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Ann Transl Med. 2020 Sep;8(17):1069. doi: 10.21037/atm-20-1856.

Hearing dysfunction in patients with Neuro-Sjögren: a cross-sectional study.

Annals of translational medicine

Tabea Seeliger, Lena Bönig, Torsten Witte, Thea Thiele, Anke Lesinski-Schiedat, Martin Stangel, Thomas Lenarz, Nils Christian Prenzler, Thomas Skripuletz

Affiliations

  1. Department of Neurology, Hannover Medical School, Hannover, Germany.
  2. Department of Clinical Immunology & Rheumatology, Hannover Medical School, Hannover, Germany.
  3. Department of Otolaryngology, Hannover Medical School, Hannover, Germany.

PMID: 33145288 PMCID: PMC7575973 DOI: 10.21037/atm-20-1856

Abstract

BACKGROUND: Sjögren's syndrome is an immunologically mediated disease with salivary and lacrimal gland destruction characterised by typical sicca symptoms of dry mouth and eyes. Awareness of extraglandular neurological manifestations such as polyneuropathy and affection of cranial nerves is rising. Hearing loss as consequence of involvement of the vestibulocochlear nerve presents a severe disability. The exact prevalence and nature of hearing dysfunction in patients with Neuro-Sjögren has been insufficiently evaluated to date.

METHODS: Thirty patients with Sjögren's syndrome (ACR-EULAR classification criteria) and polyneuropathy were included in the study in the time period between 11/2016 and 03/2018. The median age was 59 years and 57% were females. Auditory function was investigated by pure tone audiometry, Freiburg speech comprehension audiometry, transient evoked otoacoustic emissions and brainstem evoked response audiometry.

RESULTS: Pure tone audiometry revealed hearing loss in 10/30 patients (33%) with severity ranging from mild in most patients (60%) to severe in 10%. In addition, pathological audiometric test findings showed retrocochlear auditory dysfunction in 14 further patients. In total, 24/30 patients (80%) showed pathological test results on audiometric testing suggesting hearing dysfunction.

CONCLUSIONS: In conclusion, our results show that hearing dysfunction as a possible consequence of cranial neuropathy in patients with Neuro-Sjögren has been underestimated in previous studies.

2020 Annals of Translational Medicine. All rights reserved.

Keywords: Neuro-Sjögren; Sjögren’s syndrome; cochlear nerve; cranial nerve; neuropathy; polyneuropathy

Conflict of interest statement

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/atm-20-1856). Dr. MS reports personal fees from Bayer Healthcare, personal

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