Cystic fibrosis (CF) is considered one of the most prevalent lethal genetic disorders. The life expectancy of those patients has been rising. Besides the well-known prevalence of chronic rhinosinusitis, neurosensory hearing loss (NSHL) due to the large use of ototoxic antibiotics may be another problem that should receive special attention in otolaryngology (ENT). Define retrospectively the prevalence of NSHL among patients with CF from the Hospital de Clínicas de Porto Alegre (HCPA) and based on this data discuss the need for a specific out patient ENT-CF clinic. Files from CF HCPA patients were reviewed for age at diagnosis, the current age, types of antibiotics used, number of treatments, percentage with audiologic evaluation and its respective results. One hundred and seven files were reviewed. The mean age at diagnosis was 1.33 years old (SD 1.97, range 0-12). The mean age of the sample was 7.87 (SD 4.49, range 1-22). Audiologic evaluation was performed in 39.3% (42) of the patients. 28.56% (12) had some degree of NSHL, while 36.3% did not present acoustic reflex. From 667 treatments with antibiotics, 49.5% were with tobramycin, 43.4% with amycacin and 7% with gentamicin. The prevalence of NSHL among CF patients supports the need for a specific ENT-CF outpatient clinic. This clinic would help on the prevention and treatment of NSHL due to the use of ototoxic antibiotics and on the better understanding/control of rhinosinusal disorders.