The Role of Routine Hearing Screening in Children With Cystic Fibrosis on Aminoglycosides: A Systematic Review

Objective: To review the role of routine hearing screening for sensorineural hearing loss (SNHL) in children with cystic fibrosis (CF) who have been on aminoglycoside therapy. Data Sources: PubMed, Cochrane, Scopus, and Ovid databases. Review Methods: A systematic review of the literature was performed in accordance with PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. A comprehensive search was performed from 1970 to 2014. Randomized controlled trials, case-control studies, cohort studies, and case series including pediatric subjects with baseline auditory evaluations were included. Results: Twelve studies (1979-2014) were reviewed. The study population included 762 children (5 months-20 years). Hearing screening measures included pure-tone audiometry (PTA) at standard +/- high frequency threshold (HFPTA) (12/12), distortion product otoacoustic emissions (DPOAE) (4/12), transient-evoked otoacoustic emissions (1/12), and automated auditory brainstem response (1/12). The overall prevalence of SNHL ranged from 0% to 29%. However, on subset analysis of children with greater than 10 courses of intravenous (IV) aminoglycosides, up to 44% had SNHL. Eight studies recommended hearing screening in CF children on aminoglycosides; of these, two studies recommended screening even without aminoglycoside exposure, and four studies made no recommendations. HFPTA was the most commonly recommended screening measure followed by DPOAEs. Conclusion: This systematic review supports a recommendation for clinicians to perform routine hearing screening in children with CF during and after aminoglycoside exposure based on the high prevalence of SNHL in this population. Future studies should define the optimal timing for hearing screening during and after aminoglycoside therapy in children with CF.