Related Experiment Video
Updated: Oct 27, 2025

Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
Paraneoplastic cochleovestibulopathy: clinical presentations, oncological and serological associations
M Bakri Hammami1,2, Scott D Z Eggers3, Ajay Madhavan3
1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
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.
Related Concept Videos
Barrett Esophagus-II: Clinical Manifestations and Management
To diagnose Barrett's esophagus, healthcare providers often recommend an endoscopy for those showing symptoms of acid reflux. The procedure...
COPD: Pathogenesis and Clinical Features
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
Aneurysm II: Clinical Manifestations and Diagnostic Studies
Acute Pancreatitis II: Clinical Manifestations and Management
Chemotherapy-Induced Nausea and Vomiting: Dopamine Receptor Antagonists
Phenothiazines, such as prochlorperazine...
Venous Thrombosis II: Clinical Manifestations and Diagnostic Studies

