[Evaluation of auditory function in homozygous beta-thalassemia]

Area of Science:

• Hematology
• Otolaryngology
• Clinical Medicine

Background:

• Beta-thalassemia is a genetic blood disorder requiring chronic management.
• High transfusion regimens and desferrioxamine chelation therapy are standard treatments.
• Potential side effects of chronic treatment on other organ systems require investigation.

Purpose of the Study:

• To assess auditory function in patients with homozygous beta-thalassemia undergoing regular treatment.
• To compare auditory health between thalassemic patients and a healthy control group.
• To identify potential correlations between beta-thalassemia, its treatment, and auditory deficits.

Main Methods:

• Auditory function was evaluated in 29 patients with homozygous beta-thalassemia.
• Patients were on a high transfusion scheme and received desferrioxamine (40-60 mg/kg/day).
• A control group of 29 healthy subjects was included for comparison.

Main Results:

• Eight thalassemic patients exhibited conductive hearing defects (6 bilateral).
• Four thalassemic patients presented with sensory-neural hearing loss at high frequencies.
• Thalassemic patients demonstrated greater auditory impairment than controls.
• Increased incidence of tonsillar hypertrophy, adenotonsillitis, and lymph-node enlargement was noted in thalassemic patients.

Conclusions:

• Homozygous beta-thalassemia patients on high transfusion and desferrioxamine therapy are at increased risk for auditory impairment.
• Conductive and sensory-neural hearing loss are prevalent findings in this patient cohort.
• Further research is warranted to elucidate the mechanisms and long-term implications of these auditory changes.