is generally associated with iron chelation therapy. However, data are scarce, especially on adult populations, and a possible involvement of the central auditory areas has not been investigated yet. We performed a multicenter cross-sectional audiological and single-center 3Tesla brain perfusion MRI study enrolling 77 transfusion-dependent/non transfusion-dependent adult patients and 56 healthy controls. Pure tone audiometry, demographics, clinical/laboratory and cognitive functioning data were recorded. Results Half of patients (52%) presented with high-frequency hearing deficit, with overt hypoacusia (Pure Tone Average (PTA) > 25 dB) in 35%, irrespective of iron chelation or clinical phenotype. Bilateral voxel clusters of significant relative hypoperfusion were found in the auditory cortex of beta-thalassemia patients, regardless of clinical phenotype. In controls and transfusion-dependent (but not in non-transfusion-dependent) patients, the relative auditory cortex perfusion values increased linearly with age (p