scholarly journals Audiovestibular Symptoms in Systemic Autoimmune Diseases

2018 ◽  
Vol 2018 ◽  
pp. 1-14 ◽  
Author(s):  
Massimo Ralli ◽  
Vittorio D’Aguanno ◽  
Arianna Di Stadio ◽  
Armando De Virgilio ◽  
Adelchi Croce ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Autoimmune Diseases ◽  
Inner Ear ◽  
Case Reports ◽  
Autoimmune Response ◽  
Systemic Autoimmune Diseases ◽  
Immune Mediated ◽  
Autoimmune Conditions ◽  
Inner Ear Disease ◽  
Vestibular Symptoms

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.

Noise-Induced Autoimmune Sensorineural Hearing Loss

2003 ◽  
Vol 112 (7) ◽  
pp. 569-573 ◽  
Author(s):  
Reena Gupta ◽  
Robert T. Sataloff
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Environmental Noise ◽  
Sudden Hearing Loss ◽  
Sensorineural Hearing ◽  
Autoimmune Conditions ◽  
Inner Ear Disease ◽  
Asymmetric Hearing Loss ◽  
Autoimmune Inner Ear Disease

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.

Clinical Evaluation and Treatment of Immune-Mediated Inner Ear Disease

1996 ◽  
Vol 75 (5) ◽  
pp. 301-305 ◽  
Author(s):  
D. Bradley Welling
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Marked Effect ◽  
Sensorineural Hearing ◽  
Illustrative Case ◽  
Immune Mediated ◽  
Inner Ear Disease ◽  
Bilateral Sensorineural Hearing Loss ◽  
Diagnostic Finding

Immune-mediated inner ear disease, first described by McCabe’ in 1979, typically presents with an idiopathic, rapidly progressive bilateral sensorineural hearing loss. The course of the hearing loss occurs over weeks to months. It may occur in both sexes and at a variety of ages, but is most common in middle-aged females. It may be accompanied by tinnitus, Meniere's-like vertigo, or more commonly, ataxia or unsteadiness. Approximately 30% of patients will have associated systemic immune-mediated disease. Although refinements in laboratory tests for specific inner ear antigens are being made,2-4 and nonspecific laboratory indicators of inflammatory or systemic immune disease may be useful in confirming the diagnosis, the most important diagnostic finding is the improvement in hearing seen with a trial of immunosuppressants. This report includes a typical evaluation of the patient with suspected immune-mediated inner ear disease and an illustrative case. Sensorineural hearing loss due to immune-mediated inner ear disease, although unusual as a cause of hearing loss, is important to recognize because early diagnosis and treatment can have a marked effect on the clinical outcome.

Immune-mediated inner ear disease

2000 ◽  
Vol 12 (1) ◽  
pp. 32-40 ◽  
Author(s):  
John H. Stone ◽  
Howard W. Francis
Keyword(s):  
Inner Ear ◽  
Immune Mediated ◽  
Inner Ear Disease

Cochlear Implantation Outcomes in Patients With Autoimmune and Immune-Mediated Inner Ear Disease

2010 ◽  
Vol 31 (8) ◽  
pp. 1337-1342 ◽  
Author(s):  
Saba Aftab ◽  
Maroun T. Semaan ◽  
Gail S. Murray ◽  
Cliff A. Megerian
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Immune Mediated ◽  
Inner Ear Disease

Azathioprine in Combination with Steroids in the Treatment of Autoimmune Inner-Ear Disease

1993 ◽  
Vol 21 (4) ◽  
pp. 192-196 ◽  
Author(s):  
Aytac Saraçaydin ◽  
Sedat Katircioğlu ◽  
Sami Katircioğlu ◽  
M Can Karatay
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Deafness ◽  
Sensorineural Hearing ◽  
Once Daily ◽  
Uncommon Disease ◽  
Inner Ear Disease ◽  
Pure Tones ◽  
Autoimmune Inner Ear Disease

A total of twelve patients with a relatively uncommon form of progressive sensorineural deafness (autoimmune innerear disease) were treated orally with 1 mg/kg azathioprine, once daily, and with 30 mg prednisolone, every other day, for 4 weeks. Statistically significant increases in the ability to hear pure tones or in discrimination on audiometry took place in 10/12 patients. This condition was initially described as ‘sensorineural hearing loss', but it is now clear that the term ‘autoimmune inner-ear disease’ is more appropriate since the vestibular compartment as well as the cochlear compartment is involved. This relatively uncommon disease is one of the few forms of sensorineural deafness that can be successfully treated.

