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Chen JJ, Hsu CW, Chen YW, Chen TY, Zeng BS, Tseng PT. Audiological Features in Patients with Rheumatoid Arthritis: A Systematic Review. Int J Mol Sci 2024; 25:13290. [PMID: 39769062 PMCID: PMC11676466 DOI: 10.3390/ijms252413290] [Citation(s) in RCA: 3] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2024] [Revised: 12/06/2024] [Accepted: 12/07/2024] [Indexed: 01/11/2025] Open
Abstract
Hearing impairment in patients with rheumatoid arthritis has been underestimated for decades. Rheumatoid arthritis can affect both the middle ear (specifically, the incudomalleolar and incudostapedial joints) and inner ear (including the cochlea and acoustic nerve) simultaneously. Despite ongoing research, consensus on effective treatments for hearing impairment in these patients remains elusive. This systematic review aims to consolidate clinically relevant information for healthcare providers by summarizing current evidence on hearing impairment in rheumatoid arthritis patients. We conducted the current systematic review by searching platforms of PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect to retrieve eligible articles regarding hearing impairment related to rheumatoid arthritis. We extract any data on characteristics, pathophysiology, examination, and treatment related to rheumatoid arthritis. Based on the currently available evidence, we advocate for the use of specific audiometric tests to facilitate early detection of hearing impairment in these patients. Regular audiological assessments are recommended to monitor hearing ability and potentially prevent further deterioration. Finally, we propose a modified treatment protocol that integrates steroids, hydroxychloroquine, and non-invasive brain stimulation as a novel therapeutic approach for managing these symptoms. This protocol aims to offer clinicians new strategies to address hearing impairment in patients with rheumatoid arthritis effectively.
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Affiliation(s)
- Jiann-Jy Chen
- Prospect Clinic for Otorhinolaryngology & Neurology, Kaohsiung 811026, Taiwan; (J.-J.C.); (Y.-W.C.)
- Department of Otorhinolaryngology, E-Da Cancer Hospital, I-Shou University, Kaohsiung 824005, Taiwan
| | - Chih-Wei Hsu
- Department of Psychiatry, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung 833401, Taiwan;
| | - Yen-Wen Chen
- Prospect Clinic for Otorhinolaryngology & Neurology, Kaohsiung 811026, Taiwan; (J.-J.C.); (Y.-W.C.)
| | - Tien-Yu Chen
- Department of Psychiatry, Tri-Service General Hospital, School of Medicine, National Defense Medical Center, Taipei 114202, Taiwan;
- Institute of Brain Science, National Yang Ming Chiao Tung University, Taipei 112, Taiwan
| | - Bing-Syuan Zeng
- Institute of Biomedical Sciences, National Sun Yat-sen University, Kaohsiung 804201, Taiwan
- Department of Internal Medicine, E-Da Cancer Hospital, I-Shou University, Kaohsiung 824005, Taiwan
| | - Ping-Tao Tseng
- Prospect Clinic for Otorhinolaryngology & Neurology, Kaohsiung 811026, Taiwan; (J.-J.C.); (Y.-W.C.)
- Institute of Biomedical Sciences, National Sun Yat-sen University, Kaohsiung 804201, Taiwan
- Department of Psychology, College of Medical and Health Science, Asia University, Taichung413305, Taiwan
- Institute of Precision Medicine, National Sun Yat-sen University, Kaohsiung 804201, Taiwan
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Hung SH, Xirasagar S, Dang LH, Viet-Nhi NK, Cheng YF, Chen CS, Lin HC. Prevalence of hearing loss, tinnitus, vertigo and sudden deafness among patients with polymyositis and dermatomyositis. Sci Rep 2024; 14:5774. [PMID: 38459190 PMCID: PMC10923884 DOI: 10.1038/s41598-024-56372-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/01/2023] [Accepted: 03/05/2024] [Indexed: 03/10/2024] Open
Abstract
Little is known about a possible association of autoimmune inner ear disease among patients diagnosed with polymyositis (PM)/dermatomyositis (DM). This study aimed to explore differences in the prevalence of inner ear symptoms among patients with and without PM/DM using a nationwide population-based dataset. Data for this study were retrieved from the Taiwan National Health Insurance Research Database. The study sample included 1622 patients diagnosed with PM/DM and 8109 propensity-score matched comparison patients without PM/DM. We performed multivariate logistic regressions to calculate odds ratios (ORs) and 95% confidence interval (CI) for tinnitus, hearing loss, sudden deafness, and vertigo among patients with PM/DM versus comparison patients. Chi-square tests showed statistically significant differences between patients with PM/DM and comparison patients in the prevalence of tinnitus (16.1% vs. 12.7%, p < 0.001), non-conductive hearing loss (9.2% vs. 6.8%, p < 0.001), and vertigo (14.4% vs. 11.1%, p < 0.001). The adjusted ORs for tinnitus, non-conductive hearing loss, and vertigo, respectively, were 1.332 (95% CI = 1.147-1.547), 1.399 (95% CI = 1.154-1.696), and 1.374 (95% CI = 1.173-1.611) for patients with PM/DM when compared to comparison patients. Our study finds that patients with PM/DM have higher prevalence rates of tinnitus, non-conductive hearing loss, and vertigo than comparison patients.
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Affiliation(s)
- Shih-Han Hung
- Department of Otolaryngology, School of Medicine, Taipei Medical University, Taipei, 110, Taiwan
- Department of Otolaryngology, Wan Fang Hospital, Taipei, 110, Taiwan
- International Ph.D. Program in Medicine, College of Medicine, Taipei Medical University, Taipei, 110, Taiwan
| | - Sudha Xirasagar
- Department of Health Services Policy and Management, Arnold School of Public Health, University of South Carolina, Columbia, SC, USA
| | - Luong Huu Dang
- Department of Otolaryngology, Faculty of Medicine, University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Vietnam
| | - Nguyen-Kieu Viet-Nhi
- International Ph.D. Program in Medicine, College of Medicine, Taipei Medical University, Taipei, 110, Taiwan
| | - Yen-Fu Cheng
- Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan
- Department of Otolaryngology-Head and Neck Surgery, Taipei Veterans General Hospital, Taipei, Taiwan
- Department of Otolaryngology-Head and Neck Surgery, School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan
- Research Center of Data Science on Healthcare Industry, College of Management, Taipei Medical University, Taipei, Taiwan
| | - Chin-Shyan Chen
- Research Center of Data Science on Healthcare Industry, College of Management, Taipei Medical University, Taipei, Taiwan.
- Department of Economics, National Taipei University, New Taipei City, Taiwan.
| | - Herng-Ching Lin
- School of Health Care Administration, College of Management, Taipei Medical University, Taipei, Taiwan.
- Research Center of Sleep Medicine, Taipei Medical University Hospital, Taipei, Taiwan.
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Maxwell PJ, Ranjbar PA, Winkler D, Kuzy J, McGovern B, Sataloff RT. Sex differences in autoimmune inner ear disease treatment and audiological outcomes. Am J Otolaryngol 2023; 44:103875. [PMID: 37058909 DOI: 10.1016/j.amjoto.2023.103875] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/08/2023] [Accepted: 03/25/2023] [Indexed: 04/01/2023]
Abstract
OBJECTIVE The primary objective of this study was to assess possible sex-related differences in AIED treatment. The secondary aim was to assess the long-term outcomes of treatment using pre- and post-treatment audiometry and speech discrimination scores. METHODS Adult patients with a diagnosis of AIED who were treated for their condition at the senior author's (RTS) practice from 2010 to 2022 were included in this study. Patients were grouped as males or females for further analysis and comparison. Data included past medical history, medication use, surgical history and social history. Air-conduction thresholds (500 to 8000 Hz) were collected and averaged into discrete variables for pre- and post-treatment. The change (∆) and percent change (%∆) of these variables following therapy were analyzed. Speech discrimination score (SDS) testing results were taken at the same time points as pure tone averages, and patients were sub-stratified based on evidence of improvement in SDS and compared accordingly. RESULTS One hundred eighty-four patients (78 male/106 female) were included in this study. The mean age of the male participants was 57.18 ± 15.92 years, and the mean age of the female participants was 53.49 ± 16.04 years (p = 0.220). Comorbid autoimmune disease (AD) was more prevalent in females than in males (38.7 % vs. 16.7 %, p = 0.001). Of the patients treated with oral steroids, females received significantly more courses than males (2.554 ± 2.078 vs. 1.946 ± 1.301, p = 0.020). However, the average duration of oral steroids used per trial did not differ significantly between males and females (21.02 ± 18.05 vs. 20.62 ± 7.49, p = 0.135). Audiological results demonstrated that the change (∆) in pure tone average (PTA) at 0.5, 1, 2, and 3 kHz (-4.21 ± 6.394 vs. -3.91 ± 6.105) and high-frequency pure tone average (HFPTA) at 4, 6, and 8 kHz (-4.55 ± 6.544 vs. -2.19 ± 6.842) was not significantly different between the sexes following treatment (p = 0.376 and p = 0.101, respectively). Similarly, the percentage change (%∆) in PTA (-13.17 % vs. -15.01 %) and HFPTA (-8.50 % vs. -6.76 %) also did not differ significantly between males and females (p = 0.900 and p = 0.367, respectively). One hundred-five (57.1 %) patients qualified for the SDS improvement subgroup for analysis, with 50 (47.6 %) being male and 55 (51.9 %) being female (p = 0.159). The change (∆) in SDS (15.12 ± 21.159 vs. 10.62 ± 19.206) and the percentage change (%∆) in SDS (16.71 % vs. 12.40 %) also showed no significant difference between male and female patients (p = 0.312 and p = 0.313, respectively). CONCLUSION AIED is not a uniform condition regarding clinical presentation, audiological findings or progression, nor is its treatment straightforward. The utilization and duration of cytotoxic medications, as well as PTA and SDS findings, did not differ between the sexes. However, significantly more courses of oral steroids were prescribed to females than males. Sex as a biological variable and its implications in the pathogenesis and treatment of AIED warrant further study.
