Emerging options in immune‐mediated hearing loss

Abstract

Objective AIED (autoimmune inner ear disease) is an autoimmune process that leads to the dysfunction of the inner ear, resulting in fluctuating, audiovestibular symptoms. Although the pathogenesis is likely heterogeneous, immune processes within the inner ear ultimately lead to histopathologic changes and sensorineural hearing loss. This review will discuss the latest evidence on treatment options. Methods A literature search on articles pertaining to the treatment of autoimmune inner ear disease was performed on PubMed. Results Corticosteroid treatment continues to remain as first line therapy for AIED but long‐term responsiveness is poor. Cytotoxic chemotherapies can be effective alternatives for steroid nonresponsive patients, but significant side effects may limit their use. Intratympanic steroid injections are beneficial and although there is not enough evidence currently to supplant oral steroid trial they may be a useful adjunct if steroid toxicity is an issue. The efficacy of biologic agents has been variable. Compared to placebo, etanercept does not improve the hearing improvement already attained by steroids alone. However, open pilot studies of other biologic agents show hearing improvements, improvements in tinnitus/aural fullness/vertigo, ability to wean steroid dependency, or benefits in steroid‐resistant AIED. Conclusion There is currently not enough evidence that alternative treatments supersede the use of initial steroid treatment. Biologic agents and intratympanic steroid injections are relatively well tolerated and should be considered as adjunctive therapy. More studies on the efficacy of various biologics and more studies on the treatment of steroid resistant disease especially after initial benefit are still needed. For those who eventually lose their hearing, cochlear implantation remains as a viable option. Level of Evidence expert opinion.

Audiovestibular Dysfunction and Hearing Loss in Patients with Psoriasis and Psoriatic Arthritis

“…In SNHL accompanying autoimmune diseases, inner ear involvement has been observed to be more pronounced at high frequency hearing thresholds. Therefore, evaluating the high frequencies of the hearing values along with the conventional frequencies in patients who do not identify the clinical symptoms of hearing loss can help detect the presence of early hearing impairment and measures against sudden hearing loss can be taken [29][30][31]44]. Extended high-frequency audiometry, expressing sounds above 8000 Hz, has been proven as a promising tool for the early detection of hearing disorders [18,31].…”

“…The main treatment for autoimmune inner ear disease is by corticosteroids, and significant improvement of hearing impairment has also been reported in previous PsA cases following steroid administration [27][28][29]. Moreover, promising advances have been performed in the treatment of immune-mediated inner ear disease with various TNF-α inhibitors (etanercept, infliximab, golimumab; as used in other underlying autoimmune diseases or as form of local intratimpanic infusion), demonstrating them to be effective in reducing inflammation and hearing loss in cochlear diseases [44,51]. Nonetheless, if hearing loss still occurs, cochlear implantation is a safe and effective method for the auditory rehabilitation of severe SNHL [44,52].…”

“…Moreover, promising advances have been performed in the treatment of immune-mediated inner ear disease with various TNF-α inhibitors (etanercept, infliximab, golimumab; as used in other underlying autoimmune diseases or as form of local intratimpanic infusion), demonstrating them to be effective in reducing inflammation and hearing loss in cochlear diseases [44,51]. Nonetheless, if hearing loss still occurs, cochlear implantation is a safe and effective method for the auditory rehabilitation of severe SNHL [44,52]. However, flap complications are more common in cases such as psoriasis, where the risk of surgical site infection is higher than normal patients.…”

Autoimmune inner ear disease: A systematic review of management

“…46 The drug was well tolerated and side effects were limited to injection site reactions. 46,47 Other potential side effects include nausea, diarrhea, headache, sore throat, and congestion. 48 Studies assessing hearing outcomes in steroid nonresponders are limited and many of these patients eventually experience significant hearing decline.…”

“…48 Studies assessing hearing outcomes in steroid nonresponders are limited and many of these patients eventually experience significant hearing decline. 46,47 One study revealed that steroid-resistant patients have higher circulating plasma levels of IL-1β as compared with steroid-responsive patients. 51 IL-1β is aberrantly regulated in steroid nonresponders and dexamethasone showed greater ability to repress IL-1β transcription in clinical steroid responders than in nonresponders.…”