Sjogren’s syndrome;

Is there a connection between Sjögren’s syndrome and hearing loss?

FA Manian MD, MPH, , , , , , , , Leave a comment

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

Liked this post? Download the app on your smart phone and sign up below to catch future pearls right into your inbox, all for free!

Subscribe to Blog via Email

Enter your email address to subscribe to this blog and receive notifications of new posts by email.

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)

 

Disclosures/Disclaimers: The listed questions and answers are solely the responsibility of the author and do not necessarily represent the official views of Mercy Hospital-St. Louis, Massachusetts General Hospital, Harvard Catalyst, Harvard University, their affiliate academic healthcare centers, or its contributors. Although every effort has been made to provide accurate information, the author is far from being perfect. The reader is urged to verify the content of the material with other sources as deemed appropriate and exercise clinical judgment in the interpretation and application of the information provided herein. No responsibility for an adverse outcome or guarantees for a favorable clinical result is assumed by the author. Thank you!

Is there a connection between Sjögren’s syndrome and hearing loss?

My patient with primary Sjogren’s syndrome has now been diagnosed with COPD despite lack of a significant smoking history. Is there a connection between Sjogren’s syndrome and COPD?

FA Manian MD, MPH, , , , , Leave a comment

Increasing body of evidence suggests that COPD in patients with primary Sjögren’s syndrome (PSS) is not uncommon even among those who never smoked (1).

 
A 2015 study of patients with PSS reported that overall 41% of patients with PSS, including 30% of those who never smoked, fulfilled the Global Initiative for Chronic Obstructive Lung Disease (GOLD) criteria for COPD. More specifically, pulmonary function tests (PFTs) showed decreased vital capacity (VC), forced expiratory volume in 1 second (FEV-1)  and DLCO in patients with PSS. Importantly, lab inflammatory and serological features were poorly associated with PFT results, while radiographic signs of interstitial lung disease (ILG) were absent in one-half of patients with PSS and COPD (1).

 
A longitudinal study with a mean follow-up of 11 years found a 37% rate of development of COPD among patients with PSS (2). Another related study reported a poor correlation between respiratory symptoms and COPD disease as assessed by PFTs in PSS, with the authors recommending that PFTs be performed “liberally” in all patients with PSS regardless of symptoms (3).

 
Lastly, a population-based cohort study of female adults found significantly higher rate of COPD among patients with PSS compared to controls (4).

 
Although the exact pathogenic mechanism behind PSS-associated COPD is unclear, xerotrachea and impaired mucocilliary clearance, as well as inflammatory infiltrates in the exocrine glands of the airways, all leading to physical obstruction and bronchial hyperreactivity have been suggested (1).

 

Bonus Pearl: Did you know that COPD is associated with many other autoimmune diseases (eg, rheumatoid arthritis and systemic lupus erythematosus), and a genetic link has been implicated between COPD and autoimmunity? (5,6).

 

If you liked this post, sign up under MENU and catch future pearls right into your mailbox!

References
1. Nilsson AM, Diaz S, Theander E, et al. Chronic obstructive pulmonary disease is common in never-smoking patients with primary Sjögren’s syndrome. J Rheumatol 2015;42:464-71. https://www.researchgate.net/publication/270907531_Chronic_Obstructive_Pulmonary_Disease_Is_Common_in_Never-smoking_Patients_with_Primary_Sjogren_Syndrome
2. Mandl T, Diaz S, Ekberg O, et al. Frequent development of chronic obstructive pulmonary disease in primary SS-result of a longitudinal follow-up. Rheumatology 2012;51:941-46. https://www.researchgate.net/publication/221760110_Frequent_development_of_chronic_obstructive_pulmonary_disease_in_primary_SS-results_of_a_longitudinal_follow-up
3. Bolmgren VS, Olssson P, Wollmer P, et al. Respiratory symptoms are poor predictors of concomitant chronic obstructive pulmonary disease in patients with primary Sjögren’s syndrome. Rheumatol Int 2017;37:813-18. https://link.springer.com/content/pdf/10.1007/s00296-017-3678-5.pdf
4. Shen TC, Wu BR, Chen HJ, et al. Risk of chronic obstructive pulmonary disease in female adults with primary Sjögren’s syndrome. A nationwide population-based cohort study. Medicine 2016; 95:1-6. http://europepmc.org/abstract/MED/26962839
5. Hemminki K, Liu X, Ji J et al. Subsequent COPD and lung cancer in patients with autoimmune disease. Eur Respir J 2011;37:463-74. https://www.ncbi.nlm.nih.gov/pubmed/21282811
6. Ji X, Niu X, Qian J, et al. A phenome-wide association study uncovers a role for autoimmunity in the development of chronic obstructive pulmonary disease. Resp Cell Mol Biol 2018;58:777-79. https://www.atsjournals.org/doi/10.1165/rcmb.2017-0409LE

My patient with primary Sjogren’s syndrome has now been diagnosed with COPD despite lack of a significant smoking history. Is there a connection between Sjogren’s syndrome and COPD?