Stapedotomy in osteogenesis imperfecta patients.

Area of Science:

• Otolaryngology
• Genetics
• Orthopedics

Background:

• Osteogenesis Imperfecta (OI) is a genetic disorder characterized by bone fragility, blue sclera, and hearing loss.
• Hearing loss in OI is often conductive or mixed, frequently involving the middle ear ossicles.

Purpose of the Study:

• To evaluate the effectiveness of stapedotomy in improving hearing in patients diagnosed with Osteogenesis Imperfecta.
• To assess both immediate and long-term audiological outcomes following stapedotomy in OI patients.

Main Methods:

• A retrospective, one-group, pre-test-post-test study design was employed.
• Audiograms of nine OI patients undergoing stapedotomy for mixed hearing loss were compared pre- and post-operatively.
• Surgical findings such as stapes footplate fixation and promontory mucosa vascularity were documented.

Main Results:

• Significant improvements in air conduction (250-4000 Hz) and bone conduction (250-2000 Hz) were observed immediately post-surgery (p < 0.05).
• A significant closure of the air-bone gap (250-2000 Hz) was achieved post-stapedotomy (p < 0.05).
• Long-term follow-up showed sustained satisfactory results with an 8 dB HL mean threshold shift and a stable air-bone gap.

Conclusions:

• Stapedotomy is an effective surgical option for managing hearing loss associated with Osteogenesis Imperfecta.
• The procedure demonstrates significant short-term and long-term benefits with a low complication rate.
• Stapedotomy offers a promising solution for improving audiological function in OI patients.