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Paraneoplastic cochleovestibulopathy: clinical presentations, oncological and serological associations
  1. M Bakri Hammami1,2,
  2. Scott D Z Eggers3,
  3. Ajay Madhavan3,
  4. Mayra J Montalvo3,
  5. Sean J Pittock1,3,
  6. Divyanshu Dubey1,3
  1. 1 Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
  2. 2 Department of Medicine, Jacobi Medical Center-Albert Einstein College of Medicine, Bronx, New York, USA
  3. 3 Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr Divyanshu Dubey, Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Dubey.Divyanshu{at}


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.

  • vertigo
  • paraneoplastic syndrome

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  • Contributors MBH contributed to acquisition and analysis of data, drafting the manuscript and figures. SDZE, AM, MJM and SJP contributed to acquisition and analysis of data, and critical revision of the manuscript. DD contributed to conception and design of the study, acquisition and analysis of data and study supervision.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests MBH, SDZE, AM and MJM have no competing interests to disclose. DD and SJP have a patent pending for leucine zipper 4 (LUZP4) and kelch-like protein 11 (KLHL11) as a marker for neurological autoimmunity and testicular germ cell tumours.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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