Can unilateral, progressive or sudden hearing loss be immune-mediated in origin?

Atturo, Francesca; Colangeli, Roberta; Bandiera, Giorgio; Barbara, Maurizio; Monini, Simonetta

doi:10.1080/00016489.2017.1286035

Cited by 16 publications

(12 citation statements)

References 16 publications

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“…Patients are most frequently female, have the diagnosis made between their fourth and seventh decade of life and disease is usually bilateral. There is a common belief that AIED, specifically when associated with a SAID, is a progressive disease that involves both ears and many studies included only cases with bilateral hearing loss [8]. However, given that the clinical presentation of AIED can be sudden and unilateral [8], sometimes taking years to become bilateral [7], the authors chose to include patients with unilateral and bilateral hearing loss.…”

Section: Discussionmentioning

confidence: 99%

Sensorineural Hearing Loss and Systemic Autoimmune Disease: The Experience of a Systemic Immune-Mediated Diseases Unit

Ribeiro¹,

Serôdio²,

Amaral³

et al. 2021

Cureus

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Background Autoimmune inner ear disease (AIED) represents less than 1% of all cases of sensorineural hearing loss (SNHL) but its frequency may be underestimated due to lack of specific clinical and laboratory criteria. AIED can be associated with a systemic autoimmune disease (SAID) in 15%-30% of the cases. The objective of the present study was to characterize the clinical and prognostic factors of a cohort of patients with AIED. Materials and methods The authors conducted a retrospective descriptive analysis of a cohort of patients with AIED referred from the otorhinolaryngology department to a systemic immune-mediated diseases unit between March 2013 and November 2020. A consecutive sample of 39 patients with suspected AIED was referred. SNHL was defined as a fall of the hearing threshold of at least 30 decibels in three consecutive frequencies. Eight patients were excluded for not meeting the audiometric criteria or having confounding factors. The remaining 31 patients were included with a total of 50 affected ears. To classify the intensity of hearing loss, an arithmetic mean of pure tone was calculated. Normal hearing or mild hearing loss at the last pure tone audiometry of the followup were an indicator of good prognosis and were considered the outcome of interest. Results Thirty-two percent of the patients had an associated SAID. There were no differences regarding demographic and clinical characteristics when comparing patients with AIED alone and patients with AIED and a SAID, except for the positivity of antinuclear antibodies (ANA). ANA positivity was superior in patients with the association of AIED and a SAID when compared with patients with AIED alone (90% vs 50%; p=0.037). The SAID was diagnosed after the AIED in 70% of the patients, in which diagnosis of the SAID occurred a median of 4,2 (IQR 0.8-5.1) years after the diagnosis of the AIED. Normal audiometric evaluation or a mild hearing loss was achieved in 31% of the ears at the last audiometric evaluation. A normal audiometry or a mild hearing loss at the time of diagnosis was independently associated with a better outcome (31%, 14%, CI 1.71-273.69; p=0.018). Bilateral hearing loss was independently associated with a worse outcome (54%, 79%, CI 0.01-0.84; p=0.035). The use of systemic corticosteroids (p=0.941), transtympanic corticosteroids (p=0.700) and non-steroid immunomodulator drugs (p=0.986) did not affect prognosis. The presence of a SIAD did not affect the prognosis (p=0.986). Conclusions In this cohort, SAID was present in one-third of the patients with AIED. A good prognosis was achieved in one-third of the patients. A normal audiometry or mild disease at presentation was associated with a good outcome, whilst bilateral involvement was associated with a bad one. Association of a SAID did not seem to influence the hearing-related prognosis. Positivity of ANA antibodies may justify performing a complementary investigation to determine the presence of a SAID.

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Section: Discussionmentioning

confidence: 99%

Sensorineural Hearing Loss and Systemic Autoimmune Disease: The Experience of a Systemic Immune-Mediated Diseases Unit

Ribeiro¹,

Serôdio²,

Amaral³

et al. 2021

Cureus

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“…To confirm other etiologies related to ASNHL in the non-tumor group, additional investigations, including genetic examinations and immunological tests, might be performed. Atturo et al reported that 66.7% of the patients with unilateral SNHL were recognized as immune-mediated inner ear disease [38]. However, genetic examination might not be valuable in determining the cause of ASNHL.…”

Section: Discussionmentioning

confidence: 99%

Silent Vestibulopathy in Asymmetric Hearing Loss Can Be a Sign of a Cerebellopontine Angle Tumor

Sunwoo

Jeon

Park

et al. 2018

JOHBM

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Abstract:The presence of an ipsilateral cerebellopontine angle (CPA) tumor should be ruled out in patients with asymmetric sensorineural hearing loss (ASNHL). Although many patients with CPA tumors have ipsilateral vestibular hypofunction, some of them do not experience dizziness even with ipsilateral vestibular hypofunction. We analyzed the incidence of CPA tumors among patients with ASNHL without subjective dizziness based on the presence of vestibulopathy. We hypothesized that a patient with silent unilateral vestibular hypofunction (UVH) is more likely to be diagnosed with a CPA tumor. Among 157 subjects who underwent MRI for ASNHL, those who did not have "subjective dizziness" were selected. All subjects underwent hearing and vestibular function tests. UVH was diagnosed if canal paresis ≥ 25%, positive head-shake nystagmus, or gain of head-impulse test < 0.8 were detected. The diameters of the CPA tumors were measured along the petrosal ridge on the axial plane of MRI. Among the enrolled subjects, 44 (28.02%) were diagnosed with a CPA tumor. The 37 patients (84.1%) with a CPA tumor had silent UVH, while only 33 of the 113 patients (29.2%) without a CPA tumor had silent UVH (chi-square test, odds ratio = 12.8, p < 0.001). Silent UVH in patients with ASNHL may be a sign of a CPA tumor.