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Vambutas A, Davia DV. Biologics for Immune-Mediated Sensorineural Hearing Loss. Otolaryngol Clin North Am 2021; 54:803-813. [PMID: 34119332 DOI: 10.1016/j.otc.2021.05.005] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
Immune-mediated hearing losses include autoimmune inner ear disease, sudden sensorineural hearing loss, and Meniere's disease. Standard therapy for an acute decline in hearing is timely use of corticosteroids. Although 60% to 70% of patients are initially corticosteroid-responsive, that responsiveness is lost over time. In corticosteroid-resistant patients, increased expression of interleukin (IL)-1 is observed, and these patients may benefit from IL-1 inhibition. Autoinflammatory diseases are characterized by dysregulation of the innate immune response, clinically include sensorineural hearing loss, and benefit from IL-1 inhibition, thereby further establishing the relationship of IL-1 with immune-mediated sensorineural hearing loss.
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Affiliation(s)
- Andrea Vambutas
- Department of Otolaryngology, Zucker School of Medicine at Hofstra-Northwell, Hearing and Speech Center, 430 Lakeville Road, New Hyde Park, NY 11040, USA.
| | - Daniella V Davia
- Department of Otolaryngology, Zucker School of Medicine at Hofstra-Northwell, Hearing and Speech Center, 430 Lakeville Road, New Hyde Park, NY 11040, USA
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Corazzi V, Hatzopoulos S, Bianchini C, Skarżyńska MB, Pelucchi S, Skarżyński PH, Ciorba A. The Pathogenesis of secondary forms of Autoimmune Inner Ear Disease (AIED): advancing beyond the audiogram data. Expert Rev Clin Immunol 2021; 17:233-246. [PMID: 33476250 DOI: 10.1080/1744666x.2021.1879640] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Abstract
INTRODUCTION Autoimmune Inner Ear Disease (AIED) can be of a primary or secondary type. To date, a clear pathogenesis of the disease is still not available. Focusing on the secondary forms of AIED, the aim of this review is to (i) assess and describe the hearing involvement in patients affected by autoimmune diseases, (ii) describe the possible association between clinical features (among serological/laboratory data and disease activity/duration) and hearing impairment, (iii) show evidence connecting the AIED types with various etiopathogenetic mechanisms. AREAS COVERED A PRISMA-compliant systematic review was performed. Medline, Cochrane, Embase, and Cinahl were searched from 1 January 2015 through to 5 August 2020. Overall, 16 studies (involving 1043 participants) were included in the review. The data in the literature suggested that bilateral mild-to-moderate sensorineural hearing loss is a commonly reported clinical symptom of AIED. EXPERT OPINION Patients with systemic autoimmune disorders present a cochlear injury which might be associated with the humoral and/or cellular immune response against the inner ear. To date, AIED pathogenesis remains an open issue, due to the rarity of these clinical entities and due to the difficulties in investigating the inner ear immunology, considering the inner ear inaccessibility for tissue sampling.
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Affiliation(s)
- Virginia Corazzi
- ENT and Audiology Department, University Hospital of Ferrara, Ferrara, Italy
| | - Stavros Hatzopoulos
- ENT and Audiology Department, University Hospital of Ferrara, Ferrara, Italy
| | - Chiara Bianchini
- ENT and Audiology Department, University Hospital of Ferrara, Ferrara, Italy
| | - Magdalena B Skarżyńska
- Institute of Sensory Organs, Kajetany, Poland.,Department of Teleaudiology and Screening, Institute of Physiology and Pathology of Hearing, Warsaw, Poland
| | - Stefano Pelucchi
- ENT and Audiology Department, University Hospital of Ferrara, Ferrara, Italy
| | - Piotr Henryk Skarżyński
- Institute of Sensory Organs, Kajetany, Poland.,Department of Teleaudiology and Screening, Institute of Physiology and Pathology of Hearing, Warsaw, Poland.,Department of Heart Failure and Cardiac Rehabilitation, Medical University of Warsaw, Warsaw, Poland
| | - Andrea Ciorba
- ENT and Audiology Department, University Hospital of Ferrara, Ferrara, Italy
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Association between Ménière's disease and thyroid diseases: a nested case-control study. Sci Rep 2020; 10:18224. [PMID: 33106572 PMCID: PMC7588449 DOI: 10.1038/s41598-020-75404-y] [Citation(s) in RCA: 20] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/04/2020] [Accepted: 10/15/2020] [Indexed: 12/12/2022] Open
Abstract
The association of thyroid disease and Ménière’s disease would suggest that both are autoimmune diseases. This study aimed to investigate the relation of goiter, hypothyroidism, thyroiditis, hyperthyroidism, and autoimmune thyroiditis with Ménière’s disease. The Korean National Health Insurance Service-Health Screening Cohort data from 2002 through 2015 were used. The 8183 adult patients with Ménière’s disease were 1:4 matched with the 32,732 individuals of the control group for age, sex, income, and region of residence. The previous histories of thyroid disorders including goiter, hypothyroidism, thyroiditis, and hyperthyroidism were investigated using conditional logistic regression analyses. Subgroup analyses were conducted, including for age and sex. Smoking, alcohol consumption, obesity, Charlson Comorbidity Index, histories of benign paroxysmal vertigo, vestibular neuronitis, other peripheral vertigo, thyroid cancer, and levothyroxine medication were adjusted in the models. The histories of goiter (5.7% vs. 4.2%), hypothyroidism (4.7% vs. 3.6%), thyroiditis (2.1% vs. 1.6%), hyperthyroidism (3.6% vs. 2.5%), and autoimmune thyroiditis (0.99% vs. 0.67%) were higher in the Meniere’s disease group than in the control group (all P < 0.05). The histories of goiter, hypothyroidism, and hyperthyroidism were associated with Ménière’s disease (adjusted odds ratio (OR) = 1.19 [95% confidence interval (CI) = 1.04–1.36] for goiter, 1.21 [95% CI 1.02–1.44] for hypothyroidism, and 1.27 [95% CI 1.09–1.49] for hyperthyroidism, each of P < 0.05). In subgroup analyses, hypothyroidism was associated with Ménière’s disease in < 65-year-old women. Hyperthyroidism was related with Ménière’s disease in women overall. Thyroid diseases of goiter, hypothyroidism, and hyperthyroidism were associated with Ménière’s disease.
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Liu H, Zhou K, Zhang X, Peng KA. Fluctuating Sensorineural Hearing Loss. Audiol Neurootol 2019; 24:109-116. [PMID: 31315108 DOI: 10.1159/000500658] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/11/2018] [Accepted: 04/29/2019] [Indexed: 11/19/2022] Open
Abstract
BACKGROUND Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière's disease, autoimmune inner ear disease, and enlarged vestibular aqueduct. Although these 3 etiologies vary greatly, distinguishing between these conditions at initial presentation can be challenging. Furthermore, initial treatment of these conditions is often similar. In this review, we discuss historical and current perspectives on diagnosis and treatment of these conditions. SUMMARY A literature search was performed regarding fluctuating hearing loss, and current treatment of these etiologies of fluctuating hearing loss was summarized. Immediate measures at the onset of acute hearing loss include corticosteroid therapy, while preventative and chronic therapies, which can limit disease severity and frequency, vary based on the specific condition treated. Key Messages: Fluctuating hearing loss can represent a range of pathologies, but the precise etiology may not be clear at initial presentation. Timely treatment and long-term follow-up, along with appropriate diagnostics, are necessary to optimize long-term hearing.
