Autoimmune inner ear disease (AIED): A diagnostic challenge

Abstract

Autoimmune inner ear disease (AIED) has been defined as a condition of bilateral sensorineural hearing loss (SNHL), caused by an ‘uncontrolled’ immune system response. The inner ear can be the direct target of the immune response, but it can be additionally damaged by a deposition of circulating immune complexes or by systemic immune-mediated diseases. The clinical expression of immune-mediated inner ear disease shows a progressive bilateral and asymmetric SNHL profile, which typically benefits from a steroid and immunosuppressive therapy. The onset of AIED is between 3 and 90 days. Cochlear symptoms can be associated with vestibular disorders and in 15%–30% of cases, AIED occurs in the contest of a systemic autoimmune disease. Currently, the onset of immune-mediated SNHL is not a well-understood process and the pathogenetic mechanisms of AIED remain unclear. Furthermore, there are no standardized diagnostic criteria or reliable diagnostic tests for the diagnosis of AIED. Hence, the definition of immune-mediated cochleovestibular disorders is a challenging diagnosis based on exclusion. A close collaboration between otolaryngologists, audiologists and rheumatologists is recommended, in order to achieve the multidisciplinary management of this rare entity, since an early AIED identification and a prompt medical treatment might result in acceptable hearing outcomes. The paper describes the clinical features of AIED and offers a diagnostic flow-chart to use in the clinical assessment of this condition.

Human Leukocyte Antigen (HLA) Influence on Prognosis of Autoimmune Hearing Loss

“…Among the nonspecific autoimmune testings, ANA was relatively elevated in our sample (35%) ( Table 2 ). In general, the sensitivity of the ANA test for autoimmune diseases is very high, but the specificity is quite low; the ANA result must be interpreted in the specific context of every individual patient’s symptoms [ 2 , 7 ]. In our study, specific autoimmune testings, such as the lymphocyte migration inhibition assays, the lymphocyte transformation test, and assessments of tumor necrosis factor and anti-cochlear antibodies have not been used, as their diagnostic accuracy for AIHL remains undetermined [ 20 ].…”

“…Many studies [ 7 , 10 , 11 , 32 , 33 , 34 ] found that the effect of steroids on AIHL was not so beneficial, only 43% to 70% of patients with AIHL responded successfully to steroids, and over time, a further deterioration of hearing was noted. The real efficacy of steroid treatment was estimated to be only 14% [ 2 ], as indicated by our hearing outcome results.…”

“…Autoimmune hearing loss (AIHL) is a specific clinical entity and may be related to the reaction antigen—autoantibody, the activation of the complement system, a direct action of cytotoxic T cells, or immune complex-mediated damage [ 1 ]. After the activation of the immune response and the release of interleukin (IL)-1β, immune-competent lymphocytes, and immunoglobulins can cause an excessive response involving cytokines, tumor necrosis factor, and interferon-gamma into the inner ear [ 2 ]. The result is the deposition of immune complexes into the endothelial surfaces of the labyrinthine vessels, microthrombosis, vascular changes and electrochemical alterations leading to damage to hair cells and spiral ganglion of the cochlea [ 3 ].…”

Autoimmune inner ear disease: A systematic review of management

“…Patients should be screened for predisposing factors to SNHL as up to 30% of patients with AIED have a coexisting systemic autoimmune disease 19,20 . A thorough review of systems should be taken in cooperation with rheumatology to rule out a systemic autoimmune process 20,21 . MRI is typically obtained to rule out retrocochlear pathology 3 .…”

“…This system contains 28 "yes or no" questions across 5 sections: study quality (10), external validity (3), study bias (7), confounding and selection bias (6), and power of the study (1). Down and Black score ranges were then given corresponding quality levels: excellent (26)(27)(28), good (20)(21)(22)(23)(24)(25), fair (15)(16)(17)(18)(19), and poor (<15). The quality of each study included in this review was assessed using this checklist and is reported in Table 2.…”