A Case of Bilateral Sudden Sensorineural Hearing Loss Considered due to Autoimmune Inner Ear Disease

2013 ◽  
Vol 136 (0) ◽  
pp. 95-98
Author(s):  
Maki Arai ◽  
Teruyuki Kato ◽  
Yuki Misawa
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Inner Ear Disease ◽  
Autoimmune 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.

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.

Audiological outcomes in sudden sensorineural hearing loss with presumed inner ear hemorrhage

2019 ◽  
Vol 40 (2) ◽  
pp. 274-278
Author(s):  
Kaitian Chen ◽  
Lanying Wen ◽  
Ling Zong ◽  
Min Liu ◽  
Jincangjian Sun ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing

scholarly journals Sudden sensorineural hearing loss associated with inner ear lesions detected by magnetic resonance imaging

PLoS ONE ◽  
2017 ◽  
Vol 12 (10) ◽  
pp. e0186038 ◽  
Author(s):  
Jiwon Cho ◽  
Hanjae Cheon ◽  
Jung Hye Park ◽  
Hyo-Jeong Lee ◽  
Hyung-Jong Kim ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Resonance Imaging

scholarly journals Genetic Polymorphisms in Sudden Sensorineural Hearing Loss: An Update

2020 ◽  
pp. 014556132094465
Author(s):  
Virginia Corazzi ◽  
Andrea Ciorba ◽  
Chiara Bianchini ◽  
Stefano Pelucchi ◽  
Piotr Henryk Skarżyński ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Genetic Polymorphisms ◽  
Microvascular Disease ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Genetic Profile ◽  
Early Prevention ◽  
Meta Analyses

Objective: To investigate the association between genetic polymorphisms and sudden sensorineural hearing loss (SSNHL). Most of the SSNHL cases still remain idiopathic, and several etiopathogenetic hypotheses, including a genetic predisposition, have been proposed. Methods: A literature review was conducted using different databases: Medline/PubMed, EMBASE, and CINAHL, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. All databases have been searched from May 2016 to April 2020. Results: Genetic susceptibility could represent a key element in the pathogenesis of SSNHL. A number of genetic polymorphisms related to (1) inner ear microvascular disease and endothelial dysfunction and (2) to inner ear oxidative stress and inflammation have been addressed in the current literature. Conclusions: The potential identification of a genetic profile related to SSNHL could provide a more accurate prognostic evidence of idiopathic SSNHL (ISSNHL), offering to the patients not only early-prevention strategies but eventually information on various inheritance modalities.

scholarly journals Bilateral Sudden Sensorineural Hearing Loss in Waldenström’s Macroglobulinemia: Case Report and Review of the Literature

2019 ◽  
Vol 2 (02) ◽  
pp. 81-84
Author(s):  
Nael M. Shoman ◽  
Thamir Aldahmashi
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Review Of The Literature ◽  
Waldenström’S Macroglobulinemia ◽  
Waldenstrom's Macroglobulinemia ◽  
Waldenstrom’S Macroglobulinemia ◽  
Waldenström's Macroglobulinemia

Abstract Objective Waldenström’s macroglobulinemia (WM) is a rare indolent B-cell lymphoproliferative disorder, representing 1 to 2% of all hematological malignancies. Involvement of the inner ear is rare with only case reports published over the past six decades. Methods The occurrence of bilateral sudden sensorineural hearing loss is exceeding rare, with limited published data in the literature. We present a case of a 62-year-old man diagnosed with WM who presented with bilateral sequential sudden sensorineural hearing loss. Results A few months following his WM diagnosis, he experienced sudden hearing loss in his left ear. He was treated with a course of oral steroids with no improvement. Three months following this incident, he experienced a similar sudden loss of hearing in his right ear. Treatment for WM was initiated. A repeat hearing test, done 1 week later, did not show any significant improvement in his right hearing. Conclusion The clinical course of WM is highly variable, with relatively infrequent involvement of lymph nodes, spleen, or liver. The inner ear is rarely involved. In this article, otologic clinical presentation is discussed, along with a review of the literature on hearing loss in WM.

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