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Affiliation(s)
- Hui Liu
- First Hospital of Shijiazhuang City, Shijiazhuang City, China,
| | - Kunpeng Zhou
- First Hospital of Shijiazhuang City, Shijiazhuang City, China
| | - Xuemei Zhang
- First Hospital of Shijiazhuang City, Shijiazhuang City, China
| | - Kevin A Peng
- House Clinic and House Ear Institute, Los Angeles, California, USA
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Audiovestibular Symptoms in Systemic Autoimmune Diseases. J Immunol Res 2018; 2018:5798103. [PMID: 30211232 PMCID: PMC6120292 DOI: 10.1155/2018/5798103] [Citation(s) in RCA: 40] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/29/2017] [Revised: 06/11/2018] [Accepted: 07/04/2018] [Indexed: 12/16/2022] Open
Abstract
Immune-mediated inner ear disease can be primary, when the autoimmune response is against the inner ear, or secondary. The latter is characterized by the involvement of the ear in the presence of systemic autoimmune conditions. Sensorineural hearing loss is the most common audiovestibular symptom associated with systemic autoimmune diseases, although conductive hearing impairment may also be present. Hearing loss may present in a sudden, slowly, rapidly progressive or fluctuating form, and is mostly bilateral and asymmetric. Hearing loss shows a good response to corticosteroid therapy that may lead to near-complete hearing restoration. Vestibular symptoms, tinnitus, and aural fullness can be found in patients with systemic autoimmune diseases; they often mimic primary inner ear disorders such as Menière's disease and mainly affect both ears simultaneously. Awareness of inner ear involvement in systemic autoimmune diseases is essential for the good response shown to appropriate treatment. However, it is often misdiagnosed due to variable clinical presentation, limited knowledge, sparse evidence, and lack of specific diagnostic tests. The aim of this review is to analyse available evidence, often only reported in the form of case reports due to the rarity of some of these conditions, of the different clinical presentations of audiological and vestibular symptoms in systemic autoimmune diseases.
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The Role of Autoimmunity in the Pathogenesis of Sudden Sensorineural Hearing Loss. Neural Plast 2018; 2018:7691473. [PMID: 30008743 PMCID: PMC6020465 DOI: 10.1155/2018/7691473] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2018] [Accepted: 05/10/2018] [Indexed: 12/20/2022] Open
Abstract
Sudden sensorineural hearing loss (SSHL) is a clinically common acute symptom in otolaryngology. Although the incidence of SSHL has increased around the world in recent years, the etiology of the disease is still unclear. It has been reported that infections, ototoxic drugs, membrane labyrinth rupture, carcinomas, circulatory system diseases, autoimmune diseases, brain lesions, mental diseases, congenital or inherited diseases, and so on, are all risk factors for SSHL. Here, we discuss the autoimmune mechanisms behind SSHL, which might be induced by type II–IV allergic reactions. We also introduce the main immunosuppressive medications that have been used to treat SSHL, which will help us to identify potential targets for immune therapy.
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Kohlert S, Bromwich M. Mobile tablet audiometry in fluctuating autoimmune ear disease. J Otolaryngol Head Neck Surg 2017; 46:18. [PMID: 28270231 PMCID: PMC5341391 DOI: 10.1186/s40463-017-0195-1] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/18/2016] [Accepted: 03/02/2017] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Autoimmune inner ear disease (AIED) is a rare condition characterized by bilateral fluctuating sensorineural hearing loss (SNHL). The labile nature of this hearing loss makes it difficult to accurately quantify with conventional methods, and therefore it is challenging to rehabilitate. METHODS Over a 9-month period one pediatric patient with severe AIED was monitored and conducted home audiograms using a previously validated testing system (Shoebox Audiometry). During this period he also underwent several clinical audiograms. The correlation between clinical and home audiograms was analyzed with a Pearson coefficient, and the range and frequency of fluctuations was recorded. RESULTS Sixty-four automated home audiograms and nine clinical audiograms were conducted. When tested at home using a calibrated system the pure tone average (PTA) fluctuated between 12 dB and 72 dB indicating large variability in hearing. Fluctuations were frequent: on 28 occasions the PTA varied by at least 5 dB when retested within 4 days. The mean PTA was 50 dB and 95% of the thresholds were within 36 dB of the mean. Clinical audiograms obtained on the same day or within 1 day of home testing were highly concordant (with a Pearson coefficient of 0.93). CONCLUSION AIED can result in significant fluctuations in hearing over short periods of time. Home testing enables a more granular look at variations over time and correlates well with clinical testing, and thus facilitates rapid action and informed rehabilitation.
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Affiliation(s)
- Scott Kohlert
- Faculty of Medicine, University of Ottawa, Ottawa, Canada.,Department of Otolaryngology - Head and Neck Surgery, The Ottawa Hospital, Ottawa, Canada.,Department of Otolaryngology - Head and Neck Surgery, Children's Hospital of Eastern Ontario, 401 Smyth Rd, Ottawa, ON, K1H 8 L1, Canada
| | - Matthew Bromwich
- Faculty of Medicine, University of Ottawa, Ottawa, Canada. .,Department of Otolaryngology - Head and Neck Surgery, The Ottawa Hospital, Ottawa, Canada. .,Department of Otolaryngology - Head and Neck Surgery, Children's Hospital of Eastern Ontario, 401 Smyth Rd, Ottawa, ON, K1H 8 L1, Canada.
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Rossini BAA, Penido NDO, Munhoz MSL, Bogaz EA, Curi RS. Sudden Sensorioneural Hearing Loss and Autoimmune Systemic Diseases. Int Arch Otorhinolaryngol 2016; 21:213-223. [PMID: 28680488 PMCID: PMC5495599 DOI: 10.1055/s-0036-1586162] [Citation(s) in RCA: 50] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2015] [Accepted: 06/03/2016] [Indexed: 10/25/2022] Open
Abstract
Introduction Several authors have demonstrated the relationship between sudden sensorineural hearing loss (SNHL) and systemic autoimmune diseases (SAD). Immune-mediated SNHL can rarely present as unilateral sudden SNHL and manifests itself in the contralateral ear only after years. It presents clinical relevance for being one of the few SNHL that may be reversible given that early and appropriate treatment is applied. Objective The objective of this study is to describe the clinical presentations and audiological findings from patients with idiopathic sudden SNHL and SAD associated with a probable diagnosis of immune-mediated SNHL. Furthermore, we strive to estimate the prevalence of SAD in patients with sudden SNHL. Methods This is an observational retrospective cohort. We have selected and studied patients with SAD. Revision of available literature on scientific repositories. Results We evaluated 339 patients with sudden SNHL. Among them, 13 (3.83%) patients suffered from SAD. Three patients had bilateral involvement, a total of 16 ears. We evaluate and describe various clinical, epidemiological, and audiological aspects of this sample. Conclusion In our sample of patients with sudden SNHL, the prevalence of SAD was found relevant. The majority had tinnitus and dizziness concomitant hearing loss, unilateral involvement and had experienced profound hearing loss at the time of the installation. In spite of instituted treatment, most cases showed no improvement in audiometric thresholds. Apparently, patients with sudden SNHL and SAD have a more severe initial impairment, higher percentage of bilateral, lower response to treatment, and worse prognosis than patients with sudden SNHL of unknown etiology.
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Affiliation(s)
| | | | | | | | - Renata Souza Curi
- Department of ENT, Universidade Federal de São Paulo, São Paulo, Brazil
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Santosh UP, Rao MSS. Incidence of Hypothyroidism in Meniere's Disease. J Clin Diagn Res 2016; 10:MC01-3. [PMID: 27437251 DOI: 10.7860/jcdr/2016/17587.7759] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2015] [Accepted: 03/04/2016] [Indexed: 11/24/2022]
Abstract
INTRODUCTION Meniere's disease is a disorder of the membranous labyrinth of the inner ear manifesting as vertigo, tinnitus, sensory neural hearing loss and aural fullness of known or unknown origin. Some patients may present after years into typical forms. The endocrine disorders, especially hypothyroidism and the factors like inflammation, trauma, otosclerosis, autoimmunity explains the multifactorial causation for the basis of Meniere's disease. Endolymphatic hydrops is the pathologic term named for the defective regulation of endolymph volume with respect to production and absorption. However, hyper production of endolymph cannot be excluded. The cause of Meniers disease is multifactorial so clinical features of repeated attacks of variable duration cannot be explained satisfactorily based on single causation. AIM To study the incidence of hypothyroidism in Meniere's disease and to assess the success rate of improvement in subjective symptoms after treatment. MATERIALS AND METHODS The study was carried out on total 35 out patients who were clinically diagnosed of Meniere's disease and of these 35 patients, 12 patients were found to have hypothyroidism. All patients with hypothyroidism were treated with oral thyroxin supplements and they were evaluated clinically at 3 weeks, 3 months and 6 months interval for the improvement in subjective symptoms on outpatient basis. RESULTS In our study, the subjective improvement of all symptoms was seen in all cases of Meniere's disease with hypothyroidism after 12 weeks of treatment. CONCLUSION The improvement in clinical symptoms after treatment demonstrates an association between Meniere's disease and hypothyroidism which was found to be significant. Hence, clinicians should consider screening patients with Meniere disease for thyroid dysfunction who are not already taking supplements.
