While Sjögren’s syndrome (SS) is well known to cause dry eyes, mouth, skin and nose (“sicca”), its association with hearing loss is often underrecognized by patients and clinicians.  In fact, hearing loss has been reported in as many as one-third or more of patients with SS.^1-4

A 2023 systematic review and meta-analysis of 11 observational studies found a 52% pooled prevalence of any type of hearing loss in SS patients (mean age 52 years). Even after excluding studies with moderate (vs low) publication bias, the prevalence remained high (37%).¹  A subgroup analysis based on sensorineural, conductive and mixed types of hearing loss (irrespective of severity) found a prevalence of 43%, 5% and 2%, respectively, among SS patients.

A 2020 cross-sectional study involving 30 patients with SS and polyneuropathy undergoing auditory function testing (by pure tone audiometry, Freiburg speech comprehension audiometry, transient evoked otoacoustic emissions and brainstem evoked response audiometry) found hearing loss by pure tone audiometry in 33% of patients with severity ranging from mild (in 60%) to severe (in 10%).² Overall 80% of patients showed pathological test results on audiometric testing. Interestingly, in those with hearing loss on pure tone audiometry, 80% had asymmetric results.

A potential mechanism for sensorineural hearing loss in SS is the involvement of central nervous system. Of note, other autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus have also been associated with hearing loss, possibly related to the vasculitis of vasa nervorum or vasa vasorum.¹ Indeed, immunosuppressants such as corticosteroids, cyclophosphamide, azathioprine and mycophenolate mofetil have been used in the treatment of sensorineural hearing loss associated with autoimmune disorders. Dryness of Eustachian tube has been suggested as risk factor for the development of conductive hearing loss.¹

Bonus Pearl: Did you know that SS is the second most common autoimmune rheumatic disease affecting an estimated 4 million cases in the USA alone?³

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References

1. V, PartalidouS, Siolos P, et al. Prevalence of hearing loss in patients with Sjögren syndrome: a systematic review and meta-analysis. Rheumatology 2023;43:233-44. Prevalence of hearing loss in patients with Sjögren syndrome: a systematic review and meta-analysis – PubMed (nih.gov)
2. Seeliger T, Bonig L, Witte T, et al. Hearing dysfunction in patients with Neuro-sjögren: a cross-sectional study. Ann Transl Med 2020;8:1069. Hearing dysfunction in patients with Neuro-Sjögren: a cross-sectional study – PubMed (nih.gov)
3. Yang TH, Xirasagar S, Cheng YF, et al. Increased prevalence of hearing loss, tinnitus and sudden deafness among patients with Sjögren’s syndrome. RMD Open 2024;10:e003308. Increased prevalence of hearing loss, tinnitus and sudden deafness among patients with Sjögren’s syndrome – PubMed (nih.gov)
4. Kim KS, Kim HS. Successful treatment of sensorineural hearing loss in Sjögren’s syndrome with corticosteroid. Korean J Intern Med 2016;31:612-615. Successful treatmenParaschou t of sensorineural hearing loss in Sjögren’s syndrome with corticosteroid – PubMed (nih.gov)

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