Autoimmune inner ear disease (AIED): A diagnostic challenge

Autoimmune inner ear disease (AIED) has been defined as a condition of bilateral sensorineural hearing loss (SNHL), caused by an ‘uncontrolled’ immune system response. The inner ear can be the direct target of the immune response, but it can be additionally damaged by a deposition of circulating immune complexes or by systemic immune-mediated diseases. The clinical expression of immune-mediated inner ear disease shows a progressive bilateral and asymmetric SNHL profile, which typically benefits from a steroid and immunosuppressive therapy. The onset of AIED is between 3 and 90 days. Cochlear symptoms can be associated with vestibular disorders and in 15%–30% of cases, AIED occurs in the contest of a systemic autoimmune disease. Currently, the onset of immune-mediated SNHL is not a well-understood process and the pathogenetic mechanisms of AIED remain unclear. Furthermore, there are no standardized diagnostic criteria or reliable diagnostic tests for the diagnosis of AIED. Hence, the definition of immune-mediated cochleovestibular disorders is a challenging diagnosis based on exclusion. A close collaboration between otolaryngologists, audiologists and rheumatologists is recommended, in order to achieve the multidisciplinary management of this rare entity, since an early AIED identification and a prompt medical treatment might result in acceptable hearing outcomes. The paper describes the clinical features of AIED and offers a diagnostic flow-chart to use in the clinical assessment of this condition.

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immune-mediated conditions autoimmune inner ear disease autoimmune inner ear disease (AIED) Immunologic Disorders of the Inner Ear

Clinical Otology ◽

10.1055/b-0034-102411 ◽

2015 ◽

Keyword(s):

Inner Ear ◽

Immune Mediated ◽

Inner Ear Disease ◽

Autoimmune Inner Ear Disease

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

Oxford Medical Case Reports ◽

10.1093/omcr/omaa077 ◽

2020 ◽

Vol 2020 (9) ◽

Cited By ~ 1

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.

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Imaging in autoimmune inner-ear disease and endolymphatic hydrops, bone cement for improving hearing outcomes in stapes surgery, and the reporting of results. External ear canal cholesteatoma: a hypothesis

The Journal of Laryngology & Otology ◽

10.1017/s0022215118001020 ◽

2018 ◽

Vol 132 (06) ◽

pp. 469

Author(s):

Musheer Hussain ◽

Edward Fisher ◽

Jonathan Fishman

Keyword(s):

Bone Cement ◽

Inner Ear ◽

Endolymphatic Hydrops ◽

Stapes Surgery ◽

External Ear ◽

Ear Canal ◽

External Ear Canal ◽

Inner Ear Disease ◽

Hearing Outcomes ◽

Autoimmune Inner Ear Disease

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Autoimmune Inner Ear Disease

Otolaryngology ◽

10.1016/s0194-5998(05)80098-1 ◽

1995 ◽

Vol 112 (5) ◽

pp. P51-P51

Author(s):

Jeffrey P. Harris ◽

Ralph Nelson

Keyword(s):

Case Management ◽

Effective Treatment ◽

Inner Ear ◽

Diagnostic Tests ◽

Educational Objectives ◽

Problem Case ◽

Immune Mediated ◽

Inner Ear Disease ◽

Treatment Plans ◽

Autoimmune Inner Ear Disease

Educational objectives: To have a better understanding of the pathophysiology of inner ear immune-mediated disease and the most efficacious diagnostic tests available and to gain knowledge about sensible and effective treatment plans, potential complications of therapy, and problem case management.

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

The Journal of Laryngology & Otology ◽

10.1017/s002221510999137x ◽

2009 ◽

Vol 124 (1) ◽

pp. 105-107 ◽

Cited By ~ 3

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.

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Clinical Evaluation and Treatment of Immune-Mediated Inner Ear Disease

Ear Nose & Throat Journal ◽

10.1177/014556139607500509 ◽

1996 ◽

Vol 75 (5) ◽

pp. 301-305 ◽

Cited By ~ 7

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.

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