Autoimmune Inner Ear Disease Preliminary Case Report: Audiometric Findings Following Steroid Treatments

2013 ◽  
Vol 22 (1) ◽  
pp. 120-124 ◽  
Author(s):  
Dave A. Harris ◽  
Anthony A. Mikulec ◽  
Sarah L. Carls
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Steroid Treatment ◽  
Special Focus ◽  
Saint Louis ◽  
Saint Louis University ◽  
Oral Steroids ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease ◽  
Intratympanic Steroids

Purpose This study examined the audiometric findings following steroid treatment for autoimmune inner ear disease (AIED), which is characterized by bilateral subacute hearing loss, using oral and intratympanic treatment. Method The charts of 9 patients with AIED who had been seen in the Saint Louis University School of Medicine's clinic between 2004 and 2011 were reviewed, with special focus on audiometric results. Results The majority of patients benefited from treatment for AIED. Four patients (6 ears) received intratympanic therapy as part of their treatment. Conclusion Patients with AIED appeared to benefit from a clinical pathway using a prolonged course of oral steroids, followed by a trial of intratympanic steroids and/or immunosuppresants for refractory cases.

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 Unilateral autoimmune inner ear disease in a patient with lung cancer treated with nivolumab

2020 ◽  
Vol 2020 (9) ◽  
Author(s):  
Aleksandra Rajapakse ◽  
Connor O’Leary ◽  
Raefe Gundelach ◽  
Rajeev Deva ◽  
Ken O’Byrne
Keyword(s):  
Lung Cancer ◽  
Hearing Loss ◽  
Inner Ear ◽  
Metabolic Response ◽  
Early Stage ◽  
Oral Prednisolone ◽  
Gradual Improvement ◽  
Inner Ear Disease ◽  
Bilateral Sensorineural Hearing Loss ◽  
Autoimmune Inner Ear Disease

ABSTRACT A 69-year-old male presented with early stage non-small cell lung cancer in 2016. The tumor was resected; however, the patient experienced recurrence 2 years later and subsequently received paclitaxel/carboplatin concurrently with radiotherapy. Within weeks of completing this treatment, he developed a symptomatic pancoast tumor secondary to disease progression and commenced second line nivolumab. Following the second dose of nivolumab, he developed acute unilateral right hearing loss. He commenced intravenous methylprednisolone followed by a slow taper of oral prednisolone. With steroids, he noted a gradual improvement in hearing, confirmed by audiology. Restaging imaging post-nivolumab demonstrated a complete metabolic response. Two prior cases have reported bilateral sensorineural hearing loss post-immune checkpoint inhibitor (ICI). We postulate the hearing impairment relates to the development of autoimmune inner ear disease. To our knowledge, this is the only case of a patient experiencing unilateral loss of hearing following an ICI.

Simultaneous versus Sequential Intratympanic Steroid Treatment for Severe-to-Profound Sudden Sensorineural Hearing Loss

2016 ◽  
Vol 21 (6) ◽  
pp. 399-405 ◽  
Author(s):  
Myung Hoon Yoo ◽  
Won Sub Lim ◽  
Joo Hyun Park ◽  
Joong Keun Kwon ◽  
Tae-Hoon Lee ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sudden Sensorineural Hearing Loss ◽  
Steroid Treatment ◽  
Partial Recovery ◽  
Steroid Injections ◽  
Simultaneous Group ◽  
Oral Steroids ◽  
Sequential Group ◽  
Intratympanic Steroids

Objectives: Severe-to-profound sudden sensorineural hearing loss (SSNHL) has a poor prognosis. We aimed to compare the efficacy of simultaneous and sequential oral and intratympanic steroids for this condition. Methods: Fifty patients with severe-to-profound SSNHL (>70 dB HL) were included from 7 centers. The simultaneous group (27 patients) received oral and intratympanic steroid injections for 2 weeks. The sequential group (23 patients) was treated with oral steroids for 2 weeks and intratympanic steroids for the subsequent 2 weeks. Pure-tone averages (PTA) and word discrimination scores (WDS) were compared before treatment and 2 weeks and 1 and 2 months after treatment. Treatment outcomes according to the modified American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria were also analyzed. Results: The improvement in PTA and WDS at the 2-week follow-up was 23 ± 21 dB HL and 20 ± 39% in the simultaneous group and 31 ± 29 dB HL and 37 ± 42% in the sequential group; this was not statistically significant. Complete or partial recovery at the 2-week follow-up was observed in 26% of the simultaneous group and 30% of the sequential group; this was also not significant. The improvement in PTA and WDS at the 2-month follow-up was 40 ± 20 dB HL and 37 ± 35% in the simultaneous group and 41 ± 25 dB HL and 48 ± 41% in the sequential group; this was not statistically significant. Complete or partial recovery at the 2-month follow-up was observed in 33% of the simultaneous group and 35% of the sequential group; this was also not significant. Seven patients in the sequential group did not need intratympanic steroid injections for sufficient improvement after oral steroids alone. Conclusions: Simultaneous oral/intratympanic steroid treatment yielded a recovery similar to that produced by sequential treatment. Because the addition of intratympanic steroids can be decided upon based on the improvement after an oral steroid, the sequential regimen can be recommended to avoid unnecessary intratympanic injections.

