TY - JOUR T1 - Paraneoplastic cochleovestibulopathy: clinical presentations, oncological and serological associations JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry SP - 1181 LP - 1185 DO - 10.1136/jnnp-2021-326775 VL - 92 IS - 11 AU - M Bakri Hammami AU - Scott D Z Eggers AU - Ajay Madhavan AU - Mayra J Montalvo AU - Sean J Pittock AU - Divyanshu Dubey Y1 - 2021/11/01 UR - http://jnnp.bmj.com/content/92/11/1181.abstract N2 - 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%)‌ ‌and‌ ‌LUZP4-IgG‌‌ ‌(n=1,‌ ‌4%). Most common neoplastic association was ‌testicular‌/‌extra-testicular‌ ‌seminoma‌ ‌ (n=13,‌ ‌50%).‌‌ Hearing‌ impairment (bilateral, 62%) was ‌present‌ ‌in‌ ‌all‌ ‌patients.‌ ‌Fifteen patients (58%) had cochleovestibular dysfunction as their initial presentation before rhombencephalitis/encephalomyelitis manifestations (hearing loss, four; acute vertigo, eight; both, three). ‌Brain‌ ‌MRI‌ ‌demonstrated‌ ‌internal‌ ‌auditory‌ ‌canal‌ ‌enhancement‌ ‌in‌ ‌four ‌patients.‌ Audiometry commonly revealed severe-profound bilateral sensorineural hearing loss. Most patients ‌had‌ a refractory course ‌despite‌ ‌immunotherapy‌ ‌and/or‌ ‌cancer‌ ‌treatment‌.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. ER -