Genetic hearing impairment affects cochlear processing and, consequently, speech recognition in noise

Abstract The relation between speech recognition and hereditary hearing loss is not straightforward. Impaired cochlear processing of sound might be determined by underlying genetic defects. Data obtained in nine groups of patients with a specific type of genetic hearing loss were evaluated. For each group, the affected cochlear structure, or 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 genetics-related malfunctioning of the cochlea.