Paraneoplastic KLHL11 encephalitis presenting with progressive tinnitus, hearing loss and ataxia

Limbic encephalitis is associated with various tumours and antibodies. We describe a 33-year-old man with progressive cochleovestibular symptoms, cerebellar ataxia, myoclonus and antibodies against Kelch-like protein 11 (KLHL11). MR scan of the brain showed hippocampal T2 hyperintensities. FDG PET scan was normal. Biopsy of his enlarged left testes showed fibrosis without neoplasia. Immunotherapy so far has not helped significantly.