Autoimmune sensorineural hearing loss: the otology-rheumatology interface

Abstract

Autoimmune sensorineural hearing loss (SNHL) is a rare clinical entity characterized by a progressive fluctuating bilateral asymmetric SNHL that develops over several weeks to months. Vestibular symptoms, tinnitus and aural fullness are present in up to 50% of patients. Due to the lack of specific diagnostic tests, both clinical suspicion and responsiveness to corticosteroids are the pillars for the diagnosis of autoimmune SNHL. The evaluation of patients in whom this condition is suspected should include a detailed history and physical examination, an audiogram, an MRI and a limited laboratory workup to exclude secondary causes of hearing loss. The low frequency of this condition, the heterogeneity in the designs of the available studies and the absence of randomized trials comparing treatment responses and assessing long-term outcomes are some of the factors accounting for the limited evidence to guide the clinician in the approach to the diagnosis and treatment of autoimmune SNHL.

Autoimmune inner ear disease secondary to Hashimoto’s thyroiditis: a case report

“…Positive response to steroids is another requisite clinical criterion [5]. There is no definite test for AIED till now although, a number of immunological studies such as an elevated ESR, CRP, and positive ANA or RA factor may be consistent with the diagnosis.…”

Sensorineural hearing loss as the first manifestation of Sjögren's syndrome

“…Vestibular symptoms may be present. 3,5,6 SNHL can be the initial manifestation of a systemic autoimmune disease such as SS, systemic lupus erythematosus, granulomatosis with polyangiitis (Wegener's), 7 polyarteritis nodosa, rheumatoid arthritis, and Cogan's syndrome. 8,9 There is a significant association between thyroid autoimmunity and Ménière's disease, which can itself be very difficult to distinguish from AIED in the first months, 10 but our patient had no vestibular symptoms.…”

Symptomatic sensorineural hearing loss in patients with ulcerative colitis

“…The hearing level in affected patients is unstable, with periods of exacerbation alternating with periods of partial or complete remission [12]. To date, the treatment of autoimmune ear disease relies mainly on steroids and, more recently, on the use of biological agents [21,22].…”