Hearing loss is a clinical symptom, frequently mentioned in the context of mitochondrial disease.

With no cure available for mitochondrial disease, supportive treatment of clinical symptoms like hearing loss is of the utmost importance.

The aim of this study was to summarize current knowledge on hearing loss in genetically proven mitochondrial disease in children and deduce possible and necessary consequences in patient care.

Systematic literature review, including Medline, Embase, and Cochrane library.