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Affiliation(s)
- U P Santosh
- Professor, Department of ENT, JJM Medical College , Davanagere, Karnataka, India
| | - M S Sudhakar Rao
- Post Graduate Student, Department of ENT, JJM Medical College , Davanagere, Karnataka, India
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Goodall AF, Siddiq MA. Current understanding of the pathogenesis of autoimmune inner ear disease: a review. Clin Otolaryngol 2016; 40:412-9. [PMID: 25847404 DOI: 10.1111/coa.12432] [Citation(s) in RCA: 55] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/29/2015] [Indexed: 12/20/2022]
Abstract
BACKGROUND Autoimmune inner ear disease (AIED) is a poorly understood form of sensorineural hearing loss that causes bilateral, asymmetric, progressive hearing loss, sometimes with vestibular symptoms, often associated with a systemic autoimmune disease, which is noteworthy as the only sensorineural loss responsive to medical therapy. Despite much research interest of the past 25 years, its aetiopathogenesis is still unproven. OBJECTIVE OF REVIEW To succinctly consolidate research and opinion regarding the pathogenesis of autoimmune inner ear disease, in ongoing efforts to elucidate the molecular and intracellular pathways that lead to inner ear damage, which may identify new targets for pharmacotherapy. TYPE OF REVIEW Systematic review SEARCH STRATEGY PubMed/MEDLINE search using key terms to identify articles published between January 1980 and Apr 2014. Additionally, any landmark works discussed in this body of literature were obtained and relevant information extracted as necessary. EVALUATION METHOD Inclusion criterion was any information from animal or human studies with information relevant to possible aetiopathogenesis of AIED. Studies that focused on diagnosis, ameliorating symptoms or treatment, without specific information relevant to mechanisms of immune-mediated injury were excluded from this work. Articles meeting the inclusion criteria were digested and summarised. RESULTS A proposed pathogenic mechanism of AIED involves inflammation and immune-mediated attack of specific inner ear structures, leading to an excessive Th1 immune response with vascular changes and tissue damage in the cochlea. Studies have identified self-reactive T cells and immunoglobulins, and have variously implicated immune-complex deposition, microthrombosis and electrochemical disturbances causing impaired neurosignalling in the pathogenesis of AIED. Research has also demonstrated abnormalities in the cytokine milieu in subjects with AIED, which may prove a target for therapy in the future. CONCLUSION Ongoing research is needed to further elucidate the aetiopathogenesis of AIED and discern between various mechanisms of tissue injury. Large-cohort clinical studies employing IL-1 receptor blockade are warranted to determine its potential for future therapy.
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Affiliation(s)
- A F Goodall
- Department of Otolaryngology, St Helens & Knowsley Hospitals NHS Trust, St Helens, UK
| | - M A Siddiq
- Department of Otolaryngology, St Helens & Knowsley Hospitals NHS Trust, St Helens, UK
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Yıldırım A, Uzun SE, Sürücü GD, Doğan S, Karabiber M, Sarıkay Y. Evaluation of Hearing Functions with Audiological Tests in Patients with Rheumatoid Arthritis. JOURNAL OF CLINICAL AND EXPERIMENTAL INVESTIGATIONS 2016. [DOI: 10.5799/jcei.328683] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
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Headache and Dizziness: How to Differentiate Vestibular Migraine from Other Conditions. Curr Pain Headache Rep 2015; 19:31. [DOI: 10.1007/s11916-015-0502-3] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
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Monsanto RC, Neto ACL, Lorenzetti FTM. Reiter's syndrome and hearing loss: a possible association? Clin Case Rep 2014; 2:310-2. [PMID: 25548635 PMCID: PMC4270715 DOI: 10.1002/ccr3.119] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/05/2014] [Accepted: 05/05/2014] [Indexed: 11/18/2022] Open
Abstract
Key Clinical Message Patient complained of hearing loss and tinnitus after the onset of Reiter's syndrome. Audiometry confirmed the hearing loss on the left ear; blood work showed increased erythrocyte sedimentation rate and C3 fraction of the complement. Genotyping for HLA-B27 was positive. Treatment with prednisolone did not improve the hearing levels.
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Affiliation(s)
- Rafael C. Monsanto
- Centro de Estudos – Departamento de Otorrinolaringologia do Banco de Banco de Olhos de Sorocaba Hospital Praça Nabek Shiroma, 210 – Jardim Emília Sorocaba/SP CEP 18031‐060 Brazil
| | - Arlindo C. L. Neto
- Centro de Estudos – Departamento de Otorrinolaringologia do Banco de Banco de Olhos de Sorocaba Hospital Praça Nabek Shiroma, 210 – Jardim Emília Sorocaba/SP CEP 18031‐060 Brazil
| | - Fábio T. M. Lorenzetti
- Centro de Estudos – Departamento de Otorrinolaringologia do Banco de Banco de Olhos de Sorocaba Hospital Praça Nabek Shiroma, 210 – Jardim Emília Sorocaba/SP CEP 18031‐060 Brazil
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Bertoli LF, Pappas DG, Barton JC, Barton JC. Serum immunoglobulins in 28 adults with autoimmune sensorineural hearing loss: increased prevalence of subnormal immunoglobulin G1 and immunoglobulin G3. BMC Immunol 2014; 15:43. [PMID: 25339459 PMCID: PMC4210578 DOI: 10.1186/s12865-014-0043-2] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2014] [Accepted: 09/24/2014] [Indexed: 12/22/2022] Open
Abstract
BACKGROUND Our informal observations suggested that some patients with acute sensorineural hearing loss (ASNHL) have subnormal serum immunoglobulin (Ig) levels. We evaluated 28 consecutive adults (18 men, 10 women) at ASNHL diagnosis using: antibodies to 68 kD protein, 30 kD protein, and type II collagen; and serum total IgG, IgG subclasses, total IgA, and IgM. Reference ranges for Ig levels were mean ± 2 SD. We compared prevalences of subnormal IgG subclasses to those in 275 healthy European adults in previous reports. We also reviewed charts of consecutive adult index patients with primary Ig deficiency (35 common variable immunodeficiency, 406 IgG subclass deficiency) to identify other patients with probable ASHNL. RESULTS Mean age was 53 ± 10 (SD) y. Six patients (21.4%) had other autoimmunity manifestations. Antibodies to 68 kD protein, 30 kD protein, and type II collagen were detected in 21.4% (6/28), 21.1% (4/19) and 18.8% (3/16), respectively. Three patients (10.7%) had subnormal IgG1, six (21.4%) had subnormal IgG3, and four (14.3%) had subnormal IgG1 and IgG3. Some had subnormal IgG2, IgG4, IgA, and IgM (n = 1, 2, 3, and 1, respectively). Prevalences of subnormal IgG1 or IgG3 were greater in ASNHL patients (25.0% and 35.7%) than 275 controls (2.1% and 3.3%), respectively (p < 0.0001, each comparison). Relative risks of subnormal IgG1 and IgG3 in ASNHL were 11.5 [95% CI: 4.1, 31.7] and 10.9 [4.8, 25.6], respectively. Hearing improved after initial therapy in 17 patients (60.7%). Multiple regressions on Ig levels revealed no significant associations with other available variables. Logistic regressions on initial therapy response revealed a positive association with men (p = 0.0392) and a negative association with IgA (p = 0.0274). Our estimated prevalence of probable ASNHL in 35 patients with common variable immunodeficiency during a follow-up interval of 8 ± 4 y was 0% [95% CI: 0, 12.3]). Prevalence of probable ASNHL in 406 patients with IgG subclass deficiency during the same interval was 0.74% [0.19, 2.33]. CONCLUSIONS Serum levels of IgG1 or IgG3 were subnormal in 46.4% of 28 patients with ASNHL. Among adults who present with primary Ig deficiency, some may have or later develop ASNHL.
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Lobo DR, García-Berrocal JR, Ramírez-Camacho R. New prospects in the diagnosis and treatment of immune-mediated inner ear disease. World J Methodol 2014; 4:91-98. [PMID: 25332908 PMCID: PMC4202484 DOI: 10.5662/wjm.v4.i2.91] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/26/2013] [Revised: 02/10/2014] [Accepted: 04/11/2014] [Indexed: 02/06/2023] Open
Abstract
Autoimmune inner ear disease (AIED) represents a very fertile research field and the advancements in the understanding of this disease have a direct application not only in patients affected with this condition but also in other inner ear disorders that share the same injury mechanism, damage to the inner ear hair cells. AIED also presents many challenges that have still to be overcome. Firstly, access to the inner ear is limited, as many interventions such as biopsies can result in great irreversible damage. Secondly, there are no completely specific markers for AIED. Lack of a definitive diagnosis can result in the treatment of patients not affected with the disease and, therefore, no response. Finally, some patients become refractory to glucocorticoids and new therapies are needed. This review offers an overview of the animal models that have contributed to the understanding of AIED pathophysiology, the value of currently available diagnostic tests, and therapeutic options, with a special focus on new therapies for non responders or patients refractory to glucocorticoids. Among these new options for therapy, biological agents have been tested recently, whereas gene and stem cell therapy may have a role in the future. The intratympanic route of administration avoids the systemic side effects associated with currently used drugs, and may become a more frequent approach in the future.