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“…Although the mechanism of interactions between SLE and RA and SSNHL remains unclear, SLE and RA are thought to be the causes of and risk factors for SSNHL, possibly by means of immune-modulating mechanism or interruption of the vascular supply. [9][10][11]14,16 Some researchers argued that RA and SLE may cause systemic vasculitis, which was also known to stimulate extra-articular manifestations in other organs, including the auditory system. 10,11 Atturo et al 10 found increased levels of immune complexes and autoantibodies against inner ear proteins in RA patients with SSNHL, showing that immune-mediated vasculitis may induce the SSNHL and influence the hearing recovery.…”

Section: Effect Of Comorbid Sle and Ra On Hearing Recovery Of Ssnhlmentioning

confidence: 99%

“…[9][10][11]14,16 Some researchers argued that RA and SLE may cause systemic vasculitis, which was also known to stimulate extra-articular manifestations in other organs, including the auditory system. 10,11 Atturo et al 10 found increased levels of immune complexes and autoantibodies against inner ear proteins in RA patients with SSNHL, showing that immune-mediated vasculitis may induce the SSNHL and influence the hearing recovery. Several previous publications suggested an association between SSNHL and autoantibodies against phospholipids in patients with SLE, and these autoantibodies induce endothelial damage and thrombosis in the labyrinthine vessels, which may contribute to the onset of SSNHL.…”

Section: Effect Of Comorbid Sle and Ra On Hearing Recovery Of Ssnhlmentioning

confidence: 99%

“…8 Increasing experimental evidence suggesting an autoimmune component in the pathology of SSNHL has emerged. 9,10 Many published studies have demonstrated that systemic autoimmune diseases are risk factors for onset of SSNHL, a recent population-based research showed that patients with immune-mediated disease had a 4.27-fold increased the risk of SSNHL than those in the control group. 11 Similarly, Lee et al 12 found that patients with RA had a higher risk of suffering SSNHL compared with those without RA.…”

Section: Introductionmentioning

confidence: 99%

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Effect of systemic lupus erythematosus and rheumatoid arthritis on sudden sensorineural hearing loss

Xie

Ning

She³

et al. 2019

The Laryngoscope

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Objectives Recent reports have identified autoimmune systemic diseases as a significant risk factor for sudden sensorineural hearing loss (SSNHL). We investigated whether systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) were correlated with the hearing recovery of SSNHL. Study Design A retrospective study. Methods Records of 663 SSNHL patients between January 2008 and January 2019 were retrospectively reviewed, including demographic, comorbid diseases, and hearing recovery. Patients were divided into four groups (complete, partial, slight, and no recovery) according to Siegel's criteria and Chinese Medical Association of Otolaryngology (CMAO) criteria. Multinomial logistic regression was performed to evaluate the effects of onset of treatment, initial hearing threshold, audiogram pattern, diabetes mellitus, SLE, and RA on the prognosis of SSNHL according to both criteria. Results Patients in complete recovery, partial recovery, slight recovery, and no improvement were 95 (14.3%), 183 (27.6%), 170 (25.6%), and 215 (32.4%) by Siegel's criteria, and 90 (13.6%), 152 (22.9%), 188 (28.4%), and 233 (35.1%) by CMAO criteria, respectively. Among the four groups, onset of treatment, initial hearing threshold, diabetes mellitus, SLE, RA, and profound audiogram pattern were found to be associated with recovery outcome by both Siegel's criteria and CMAO criteria (P < .05). According to analysis results, presence of SLE, RA, diabetes mellitus and higher initial hearing threshold were significantly correlated with a poor prognosis by both Siegel's criteria and CMAO criteria (P < .05). Conclusion Comorbid SLE or RA may negatively affect the prognosis of SSNHL. Lower initial hearing threshold and absence of diabetes mellitus are associated with favorable hearing recovery. Level of Evidence 4. Laryngoscope, 130:2475–2480, 2020

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Can unilateral, progressive or sudden hearing loss be immune-mediated in origin?

Abstract: The nonspecific autoimmune tests are worth to be performed also when SNHL is not bilateral and progressive, since an immunological mechanism could also underlie unilateral and sudden SNHL cases.

Cited by 16 publications

References 16 publications

Sensorineural Hearing Loss and Systemic Autoimmune Disease: The Experience of a Systemic Immune-Mediated Diseases Unit

Sensorineural Hearing Loss and Systemic Autoimmune Disease: The Experience of a Systemic Immune-Mediated Diseases Unit

Silent Vestibulopathy in Asymmetric Hearing Loss Can Be a Sign of a Cerebellopontine Angle Tumor

Effect of systemic lupus erythematosus and rheumatoid arthritis on sudden sensorineural hearing loss

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