Patients with cystic fibrosis (CF) are at increased risk for sensorineural hearing loss (SNHL) due, at least in part, to the ototoxic side effects of routine CF therapies. However, the prevalence of SNHL and additional factors contributing to the development of SNHL are unknown. To identify risk factors associated with the development of SNHL in a large cohort of CF patients who had been referred for audiometric testing. A retrospective study of audiometric results and medication information in a cohort of patients with CF. Records of 178 CF patients seen at Boston Children's Hospital for audiometric testing from 2007 to 2010 were reviewed. Mean age of patients was 18 yr (standard deviation = 10 yr), and 98 (55%) of the patients were female. Audiometric results, medications, and hospitalizations were recorded. Multivariable logistic regression was used to evaluate the association between SNHL and the number of hospitalizations and chronic antibiotic use in the year prior to the patients' audiometry. In this sample, 37/178 (21%) patients had SNHL. Twenty-nine (78%) of the 37 patients had bilateral SNHL and 8 (22%) had unilateral SNHL. Across all age groups, the majority of patients had a bilateral hearing loss (HL). A multivariable model showed that older age and more frequent hospitalizations were associated with SNHL. The number of courses of chronic antibiotics in the year prior to audiometric testing was not correlated with rate of HL. This study suggests that age and frequency of hospitalizations are key predictors of HL development. Increased awareness and regular screening for SNHL should be included in the routine care of CF patients, particularly those at the highest risk.