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Assessment of diagnostic parameters in meniere's disease. Indian J Otolaryngol Head Neck Surg 2012; 53:108-11. [PMID: 23119769 DOI: 10.1007/bf02991501] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022] Open
Abstract
Meniere's disease has been ever served as one of the most debatable issue amongst the otologist. Various proposals for it's accurate diagnosis have arised in medical forefronts. The randomized study over here, of various diagnostic criteria in 52 nos are critically analysed in view of confirmation of clinically suspected Meniere's disease.Conclusions are drawn on basis of the autcomes of BERA,EcochG and caloric tests in form of individual as well cobined sensitivity in favour to attempt to standardize the deciding parameters in relation to the stage of the disease.
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Hornibrook J, George P, Spellerberg M, Gourley J. HSP70 antibodies in 80 patients with "clinically certain" Meniere's disease. Ann Otol Rhinol Laryngol 2011; 120:651-5. [PMID: 22097150 DOI: 10.1177/000348941112001004] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
OBJECTIVES We tested the claim that a significant proportion of patients with Meniere's disease have antibodies to heat shock protein 70 (HSP70) antigen, which may lead to defective endolymphatic sac function and vertigo attacks. METHODS Serum samples were taken from 80 subjects with a "certain" diagnosis of Meniere's disease (American Academy criteria plus electrocochleographic confirmation of endolymphatic hydrops with tone burst stimuli) and were tested for HSP70 antibodies with the OTOblot (hsp70) Western blot assay. The response was recorded as negative, positive, or equivocal. Samples from 80 sex- and age-matched blood donors were used as controls. RESULTS Of 80 patients with "clinically certain" Meniere's disease, 14 were positive for HSP70 antibodies or equivocal; of 80 controls, 10 were positive or equivocal. There was no significant difference (p=0.239). There was no correlation with bilateral disease, "activity" of Meniere's disease, or stage of Meniere's disease. CONCLUSIONS Patients with an unequivocal diagnosis of Meniere's disease do not have a significantly raised incidence of HSP70 antibodies.
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Affiliation(s)
- Jeremy Hornibrook
- Department of Otolaryngology-Head and Neck Surgery and Audiology, Christchurch Hospital, and the Department of Communication Disorders, University of Canterbury, Christchurch, New Zealand
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Semaan MT, Megerian CA. Ménière's Disease: A Challenging and Relentless Disorder. Otolaryngol Clin North Am 2011; 44:383-403, ix. [DOI: 10.1016/j.otc.2011.01.010] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
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Yukawa K, Hagiwara A, Ogawa Y, Nishiyama N, Shimizu S, Kawaguchi S, Nakamura M, Ito H, Tomiyama S, Suzuki M. Bilateral progressive hearing loss and vestibular dysfunction with inner ear antibodies. Auris Nasus Larynx 2009; 37:223-8. [PMID: 19709829 DOI: 10.1016/j.anl.2009.06.005] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2008] [Revised: 05/01/2009] [Accepted: 06/03/2009] [Indexed: 10/20/2022]
Abstract
Autoimmune inner ear disease (AIED) is a clinical syndrome of uncertain etiology. We present the neuro-otological findings of 2 cases of bilateral hearing loss, dizziness and the antibody profiles of the inner ears. Case 1 had bilateral progressive hearing loss, vestibular dysfunction and abnormal eye movement as the disease progressed. She had inner ear antibodies against 42 and 58kDa protein antigency on Western blot immune assay, and responded to glycocorticosteroid but not to immunosuppressant treatment. Intratympanic steroid injection temporally eliminated her symptoms. However, she developed idiopathic Cushing's syndrome and underwent labyrinthectomy. Case 2 became deaf as a teenager and experienced dizziness 10 years after becoming deaf. He reacted strongly to 68kDa protein and was a good responder to immunosuppressant with steroid. As we still lack a definitive diagnostic test for AIED, careful observation of the clinical course is critical for differential diagnosis regarding the bilateral progressive hearing loss.
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Affiliation(s)
- Kumiko Yukawa
- Department of Otolaryngology, Tokyo Medical University, 6-7-1 Nishishinjuku Shinjuku-ku, Tokyo, Japan.
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Agrup C. Immune-mediated audiovestibular disorders in the paediatric population: A review. Int J Audiol 2009; 47:560-5. [DOI: 10.1080/14992020802282268] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
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García-Berrocal JR, Ramírez-Camacho R, González-García JA, Verdaguer JM, Trinidad A. Does the Serological Study for Viral Infection in Autoimmune Inner Ear Disease Make Sense? ORL J Otorhinolaryngol Relat Spec 2008; 70:16-19; discussion 19-20. [DOI: 10.1159/000111043] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
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Fattori B, Nacci A, Dardano A, Dallan I, Grosso M, Traino C, Mancini V, Ursino F, Monzani F. Possible association between thyroid autoimmunity and Menière's disease. Clin Exp Immunol 2008; 152:28-32. [PMID: 18241228 DOI: 10.1111/j.1365-2249.2008.03595.x] [Citation(s) in RCA: 32] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022] Open
Abstract
Various aetiopathological mechanisms have been postulated to be at the root of Menière's disease (MD), and some data suggest that there may be also an underlying autoimmune factor. In fact, Menière patients manifest certain characteristics that are typical of autoimmune involvement association of particular human leucocyte antigen haplotypes, the presence of antibodies against internal ear antigens. In this study, we evaluated the association between thyroid autoimmunity and MD in a non-selected group of patients. We recruited 50 consecutive MD patients and two groups as controls: group A, 82 healthy volunteers; and group B, 50 subjects suffering from acute unilateral peripheral vestibulopathy. All subjects were submitted to instrumental assessment of cochlear-vestibular function and analysis of thyroid-stimulating hormone (TSH), free triiodothyronine, free thyroxine, anti-TSH receptor antibody (TR-Ab), anti-thyroperoxidase antibody (TPO-Ab) and anti-thyroglobulin antibody (Tg-Ab) in the blood. The prevalence of autoimmune thyroiditis in group B [6/50 (12%); 66.7% TPO-Ab and 33.3% Tg-Ab] was superimposable with the healthy controls [6/82 (7%); 66.7% TPO-Ab and 33.3% Tg-Ab]. In contrast, 38% of the MD patients (P = 0.0001 versus group A and group B) had significant autoantibody levels (68.4% TPO-Ab; 15.8% TPO-Ab + TR-Ab; 10.5% Tg-Ab; 5.2% TPO-Ab + Tg-Ab). Furthermore, 14% of the MD patients were hyperthyroid under l-thyroxine therapy, while no dysfunction was seen in the control groups. Overall, our data demonstrate a significant association between MD and thyroid autoimmunity, which suggests that an autoimmune factor is involved in the aetiopathogenesis of this disease. These findings suggest that it should be useful to submit MD patients to multi-disciplinary clinical investigation.
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Affiliation(s)
- B Fattori
- Department of Neuroscience, University of Pisa, Pisa, Italy.
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Mazlumzadeh M, Matteson EL. Cogan's Syndrome: An Audiovestibular, Ocular, and Systemic Autoimmune Disease. Rheum Dis Clin North Am 2007; 33:855-74, vii-viii. [DOI: 10.1016/j.rdc.2007.07.015] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
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Lobo D, López FG, García-Berrocal JR, Ramírez-Camacho R. Diagnostic tests for immunomediated hearing loss: a systematic review. The Journal of Laryngology & Otology 2007; 122:564-73. [PMID: 17908357 DOI: 10.1017/s002221510700059x] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
OBJECTIVE To quantitatively evaluate the diagnostic accuracy of diagnostic tests for immunomediated hearing loss. DATA SOURCES We searched Medline and the Cochrane Database of Systematic Reviews for potentially relevant studies. STUDY SELECTION Twenty-five studies met the inclusion criteria of this systematic review. The diagnosis of immunomediated hearing loss was based on the clinical presentation and the response to corticosteroid administration. DATA EXTRACTION The following data were extracted from the selected studies and entered into a standardised database: population demographics; exclusion and inclusion criteria; diagnostic tests; sensitivity; specificity; the number of true positive, true negative, false positive and false negative values; therapy used, including dose and duration; and delay between symptom onset and therapy commencement. DATA SYNTHESIS This systematic review combined data from 679 patients with immunomediated hearing loss, reported by 22 research teams. Substantial heterogeneity was found among the included studies; for this reason, summary sensitivity and specificity values were not computed. CONCLUSIONS The results of diagnostic tests for immunomediated hearing loss depend on many factors, and there is a risk of potential bias. This is the first time that such a systematic review has been presented; such a review is a more rigorous method of demonstrating the utility of the available diagnostic tests.
