Cochlear implantation in osteogenesis imperfecta: a case series on feasibility, challenges, and outcomes

Article in CI Journal

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The following article is in Cochlear International Journal,  BCIG have access to the entire Journal as a membership benefit:

Cochlear implantation in osteogenesis imperfecta: a case series on feasibility, challenges, and outcomes

Gabrielle Boone, Joshua Sappington

Abstract

Objective:
The primary objectives of this investigation were to describe the relationship between osteogenesis imperfecta and hearing loss, evaluate the feasibility and challenges of cochlear implantation in osteogenesis imperfecta patients, and report on postoperative hearing outcomes and overall impact on quality of life.

Patients:
This case series details two female patients with osteogenesis imperfecta with a history of multiple fractures. Both patients experienced profound bilateral sensorineural hearing loss and had limited benefit from hearing aids.

Intervention(s):
Binaural cochlear implantation was performed to address profound sensorineural hearing loss in osteogenesis imperfecta patients.

Main outcome measure(s):
Preoperative and postoperative hearing assessments, along with additional clinical, radiological, and surgical data were utilized to evaluate surgical feasibility, describe specific surgical considerations and challenges in osteogenesis imperfecta patients, and determine the overall impact of cochlear implantation on patient outcomes.

Results:
Despite encountering significant surgical challenges due to the unique anatomical features of osteogenesis imperfecta, successful cochlear implantation was achieved in both patients. Postoperative evaluations demonstrated improvement in hearing performance and increased patient satisfaction and well-being.