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.
No practice change warranted from a single case report; clinicians should be aware that cochlear implantation may be considered in autoimmune-related bilateral hearing loss, but outcomes data remain extremely limited.
This report expands the rare-disease evidence base for cochlear implantation in autoimmune-mediated hearing loss, informing future candidacy discussions for this emerging diagnosis.
- 01Single case report of cochlear implant use in a patient with bilateral rapidly progressive hearing loss caused by KLHL11 IgG autoimmune encephalitis.
- 02KLHL11 IgG-associated rhombencephalitis is a rare autoimmune condition affecting the brainstem and inner ear.
- 03Reports functional hearing outcomes following cochlear implantation in this rare disease context.
- 04Published in Otology & Neurotology (doi:10.1097/MAO.0000000000004935).
- 05Findings are limited to a single patient and cannot be generalized without further study.
Cochlear implantation can provide functional hearing benefit in a patient with bilateral hearing loss secondary to KLHL11 IgG-associated rhombencephalitis.
studyunclear- PMID
- 42348851
- DOI
- 10.1097/MAO.0000000000004935.
- Journal
- Otology & Neurotology
- Publication type
- case_report
- Evidence level
- 4
- Sample size
- 1
- Population
- Adult patient with bilateral rapidly progressive hearing loss secondary to KLHL11 IgG-associated rhombencephalitis
- Intervention
- Cochlear implantation
Primary outcomes
Hearing outcomes following cochlear implantation in KLHL11 IgG-associated autoimmune hearing loss