Auditory cortex hypoperfusion: a metabolic hallmark in Beta Thalassemia.

Area of Science:

• Hematology
• Neurology
• Audiology

Background:

• Sensorineural hearing loss is a frequent complication in beta-thalassemia patients, often attributed to iron chelation therapy.
• Limited data exist on hearing loss in adult beta-thalassemia populations, particularly concerning central auditory pathway involvement.
• This study investigates audiological and brain perfusion changes in adult beta-thalassemia patients.

Purpose of the Study:

• To assess the prevalence and characteristics of hearing loss in adult beta-thalassemia patients.
• To investigate potential alterations in central auditory areas using brain perfusion MRI.
• To explore the relationship between hearing loss, iron chelation, clinical phenotype, and cognitive function.

Main Methods:

• A multicenter, cross-sectional study involving 77 adult beta-thalassemia patients and 56 healthy controls.
• Audiological assessments included pure tone audiometry.
• 3 Tesla brain perfusion MRI was used to evaluate auditory cortex perfusion.

Main Results:

• 52% of patients exhibited high-frequency hearing deficit, with 35% having overt hypoacusis, irrespective of iron chelation or clinical status.
• Reduced relative perfusion in the auditory cortex was observed in beta-thalassemia patients, independent of clinical phenotype.
• Auditory cortex perfusion correlated with age in controls and transfusion-dependent patients, and showed a U-shaped correlation with hearing loss severity in affected patients.

Conclusions:

• Primary auditory cortex perfusion changes represent a metabolic hallmark in adult beta-thalassemia.
• These perfusion alterations suggest complex hearing function remodeling, occurring independently of chelation therapy and prior to overt hearing loss.
• The cognitive implications of these perfusion changes warrant further investigation.