Patients with Sjögren's are at increased risk of hearing loss, both conductive (hearing loss due to problems with the ear canal, eardrum, Eustachian tube or ear bones) and sensorineural (nerve deafness) types. Autoimmune hearing loss can occur; this is a type of sensorineural hearing loss that results when a person's antibodies attack the auditory nervous system. Treatment is similar to other forms of autoimmune hearing loss and centers on oral or intratympanic steroids and, at times, antivirals. Occasionally, other immunosuppressant drugs are used for steroid-sparing effects. Partial return of hearing can occur if treatment is prompt, although progressive deterioration is more common. Audiograms should be ordered in all Sjögren's patients who complain of hearing loss, and all other causes of hearing loss that are treatable must be excluded before the patient diagnosed with Sjögren's-related autoimmune hearing loss.
Tinnitus, or ringing in the ears, also occurs more frequently in patient with Sjögren's. In patients with sensorineural hearing loss, tinnitus is often a byproduct of the nerve damage. However, even Sjögren's patients with normal hearing often complain of tinnitus for reasons that are unknown. Tinnitus occurs more often in patients with anxiety and depression; on many occasions, treating the anxiety and depression can improve the patient's subjective tinnitus. While most patients are able to live with the tinnitus, some patients suffer so greatly that more aggressive treatment such as tinnitus maskers (electronic hearing aids that emit sounds to mask the ringing caused by tinnitus), biofeedback, or medication such as antidepressants may be necessary. Acupuncture has also been found to be helpful in many patients.
Otalgia, or ear pain, can occur in as many as a quarter of Sjögren's patients. The origin of the ear pain is also largely unknown, although many suspect that the dryness in the upper airway can lead to Eustachian tube dysfunction. Because the Eustachian tube serves to equalize pressure within the middle ear, when the Eustachian tube is diseased or dry the middle ear develops negative pressure, which can lead to pain, hearing loss, fluid accumulation, and infection. The treatment of Eustachian tube dysfunction depends on the severity of the disease; avoiding decongestants and using a nasal steroid and nasal saline to improve function is usually all that is necessary. In severe cases of Eustachian tube dysfunction, patients may suffer from recurrent fluid buildup in the middle ear and middle ear infections. Patients with this level of disease may benefit from antibiotics or even a myringotomy tube (a tube placed in the eardrum to drain the middle ear). Dryness in the oropharynx due to Sjögren's can also potentially cause referred pain to the ears.
Ear pain, redness, and swelling may also occur due to relapsing polychondritis. This condition causes autoimmune inflammation of ear cartilage as well as other cartilaginous structures in the head and neck and may occur as an isolated condition or in association with other autoimmune diseases like Sjögren's. It is usually treated with high-dose oral steroids and other immunosuppressant drugs, and antibiotics for bacterial infections.
*Excerpt from “The Sjögren’s Book”, Fifth Edition, Wallace DJ. 2022 pp.260-261
