Paraneoplastic cochleovestibulopathy: Clinical presentations, oncological and serological associations

M. Bakri Hammami, Scott D.Z. Eggers, Ajay Madhavan, Mayra J. Montalvo, Sean J. Pittock, Divyanshu Dubey

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish (US)
Pages (from-to)1181-1185
Number of pages5
JournalJournal of Neurology, Neurosurgery and Psychiatry
Issue number11
StatePublished - Nov 1 2021


  • paraneoplastic syndrome
  • vertigo

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology
  • Psychiatry and Mental health


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