(A246) The use of bone-conduction implants in patients with rare genetic syndromes associated with ear malformations

Introduction: The purpose of the study is to evaluate the effectiveness and safety of bone-conduction implants in patients with rare genetic syndromes, such as Treacher-Collins and Goldenhar syndromes, presenting with ear malformations and associated conductive or mixed hearing loss.

Methods: Study

Design: Retrospective cohort study.
Setting: World Hearing Center, Institute of Physiology and Pathology of Hearing, Kajetany, Poland.
Patients: The study included 9 patients diagnosed with rare genetic syndromes, including Treacher-Collins and Goldenhar syndromes, who exhibited conductive or mixed hearing loss due to external and/or middle ear malformations.
Interventions: Bone-conduction implants were surgically implanted in patients with ear malformations who were not candidates for traditional hearing aids due to anatomical constraints.
Main Outcome Measures: Hearing improvement was evaluated using pure-tone audiometry and speech audiometry. Subjective satisfaction with the implants was assessed using the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire.

Results: All patients showed significant improvement in hearing and speech comprehension after the implantation of bone-conduction devices. Pure-tone audiometry and speech audiometry results demonstrated a marked improvement in both quiet and noisy environments. Patients reported a high level of satisfaction, with notable improvements in daily communication. The surgical procedures were safe, with a low incidence of minor complications.

Conclusions: Bone-conduction implants are an effective and safe solution for managing conductive hearing loss in patients with rare genetic syndromes associated with ear malformations. Early intervention with these implants supports auditory rehabilitation and improves quality of life, enabling better speech development, especially in children.