Systemic Corticosteroids Remain Top Treatment for Autoimmune Inner Ear Disease

What does a systematic review of autoimmune inner ear disease (AIED) management reveal about the prevalence, efficacy, and potential of various treatment modalities?

BOTTOM LINE: Systemic corticosteroids are the first-line treatment of AIED, with intratympanic steroids as a possible adjuvant or alternative; prospective studies investigating biologic and nonsteroidal therapy efficacy are warranted.

BACKGROUND: AIED is an uncommon inner ear disorder characterized by progressive and fluctuating sensorineural hearing loss (SNHL). Its pathogenesis is not fully understood, and there is a lack of standardized diagnostic criteria or reliable pathognomonic tests for AIED. Steroids are the mainstay treatment for AIED, but responsiveness varies.

STUDY DESIGN: Systematic review.

SETTING: University of Florida College of Medicine, Gainesville, Fla.

SYNOPSIS: Researchers conducted a data search to identify prospective or retrospective studies on AIED treatment published between April 1990 and April 2020. Sixteen studies were chosen for review. Investigators extracted data from each study on sample size, study design, treatment modality, audiometric data, and reported vestibular symptoms. Primary and secondary outcomes were hearing improvement and vestibular symptom improvement, respectively. Researchers found that steroids have remained the first-line treatment for AIED. They noted, however, that long-term use of systemic steroid therapy is associated with significant morbidity, adverse effects, and decreased patient compliance, and that treatment of steroid-resistant AIED remains challenging. Among the 16 studies reviewed, results of alternatives such as intratympanic steroid injections, nonsteroidal immunosuppressants, and biologic agents varied widely. Authors concluded that prospective clinical trials to guide treatment and compare the effectiveness of treatments are warranted. The study was limited by the heterogeneity of the inclusion criteria for the AIED diagnosis and a paucity in the literature formally investigating potential treatment options in steroid-resistant patients.

CITATION: Breslin NK, Varadarajan VV, Sobel ES, et al. Autoimmune inner ear disease: A systematic review of management. Laryngoscope Investig Otolaryngol. 2020;5:1217-1226.