Treatments for Hearing Loss in Osteogenesis Imperfecta: A Systematic Review and Meta-Analysis on Their Efficacy

Scientific Reports

Ugarteburu, M., Cardoso, L., et al. (2022).

Scientific Reports, 12(1), 17125.

This systematic review and meta-analysis investigates the effects of treatment on hearing outcomes for individuals with osteogenesis imperfecta.

National Science Foundation



From database inception to November 2020

Peer-reviewed studies

13 studies included in the systematic review; 12 studies included in the meta-analysis

Findings from the systematic review indicate improved speech perception with cochlear implants for individuals with osteogenesis imperfecta. Improved hearing thresholds with bone-anchored hearing aids and implantable hearing aids were additionally noted for this population. Success was limited by bone fragility and high vascularity. The meta-analysis showed stapes surgeries have a low success rate due to atrophy and fracture of the ossicles. Limitations to this review include the small number of studies due to the rarity of this condition.