Power stapes: an alternative method for treating hearing loss in osteogenesis imperfecta?

Area of Science:

• Otolaryngology
• Audiology
• Genetics

Background:

• Osteogenesis imperfecta (OI) is a genetic disorder characterized by brittle bones, often leading to conductive or mixed hearing loss.
• Traditional hearing rehabilitation methods may have limitations in OI patients.

Purpose of the Study:

• To evaluate the efficacy and safety of a one-step surgical approach combining power stapes, stapedotomy, and middle ear implantation with Vibrant SoundBridge (VSB) for hearing rehabilitation in patients with Osteogenesis Imperfecta (OI).

Main Methods:

• A retrospective case series involving two patients (mother and son) with genetically confirmed OI Type I and severe to profound mixed hearing loss.
• Surgical interventions included sequential unilateral and bilateral power stapes procedures with VSB implantation.
• Audiological assessments (pure-tone, free-field audiometry, word recognition) and high-resolution computed tomography of temporal bones were used for pre- and post-operative evaluation.

Main Results:

• All patients experienced favorable hearing outcomes, with an average improvement of 36.8 dB compared to preoperative values.
• The primary intraoperative complication was manageable severe middle ear mucosal bleeding.
• No instances of inner ear trauma were reported.

Conclusions:

• Power stapes surgery, when combined with VSB implantation, is a safe and promising option for hearing loss management in select OI patients.
• This approach offers a new, advantageous application of VSB for mixed hearing loss associated with severe otosclerosis and elevated bone-conduction thresholds.