Related Experiment Videos
Hearing loss and desferrioxamine in homozygous beta-thalassemia
Insights
Beta-thalassemia patients treated with desferrioxamine experienced sensorineural hearing loss, particularly younger individuals. Hearing loss correlated with desferrioxamine dose, suggesting an ototoxic effect even with good iron chelation.
Area of Science:
- Pediatrics
- Hematology
- Ototoxicology
Background:
- Beta-thalassemia requires regular blood transfusions, leading to iron overload.
- Iron chelation therapy, often with desferrioxamine, is crucial for managing iron overload.
- Ototoxicity is a potential concern with chelation therapies.
Purpose of the Study:
- To assess audiometric findings in children with beta-thalassemia undergoing desferrioxamine treatment.
- To investigate the relationship between desferrioxamine administration and hearing loss.
- To determine the prevalence and characteristics of hearing impairment in this patient group.
Main Methods:
- Audiometric screening was conducted on 153 children (aged 5-18) with beta-thalassemia.
- Patients received regular blood transfusions and desferrioxamine chelation.
- Hearing loss was analyzed in relation to age, desferrioxamine dosage, and iron load.
Main Results:
- 38% of patients exhibited significant high-frequency sensorineural hearing loss with recruitment.
- Hearing loss was more pronounced in younger patients, suggesting cochlear damage.
- Hearing impairment correlated with desferrioxamine dose and was higher with lower iron load, indicating ototoxicity.
Conclusions:
- Desferrioxamine treatment in beta-thalassemia patients can lead to sensorineural hearing loss.
- The ototoxic effect appears dose-dependent and may be more evident with effective iron chelation.
- Regular audiometric monitoring is recommended for children with beta-thalassemia receiving desferrioxamine.
Abstract:
The authors present the results obtained during an audiometric screening of 153 children aged 5-18 years, affected by beta-thalassemia and treated with regular blood transfusions and iron overload chelation by means of desferrioxamine. Thirty-eight percent of the patients showed a significant sensorineural hearing loss at high frequencies with recruitment. Younger patients had a greater hearing loss, indicating that cochlear damage was not due to the disease itself. Furthermore, hearing loss appeared to be correlated with the mean and peak desferrioxamine doses administered and was higher in subjects with lower iron load. Thus, the ototoxic effect seems to have been higher when a good iron chelation had been obtained. Among our patients, conductive hearing loss was not more frequent than in patients without beta thalassemia.