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.

Sweet's disease and profound, bilateral, sensorineural hearing loss

2009 ◽  
Vol 124 (1) ◽  
pp. 105-107 ◽  
Author(s):  
S Cheng ◽  
M da Cruz
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Definitive Diagnosis ◽  
Sensorineural Hearing ◽  
Immune Mediated ◽  
Inner Ear Disease ◽  
Bilateral Sensorineural Hearing Loss ◽  
Loss Of Hearing ◽  
Hearing Outcomes

AbstractObjective:We report a case of Sweet's disease associated with rapid, profound loss of hearing, against a background of progressive, bilateral, sensorineural hearing loss.Results:The clinical features were indistinguishable from those of immune-mediated inner ear disease. Establishment of a definitive diagnosis was a challenge due to the absence of a reliable diagnostic test. The patient was unresponsive to extensive immunosuppressive therapy and subsequently underwent cochlear implantation, with good hearing outcomes.Conclusions:Profound, bilateral, sensorineural hearing loss in the context of Sweet's disease may be related to the underlying immunological aetiology. Cochlear implantation can successfully restore hearing when immunotherapy fails.

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.

scholarly journals Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
G. Psillas ◽  
M. Daniilidis ◽  
A. Gerofotis ◽  
K. Veros ◽  
A. Vasilaki ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Cyclosporine A ◽  
Immunosuppressive Treatment ◽  
Clinical Manifestations ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease

Sudden sensorineural hearing loss may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without another organ involvement (autoimmune inner ear disease). The diagnosis of autoimmune inner ear disease is still predicated on clinical features, and to date specific diagnostic tests are not available. We report a case of bilateral sudden hearing loss, tinnitus, intense rotatory vertigo, and nausea in a female patient in which the clinical manifestations, in addition to raised levels of circulating immune complexes, antithyroglobulin antibodies, and the presence of the HLA A1-B8-DR3 haplotype, allowed us to hypothesize an autoimmune inner ear disease. Cyclosporine-A immunosuppressive treatment in addition to steroids helped in hearing recovery that occurred progressively with normalization of the hearing function after a five-month treatment. Cyclosporine-A could be proposed as a therapeutic option in case of autoimmune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events.

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.

Congenital heart block and immune mediated sensorineural hearing loss: possible cross reactivity of immune response

Lupus ◽  
2016 ◽  
Vol 26 (8) ◽  
pp. 835-840 ◽  
Author(s):  
C Bason ◽  
I Pagnini ◽  
A Brucato ◽  
S Maestroni ◽  
A Puccetti ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Heart Block ◽  
Congenital Heart ◽  
Sensorineural Hearing ◽  
Congenital Heart Block ◽  
Cardiac Conduction ◽  
Immune Mediated ◽  
Cogan Syndrome

Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system. We wanted to evaluate whether mothers with anti-Ro/SSA antibodies who gave birth to children with congenital heart block have antibodies directed against inner ear antigens and whether these antibodies are connected with the presence of immune-mediated sensorineural hearing loss. We did not find anti-inner ear antibodies in the majority of the mothers. On the contrary a 13-year-old boy with congenital heart block and sensorineural hearing loss was positive for the presence of anti-inner ear antigens antibodies. Moreover his serum was positive for the presence of anti-Ro60 peptide antibodies but did not recognize the entire protein Ro60 (TROVE2), a behaviour similar to that of sera from patients with Cogan syndrome. In conclusion the data obtained so far show that anti-inner ear antibodies do not recognize the entire protein TROVE2 and do not support the hypothesis that such antibodies may be involved in the pathogenesis of congenital heart block.

Cochlear and Retrocochlear Immune-Mediated Inner Ear Disorders

1986 ◽  
Vol 95 (5) ◽  
pp. 535-540 ◽  
Author(s):  
Jan E. Veldman
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Neural Tissue ◽  
Sensorineural Hearing ◽  
Immune Mediated ◽  
Inner Ear Disorders

Three different forms of immune-mediated sensorineural hearing loss are described. The pathogeneses of these three cases with severe audiovestibular deficits are completely different. To make an appropriate diagnosis remains a dilemma. Autoimmunity plays a certain role, but is not always present. Vascular and neural tissue can become involved. Immune-mediated forms of sensorineural hearing loss can be of cochlear or retrocochlear origin.

