Audiological Patterns in Patients with Autoimmune Hearing Loss

Abstract

<b><i>Introduction:</i></b> The aim of this study was to illustrate clinical and audiological patterns of hearing impairment in patients with autoimmune hearing loss (AIHL). <b><i>Methods:</i></b> Fifty-three patients with AIHL were retrospectively recruited, and a tapering schema of steroid treatment was administered in all these patients. The diagnosis of AIHL was essentially based on clinical symptoms, such as recurrent, sudden (sensorineural hearing loss [SSHL]), fluctuating, or quickly progressing (<12 months) SSHL (uni-/bilateral), in association with the coexistence of autoimmune diseases, high antinuclear antibodies (ANA) and the presence of human leukocyte antigen (HLA) B27, B35, B51, C04, and C07. Logistic regression analysis was applied to correlate the clinical data and laboratory features of AIHL with final outcomes. <b><i>Results:</i></b> The onset of AIHL was mainly progressive (49%), followed by SSHL (39.6%) or fluctuating (11.3%). The pure-tone audiogram showed more commonly a downsloping pattern (42.6% of ears), but also an upsloping, flat, cookie-bite, or inverse cookie-bite shape. Bilateral progressive AIHL was more frequently simultaneous (23 patients) than heterochronous (4 patients). Nineteen patients (35.8%) showed a favorable response to steroid therapy. The presence of recurrent, bilateral SSHL versus recurrent, unilateral SSHL had statistically negative effect on hearing recovery (OR = 0.042, <i>p</i> < 0.05). The heterochronous bilateral SSHL may have better prognosis than simultaneous bilateral SSHL (OR = 10.000, <i>p</i> = 0.099). The gender, age, concomitant autoimmune disease, high ANA, HLA alleles, tinnitus, and vestibular symptoms had no statistical effect on a favorable outcome of AIHL. <b><i>Conclusions:</i></b> A bilateral, simultaneous, and progressive hearing loss combined with downsloping audiogram occurred more often in patients with AIHL. Bilateral simultaneous SSHL with recurrences represents the worse prognostic form of AIHL.

Recurrent sudden sensorineural hearing loss—A literature review

“…Progressive hearing loss implies an autoimmune inner ear disorder or central lesion. Recurrent hearing loss in patients with an underlying autoimmune disease is sometimes considered as autoimmune hearing loss 23,24 …”

“…Recurrent hearing loss in patients with an underlying autoimmune disease is sometimes considered as autoimmune hearing loss. 23 , 24 …”

“…Recurrent hearing loss in patients with an underlying autoimmune disease is sometimes considered as autoimmune hearing loss. 23,24 A thorough investigation may be needed when patients suffer from recurrent sudden hearing loss after their first SSNHL episode.…”

Autoimmune Hearing Loss: A Diagnostic Challenge

“…The diagnosis AIHL is still unclear and there are no serologic or radiologic criteria for its identification. When initially described, AIHL was recognized as always being both bilateral and progressive [ 2 ]; however, AIHL may involve only one ear [ 3 ] and a significant proportion of patients with AIHL may experience a sudden onset of symptoms [ 4 , 5 ]. Moreover, positive response to steroid administration was considered as a clinical prerequisite for AIHL [ 2 ]; nevertheless, less than half of patients respond favorably to steroid therapy [ 5 , 6 ] and, over time, a progression of hearing loss has been observed.…”

“…Different pure-tone audiogram shapes in patients with AIHL have been recorded, including down-sloping (which is the most frequent), up-sloping, flat, cookie-bite, and inverse cookie-bite shapes; almost half of cases have moderate hearing loss [ 1 , 5 ]. AIHL is a long-standing disease with relapses and is more commonly observed after unilateral or bilateral sudden hearing loss [ 7 ].…”