Analysis of Audiometric Relapse-Free Survival in Patients With Immune-Mediated Hearing Loss Exclusively Treated With Corticosteroids

Mata‐Castro, Nieves; García-Chilleron, Raimon; Gavilanes-Plasencia, Javier; Ramírez‐Camacho, Rafael; García‐Fernández, Alfredo; García-Berrocal, José Ramón

doi:10.1016/j.otoeng.2017.08.010

Cited by 3 publications

(4 citation statements)

References 15 publications

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“…The onset of AIHL in our patients was found to be mainly progressive (49%), 43.3% of cases had HL, and most of the cases had both bilateral HL (67.9%) and downsloping audiogram (42.6%). Moreover, a relatively significant proportion of our AIHL cases had sudden onset (39.6%), higher than 38.7% [Mata-Castro et al, 2018] and 15% [Zeitoun et al, 2005], as reported in other studies. Sudden bilateral installation of HL, which is usually a dramatic condition, happened to 12 of our patients (Fig.…”

Section: Discussionsupporting

confidence: 73%

“…It has been postulated that AIHL is a chronic disease with recurrences. One study showed that half of AIHL occurred before 6 months of follow-up, and 87.1% of cases occurred during the 1st year [Mata-Castro et al, 2018]. Similarly, in our study, the recurrences were more commonly observed after SSHL (9 after unilateral SSHL, 4 The presence of recurrent bilateral (vs. unilateral); sudden SSHL was a factor that influenced negatively the probability of achieving a favorable response to therapy yes, favorable response to therapy; no, no response to therapy; heteroch, heterochronous; simult, simultaneous; bil, bilateral; uni, unilateral; ANA, antinuclear antibodies; AIHL, autoimmune hearing loss; SSHL, sensorineural hearing loss; HL, hearing loss; HLA, human leukocyte antigen.…”

Section: Discussionmentioning

confidence: 99%

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Audiological Patterns in Patients with Autoimmune Hearing Loss

et al. 2021

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Introduction: The aim of this study was to illustrate clinical and audiological patterns of hearing impairment in patients with autoimmune hearing loss (AIHL). Methods: 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. Results: 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, p < 0.05). The heterochronous bilateral SSHL may have better prognosis than simultaneous bilateral SSHL (OR = 10.000, p = 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. Conclusions: 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.

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Section: Discussionsupporting

confidence: 73%

Section: Discussionmentioning

confidence: 99%

Audiological Patterns in Patients with Autoimmune Hearing Loss

et al. 2021

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“…[39] Regarding biological agents, some treatment studies of anakinra and infliximab show higher percentages of hearing improvement. [40] Systemic injection of etanercept exerted its effects through decreasing cochlear inflammation and fibrosis with reduced cochlear infiltrating cell numbers in animal studies, [41][42][43] and showed its equivalence to corticosteroids in treating induced labyrinthitis. [44] Table 3 Diagnosis approach and treatment.…”

Section: Neoplasmsmentioning

confidence: 99%

Relapsing autoimmune inner ear disease with significant response to methotrexate and azathioprine combination therapy: A case report and mini literature review

Huang

Lin

et al. 2023

Medicine

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Rationale: Autoimmune inner ear disease typically presents with bilateral hearing loss that progresses over weeks or months though its mechanisms are unknown. Corticosteroids are the first-line treatment, but their responses are variable and relapses are frequent. Thus, many experts have sought to replace corticosteroids with immunosuppressive agents. Patient concerns: A 35-year-old woman experienced a progressive hearing impairment, initially on the left side and later becoming bilateral. Her response to corticosteroid monotherapy was temporary, and there have been two relapse episodes over several months. Diagnoses: Autoimmune inner ear disease was considered due to evidence of autoimmunity combined with a clinical course of bilateral and recurrent sensorineural hearing loss and a partial response to corticosteroid therapy. Interventions: The patient received a 3-day mini-pulse of methylprednisolone at 250 mg/d, followed by 12 mg/d maintenance, and concurrently began an azathioprine regimen gradually increasing to 100 mg/day as a corticosteroid-sparing agent. Outcomes: Three weeks after immunosuppressive therapy, hearing and pure-tone audiometry improved, and after 7 weeks, methylprednisolone was tapered to 8 mg/d. The dosage was further reduced by adding methotrexate at 7.5 mg/week, resulting in a reduction to 4 mg/d as maintenance therapy after 4 weeks. Lessons: For patients who are unresponsive to corticosteroids or experience difficulty tolerating them, a combination therapy of methotrexate and azathioprine is recommended as a viable alternative as this regimen is well-tolerated and yields positive outcomes.

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“…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 ].…”

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confidence: 99%