Health of the Auditory Nerve in Hereditary Sensorineural Hearing Loss: Insights from Electrophysiological Measurements in Individuals with Pathogenic Variants of TMPRSS3, COCH, GJB2 and SLC26A4
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2024-09-24
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en
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This study investigated the health of the auditory nerve in individuals with hereditary sensorineural hearing loss (SNHL) using neural response telemetry (NRT). The eCAP thresholds (T-NRT), amplitude growth slopes (AGFs), and refractoriness of spiral ganglion neurons (SGNs) were compared among subjects with TMPRSS3-, COCH-, GJB2-, and SLC26A4-mutations. Results indicated that individuals with TMPRSS3- and COCH-variants exhibited lower eCAP thresholds and higher amplitude growth slopes, suggesting better SGN survival. In contrast, prolonged auditory deprivation in those with GJB2 and SLC26A4 mutations might have led to SGN degeneration, as indicated by higher thresholds and flatter slopes. The lowest eCAP thresholds were found in the apical cochlear region. While the eCAP parameters tended to predict speech perception poorly, the study concluded that the investigated parameters may not reliably predict speech perception outcomes, highlighting the complexity of individual variability in interpreting eCAP data.
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