Revised Swedish visual field standards for a driver's licence: Threshold perimetry as a predictor of eligibility according to the current Swedish and current Norwegian suprathreshold standards.

The term “autoimmune inner‐ear disease” (AIED) is referred to as an idiopathic, rapidly progressive, bilateral sensorineural hearing loss (SNHL) that characteristically occurs over a period of weeks or months []. Although understanding of systemic autoimmune manifestations has advanced markedly, reflected in updated ACR/EULAR criteria (American College of Rheumatology/European League Against Rheumatism, 2010 and 2022), knowledge of AIED remains limited, and definitive diagnostic criteria are still lacking [, , ]. AIED may present as an isolated inner‐ear disorder (primary AIED) or in association with systemic autoimmune disease (secondary AIED), the latter accounting for ~15%–30% of cases [, , , ]. Systemic autoimmune conditions associated with AIED can be grouped into four categories (Figure ): systemic vasculitides (e.g., ANCA‐associated vasculitis), connective‐tissue and systemic autoimmune diseases (e.g., rheumatoid arthritis), granulomatous disorders (e.g., sarcoidosis), and other immune‐mediated syndromes [, , , ]. Epidemiological data for AIED is limited due to the heterogeneity of the associated systemic immune diseases and lack of reproducible diagnostic criteria for the locally confined form of AIED, therefore estimating 5/100,000 cases per year []. AIED predominantly affects females and symptoms develop during the third to the sixth decade of life []. Although progressive bilateral hearing loss is characteristic for AIED, early unilateral and fluctuating patterns are also reported, often accompanied by tinnitus, vertigo, or aural fullness, which may mimic Ménière's disease [, ]. A study looking at audiometric data of 53 AIED patients reported downsloping audiograms for 43% of the cohort, while 21% showed flat or upsloping patterns []. Intraoperatively, intracochlear fibrosis as well as ossification is encountered in approximately 54% of AIED patients undergoing cochlear implantation []. A systematic review also reported disease‐related intraoperative adjustments in 57.7% of studies, most commonly cochlear drill‐out (53.3%), difficult round‐window insertion (26.7%), and scala‐vestibuli insertion (26.7%) []. Diseases related to secondary AIED grouped according to their rheumatological classification as vasculitis, connective tissue disease, systemic or granulomatous disease, and other not further classified pathologies. Created in . [Color figure can be viewed in the online issue, which is available at ] To date, no reliable serological panel exists for diagnosing AIED. Although candidate markers such as heat shock protein 70 (HSP70) have been proposed, diagnosis still relies on excluding alternative etiologies and assessing corticosteroid responsiveness [, ]. High‐dose systemic corticosteroids remain the first‐line treatment, with intratympanic administration reserved for non‐responders []. Due to steroid resistance and side effects, disease‐modifying antirheumatic drugs (DMARDs) are increasingly used as a second line therapy [, ]. Reviewing 16 studies including 459 patients with AIED, Breslin et al. noted consistent use of cyclophosphamide, methotrexate and azathioprine besides corticosteroids, yet evidence for hearing preservation remains inconsistent. Emerging biologic therapies including TNFα‐inhibitors, IL‐1‐inhibitors and the CD20‐inhibitor rituximab show promising but inconclusive results [, , , ].