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Affiliation(s)
- D Lobo
- Ear Research Group, Department of Otorhinolaryngology, Hospital Universitario Puerta de Hierro, Universidad Autónoma de Madrid, Spain.
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García Berrocal JR, Lobo D, García López F, Ramírez-Camacho R. Enfermedad inmunomediada del oído interno: validación diagnóstica mediante un análisis sistemático de la literatura científica. ACTA OTORRINOLARINGOLOGICA ESPANOLA 2007. [DOI: 10.1016/s0001-6519(07)74914-2] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
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Abstract
Inner ear disorders are common and patients with vestibular failure often present to a neurology clinic because of their dizziness, gait unsteadiness and oscillopsia. Vestibular disorders can be divided into peripheral and central vestibular disorders. Most of the peripheral vestibular disorders have a clinical diagnosis, and a thorough history and examination will often provide a clear direction as to the diagnosis. Correct diagnosis allows treatment for many of the peripheral and central vestibular disorders. As inner ear damage is generally irreversible, early diagnosis allowing prompt treatment is important. The aim of this review is to discuss some audiovestibular conditions that may well appear in a neurology clinic, and to discuss some recent advances within the audiovestibular field that may be of interest to neurologists. Some of the most common audiovestibular conditions will be discussed along side more uncommon conditions.
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Affiliation(s)
- Charlotte Agrup
- The National Hospital for Neurology and Neurosurgery, London, UK.
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García Berrocal JR, Lobo D, García López F, Ramírez-Camacho R. Immunomediated Inner Ear Disease: Diagnostic Validation by Means of a Systematic Analysis of the Scientific Literature. ACTA OTORRINOLARINGOLOGICA ESPANOLA 2007. [DOI: 10.1016/s2173-5735(07)70336-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/14/2022]
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Abstract
PURPOSE OF REVIEW Immune-mediated inner-ear disorders may present to different medical disciplines and new research findings emerge rapidly. The purpose of this review is to draw the different strands together to produce an overview describing the clinical presentation of immune-mediated inner-ear disorders and to discuss useful diagnostic criteria with a focus on tissue-specific and tissue-non-specific antibodies. RECENT FINDINGS The importance of diagnosing an immune-mediated inner-ear disorder is highlighted in the context of it being one of few forms of treatable inner-ear disorder with a good response to immunosuppressive therapy. Due to a lack of reliable tests, the criteria upon which the diagnosis of immune-mediated inner-ear disease is based are often arbitrary. Previous and current research focuses mainly on the investigation of the aetiology of immune-mediated disorders, studying the presence of autoantibodies and the antigens responsible for their production. The prognostic and therapeutic values of inner-ear-specific antibodies are still unclear. Various antigens have been suggested. However, most antigens identified have been ubiquitous proteins not specific to the inner ear and therefore lack logical association with localized inner-ear pathology. SUMMARY Early diagnosis of immune-mediated inner-ear disorders with prompt treatment may prevent irreversible damage to inner-ear structures. Accordingly, it is important to include immune-mediated inner-ear disorders in the differential diagnosis of patients presenting with 'idiopathic' audiovestibular dysfunction. To enable early diagnosis, we recommend that the current available routine immunological laboratory tests (antinuclear, antineutrophil cytoplasmic, antiendothelial cell, antiphospholipid/anticardiolipin and antithyroid antibodies) are used when assessing a patient with suspected immune-mediated inner-ear disorder.
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Affiliation(s)
- Charlotte Agrup
- Department of Neuro-Otology, National Hospital for Neurology and Neurosurgery, London, UK.
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García Callejo FJ, Corts J, de Paula Vernetta C, Laporta P, Ramírez Sabio J, Marco Algarra J. Estudio de comorbilidad de la hipoacusia neurosensorial y otras enfermedades de patogenia autoinmune. Utilidad de las pruebas de laboratorio. ACTA OTORRINOLARINGOLOGICA ESPANOLA 2006; 57:28-33. [PMID: 16503030 DOI: 10.1016/s0001-6519(06)78659-9] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
Abstract
OBJECTIVES To value if the sensorineural hearing loss (SHL) in several autoimmune diseases (AD) is also secondary to immunomediation. PATIENTS AND METHODS In 342 cases with SHL and any AD and 301 normoacoustic controls with AD a non-specific immunologic battery of tests and a Western-blot (WB) for anticochlear autoantibodies were developed, and cases with SHL were treated with deflazacort. RESULTS Alterations in non-specific battery were similar between cases and controls. But a 12,5% of cases with SHL and a 1,6% of normoacoustic showed a positive WB (p<0,001). Steroid therapy improved hearing in 14,3% of cases. In them, sensitivity and specificity of non-specific battery were 78,4% and 75,5%, respectively, to predict response to therapy. These were 98,9% and 81,6% for WB. CONCLUSIONS The SHL in cases with AD can be considered immunomediated in a discrete average, but the diagnostic efficiency of WB to predict an improvement after steroid therapy is high.
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Affiliation(s)
- F J García Callejo
- Servicio de Otorrinolaringología, Hospital Clínico Universitario de Valencia.
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Kremmer S, Kreuzfelder E, Bachor E, Jahnke K, Selbach JM, Seidahmadi S. Coincidence of normal tension glaucoma, progressive sensorineural hearing loss, and elevated antiphosphatidylserine antibodies. Br J Ophthalmol 2004; 88:1259-62. [PMID: 15377546 PMCID: PMC1772360 DOI: 10.1136/bjo.2003.040832] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Abstract
BACKGROUND Recently, in patients with normal tension glaucoma (NTG) elevated levels of antiphosphatidylserine antibodies (APSA), a subgroup of antiphospholipid antibodies (APLA) were found. Progressive sensorineural hearing loss (PSHL) is associated with autoimmune diseases and also the presence of APLA. METHODS To investigate a possible association between NTG and PSHL, 34 patients (age range 31-81 years) with NTG were evaluated for evidence of audiovestibular disorders. Besides ophthalmological standard examinations (slit lamp, IOP, funduscopy, perimetry) scanning laser tomography and polarimetry were performed. From all patients' audiograms, stapedial thresholds and otoacoustic emissions were obtained. The serological testing of patients and controls (40 healthy blood donors older than 50 years) concerned IgG and IgM levels of antibodies against phosphatidylserine (APSA) and beta2 glycoprotein. RESULTS 23 of 34 NTG patients had hearing loss (PSHL n = 11; presbyacusis n = 12). The NTG patients had significantly higher APSA levels than controls. Elevated APSA concentrations were significantly more frequent in patients with NTG and hearing loss compared with NTG patients with normacusis. CONCLUSIONS These findings show that NTG and hearing loss have a high coincidence. The elevated APSA levels may indicate an association with similar systemic autoimmune processes.
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Affiliation(s)
- S Kremmer
- Department of Ophthalmology, University Hospital Essen, Hufelandstrasse 55, 45122 Essen, Germany.
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Abstract
BACKGROUND Autoimmune inner ear disease (AIED) was first described in 1979 and the disease has become more widely recognized over the last decade. Limited information is available regarding clinical features of the disease, disease course, and response to treatment. OBJECTIVE To analyze data from 42 patients with documented immune mediated inner ear disease to further define this syndrome. METHODS A retrospective chart review was conducted on all patients considered to have AIED by the Otolaryngology division physicians and on all patients positive for antibody testing to inner ear antigens from 1990 to 1999. Patients who were antibody positive with a clinical diagnosis of AIED were included in this review. RESULTS Patients with AIED presented with rapidly progressive, frequently bilateral (79%), often fluctuating sensironeural hearing loss. Mean age at presentation was 50 years (22-80) with no gender predilection. Tinnitus (83%), vestibular complaints (79%), and Menieres (50%) were common concomitant symptoms along with hearing loss. Seven of 42 (17%) of the patients had evidence for other systemic autoimmune disorders. In 4 of the patients the onset of vestibuloauditory complaints preceded the diagnosis of autoimmune disorder. Thirty-three of 42 demonstrated antibodies to inner ear antigens but other autoantibodies were infrequent except in patients with systemic autoimmune disorders. Twenty-three of 33 (70%) of patients treated with corticosteroids improved clinically, often short-term. Sixteen patients received treatment with other immunosuppressive drugs including methotrexate, cyclophosphamide, azathioprine, mycophenolic mofetil, and intravenous immunoglobulin (IVIg) generally with a limited modest response. Using clinical trial criteria for response, only 5/35 (14%) demonstrated improvement over a mean 34.4 months follow-up. CONCLUSIONS Immune-mediated inner ear disease is not a uniform disease with simple diagnosis or treatment. The course of the disease often results in significant long-term disability due to hearing loss and response to aggressive immunosuppression including corticosteroids is poor.
