Therapeutic potential of mitochondrial transfer in reversing mutant-to-wild-type mtDNA ratio and improving mitochondrial dysfunction in 1555A>G mtDNA mutation-associated hearing loss

Mitochondrial DNA (mtDNA) mutations are a major cause of sensorineural hearing loss (SNHL). The m.1555A >G mutation in the mitochondrial 12S rRNA gene is closely linked to nonsyndromic and aminoglycoside-induced hearing loss, leading to impaired oxidative phosphorylation (OXPHOS) and ATP production. Current treatments focus on auditory rehabilitation without addressing mitochondrial pathology....

No actionable change — this is early-stage laboratory (in-vitro or preclinical) research; mitochondrial transfer as a therapy for m.1555A>G-related hearing loss is not ready for clinical application.

Mitochondrial transfer represents a potentially transformative gene-correction strategy for inherited hearing loss, and proof-of-concept success could accelerate a new therapeutic class for currently untreatable genetic auditory conditions.

1. 01Targets the m.1555A>G mitochondrial DNA mutation, which causes aminoglycoside-induced (antibiotic-related) hearing loss.
2. 02Mitochondrial transfer aimed to increase the ratio of healthy (wild-type) to mutant mtDNA in affected cells.
3. 03Study evaluated improvement in mitochondrial function following the transfer.
4. 04Published in Scientific Reports; likely in-vitro or preclinical model.
5. 05Represents an early but novel step toward gene-level therapy for mitochondrial hearing loss.

PMID

42115265

DOI

10.1038/s41598-026-51402-4.

Journal

Scientific Reports

Publication type

research_article

Evidence level

4

Population

Cell or animal models carrying the m.1555A>G mitochondrial DNA mutation associated with sensorineural hearing loss

Intervention

Mitochondrial transfer to correct mutant-to-wild-type mtDNA ratio