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Introduction: The treatment of patients with cystic fibrosis involves the use of ototoxic drugs, mainly aminoglycoside antibiotics. Due to the use of these drugs, fibrocystic patients are at risk of developing hearing loss., Objective: To evaluate the hearing of patients with cystic fibrosis by High Frequency Audiometry and Distortion Product Otoacoustic Emissions., Methods: Cross-sectional study. The study group consisted of 39 patients (7-20 years of age) with cystic fibrosis and a control group of 36 individuals in the same age group without otologic complaints, with normal audiometric thresholds and type A tympanometric curves. High Frequency Audiometry and Distortion Product Otoacoustic Emissions tests were conducted., Results: The study group had significantly higher thresholds at 250, 1000, 8000, 9000, 10,000, 12,500, and 16,000Hz (p=0.004) as well as higher prevalence of otoacoustic emission alterations at 1000 and 6000Hz (p=0.001), with significantly lower amplitudes at 1000, 1400, and 6000Hz. There was a significant association between alterations in hearing thresholds in High Frequency Audiometry with the number of courses of aminoglycosides administered (p=0.005). Eighty-three percent of patients who completed more than ten courses of aminoglycosides had hearing loss in High Frequency Audiometry., Conclusion: A significant number of patients with cystic fibrosis who received repeated courses of aminoglycosides showed alterations in High Frequency Audiometry and Distortion Product Otoacoustic Emissions. The implementation of ten or more aminoglycoside cycles was associated with alterations in High Frequency Audiometry., (Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.)