Early Deficits in Speech Perception in Carriers of the p.Pro51Ser Variant in the COCH Gene: A Prospective Longitudinal Evaluation of Speech Perception in Quiet and Noise

Abstract

Background and Aim: The primary concern among adults with regard to their hearing is the difficulty in comprehending speech, particularly in noisy environments. The constant need to listen attentively leads to heightened frustration, fatigue, and decreased concentration. According to research, high-frequency hearing loss could have negative implications on speech perception and make it even harder to communicate. In this study, the focus is on patients with DFNA9 as it is one of the most common forms of non-syndromic high-frequency hearing loss. These patients carry the p.Pro51Ser variant in the COCH gene, which leads to progressive decline of hearing and vestibular function. Despite various cross-sectional studies on the natural progression of hearing levels, speech perception in silence and noisy surroundings is largely unexplored in this group of patients.