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Hearing Loss in Neuromyelitis Optica Spectrum Disorder: Case Report and Systematic Review.

Stefania Kalampokini1, Effrosyni Koutsouraki1, George Psillas2

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Summary

Sudden hearing loss is rare in Neuromyelitis Optica Spectrum Disorder (NMOSD). This study highlights a case and reviews literature, finding hearing loss can be an early NMOSD symptom, impacting outcomes if not recognized.

Keywords:
NMOSDdeafnesshearing lossneuromyelitis optica spectrum disorderplasmapheresis

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Area of Science:

  • Neurology
  • Ophthalmology
  • Immunology

Background:

  • Sudden, non-traumatic hearing loss is linked to vascular/inflammatory conditions.
  • Hearing loss is an exceptionally rare manifestation of Neuromyelitis Optica Spectrum Disorder (NMOSD).
  • Aquaporin-4 antibodies are key biomarkers in NMOSD pathogenesis.

Purpose of the Study:

  • To report a rare case of peripheral auditory pathway involvement in aquaporin-4-positive NMOSD.
  • To systematically review existing literature on hearing loss in NMOSD patients.
  • To elucidate the clinical presentation, localization, and treatment outcomes of hearing loss in NMOSD.

Main Methods:

  • Case presentation of a 58-year-old female with NMOSD experiencing bilateral tinnitus and unilateral deafness.
  • Auditory brainstem response testing to identify the auditory pathway lesion.
  • Systematic literature review of MEDLINE and Scopus for NMOSD-associated hearing loss cases.

Main Results:

  • A total of 15 cases, including the current one, were identified.
  • Hearing loss predominantly affects females (73.3%) aged 26-70, often in AQP4-positive NMOSD.
  • Auditory impairment can be central or peripheral and may present with tinnitus, ataxia, or vomiting.

Conclusions:

  • Peripheral auditory pathway affection is a rare but significant presentation of NMOSD.
  • Early diagnosis and treatment of hearing loss during NMOSD relapses are critical for patient outcomes.
  • This study expands understanding of NMOSD as an autoimmune aquaporin-4 channelopathy affecting auditory pathways.