Bilateral Cochlear Implantation in Vogt-Koyanagi-Harada Syndrome: A Case Report

Objective: To evaluate cochlear implantation in Vogt-Koyanagi-Harada syndrome with regard to surgical difficulties and hearing outcomes. Patients: Single case report. Intervention(s): Therapeutic complete electrode insertion during surgery despite evident intra-cochlear fibrosis. Main Outcome Measure(s): Postoperative speech audiometry, categories of auditory performance, and speech intelligibility rating. Results: Vogt-Koyanagi-Harada syndrome is a multisystem autoimmune disease that affects tissues containing melanin. It is characterized by bilateral uveitis with auditory, vestibular, and dermatologic manifestations. Standard treatment comprises aggressive administration of systemic corticosteroids. This report describes the use of bilateral cochlear implants in a 30-year-old Saudi woman who presented with uveitis and was diagnosed with Vogt-Koyanagi-Harada syndrome. She had progressive hearing loss in both ears and experienced minimal improvement with hearing aids. The patient underwent sequential cochlear implantation, which was challenging because it was difficult to insert the electrode in the right ear due to intra-cochlear fibrosis. After more than 5 years of follow-up, she has good hearing in both ears, despite the advancement of ocular disease and recurring visual complaints. Conclusion: This study suggests that patients with Vogt-Koyanagi-Harada syndrome could develop intra-cochlear fibrosis during cochlear implantation due to the autoimmune nature of disease. Moreover, cochlear implantation becomes more difficult with disease advancement. Therefore, it is essential that healthcare professionals consider early detection and prompt treatment of hearing loss in patients with this syndrome.