Successful cochlear implantation in a case of Vogt-Koyanagi-Harada disease.

Objective: To characterize the auditory phenotype of Vogt-Koyanagi-Harada disease (VKH) and highlight the potential for successful cochlear implantation (CI).

Patients: Single case report

Intervention(s): Therapeutic

Main Outcome Measure(s): AzBio Sentences in quiet and noise, CNC Words, BKB-SIN RESULTS: VKH is a systemic autoimmune disorder characterized by granulomatous uveitis accompanied by neurologic and auditory-vestibular symptoms. Development is attributed to an autoimmune response against pigmented cells, with destruction of melanocytes by T lymphocytes directed against an unidentified antigen; accordingly, the disease typically affects races with darkly pigmented skin. VKH presents as blurring of vision and eye inflammation, severe headaches, and meningismis. Auditory symptoms include sensorineural hearing loss (SNHL), tinnitus, and imbalance. SNHL can persist despite immunosuppressant therapy.This report describes successful cochlear implantation in a 26-year-old female patient with VKH who presented with complaints of blurred vision and bilateral sudden SNHL. Oral steroids initially restored hearing, but benefit ceased upon discontinuation of oral steroids and was not restored following a series of intratympanic steroid injections. The patient underwent bilateral CI, which was fast-tracked because of the autoimmune nature of her hearing loss. After implantation, her speech understanding in both quiet and noise far exceeds her pre-implant scores.

Conclusion: This unique case is the first to report successful CI in VKH. It is important for hearing health professionals to be aware of the signs and symptoms of this disease as patients may present initially with hearing loss, tinnitus, or imbalance, and may benefit from CI in cases of persistent, steroid-unresponsive, severe-to-profound SNHL.