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Affiliation(s)
- Shelley S Broughton
- Otolaryngology, University of Texas Southwestern Medical School, Dallas, Texas, USA
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Abstract
Typically, autoimmune sensorineural hearing loss has been described as a slowly progressive, asymmetric hearing loss that is responsive to medications traditionally used in the treatment of other autoimmune conditions. These medications include steroids and cytotoxic drugs. Inciting factors in autoimmune inner ear disease are rarely cited. We describe a case of episodic sudden hearing loss triggered consistently by environmental noise. The hearing loss was responsive to steroids at the time of each occurrence and was determined to be autoimmune. This case raises questions about the relationship between autoimmune inner ear disease and sensitivity to environmental noise that warrant further research.
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Affiliation(s)
- Reena Gupta
- Department of Otolaryngology-Head and Neck Surgery, Thomas Jefferson Medical College, Thomas Jefferson University Hospital, Philadelphia, PA 19103-6771, USA
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38
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Spencer RF, Sismanis A, Kilpatrick JK, Shaia WT. Demyelination of Vestibular Nerve Axons in Unilateral Ménière's Disease. EAR, NOSE & THROAT JOURNAL 2002. [DOI: 10.1177/014556130208101113] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
We conducted a study to determine whether vestibular nerves inpatients with unilateral Ménière's disease whose symptoms are refractory to medical management exhibit neuropathologic changes. We also endeavored to determine whether retrocochlear abnormalities are primary or secondary factors in the disease process. To these ends, we obtained vestibular nerve segments from five patients during retrosigmoid (posteriorfossa) neurectomy, immediately fixed them, and processed them for light and electron microscopy. We found that all five segments exhibited moderate to severe demyelination with axonal sparing. Moreover, we noted that reactive astrocytes produced an extensive proliferation of fibrous processes and that the microglia assumed a phagocytic role. We conclude that the possible etiologies of demyelination include viral and/or immune-mediated factors similar to those seen in other demyelinating diseases, such as multiple sclerosis and Guillain-Barré syndrome. Our findings suggest that some forms of Ménière's disease that are refractory to traditional medical management might be the result of retrocochlear pathology that affects the neuroglial portion of the vestibular nerve.
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Affiliation(s)
- Robert F. Spencer
- Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Medical College of Virginia, Virginia Commonwealth University, Richmond
- Department of Anatomy, School of Medicine, Medical College of Virginia, Virginia Commonwealth University, Richmond
| | - Aristides Sismanis
- Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Medical College of Virginia, Virginia Commonwealth University, Richmond
| | - Jefferson K. Kilpatrick
- Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Medical College of Virginia, Virginia Commonwealth University, Richmond
| | - Wayne T. Shaia
- Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Medical College of Virginia, Virginia Commonwealth University, Richmond
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39
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García Berrocal JR, Ramírez-Camacho R, Vargas JA, Millan I. Does the serological testing really play a role in the diagnosis immune-mediated inner ear disease? Acta Otolaryngol 2002; 122:243-8. [PMID: 12030569 DOI: 10.1080/000164802753648105] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/17/2022]
Abstract
In order to analyse the clinical presentation and laboratory tests used for the diagnosis of immune-mediated sensorineural hearing loss (IMSNHL) a prospective study was conducted involving 125 patients affected by diverse clinical forms of the disease. The following methods were used: analysis of clinical evolution, pure-tone audiometry, determination of the immunophenotype of peripheral blood lymphocytes, immunoglobulins, complement factors C3 and C4, erythrocyte sedimentation rate, antinuclear autoantibodies (ANA) and heat shock protein 70 and the response to therapy. Levels CD4 (p = 0.03) and CD4CD45RA (p = 0.002) T cells were significantly reduced in patients with sudden sensorineural hearing loss. ANA were found in 34.4% of patients and the incidence of antibodies to HSP-70 was similar in both patients and control subjects. Diagnosis of IMSNHL is still based on clinical impressions as laboratory testing is not sufficiently diagnostic. However, the long-term evolution of IMSNHL presented by the patients included in this study may explain the negative results obtained in the tests. The existence of a typical profile patient, including the clinical course, immunological changes (presence of ANA and detection of abnormalities in T-cell subpopulations) and the response to steroid therapy can facilitate diagnosis. Further investigation to help identify a specific marker characteristic of IMSNHL is required.
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Affiliation(s)
- J R García Berrocal
- Otorhinolaryngology Department, Clínica Puerta de Hierro, Universidad Autónoma de Madrid, Spain.
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40
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García Berrocal JR, Ramírez-Camacho R, Arellano B, Vargas JA. Validity of the Western blot immunoassay for heat shock protein-70 in associated and isolated immunorelated inner ear disease. Laryngoscope 2002; 112:304-9. [PMID: 11889388 DOI: 10.1097/00005537-200202000-00019] [Citation(s) in RCA: 46] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
OBJECTIVE To assess the validity of the Western blot immunoassay for heat shock protein-70 (hsp-70) for diagnosis of autoimmune inner ear disease. STUDY DESIGN Retrospective study of 53 patients affected by sudden deafness (n = 19), idiopathic progressive sensorineural hearing loss (n = 24), and Meniere's disease (n = 10) who were treated from 1995 to 1999. The clinical course and response to corticosteroid were evaluated. METHODS A purified hsp-70 antigen from bovine kidney cell line was used for the Western blot immunoassay. RESULTS Only five patients (9.4%) showed anti--hsp-70 antibodies: Two presented a sudden sensorineural hearing loss (sudden deafness group), two showed an idiopathic progressive sensorineural hearing loss (idiopathic progressive sensorineural hearing loss group), and one was affected by fluctuating hearing loss (Meniere's disease group). A systemic autoimmune condition was observed in 29.1% of patients with idiopathic progressive sensorineural hearing loss. CONCLUSIONS The low sensitivity of Western blot immunoassay for patients affected by idiopathic progressive sensorineural hearing loss and Meniere's disease may result from either the long time elapsed from the hearing loss and vertigo to the initial examination or from the increased percentage of cases of systemic autoimmune disease present in patients with idiopathic progressive sensorineural hearing loss. More studies to detect the immune-mediated inner ear disease in Western blot immunoassay-negative patients are required.
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Affiliation(s)
- José Ramón García Berrocal
- Servicio de Otorrinolaringología, Clínica Puerta de Hierro, Universidad Autónoma de Madrid, Madrid, Spain.
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41
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Lasak JM, Sataloff RT, Hawkshaw M, Carey TE, Lyons KM, Spiegel JR. Autoimmune Inner Ear Disease: Steroid and Cytotoxic Drug Therapy. EAR, NOSE & THROAT JOURNAL 2001. [DOI: 10.1177/014556130108001110] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
The goal of this study was to assess the effects of immunosuppressive therapy on hearing inpatients with presumed autoimmune sensorineural hearing loss (AISNHL) and a Western blot assay positive for a 68 kD inner ear antigen. To achieve this objective, we conducted a retrospective review of 39 such patients who were treated with either a steroid alone or with a steroid followed by a cytotoxic agent. Pure-tone average (PTA) at 500 Hz, 1 kHz, 2 kHz, and 3 kHz and speech discrimination scores (SDS) were used as objective measures of outcome. At the completion of treatment, 23 of the 39 patients (59.0%) exhibited a positive response to therapy. The steroid-only responders (n = 6) tended to demonstrate a greater improvement in PTA (14.8 vs 4.5 dB), while the cytotoxic-agent responders (n = 17) had a significantly greater improvement in SDS (26.2 vs 6.9%; p< 0.01). We conclude that most patients with AISNHL benefit from immunosuppressive therapy and that cytotoxic medications appear to improve SDS, even in some patients who have not responded to corticosteroid therapy.
