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. 2022 Oct 22;13(11):1923.
doi: 10.3390/genes13111923.

Genetic Hearing Loss Affects Cochlear Processing

Cris Lanting  1 , Ad Snik  1 , Joop Leijendeckers  1 , Arjan Bosman  1 , Ronald Pennings  1
Affiliations

Genetic Hearing Loss Affects Cochlear Processing

Cris Lanting et al. Genes (Basel). .

Abstract

The relationship between speech recognition and hereditary hearing loss is not straightforward. Underlying genetic defects might determine an impaired cochlear processing of sound. We obtained data from nine groups of patients with a specific type of genetic hearing loss. For each group, the affected cochlear site-of-lesion was determined based on previously published animal studies. Retrospectively obtained speech recognition scores in noise were related to several aspects of supra-threshold cochlear processing as assessed by psychophysical measurements. The differences in speech perception in noise between these patient groups could be explained by these factors and partially by the hypothesized affected structure of the cochlea, suggesting that speech recognition in noise was associated with a genetics-related malfunctioning of the cochlea. In particular, regression models indicate that loudness growth and spectral resolution best describe the cochlear distortions and are thus a good biomarker for speech understanding in noise.

Keywords: frequency discrimination; gap detection; hereditary hearing loss; loudness growth; otogenetics; speech-in-noise.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Mean audiogram of the patient groups showing the mean threshold for the frequencies tested (right and left ear averaged), as well as the 95% confidence interval around the mean.
Figure 2
Figure 2
Boxplots of the eight patient groups, rank-ordered by the mean CDF for each group. The groups vary with respect to their mean CDF, but also with respect to the distribution within each group. The highest mean CDF (i.e., poorest speech understanding in noise) and the broadest distribution of individual patients’ CDF-values can be observed in the USH2a group. CDF = 0 means normal speech understanding in noise.
Figure 3
Figure 3
Cochlear distortion factor (CDF) as a function of the hearing loss (PTA) of the eight patient groups (individual data shown as circles where each patient group has a different color. The average for each patient group across PTA and CDF is indicated by a diamond-shaped symbol). The linear regression line presents the calculated best-fit curve and the 95% confidence interval of the fit.
See this image and copyright information in PMC

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