Paraneoplastic cochleovestibulopathy: clinical presentations, oncological and serological associations.
| Autor: | Hammami MB; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.; Department of Medicine, Jacobi Medical Center-Albert Einstein College of Medicine, Bronx, New York, USA., Eggers SDZ; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA., Madhavan A; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA., Montalvo MJ; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA., Pittock SJ; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA., Dubey D; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA Dubey.Divyanshu@mayo.edu.; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA. |
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| Jazyk: | angličtina |
| Zdroj: | Journal of neurology, neurosurgery, and psychiatry [J Neurol Neurosurg Psychiatry] 2021 Nov; Vol. 92 (11), pp. 1181-1185. Date of Electronic Publication: 2021 Jul 20. |
| DOI: | 10.1136/jnnp-2021-326775 |
| Abstrakt: | 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. Competing Interests: 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. (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.) |
| Databáze: | MEDLINE |
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