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

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

Tabea Seeliger  1 , Lena Bönig  1 , Torsten Witte  2 , Thea Thiele  2 , Anke Lesinski-Schiedat  3 , Martin Stangel  1 , Thomas Lenarz  3 , Nils Christian Prenzler  3 , Thomas Skripuletz  1
Affiliations

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

Tabea Seeliger et al. Ann Transl Med. 2020 Sep.

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.

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

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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 fees from Takeda, personal fees from CSL Behring, grants and personal fees from Sanofi-Genzyme, personal fees from Grifols, grants and personal fees from Merck-Serono, personal fees from Roche, grants and personal fees from Novartis, personal fees from Teva, personal fees from MedDay, personal fees from Alexion, grants and personal fees from Biogen, personal fees from Janssen, outside the submitted work. Dr. ThS reports personal fees from Alexion, Bayer Vital, Biogen, Celgene, CSL Behring, Merck, Novartis, Roche, Sanofi Aventis, outside the submitted work. Dr. ThS serves as an unpaid editorial board member of Annals of Translational Medicine from Jan 2020 to Dec 2021. The other authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Correlation of age and pure tone average 4 (PTA4) in the better ear with classification of the degree of hearing loss in accordance with the European Working Group on Genetics of Hearing Impairment. Rectangles indicate patients with evidence of retrocochlear auditory dysfunction.
Figure 2
Figure 2
Retrocochlear auditory dysfunction in 19/30 patients (63%) specified for essential pathological findings. ABR, auditory brainstem response.
See this image and copyright information in PMC

References

    1. Sjögren H. Zur Kenntnis der Keratoconjunctivitis Sicca. Acta Ophthalmol 1933;13:1-39. 10.1111/j.1755-3768.1935.tb04186.x - DOI - PubMed
    1. Moreira I, Teixeira F, Martins Silva A, et al. Frequent involvement of central nervous system in primary Sjögren syndrome. Rheumatol Int 2015;35:289-94. 10.1007/s00296-014-3097-9 - DOI - PubMed
    1. Pars K, Pul R, Schwenkenbecher P, et al. Cerebrospinal Fluid Findings in Neurological Diseases Associated with Sjögren’s Syndrome. Eur Neurol 2017;77:91-102. 10.1159/000454765 - DOI - PubMed
    1. Iwai K, Amo K, Kuki I, et al. An unusual manifestation of Sjögren syndrome encephalitis. Brain Dev 2019;41:217-20. 10.1016/j.braindev.2018.08.004 - DOI - PubMed
    1. Yuan J, Gong L, Wu H, et al. Case report of primary Sjögren Syndrome with simple trigeminal lesion as initial symptom. J Neuroimmunol 2018;324:126-8. 10.1016/j.jneuroim.2018.08.005 - DOI - PubMed