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Updated: May 4, 2026
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Subclinical high-frequency cochlear dysfunction in adolescents with β-thalassemia major: A distortion product
Ozlem Yagiz Agayarov1, Rojbin Kilic1, Murat Gumussoy1
1Tepecik Training and Research Hospital, Department of Otolaryngology Head and Neck Surgery, Güney Mahallesi 1140/1 Sokak No:1, Yenişehir-Konak, İzmir, Türkiye.
Objective:
To evaluate cochlear function using distortion product otoacoustic emissions (DPOAEs) in adolescents with β-thalassemia major receiving regular desferrioxamine chelation therapy who exhibit normal pure-tone audiometric thresholds, and to compare the findings with those of healthy controls.
Methods:
This cross-sectional controlled study included 21 adolescents with β-thalassemia major aged 15-18 years receiving regular desferrioxamine therapy and 21 age- and sex-matched healthy controls. All participants demonstrated normal pure-tone audiometry. DPOAE measurements were performed at frequencies ranging from 500 Hz to 10,000 Hz, and distortion product amplitudes (DP1) and signal-to-noise ratio (SNR) values were analyzed. Mean values of both ears were used for statistical comparisons. To control for type I error due to multiple frequency comparisons, p values were adjusted using the Holm-Bonferroni method separately for DP1 and SNR analyses.
Results:
At 1 kHz, the mean DP1 amplitude was significantly higher in the patient group compared to controls (10.07 ± 5.73 vs. 4.95 ± 5.63 dB SPL; p = 0.005). At 10 kHz, DP1 amplitude was significantly lower in patients than in controls (p = 0.004), and this difference remained statistically significant after Holm-Bonferroni correction. Although SNR values at 10 kHz were lower in the patient group in the unadjusted analysis (p = 0.039), this difference did not retain statistical significance after correction for multiple comparisons. No significant differences were observed at the other tested frequencies (p > 0.05).
Conclusion:
Adolescents with β-thalassemia major receiving regular desferrioxamine chelation therapy may exhibit subclinical high-frequency cochlear dysfunction despite normal pure-tone audiometric findings. The presence of significantly reduced DP1 amplitudes at 10 kHz highlights the sensitivity of DPOAE testing in detecting early cochlear alterations not identified by conventional audiometry. Incorporating high-frequency DPOAE measurements into routine audiological monitoring may facilitate earlier detection of cochlear involvement and improve long-term auditory surveillance in this patient population.
