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Hearing abnormalities in multiple sclerosis: clinical semiology and pathophysiologic mechanisms

  • Neurological Update
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Abstract

Auditory manifestations from multiple sclerosis (MS) are not as common as the well-recognized sentinel exacerbations of optic neuritis, partial myelitis, motor weakness, vertiginous episodes, heat intolerance, and eye movement abnormalities. This paper discusses four cases of auditory changes, secondary to MS, and describes the first case, to our knowledge, of palinacousis, the perseveration of hearing, despite cessation of the sound stimulus. For each we characterize the initial complaint, the diagnostic work up, and ultimately, underscore the individualized treatment interventions, that allowed us to achieve a remission in all four cases. Individually codifying the treatment regimens served to mitigate, if not to abolish, the clinical derangements in hearing. Special attention is focused upon examination of the clinical manifestations and the pathophysiologic mechanisms which are responsible for them. We further emphasize the differential diagnostic considerations, and physical exam findings, along with the results of laboratory testing, neuro-imaging sequences, and lesion localization. Taken together, such information is germane to organizing cogently coherent strategic treatment plan(s). We believe that this small case series represents a clinically pragmatic example of ‘precision medicine’; a principal theme and goal throughout this paper, the achievement of such in MS, but also as an illustration for the assessment and management schema for neuroimmunologic disorders in general.

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Acknowledgements

The authors wish to thank Mr. Jason Ooi and Dr. Matthew Parsons for their rendering of the auditory system illustration (Figure 7), based upon the conceptions provided by Elliot and Teresa Frohman.

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Authors and Affiliations

  1. Department of Neurology, Doctor’s Health at Renaissance Health Neurology Institute, Austin, USA

    Roberto A. Cruz

  2. Department of Neurology, University of Texas Rio Grande Valley School of Medicine, Edinburg, USA

    Roberto A. Cruz

  3. Dell Medical School, University of Texas at Austin, Austin, USA

    Thomas Varkey

  4. Colangelo College of Business, Grand Canyon University, Phoenix, Az, USA

    Thomas Varkey

  5. Kent State University (KSU), Pontifícia Universidade Católica do Paraná (PUR-PR), Curitiba, Brazil

    Ana Flavia

  6. The Frohman Foundation, Austin, TX, USA

    Ana Flavia, Ana Paula Amatuzzi Samways, Alejandro Garza, Gabrielle Greenlee, Margaret Friess, Jayne Sconzert, Afaf Aijaz, Teresa C. Frohman & Elliot M. Frohman

  7. Dow International Medical University, Karachi, Pakistan

    Afaf Aijaz

  8. Department of Internal Medicine, Universidade Federal do Parana, Curitiba, PR, Brazil

    Walter Arruda

  9. Department of Neurology, Nossa Senhora das Graças Hospital, Curitiba, PR, Brazil

    Juliana Khouri

  10. Department of Neurology, Dell Medical School, University of Texas at Austin, Austin, USA

    Kent Ellington

  11. Laboratory of Neuroimmunology, Professor Lawrence Steinman, Stanford University School of Medicine, Palo Alto, USA

    Teresa C. Frohman & Elliot M. Frohman

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  1. Roberto A. Cruz
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Contributions

RAC: conception and critical revision of the manuscript for intellectual content. TV: conception and critical revision of the manuscript for intellectual content. AF: critical revision of the manuscript for intellectual content. APAS: critical revision of the manuscript for intellectual content. AG: critical revision of the manuscript for intellectual content. GG: critical revision of the manuscript for intellectual content. MF: critical revision of the manuscript for intellectual content. JS: critical revision of the manuscript for intellectual content. AA: critical revision of the manuscript for intellectual content. WA: critical revision of the manuscript for intellectual content. JK: critical revision of the manuscript for intellectual content. KE: critical revision of the manuscript for intellectual content. TCF: conception and critical revision of the manuscript for intellectual content; final revision, organizational review, reduction in the size of the paper, and elimination of redundancies. EMF: conception and critical revision of the manuscript for intellectual content; final revision, size reduction, and critical review with TCF.

Corresponding author

Correspondence to Elliot M. Frohman.

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Conflicts of interest

Roberto A. Cruz: MS fellowship funded by the MS society. Thomas Varkey: Has nothing to disclose. Ana Flavia: Has nothing to disclose. Ana Paula Amatuzzi Samways: Has nothing to disclose. Alejandro Garza: Has nothing to disclose. Gabrielle Greenlee: Has nothing to disclose. Margaret Freis: Has nothing to disclose. Jayne Sconzert: Has nothing to disclose. Afaf Aijaz: Has nothing to disclose. Walter Arruda: Has nothing to disclose. Juliana Khouri: Has nothing to disclose. Kent Ellington: Has nothing to disclose. Teresa C. Frohman: Has received consulting honorarium from Genzyme. Elliot M. Frohman: Has received speaker and consulting fees from Novartis, Genzyme, Biogen, Alexion and Janssen.

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Cruz, R.A., Varkey, T., Flavia, A. et al. Hearing abnormalities in multiple sclerosis: clinical semiology and pathophysiologic mechanisms. J Neurol 269, 2792–2805 (2022). https://doi.org/10.1007/s00415-021-10915-w

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  • DOI: https://doi.org/10.1007/s00415-021-10915-w

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