
This study aimed to summarize current knowledge on hearing loss in genetically proven mitochondrial disease in children and deduce possible and necessary consequences in patient care. Overall, the findings do not allow for a general statement on hearing loss in children with mitochondrial disease. However, retro-cochlear hearing loss seems to be found more often than expected. A common feature is a progression of hearing loss over time. However, hearing loss in these patients shows various characteristics. Therefore, awareness of mitochondrial disease as a possible causative background is essential for otolaryngologists. Future attempts rely on standardized reporting and long‐term follow‐up.
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