Functional Impacts of Aminoglycoside Treatment on Speech Perception and Extended High-Frequency Hearing Loss in a Pediatric Cystic Fibrosis Cohort

Abstract

Purpose The purpose of this study is to better understand the prevalence of ototoxicity-related hearing loss and its functional impact on communication in a pediatric and young adult cohort with cystic fibrosis (CF) and individuals without CF (controls). Method We did an observational, cross-sectional investigation of hearing function in children, teens, and young adults with CF (n = 57, M = 15.0 years) who received intravenous aminoglycoside antibiotics and age- and gender-matched controls (n = 61, M = 14.6 years). Participants completed standard and extended high-frequency audiometry, middle ear measures, speech perception tests, and a hearing and balance questionnaire. Results Individuals with CF were 3-4 times more likely to report issues with hearing, balance, and tinnitus and performed significantly poorer on speech perception tasks compared to controls. A higher prevalence of hearing loss was observed in individuals with CF (57%) compared to controls (37%). CF and control groups had similar proportions of slight and mild hearing losses; however, individuals with CF were 7.6 times more likely to have moderate and greater degrees of hearing loss. Older participants displayed higher average extended high-frequency thresholds, with no effect of age on average standard frequency thresholds. Although middle ear dysfunction has not previously been reported to be more prevalent in CF, this study showed that 16% had conductive or mixed hearing loss and higher rates of previous otitis media and pressure equalization tube surgeries compared to controls. Conclusions Individuals with CF have a higher prevalence of conductive, mixed, and sensorineural hearing loss; poorer speech-in-noise performance; and higher rates of multiple symptoms associated with otologic disorders (tinnitus, hearing difficulty, dizziness, imbalance, and otitis media) compared to controls. Accordingly, children with CF should be asked about these symptoms and receive baseline hearing assessment(s) prior to treatment with potentially ototoxic medications and at regular intervals thereafter in order to provide otologic and audiologic treatment for hearing- and ear-related problems to improve communication functioning.

Figure 1.

Standard and extended high-frequency audiometric thresholds measured in hearing level (dB HL) and calibrated according to ISO 389–8 and 389–1 (International Organization for Standardization, 2004, 2017). Top panel: Mean and 95% confidence interval (CI) for the cystic fibrosis (CF) and control groups. Bottom panel: Mean and 95% CI for controls with the CF group further classified into normal hearing (CF–NH) and hearing loss (CF–HL) groups.

Figure 2.

Participant age at test plotted as a function of average hearing thresholds for the control and cystic fibrosis (CF) groups. Standard frequency average thresholds (SF; 0.25–8 kHz) are displayed on the left, and extended high-frequency average thresholds (EHF; 10–16 kHz) are displayed on the right. Regression lines and the coefficient of determination are displayed in each figure. *p < .05, **p < .01, ***p < .001.

Figure 3.

Ipsilateral (IPSI) and contralateral (CONTRA) middle ear muscle reflex (MEMR) thresholds box plots for the cystic fibrosis (CF) and control groups (top panel) and hearing status (bottom panel). Hearing loss was defined as thresholds of > 15 dB HL at any test frequency (0.25–16 kHz) and included normal hearing (NH) and hearing loss (HL) categories. Box plots show median (line), interquartile range (boxes), 95% confidence interval (stems), and outliers (dots). *p < .05, **p < .01, ***p < .001.

Figure 4.

SNR-Loss displayed based on participant group (left), hearing status (middle), and lastly with the cystic fibrosis (CF) group further classified into normal hearing (CF–NH) and hearing loss (CF–HL) groups (right). The SNR-Loss was calculated as the individual SNR–50 minus age-matched normative SNR-50 values. Error bars indicate 95% confidence intervals. *p < .05, **p < .01, ***p < .001.

Figure 5.

SNR-Loss plotted as a function of average hearing thresholds for the control group with the cystic fibrosis (CF) group further classified into normal hearing (CF–NH) and hearing loss (CF–HL) groups. Standard frequency average thresholds (SF; 0.25–8 kHz) are displayed on the left, and extended high-frequency average thresholds (EHF; 10–16 kHz) are displayed on the right. Regression lines and the coefficient of determination are displayed in each figure. *p < .05, **p < .01, ***p < .001.