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Robotic Cochlear Implantation for Direct Cochlear Access
Published on: June 16, 2022
Cochlear implantation in 3 patients with osteogenesis imperfecta: imaging, surgery and programming issues
Liselotte J C Rotteveel1, Andy J Beynon, Lucas H M Mens
1Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. l.rotteveel@kno.umcn.nl
Audiology & Neuro-Otology
|December 7, 2007
Summary
Cochlear implantation in osteogenesis imperfecta (OI) patients presents unique surgical challenges due to bone fragility. Despite difficulties like nonauditory stimulation, successful hearing outcomes are achievable with careful planning and management.
Area of Science:
- Otolaryngology
- Genetics
- Biomaterials Science
Background:
- Osteogenesis imperfecta (OI) is a genetic connective tissue disorder affecting collagen type I production, leading to bone fragility.
- Hearing loss is common in OI patients (35-60%), with a subset (2-11%) potentially benefiting from cochlear implantation.
Observation:
- Surgical findings in OI patients included brittle scutum, sclerotic cochlea, hyperplastic middle ear mucosa, and persistent bleeding.
- One case experienced electrode misplacement into the semicircular canal due to severe CT-identified deformities.
- All cases faced programming challenges due to nonauditory stimulation, impacting device usability.
Findings:
- Deviant electrode voltages and spatial spread of neural excitation indicated altered otic capsule conductivity and intracochlear channel interaction.
- Facial nerve stimulation (FNS) occurred frequently, with consistent pitch variations related to FNS in one patient.
- Despite electrophysiological anomalies, significant open-set phoneme scores (81% and 78%) were achieved in two patients after one year.
Implications:
- Cochlear implantation in OI is feasible and safe when surgeons are prepared for temporal bone abnormalities.
- Preoperative imaging is crucial for understanding petrosal bone morphology and counseling patients on potential risks like misplacement.
- Managing FNS requires frequent device refitting, highlighting the need for specialized rehabilitation protocols for OI patients undergoing cochlear implantation.