Download Full Text (407 KB)

Publication Date



Introduction: Aminoglycoside antibiotics (AG) are essential to treat infections in cystic fibrosis (CF). Potential nephrotoxicity and ototoxicity make appropriate monitoring critical. The 2016 CFF Patient Registry reports a low incidence of hearing loss (2.2% overall). However, in the United States, 13% of the total population > 12 years has hearing loss. A standardized aminoglycoside induced ototoxicity algorithm (AIOA) was implemented in 2017 at Children’s Mercy Kansas City (CMKC) to assess CF patients treated with intravenous (IV) and/or inhaled AG.

Methods: The AIOA was developed using information from CF provider surveys, retrospective chart review, observational cohort analysis, and a literature review. The algorithm is a visual reference for clinicians and provides specific AG monitoring instructions. It identifies risk factors warranting more frequent screening. The CF Center Coordinator and pharmacist are responsible for monitoring adherence to the AIOA including 1) identification of patients for monitoring during pre-clinic huddles and hospitalizations 2) review of monthly AG prescriptions, and 3) inpatient AG order review.

Results: Prior to AIOA implementation, 14 of 52 patients (27%) treated with IV AG between 2014 and 2015 hadan audiogram. Of the 70 patients that received at least two courses of inhaled AG in 2016, 18 (26%) had an audiogram. In the 24 months post-AIOA implementation, 43 of 44 patients (98%) treated with IV AG had an audiogram and of these, 27 (63%) were abnormal. Over the same period, 19 of 33 patients (58%) receiving inhaled AG for > 5 years had an audiogram completed per the AIOA. Among these, 10 (53%) were abnormal. The majority of patients (90%) received concomitant IV AG. Ten of the 30 (33%) patients that had repeated audiograms had clinically significant changes. A post-implementation provider survey demonstrated AIOA support and use of this information to influence treatment decisions. Specific interventions based on audiogram data included referral to otolaryngology for hearing aid evaluation and modifications to pulmonary exacerbation treatment regimens.

Conclusions: Implementation of an AIOA increased the frequency of audiogram screening among CF patients treated with IV and inhaled AG. The prevalence of hearing abnormalities at CMKC is higher than reported in the CFF Patient Registry and the overall US population. This discrepancy may be secondary to AG usage at CMKC or underreporting due to lack of testing. The frequent use of AG among CF patients and the probability of AG induced hearing loss suggest an urgent need to establish an AIOA nationally.

Document Type


Sustainability and Outcomes of a Standardized Aminoglycoside Induced Ototoxicity Monitoring Algorithm in Patients with Cystic Fibrosis