Investigating the Potential Hearing Impairment and Ototoxicity in Children up to Six Years With Cystic Fibrosis After Aminoglycoside Exposure (PIANO-CF Extension).

Abstract

Background: People with CF (pwCF) are often treated with prolonged courses of aminoglycosides (AGs), for which known adverse effects include ototoxicity as a subset of hearing impairment (HI).

Methods: The PIANO-CF trial was a single-center study conducted at The Royal Children's Hospital where 28 pediatric patients aged < 7 years underwent sequential hearing tests at increased range up to 12,500 Hz in relation to receiving intravenous (IV) AGs. More than 85% of the cohort (n = 24) participated in the follow-up hearing testing up to 1 year.

Results: HI was defined by degree (dB) and frequency (Hz) on the audiogram. This was further reviewed to determine if the type of HI was consistent with ototoxicity as there are frequently other causes of HI in this age group. At baseline the prevalence of HI and ototoxicity were 11% and 7%, respectively. Over a period of 1 year, HI was identified in 12.5% and that of ototoxicity in 6%. No correlation was found between degree of IV AG exposure and HI or ototoxicity.

Discussion: The finding of HI in young children with CF, including in those with minimal IV AG exposure, has implications for CF services to proactively screen for HI. Undetected HI may compromise learning outcomes and given the age of children studied, this is not insignificant during the acquisition and development of language skills.

Conclusion: Routine audiometric testing for pwCF up to 12,500 Hz or beyond may increase sensitivity in detection of ototoxicity and should be considered for use in screening, monitoring, and future research.