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.

Cochlear Implantation in Autoimmune Inner Ear Disease

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P53-P53 ◽  
Author(s):  
Katrina R Stidham ◽  
Joseph B Roberson
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Immunosuppressive Therapy ◽  
Cochlear Implantation ◽  
Hearing Aid ◽  
Speech Discrimination ◽  
Inner Ear Disease ◽  
Potential Risks ◽  
The Impact ◽  
Autoimmune Inner Ear Disease

Objective 1) Evaluate the success of cochlear implantation (CI) in patients with autoimmune inner ear disease (AIED). 2) Discuss the impact of CI on consideration of immunosuppressive therapy for patients with fluctuating hearing loss due to AIED. Methods Retrospective chart review was conducted of patients undergoing CI with diagnosis of AIED between 6/02 and 6/07. Patients’ autoimmune and immunotherapy treatment history was explored. Non-operative ear hearing thresholds and speech discrimination scores (SDS) were documented pre- and postoperatively. Objective and subjective implant success in setting of fluctuating hearing in non-implant ear was evaluated. Results Four patients with AIED, aged 5–55, underwent CI. Two had isolated otologic symptoms and 2 had other systemic autoimmune diagnoses. All had been previously treated with steroids. Three had received other immunosuppressive therapy. One declined other treatment due to potential risks. At the time of implantation, 3 patients had functional hearing in the non-implant ear and received benefit from a hearing aid. Each continued to have usable hearing, with fluctuating improvements post-implant at times to much better SDS than would normally meet candidacy requirements for CI. All patients found the implant provided better hearing than the hearing-aid ear. Conclusions CI is a viable option for patients with AIED. Implanting a profoundly deafened ear where opposite ear has fluctuating but usable hearing does not negatively impact outcomes. With the success of CI for AIED, the potential risks of long-term immunosuppressive therapy may not warrant its use in patients with isolated hearing loss symptoms.

Autoimmune Inner Ear Disease: Diagnostic and Therapeutic Approaches in a Multidisciplinary Setting

2003 ◽  
Vol 14 (04) ◽  
pp. 225-230 ◽  
Author(s):  
Eric L. Matteson ◽  
David A. Fabry ◽  
Scott E. Strome ◽  
Colin L. W. Driscoll ◽  
Charles W. Beatty ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Disease Process ◽  
Clinical Syndrome ◽  
Point Of View ◽  
Clinical Aspects ◽  
Inner Ear Disease ◽  
Circulating Autoantibodies ◽  
Sensitivity Specificity ◽  
Autoimmune Inner Ear Disease

Autoimmune Inner Ear Disease (AIED) is a clinical syndrome of uncertain pathogenesis. It is associated with bilateral rapidly progressive hearing loss. The hearing loss may be associated with vestibular symptoms. Autoimmunity has been proposed as the pathogenesis of this sort of hearing loss, although the mechanism of the disease is poorly understood. It is well accepted that the endolymphatic sac is an immunocompetent organ and circulating autoantibodies against inner ear antigens have been reported, as have viral antigens in the endolymph, although the sensitivity, specificity, and roles of those antibodies in a disease process are poorly defined.We will describe the clinical aspects of the disease, the histopathology, the immunologic indicators, the types of presentation, both from the audiologic and vestibular point of view, clinical trials for treatment and the follow-up. One of our conclusions is that many of these patients respond favorably to the treatment Methotrexate.

scholarly journals Autoimmune ear disease: clinical and diagnostic relevance in Cogan’s sydrome

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Luigi Maiolino ◽  
Salvatore Cocuzza ◽  
Angelo Conti ◽  
Luisa Licciardello ◽  
Agostino Serra ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Systemic Vasculitis ◽  
Clinical Syndrome ◽  
Inflammatory Disorder ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease

The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan’s syndrome. Cogan’s syndrome is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis, vestibular and auditory dysfunction. Associations between Cogan’s syndrome and systemic vasculitis, as well as aortitis, also exist. We report a case of a young woman who presented audiological and systemic characteristics attributable to Cogan’s syndrome. In the description of the case we illustrate how the appearance and evolution of the disease presented.

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