Autoimmune Inner Ear Disease: Steroid and Cytotoxic Drug Therapy

Abstract

The goal of this study was to assess the effects ofimmun osupp ressiv e therapy on hearing in patients with presumed autoimmune sensorin eural hearin g loss (AISNHL) and a Western blot assay positivefor a 68 kD inner ear antig en. To achieve this objective, we conducted a retrosp ective review of39 such patients who were treated with either a steroid alone or with a steroid followed by a cytotoxic agent. Pure-tone avera ge (PTA) at 500 Hz, 1 kHz, 2 kHz, and 3 kHz and speecli discrimination scores (SDS) were used as objective tneasu res of outcome. At the compl etion of treatm ent, 23 of the 39 patients (59.0%) exhibited a positive respo nse to therapy. The steroid-only responders (n = 6) tend ed to demonstrat e a greater impro vement in PTA (14.8 vs 4.5 dB), while the cytotoxic-agent responders (n = 17) had a significantly greater impro vement in SDS (26.2 vs 6.9 %; p<O.OI). We conelude that most patients with AISNHL benefit from immunosuppressive therapyand that cytotoxic medications appear to imprave SDS, even in som e pati ents who have not responded to cortico steroid therap y.

Safety of High-Dose Corticosteroids for the Treatment of Autoimmune Inner Ear Disease

“…A number of case series have described the efficacy of prednisone for treatment of AIED (2,9,19Y21). Of these, only Lasak et al (20) reports detailed side effects for patients treated with steroids alone. In this retrospective case series, 12 patients were treated for a mean total of 3 weeks (range, 2 wk to 3 mo), initially with 1 mg/kg of prednisone or equivalent, followed by taper of unspecified duration.…”

Systematic Review of Treatments for Autoimmune Inner Ear Disease

“…The available data are limited to two retrospective trials and a series of case reports. One trial showed a 57% response rate to cyclophosphamide in patients who initially responded to steroids, but were unable to wean (18), whereas the other showed no difference in outcomes for cyclophosphamide plus steroids, versus steroids alone (17). Given the available data and potentially serious side-effects of this medication, the wisdom of its use remains questionable.…”

“…Another retrospective study, published in a non-peerreviewed journal, evaluated 62 subjects with clinical AIED and whose Western blot analyses were positive for a 68 kD inner ear antigen (18). Thirty-nine of these patients were considered to have active immune hearing loss and underwent treatment, which included eight with sudden sensorineural hearing loss.…”

“…In a retrospective trial involving multiple immunosuppressant agents described above, Methotrexate was given to 23 subjects, 21 as first-line treatment, and two after failure of cyclophosphamide (18). Fourteen subjects ultimately responded to methotrexate.…”

“…In a retrospective trial involving multiple immunosuppressant agents described above, seven subjects received azathioprine, all after trials of cyclophosphamide, methotrexate, or both (18). Five subjects ultimately responded to azathioprine.…”