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 obliteration in ulcerative colitis patients with sensorineural hearing loss

2007 ◽  
Vol 122 (8) ◽  
pp. 871-874 ◽  
Author(s):  
S Kariya ◽  
K Fukushima ◽  
Y Kataoka ◽  
S Tominaga ◽  
K Nishizaki
Keyword(s):  
Magnetic Resonance Imaging ◽  
Ulcerative Colitis ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Three Dimensional ◽  
Sensorineural Hearing ◽  
Resonance Imaging ◽  
Published Report

AbstractObjective:Systemic autoimmune diseases, including ulcerative colitis, may involve the inner ear. Several ulcerative colitis cases presenting with sensorineural hearing loss have been reported. We report the T2-weighted, three-dimensional, inner-ear magnetic resonance imaging findings in the inner ears of two such patients.Methods:Case reports and a review of the literature concerning autoimmune disease and sensorineural hearing loss are presented.Results:We describe two cases of ulcerative colitis with sensorineural hearing loss in which three-dimensional magnetic resonance imaging revealed obliteration of the inner ear. Those inner ears with obliteration had severe hearing loss, and responded poorly to steroid therapy.Conclusion:To our knowledge, there has been no previous published report of the T2-weighted, inner-ear magnetic resonance imaging findings of cases of ulcerative colitis with sensorineural hearing loss. This paper represents the first published report in the world literature of inner-ear obliteration in such patients. Three-dimensional magnetic resonance imaging is beneficial in elucidating the pathophysiology of the inner-ear involvement seen in ulcerative colitis.

scholarly journals Sensorineural hearing loss and status epilepticus associated with ulcerative colitis: Is there enough evidence to support immune-related mechanisms?

2015 ◽  
Vol 6 (01) ◽  
pp. 077-079 ◽  
Author(s):  
Sinem Yazici ◽  
Gulcin Benbir ◽  
Birsen Ince
Keyword(s):  
Ulcerative Colitis ◽  
Hearing Loss ◽  
Status Epilepticus ◽  
Sensorineural Hearing Loss ◽  
Early Recognition ◽  
Systemic Disease ◽  
Sensorineural Hearing ◽  
Inflammatory Disorder ◽  
Major Morbidity ◽  
Immune Mediated

ABSTRACTUlcerative colitis (UC) is characterized by an inflammatory disorder of the gastrointestinal tract. Immune-mediated extraintestinal manifestations of UC have increasingly attracted attention in the literature recently, for which UC is now considered as a systemic disease. Neurologic involvement associated with UC is probably under-reported because of the unawareness of many physicians, although early recognition and treatment are crucial in preventing major morbidity and sequel. In this case report is presented a patient newly diagnosed as UC, who developed both sensorineural hearing loss and intractable status epilepticus that we suggest to have resulted from immune-mediated mechanisms.

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.

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.

Drug Delivery to the Inner Ear: Strategies and Their Therapeutic Implications for Sensorineural Hearing Loss

2012 ◽  
Vol 9 (3) ◽  
pp. 231-242 ◽  
Author(s):  
Teresa Rivera ◽  
Lorena Sanz ◽  
Guadalupe Camarero ◽  
Isabel Varela-Nieto
Keyword(s):  
Drug Delivery ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Therapeutic Implications

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.

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