Autoimmune sensorineural hearing loss: is it still a clinical diagnosis?

2003 ◽  
Vol 117 (3) ◽  
pp. 212-214 ◽  
Author(s):  
J. Mathews ◽  
S. Rao ◽  
B. N. Kumar
Keyword(s):  
Ulcerative Colitis ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Laboratory Diagnosis ◽  
Sensorineural Hearing ◽  
Pathogenetic Mechanism ◽  
Diagnostic Assays ◽  
Immune Mediated ◽  
The Many

Inner ear involvement with sensorineural hearing loss (SNHL) has been reported in many autoimmune disorders including ulcerative colitis. The pathogenetic mechanism of hearing loss in ulcerative colitis is thought to be immune mediated. Diagnostic tests are being developed to identify inner ear autoantibodies, that may be the cause of such hearing loss. The only test that is currently available for clinical use is the Otoblot test. This, however, tests only for antibodies against bovine heat shock protein 70 which is only one of the many cross-reacting proteins against the inner ear in suspected immune-mediated hearing loss. The clinical response to steroid therapy is thus the mainstay in the diagnosis of immune-mediated hearing loss. This paper presents a series of patients with clinically suspected autoimmune hearing loss. Diagnostic assays for this condition are discussed along with a review of the recent advances in the pathogenesis and laboratory diagnosis of immune-mediated sensorineural hearing loss.

Inner ear involvement in rheumatoid arthritis: a prospective clinical study

1995 ◽  
Vol 109 (8) ◽  
pp. 713-718 ◽  
Author(s):  
Ioannis Kastanioudakis ◽  
Antonios Skevas ◽  
Vasilios Danielidis ◽  
Eleni Tsiakou ◽  
A. Alexandros Drosos ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Disease Duration ◽  
Sensorineural Hearing ◽  
Joint Involvement ◽  
Acoustic Reflex ◽  
Reflex Latency ◽  
Bilateral Sensorineural Hearing Loss

AbstractSensorineural hearing loss in rheumatoid arthritis (RA) has been reported to be the result of the extraarticular manifestation of the disease (rheumatoid nodular vasculitis) or due to drug ototoxicity.In an attempt to investigate the presence of sensorineural hearing loss and the possible causes for it we investigated prospectively 45 RA patients (42 female; three male) with a mean age of 52.5±10.7 years and a mean disease duration of 8.5±7.3 years. All patients underwent a complete physical examination and audiological evaluation which included pure tone audiometry and impedance audiometry (tympanogram, static compliance, acoustic reflex, reflex decay, acoustic reflex latency test.We found a sensorineural hearing loss >20 dB HL in 44.4 per cent (40/90) ears. In all cases the site of hearing loss was the cochlea and in most of them it was bilateral and symmetric (16 patients out of 45 had bilateral sensorineural hearing loss i.e. 35.5 per cent.There was no correlation between sensorineural hearing loss and age, sex, disease duration, articular and extra-articular manifestations and the presence of autoantibodies in our patients. In addition, no correlation was found between sensorineural hearing loss and drug therapy for one at least of the following drugs: NSAIDs, d-penicillamine, plaquenil and methotrexate.We noticed a prologation of acoustic reflex latency in five patients (10 per cent) which was found to be correlated with the temporomandibular joint involvement and the presence of rheumatoid factor (RF).We conclude that inner ear involvement in RA is expressed by: (1) mild symmetric, bilateral sensorineural hearing loss of cochlear type in 35.5 per cent of patients; (2) normal acoustic reflex thresholds; (3) nondecay; and (4) prologation of acoustic reflex latency which appeared in a small number of patients (10 per cent).

Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss

2020 ◽  
pp. 624-624
Author(s):  
S. I. Ereniev ◽  
O. V. Plotnikova
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Biological Age ◽  
Biological Aging ◽  
Vibration Disease ◽  
Bilateral Sensorineural Hearing Loss

Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss were studied. The biological age of patients exceeded the calendar age by an average of 7.36±0.36 years and the proper biological age by 10.79±0.72 years. The rate of biological aging of the examined patients was 1.14±0.08 times higher than the rate of aging of their healthy peers.

Sign in / Sign up

Export Citation Format

Share Document