Paraneoplastic cochleovestibulopathy: Clinical presentations, oncological and serological associations

Objective Cochleovestibulopathy is a distinguishable paraneoplastic phenotype. In this study, we evaluate clinical presentation, serological/cancer associations and outcomes of paraneoplastic cochleovestibulopathy. Methods Retrospective chart review of patients with hearing impairment and/or vestibulopathy who underwent serological evaluations for paraneoplastic antibodies between January 2007 and February 2021 was performed. Results Twenty-six patients were identified (men, n=23; median age, 45 years, range: 28-70). Biomarkers detected included: KLHL11-IgG(n=20,77% (coexisting LUZP4-IgG, n=8)), ANNA1-IgG (n=3,12%),amphiphysin-IgG(n=2,8%)andLUZP4-IgG(n=1,4%). Most common neoplastic association was testicular / extra-testicularseminoma(n=13,50%). Hearing impairment (bilateral, 62%) was presentinallpatients.Fifteen patients (58%) had cochleovestibular dysfunction as their initial presentation before rhombencephalitis/encephalomyelitis manifestations (hearing loss, four; acute vertigo, eight; both, three). BrainMRIdemonstratedinternalauditorycanalenhancementinfour patients. Audiometry commonly revealed severe-profound bilateral sensorineural hearing loss. Most patients had a refractory course despiteimmunotherapyand/orcancertreatment. Conclusion Cochleovestibulopathy commonly presents with rapidly progressive bilateral hearing loss and/or acute vertigo. However, in some patients, these symptoms present along with or following brainstem/cerebellar manifestations. KLHL11-IgG and seminoma are the most common serological and cancer associations, respectively. Recognition of this phenotype may aid in earlier diagnosis of paraneoplastic autoimmunity and associated cancer.