Paraneoplastic cochleovestibulopathy: clinical presentations, oncological and serological associations
M Bakri Hammami, Scott D.Z. Eggers, Ajay A. Madhavan, Mayra Montalvo, Sean J. Pittock, Divyanshu Dubey
Abstract
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.