Trajectory of hearing loss in children with unilateral hearing loss

Abstract

Introduction: The aim of this study was to quantify the amount of deterioration in hearing and to document the trajectory of hearing loss in early identified children with unilateral hearing loss (UHL). We also examined whether clinical characteristics were associated with the likelihood of having progressive hearing loss.

Methods: As part of the Mild and Unilateral Hearing Loss Study, we followed a population-based cohort of 177 children diagnosed with UHL from 2003 to 2018. We applied linear mixed models to examine hearing trends over time including the average amount of change in hearing. Logistic regression models were used to examine the relationship between age and severity at diagnosis, etiology, and the likelihood of progressive loss and amount of deterioration in hearing.

Results: The median age of the children at diagnosis was 4.1 months (IQR 2.1, 53.9) and follow-up time was 58.9 months (35.6, 92.0). Average hearing loss in the impaired ear was 58.8 dB HL (SD 28.5). Over the 16-year period, 47.5% (84/177) of children showed deterioration in hearing in one or both ears from their initial diagnostic assessment to most recent assessment including 21 (11.9%) who developed bilateral hearing loss. Average deterioration in the impaired ear ranged from 27 to 31 dB with little variation across frequencies. Deterioration resulted in a change in category of severity for 67.5% (52/77) of the children. Analysis for children who were followed for at least 8 years showed that most lost a significant amount of hearing rapidly in the first 4 years, with the decrease stabilizing and showing a plateau in the last 4 years. Age and severity at diagnosis were not significantly associated with progressive/stable loss after adjusting for time since diagnosis. Etiologic factors (ENT external/middle ear anomalies, inner ear anomalies, syndromic hearing loss, hereditary/genetic) were found to be positively associated with stable hearing loss.

Conclusion: Almost half of children with UHL are at risk for deterioration in hearing in one or both ears. Most deterioration occurs within the first 4 years following diagnosis. Most children did not experience sudden "large" drops in hearing but more gradual decrease over time. These results suggest that careful monitoring of UHL especially in the early years is important to ensure optimal benefit from early hearing loss detection.

Keywords: children; etiology; progressive loss; trajectory; unilateral hearing loss.

Figure 1

Selection of study participants. ANSD, auditory neuropathy spectrum disorder; HL, hearing loss.

Figure 2

Average change in hearing thresholds across frequencies from initial diagnosis to most recent assessment (n = 77). The boxes indicate the 25th, 50th, and 75th percentiles. The whiskers above and below the box boundaries show the largest and smallest observed values. x on graph refers to mean thresholds.

Figure 3

Category of hearing loss severity at diagnosis and at most recent assessment. The shaded boxes represent the degree of hearing loss in the impaired ear at diagnosis and the unshaded boxes the degree at final assessment. For the 21 ears that developed hearing loss (previously normal hearing), degree of hearing loss at final assessment is shown. UHL, unilateral hearing loss; HL, hearing loss.

Figure 4

Trajectory of hearing loss across four frequencies (n = 77). Error bars represent one standard deviation.