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Affiliation(s)
- John M. Lasak
- Department of Otolaryngology, Ohio State University, Columbus
| | - Robert T. Sataloff
- Department of Otolaryngology–Head and Neck Surgery, Thomas Jefferson University, Philadelphia
| | - Mary Hawkshaw
- American Institute for Voice and Ear Research, Philadelphia
| | - Thomas E. Carey
- Immunology and Cell Biology Laboratory, Kresge Hearing Research Institute, the University of Michigan, Ann Arbor
| | - Karen M. Lyons
- Department of Otolaryngology–Head and Neck Surgery, Thomas Jefferson University, Philadelphia
| | - Joseph R. Spiegel
- Department of Otolaryngology–Head and Neck Surgery, Thomas Jefferson University, Philadelphia
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42
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Yeo SW, Park SN. Immune-mediated sensorineural hearing loss in a patient with ankylosing spondylitis: a case report. Otolaryngol Head Neck Surg 2001; 125:113-4. [PMID: 11458230 DOI: 10.1067/mhn.2001.115524] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Affiliation(s)
- S W Yeo
- Department of Otolaryngology-Head and Neck Surgery, Kangnam St. Mary's Hospital, The Catholic University of Korea, College of Medicine, Seoul, Korea
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43
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Abstract
Autoimmune vestibulo-cochlear disorders (AVCD) represent a group of syndromes with overlapping clinical features, manifesting as sensorineural hearing loss, often associated with vertigo, tinnitus, and aural fullness, and believed to be caused by an autoimmune mechanism. Although definitive evidence of a classic "autoimmune process" is still lacking, substantial indirect evidence has accumulated to strongly indicate such a pathogenesis. Rapidly progressing AVCD is analogous to rapidly progressive glomerulonephritis in that inner ear inflammation progresses to severe, irreversible damage within 3 months of onset (and often much more quickly). Thus patients with rapidly progressive AVCD are treated with a sense of urgency. Prompt treatment with corticosteroids and other antirheumatic/immunosuppressive agents can preserve hearing and vestibular functions. We are not aware of any randomized controlled clinical trials evaluating the efficacy of antirheumatic/immunosuppressive agents in AVCD. In this article we review reports of various therapies that have been tried in this condition and our experience of etanercept therapy in AVCD.
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Affiliation(s)
- M U Rahman
- Rheumatology Unit, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts, USA.
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44
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Dornhoffer JL, Arenberg JG, Arenberg IK, Shambaugh GE. Pathophysiological mechanisms in immune inner ear disease. ACTA OTO-LARYNGOLOGICA. SUPPLEMENTUM 2001; 526:30-6. [PMID: 9107353 DOI: 10.3109/00016489709124018] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Immune inner ear disease is a somewhat controversial entity which describes cochleovestibular dysfunction that is related to immune-mediated mechanisms. The diagnosis of this disease is based on clinical presentation and response to various treatment protocols. Unfortunately, the presentation is variable and the treatment empirical, and this has caused much confusion in the diagnosis and management of the condition. To elucidate the variable nature of the disease, it is important to understand that more than one mechanism of immune injury may be involved. This paper attempts to classify clinical and experimental cases of immune inner ear disease with regard to the Gell and Coombs classification scheme of immune-mediated injury. By understanding the different pathophysiological mechanisms involved, the clinician should be better able to diagnose and manage this difficult problem in a directed fashion. The ramifications of the proposed classification system on the diagnosis, treatment and future research of immune inner ear disease are discussed.
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Affiliation(s)
- J L Dornhoffer
- Department of Otolaryngology/Head and Neck Surgery, University of Arkansas for Medical Sciences, Little Rock, USA
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45
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46
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Bouman H, Klis SF, Meeuwsen F, de Groot JC, Smoorenburg GF, Veldman JE. Experimental autoimmune inner ear disease: an electrocochleographic and histophysiologic study. Ann Otol Rhinol Laryngol 2000; 109:457-66. [PMID: 10823474 DOI: 10.1177/000348940010900504] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Systemic immunization with swine inner ear antigens in complete Freund's adjuvant induces functional disturbances in the cochlea. Morphometric data indicate that an endolymphatic hydrops develops within 2 weeks. It diminishes 6 weeks after immunization. A progressive decrease in the compound action potential amplitude is observed from 2 to 6 weeks after immunization. Enhancement of the amplitude of the summating potential is present without a clear overall correlation to the presence of endolymphatic hydrops. The amplitude of the cochlear microphonics shows no significant changes after immunization. Western blot analysis of the sera performed 2 and 6 weeks after immunization shows enhanced reactivity at 68, 50, 45, and 27 kd molecular weights, as compared to controls. The same spectrum of cross-reacting antibodies is believed to be instrumental in immune-mediated sensorineural hearing loss in patients. Apparently, cross-reacting antibodies and released mediators disturb cochlear homeostasis, resulting in the observed changes in the electrophysiological responses. However, these changes are not clearly related to structural changes at the light and electron microscopic levels.
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Affiliation(s)
- H Bouman
- Department of Otorhinolaryngology, University Medical Center Utrecht, The Netherlands
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47
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Abstract
The effect of allergy immunotherapy and elimination of suspected food allergens was evaluated in patients with Meniere's disease. A total of 137 patients with Meniere's disease for whom allergy treatment had been recommended were identified and were mailed and returned a symptoms questionnaire. One hundred thirteen had received allergy treatment; 24 did not have treatment and served as a control group. Information regarding history, signs and symptoms, allergy test results, and audiologic data were obtained by chart review. The 113 patients treated with desensitization and diet showed a significant improvement from pretreatment to posttreatment in both allergy and Meniere's symptoms. Ratings of frequency, severity, and interference with everyday activities of their Meniere's symptoms also appeared better after allergy treatment than ratings from the control group of untreated patients. Vertigo control results, by use of the American Academy of Otolaryngology-Head and Neck Surgery classification, categorized 47.9% as class A or B. Hearing was stable or improved in 61.4%. Patients with Meniere's disease can show improvement in their symptoms of tinnitus and vertigo when receiving specific allergy therapy. The inner ear may be the target, directly or indirectly, of an allergic reaction.
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Affiliation(s)
- M J Derebery
- House Ear Clinic and House Ear Institute, Los Angeles, CA 90057, USA
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48
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Abstract
Immune-mediated inner ear disease (IMIED) is a syndrome that includes the subacute onset of sensorineural hearing loss, often accompanied by vertigo and tinnitus. This constellation of symptoms may occur as a primary disorder in which no other organ involvement is evident, or it may complicate certain systemic conditions, including Wegener's granulomatosis, Cogan's syndrome, polyarteritis nodosa, and systemic lupus erythematosus. The precise disease mechanisms remain undefined, largely because of the difficulty obtaining relevant tissue specimens in untreated patients. However, if treated promptly with aggressive immunosuppression, the devastating sequelae of IMIED may be avoided. In this article, we review the pathophysiology, clinical evaluation, diagnostic testing, and therapy of IMIED.
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Affiliation(s)
- J H Stone
- Division of Rheumatology, Johns Hopkins Vasculitis Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA.
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49
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Abstract
OBJECTIVES To assess the utility of various laboratory tests used to diagnose autoimmune inner ear disease. STUDY DESIGN Retrospective study of 82 patients evaluated at the University of Washington Otology Clinic from 1996 through 1998 with review of clinical history, laboratory tests, audiograms, response to therapy, and final diagnoses. METHODS Charts were reviewed for presenting history and initial workup including test results for erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Clq binding assay, anticardiolipin antibody (aCL), antineutrophil cytoplasmic antibody (ANCA), microhemagglutinin assay for Treponema pallidum (microhemagglutination assay), Lyme disease titers, and the Western blot for heat shock protein 70 (hsp 70). RESULTS The Western blot for hsp 70 is the best test for predicting corticosteroid responsiveness. The sensitivity was low at 42%, although the specificity was 90%, and the positive predictive value of this test was excellent at 91%. The ESR was as good as the CRP in detecting acute-phase reactants. The other, more specific tests in the laboratory panel (aCL, ANCA, MHA, and Lyme disease titers) did not detect any new cases of autoimmune disease in addition to those which were already identified by an abnormal ESR. CONCLUSIONS A diagnostic test panel for autoimmune inner ear disease should include an ESR and the Western blot for hsp70. More specific laboratory testing for systemic disease is warranted when the ESR is elevated. In patients with a positive Western blot, a trial of corticosteroid therapy can be given with good conviction because the test is quite specific. However, many people who are Western blot negative may also respond to corticosteroid therapy because the test lacks sensitivity.
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Affiliation(s)
- K Hirose
- Department of Otolaryngology, University of Washington, Seattle 98195-6515, USA
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50
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Gloddek B, Lassmann S, Gloddek J, Arnold W. Role of S-100beta as potential autoantigen in an autoimmune disease of the inner ear. J Neuroimmunol 1999; 101:39-46. [PMID: 10580812 DOI: 10.1016/s0165-5728(99)00131-9] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
In Lewis rats, adoptive transfer of T cells specific for the calcium-binding protein S-100beta mediates experimental autoimmune encephalomyelitis, but surprisingly also induced a marked labyrinthitis associated with impairment of hearing. This suggests that transfer of S-100beta-specific T cells into susceptible animals could be a novel model to study autoimmunity of inner ear diseases. The investigation demonstrated in detail an inner ear involving, central nervous system (CNS)-specific autoimmune disease in order to identify a putatively shared autoantigen(s) in these pathologies. In fact, the model will be a useful tool to investigate in detail, the pathological mechanisms of the human inner ear disease associated with Vogt-Koyanagi-Harada disease.
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Affiliation(s)
- B Gloddek
- Department of Otolaryngology/Head and Neck Surgery, Klinicum rechts der Isar der, Technical University München, Munich, Germany.
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