Holt-oram syndrome ad conductive hearing loss: treatment with bilateral bone conduction implant (OSIA)

Holt-Oram Syndrome is a rare genetic disorder characterized by congenital malformations of the upper limbs and cardiac anomalies, with possible involvement of the auditory system. We present the case of a 6-year-old girl diagnosed with Holt-Oram Syndrome in 2019, who also presented bilateral conductive hearing loss that did not respond to previous surgical interventions. In May 2020, a bilateral BAHA 6 with soft band was applied; however, in February 2025, following adequate temporal bone development, a bilateral bone conduction implant (OSIA) was performed, leading to significant improvement in auditory thresholds. This case highlights the importance of early monitoring and treatment of auditory anomalies in patients with Holt-Oram Syndrome and confirms the effectiveness of bone conduction implants as a therapeutic solution.