Cochlear Implantation in a Patient With Pfeiffer Syndrome and Temporal Bone Vascular Anomalies

Objective: Pfeiffer syndrome is a rare craniosynostotic disorder resulting in premature bony fusion of the skull, which can result in abnormal temporal bone and vascular anatomy and hearing loss. Cochlear implantation in these patients requires thoughtful surgical planning given the potential for limited access to the cochlea because of subcutaneous or intratemporal vasculature. Herein, we present a patient with Pfeiffer syndrome who underwent successful cochlear implantation using preoperative Doppler ultrasound to identify large extracranial venous anatomy followed by a modified transcanal surgical approach. Patients: An adult female patient with Pfeiffer syndrome. Intervention(s): Preoperative axial computed tomography, catheter angiography, Doppler ultrasound, and right side cochlear implant. Main Outcome Measure(s): Surgical feasibility and audiometric outcomes including aided thresholds and word recognition score. Results: Successful cochlear implantation was performed via a modified transcanal approach with blind sac closure of the external auditory meatus. Full electrode insertion was obtained through a round window approach. No extracranial or intratemporal vessels were encountered during surgery. Conclusion: Patients with significant craniosynostoses and vascular malformations of the temporal bone can undergo successful cochlear implantation. Careful preoperative planning with high-resolution CT, MRA, and MRV, and/or traditional catheter angiography can assist in determining surgical feasibility and minimizing risk. We recommend preoperative Doppler ultrasound of abnormal extracranial vessels to assist in safe placement of incisions and hardware.

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