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The neuro-otology of Susac syndrome

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Abstract

Objective

We characterised the clinical and neuro-otological characteristics of patients with Susac syndrome.

Methods

The medical records of 30 patients with Susac syndrome were reviewed for details of their clinical presentation and course, neuro-otological symptoms, investigation results including audiology and vestibular function tests, treatment and outcomes.

Results

Our findings demonstrate that 29 of our 30 patients with Susac syndrome developed neuro-otological symptoms such as hearing loss, disequilibrium, tinnitus or vertigo during their disease course. Hearing loss was the most common neuro-otological symptom occurring in 93% of patients. A rising configuration of low-frequency greater than the high-frequency sensorineural hearing loss was the most characteristic finding on audiological testing (37% of reviewed audiograms). Disproportionately poor speech discrimination was identified in 20% of cases, and one case demonstrated a retrocochlear pattern on electrophysiological testing. Four patients required hearing aids and a further two patients required a cochlear implant due to severe hearing loss. Two out of two treated patients had improvements in hearing after the prompt administration of corticosteroids, indicating the potential for recoverable hearing loss if relapses are treated early. Effects on vestibular function were variable in ten patients who were tested, with most showing preservation of function despite significant hearing loss.

Conclusions

Neuro-otological symptoms in Susac syndrome are almost universal. In the correct clinical context, a rising configuration of low to high-frequency sensorineural hearing loss should prompt consideration of Susac syndrome. Treatment of inner ear symptoms in Susac syndrome requires further research as early immunotherapy may be beneficial.

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Author information

Author notes

  1. Todd A. Hardy and Rachael L. Taylor contributed equally to the work.

Authors and Affiliations

  1. Department of Neurology, Concord Hospital, University of Sydney, Sydney, NSW, Australia

    Todd A. Hardy, Jessica Qiu, Benjamin Trewin, Penelope J. Spring & Stephen W. Reddel

  2. Brain and Mind Centre, University of Sydney, Sydney, NSW, Australia

    Todd A. Hardy, Jerome Ip, Luke Chen, Heidi N. Beadnall, Michael H. Barnett & John D. E. Parratt

  3. Department of Physiology and Centre for Brain Research, The University of Auckland, Auckland, New Zealand

    Rachael L. Taylor

  4. Department of Neurology, Gosford Hospital, Gosford, NSW, Australia

    Billy O’Brien

  5. Department of Neurology, Campbelltown Hospital, Sydney, NSW, Australia

    Sumana Gopinath

  6. University of Notre Dame, Sydney, NSW, Australia

    Mohamed Shaffi

  7. Department of Neurology, St Vincent’s Hospital, Sydney, NSW, Australia

    Sam J. Bolitho

  8. Department of Immunology, Royal Prince Alfred Hospital, Sydney, NSW, Australia

    Roger J. Garsia & Heidi N. Beadnall

  9. Department of Neurology, Auckland City Hospital, Auckland, New Zealand

    Richard Roxburgh

  10. Department of Neurology, Christchurch Hospital, Christchurch, New Zealand

    Deborah F. Mason

  11. Department of Neurology, Sunshine Coast Hospital, Sunshine Coast, Qld, Australia

    Fiona Chan & Benjamin Tsang

  12. Department of Neurology, Cairns Hospital, Cairns, Qld, Australia

    Ian Wilson

  13. Department of Neurology, Royal Prince Alfred Hospital, Sydney, NSW, Australia

    Michael H. Barnett, Miriam S. Welgampola & G. Michael Halmagyi

  14. Department of Neurology, Royal North Shore Hospital, Sydney, NSW, Australia

    John D. E. Parratt

  15. Section of Neurology, Sydney Adventist Hospital, Sydney, NSW, Australia

    John D. G. Watson

  16. Discipline of Medicine, University of NSW, Sydney, Australia

    John D. G. Watson

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  1. Todd A. Hardy
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  2. Rachael L. Taylor
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  3. Jessica Qiu
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  16. Ian Wilson
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  20. John D. G. Watson
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  21. Miriam S. Welgampola
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  22. Stephen W. Reddel
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  24. G. Michael Halmagyi
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Corresponding author

Correspondence to Todd A. Hardy.

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Conflicts of interest

The authors declare no relevant competing interests. This work received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Ethical standards

This study was approved as a clinical audit by the Sydney Local Health District Human Research Ethics Committee—CRGH with a waiver of patient consent (HREC/16/CRGH/293), and was, therefore, performed in accordance with the 1964 Declaration of Helsinki and its later amendments.

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Hardy, T.A., Taylor, R.L., Qiu, J. et al. The neuro-otology of Susac syndrome. J Neurol 267, 3711–3722 (2020). https://doi.org/10.1007/s00415-020-10086-0

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