Stria Vascularis Ultrastructural Pathology in the C3H/lpr Autoimmune Strain Mouse: A Potential Mechanism for Immune-Related Hearing Loss

1992 ◽  
Vol 106 (3) ◽  
pp. 288-295 ◽  
Author(s):  
Ilsa Schwartz ◽  
Sean O. McMenomey ◽  
Nancy J. Russell ◽  
Jane I. Morton ◽  
Dennis R. Trune
Keyword(s):  
Hearing Loss ◽  
Autoimmune Disease ◽  
Basement Membrane ◽  
Autoimmune Diseases ◽  
Stria Vascularis ◽  
Disease Onset ◽  
Systemic Autoimmune Disease ◽  
Potential Mechanism ◽  
Systemic Autoimmune Diseases ◽  
Strain Mouse

The stria vascularis in the C3H/ lpr autoimmune strain mouse was ultrastructurally examined in order to better understand the potential mechanisms by which systemic autoimmune disease affects the ear. The inner ears from C3H/ lpr mice before disease onset and C3H/HeJ controls showed no apparent pathology. However, the stria vascularis from older C3H/ lpr mice after systemic autoimmune disease onset showed considerable intercellular edema around the stria capillaries and thickening of the capillary basement membrane, compared to controls. These observations suggest that perivascular abnormalities, which are the hallmark of systemic autoimmune diseases, may underlie the stria dysfunction and hearing loss seen in autoimmune diseases in humans.

Review of the Biological Agents Used for Immune-Mediated Inner Ear Disease

2013 ◽  
Vol 64 (3) ◽  
pp. 223-229 ◽  
Author(s):  
David Lobo ◽  
José R. García-Berrocal ◽  
Almudena Trinidad ◽  
José M. Verdaguer ◽  
Rafael Ramírez-Camacho
Keyword(s):  
Inner Ear ◽  
Biological Agents ◽  
Immune Mediated ◽  
Inner Ear Disease

scholarly journals Tocilizumab for the Treatment of Rheumatoid Arthritis and Other Systemic Autoimmune Diseases: Current Perspectives and Future Directions

2012 ◽  
Vol 2012 ◽  
pp. 1-14 ◽  
Author(s):  
Atsushi Ogata ◽  
Toshio Tanaka
Keyword(s):  
Rheumatoid Arthritis ◽  
Autoimmune Diseases ◽  
Host Defense ◽  
Lupus Erythematosus ◽  
Therapeutic Strategy ◽  
Case Reports ◽  
Systemic Autoimmune Diseases ◽  
Future Directions ◽  
Pilot Studies ◽  
Beneficial Effects

Interleukin (IL)-6 is a cytokine featuring redundancy and pleiotropic activity. While IL-6, when transiently produced, contributes to host defense against acute environmental stress, continuous dysregulated IL-6 production plays a significant pathological role in several systemic autoimmune diseases. In response to the expectation that IL-6 blockade would constitute a novel therapeutic strategy for the treatment of these diseases, tocilizumab, a humanized anti-IL-6 receptor antibody, was developed. Clinical trials have verified the efficacy and the safety of tocilizumab for patients with rheumatoid arthritis, resulting in approval of this innovative biologic for the treatment of rheumatoid arthritis in more than 90 countries worldwide. Pathological analyses of the effect of IL-6 on the development of autoimmune diseases and a considerable number of case reports and pilot studies have also indicated the beneficial effects of this antibody on other systemic autoimmune diseases, including systemic lupus erythematosus, systemic sclerosis, polymyositis, and large-vessel vasculitis.

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