Mitochondrial Hearing Loss: Diagnosis and Management

Abstract

Mitochondrial hearing loss (MHL) arises from mutations in mitochondrial DNA (mtDNA) or in nuclear genes coding for mitochondrial proteins, which impair inner ear function resulting in hearing loss. Diagnosis of MHL requires a comprehensive evaluation, including genetic tests, clinical assessments, and audiological examination. Treatment options for MHL are limited, with supportive measures to enhance communication and restore hearing function being the primary options. Ongoing research is investigating new therapies that target mitochondrial dysfunction and regenerative techniques to restore hearing function. It is crucial to understand the underlying mechanisms of MHL and develop effective interventions to mitigate its negative impact.