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Bilateral hearing loss preceding rhomboencephalitis – a hint for Kelch-like 11 syndrome

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Abstract

Background

Paraneoplastic neurological syndromes have diverse clinical presentations and offer an opportunity for early diagnosis of malignancy and treatment. Recently, a new paraneoplastic syndrome associated with seminoma was described, consisting of rhombencephalitis with antibodies targeting the Kelch-like protein 11 (KLHL11). Questions were raised as to the spectrum of clinical symptoms and strength of association to seminoma.

Methods

We present a 45-year-old man with bilateral sensorineural hearing loss, vertigo, and progressive ataxia. An extensive diagnostic workup led to the diagnosis of anti-KLHL11 paraneoplastic syndrome based on an immunofluorescence assay showing a typical pattern and a confirmatory serological assay. As a result, the patient underwent a meticulous search for an underlying seminoma.

Results

Although initially, all images were interpreted as negative, a revision of the positron emission tomography-CT (PET-CT) examination identified a small mediastinal suspicious mass. The mass was resected, and pathological examination confirmed it to be an extra-testicular seminoma.

Conclusions

Patients presenting with progressive sensorineural hearing loss, vertigo, and ataxia should be evaluated for KLHL11 paraneoplastic syndrome. Furthermore, we support a strong association between anti-KLH11 rhombencephalitis and an underlying seminoma and recommend a thorough search for an undiagnosed germ cell tumor in these patients.

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Authors and Affiliations

  1. Department of Neurology, Tel Aviv Medical Center, Tel Aviv, 6243906, Israel

    David Krivitski, Yifat Alcalay & Avi Gadoth

  2. Immunology Laboratory, Epilepsy Service Imaging Unit, Tel Aviv Medical Center, 6243906, Tel Aviv, Israel

    Yifat Alcalay

  3. Encephalitis Center, Epilepsy Service Imaging Unit, Tel Aviv Medical Center, 6243906, Tel Aviv, Israel

    Yifat Alcalay, Yael Paran, Orna Eisenstein & Avi Gadoth

  4. Department of Thoracic Surgery, Epilepsy Service Imaging Unit, Tel Aviv Medical Center, 6243906, Tel Aviv, Israel

    Michael Peer

  5. Infectious Disease Unit, Epilepsy Service Imaging Unit, Tel Aviv Medical Center, 6243906, Tel Aviv, Israel

    Yael Paran

  6. Imaging Division, Epilepsy Service Imaging Unit, Tel Aviv Medical Center, 6243906, Tel Aviv, Israel

    Orna Eisenstein

  7. Department of Nuclear Medicine, Sheba Medical Center, Tel Hashomer, Israel

    Tima Davidson

Authors
  1. David Krivitski
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  2. Yifat Alcalay
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  3. Michael Peer
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  4. Yael Paran
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  5. Orna Eisenstein
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  6. Tima Davidson
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  7. Avi Gadoth
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Corresponding author

Correspondence to David Krivitski.

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The patient has consented to the submission of the case report to the journal. This is an observational study with a description of a single clinical case – No ethical approval is required.

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Informed consent was obtained from the patient.

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All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript.

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Krivitski, D., Alcalay, Y., Peer, M. et al. Bilateral hearing loss preceding rhomboencephalitis – a hint for Kelch-like 11 syndrome. Neurol Sci 44, 369–372 (2023). https://doi.org/10.1007/s10072-022-06369-1

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  • DOI: https://doi.org/10.1007/s10072-022-06369-1

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