Efficacy of Cochlear Implantation in Kelch-like Protein–11 (KLHL11) IgG–Associated Rhombencephalitis
Karl R. Khandalavala, Ty J. Skyles, Kayla R. Goulson, John I. Lane, Divyanshu Dubey, Matthew L. CarlsonObjective:
To describe cochlear implant outcomes in a patient with bilateral rapidly progressive hearing loss secondary to Kelch-like protein–11 (KLHL11) IgG rhombencephalitis.
Patient:
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.
Interventions:
Bilateral simultaneous cochlear implantation.
Main Outcome Measures:
Postimplant speech testing (CNC word, AzBio sentence scoring), and changes to hearing function with fluctuations in systemic disease symptomatology.
Results:
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%).
Conclusions:
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.