A Pilot Study of Rituximab in Immune-Mediated Inner Ear Disease

Abstract

Immune-mediated inner ear disease (IMED) is a cause of rapidly progressive auditory dysfunction. Patients are often responsive to high-dose corticosteroids and the disease is believed to be mediated by an antibody to inner ear proteins. To date, no therapies have proven effective as corticosteroid-sparing agents. Rituximab is a monoclonal antibody that depletes B cells, resulting in a reduction in autoantibody production. For that reason, rituximab was evaluated in a small pilot study in patients with IMED to see if there was a signal suggesting benefit. In all, 5/7 patients met the primary endpoint of an improvement in pure tone average (500–3000 Hz) by 10 dB in at least one ear, or an improvement in word identification score by at least 12% at 24 weeks, both relative to screening precorticosteroid values after 1 course of treatment. No significant adverse events were reported. The results of this study suggest further evaluation of rituximab as a treatment for IMED is indicated.

Autoimmune Inner Ear Disease: A Retrospective Review of Forty-Seven Patients

“…With respect to serial rituximab infusions, 5 of 7 patients maintained their postcorticosteroid improvement in the pure-tone audiogram at 24 weeks, suggesting certain benefits of this treatment [Cohen et al, 2011]. The number of subjects in these previous studies tended to be small, and the studies were not prospective or randomized in nature due to the rarity of AIED.…”

“…Recently, small open-label studies have demonstrated certain benefits of an IT injection of infliximab (Remicade) and intravenous infusions of adalimumab and rituximab. These benefits range from maintaining the level of baseline hearing loss to the improvement of the AIED [van Wijk et al, 2006;Vergles et al, 2010;Cohen et al, 2011].…”

“…New biological modifiers i.e. adalimumab (Humira ® ) and rituximab (Rituxan ® ) may also be useful for treating AIED; however, the effectiveness of these medications has not been established [Vergles et al, 2010;Cohen et al, 2011]. Currently, however, both the open-label nature of these medications and their relatively high cost make their use for treating AIED limited.…”

Systematic Review of Treatments for Autoimmune Inner Ear Disease

“…One open-label study evaluated the effects of intravenous administration of the antiCD20 antibody rituximab combined with a steroid taper on PTA and WRS (36). Subjects were those suspected clinically of having AIED and who responded to a prednisone trial.…”

New prospects in the diagnosis and treatment of immune-mediated inner ear disease

“…Rituximab is a chimeric monoclonal antibody that binds to the CD20 receptor of B lymphocytes, thereby inducing apoptosis and reducing their number. The few studies that have used rituximab in AIED patients have yielded encouraging results [35,36] . However, more studies are needed for reliable conclusions to be reached.…”