Beyond the cochlea: exploring the multifaceted nature of hearing loss in primary mitochondrial diseases.

ABSTRACT: Primary mitochondrial diseases, with diverse systemic manifestations, often present with auditory impairments due to mitochondrial dysfunction. This study provides an in-depth exploration of auditory deficits in primary mitochondrial diseases, highlighting the impact of various pathogenic variants on both cochlea and neural/central auditory functions. An observational study involving 72 adults with primary mitochondrial diseases was conducted. Participants underwent extensive audiological evaluations including pure-tone audiometry, tympanometry, acoustic reflex thresholds, quick speech-in-noise test, listening in spatialized noise-sentences test, auditory-evoked brainstem responses and distortion product otoacoustic emissions. Multivariate analysis of covariance and logistic regression ana

SUBMITTER: Koohi N 

PROVIDER: S-EPMC11583428 | biostudies-literature | 2024

REPOSITORIES: biostudies-literature