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Auditory involvement in thalassemia major
Insights
Children with beta-thalassemia may experience hearing loss. Higher ferritin levels in these patients suggest iron overload could damage auditory function, particularly high frequencies.
Area of Science:
- Pediatric Hematology
- Otolaryngology
- Auditory Science
Background:
- Homozygous beta0-thalassemia requires management with blood transfusions.
- Iron overload is a common complication of thalassemia treatment.
- Auditory function can be affected by chronic illnesses and their treatments.
Purpose of the Study:
- To investigate auditory function in children with homozygous beta0-thalassemia.
- To explore the relationship between iron overload and hearing impairment in this population.
- To identify potential causes of auditory dysfunction in beta-thalassemia patients.
Main Methods:
- Auditory function assessment in 75 children with beta0-thalassemia and 75 controls.
- Evaluation of hearing impairment types (conductive and sensorineural).
- Measurement of ferritin levels in a subgroup of patients with and without hearing loss.
Main Results:
- 12 patients had mild conductive hearing impairment (bony/adenoid hypertrophy).
- 43 patients exhibited moderate sensorineural hearing loss (high frequencies, recruitment).
- Significantly higher mean ferritin levels were found in patients with sensorineural hearing loss compared to those without.
Conclusions:
- Iron overload may contribute to sensorineural hearing loss in beta-thalassemia patients.
- High ferritin levels correlate with auditory damage, especially in high frequencies.
- Other factors like intermittent hypoxia and bony hypertrophy may also play a role in auditory impairment.
Abstract:
The auditory function of 75 children affected by homozygous beta0-thalassemia, managed with a low transfusion scheme and treated irregularly with low doses of desferrioxamine, and of 75 controls were examined. In 12 patients a mild bilateral conductive hearing impairment due to bony hypertrophy and/or adenoid hypertrophy was found. In 43 cases a moderate monolateral or bilateral sensory-neural hearing loss at high frequencies with recruitment phenomenon was observed. Ferritin levels were determined in a randomly chosen group of these patients with (14) and without heaing loss (11). In the subjects with sensory-neural hearing loss the mean ferritin levels were significantly higher than in those with no hearing defect. There was no obvious relation between sensory-neural damage on the one hand and Hb levels and unit of blood transfused on the other. The results of this study suggest that iron overload could be a cause of damage in the high frequency elements of the auditory mechanism. Intermittent hypoxia and slow 8th nerve compression due to bony hypertrophy as causes of auditory involvement are also discussed.