Outcomes of Bonebridge Implantation in 10 Patients with Rare Genetic Syndromes and Difficult Anatomy.

Background

Congenital hearing loss occurs in about 2 of every 1000 newborns, of which half probably have a genetic origin. In syndromic patients, hearing impairment often results from craniofacial malformations affecting the outer and middle ear. Anatomical limitations such as microtia or external auditory canal atresia often preclude conventional air-conduction hearing aids, leaving bone-conduction devices as one viable option. However, surgical intervention in such patients is challenging. This study aimed to evaluate the audiological outcomes, safety, and effectiveness of the Bonebridge BCI 602 implant in 10 patients with genetic syndromes.

Methods

The case series was made up of 10 patients aged 6-45 years, each diagnosed with a congenital syndrome affecting the external and/or middle ear. All cases involved surgical implantation of the Bonebridge system. Audiological outcomes were evaluated in free-field conditions on the day of sound processor activation and at 3-6 months follow-up via pure-tone and speech audiometry.

Results

All surgical procedures were completed without serious adverse events, and the incidence of postoperative complications was low. Audiological outcomes showed clinically significant hearing improvement in all patients following Bonebridge implantation. Post-implantation hearing thresholds ranged from 25 to 40 dB HL, with notable gains in speech perception in both quiet and noisy environments.

Conclusions

The Bonebridge implant appears to be a safe and effective option for auditory rehabilitation in patients with hearing loss associated with various genetic syndromes involving craniofacial malformation. However, this complex patient population requires individual assessment, interdisciplinary evaluation, and careful surgical planning.