Efficacy of Cochlear Implantation in Kelch-like Protein-11 (KLHL11) IgG-Associated Rhombencephalitis

To describe cochlear implant outcomes in a patient with bilateral rapidly progressive hearing loss secondary to Kelch-like protein-11 (KLHL11) IgG rhombencephalitis.

A 26-year-old male presenting to neurotology clinic at a tertiary care center for evaluation of bilateral rapidly progressive sensorineural hearing loss, unresponsive to systemic corticosteroid therapy.

Bilateral simultaneous cochlear implantation.

Postimplant speech testing (CNC word, AzBio sentence scoring), and changes to hearing function with fluctuations in systemic disease symptomatology.

Preoperative testing demonstrated poor speech perception (bilateral AzBio sentence in quiet: 43%, right-sided CNCw score: 38%, and left-sided CNCw score: 0%). Despite systemic disease progression of KLHL11-IgG rhombencephalitis, including worsening cerebellar ataxia and dysarthria, cochlear implant performance did not decrease at the time of last follow-up. At 1-year, binaural and monaural speech testing were both significantly improved compared with the 1-month testing (bilateral AzBio sentence in quiet: 91%, right-sided CNCw score: 70%, and left-sided CNCw score: 32%).

Cochlear implantation may offer meaningful auditory rehabilitation in patients with KLHL11-IgG associated rhombencephalitis with relative auditory nerve sparing, despite ongoing disease progression. Further studies are required to evaluate the durability and generalizability of cochlear implantation across the spectrum of disease-related hearing loss.