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Autoimmune Inner Ear Disease
Built from Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
What’s inside
9 sections · 103 slides
Overview
- Scope of this presentation
Background and Inner Ear Immunology
- Cogan’s original case reports
- McCabe’s description of autoimmune SNHL
- Immunological status of the inner ear
- Immune cells within the endolymphatic sac
- Evidence for local antibody production
- Spiral modiolar vein and collecting venules
- Vascular adhesion changes during inflammation
- Sequence of immune-mediated cochlear injury
Clinical Presentation
- Incidence of autoimmune inner ear disease
- Audiological pattern of AIED
- Differentiating AIED from other causes of SNHL
- Overlap with Ménière’s disease
- Value of a systemic review of systems
- Autoimmune hearing loss and associated systemic features
- Autoimmune hearing loss and associated systemic features (continued)
Mechanisms of Inner Ear Injury
- Molecular mimicry as a trigger
- The Cogan peptide
- The 68 kDa antigen and heat shock protein 70
- Other inner ear target antigens
- Collagen antibodies in relapsing polychondritis
- T cell mediated mechanisms
- Endolymphatic hydrops in AIED
- Cochlear vasculitis in granulomatosis with polyangiitis
- Otopathology of Sjögren’s syndrome and SLE
- Labyrinthine fibrosis and new bone in lupus
- Perilymphatic fibrosis and ossification in an animal model
- Findings in animal models of AIED
- Schuknecht and Nadol assessment in Cogan’s syndrome
Investigation
- Absence of a gold standard test
- The OTOblot assay
- Limitations of Hsp70 immunoassay
- Exclusion of infectious causes
- Autoimmune serological panel
- Role of serial audiometry
- Indications for imaging
Harris Classification
- Six categories of the Harris classification
- Category 1: organ (ear)-specific disease
- Category 2: SNHL with systemic autoimmune disease
- Category 3: immune-mediated Ménière’s disease
- Category 4: SNHL with associated inflammatory disease
- Category 5: SNHL with discrete organ system disease
- Category 6: nonimmune rapidly progressive SNHL
Autoimmune Disorders Associated with AIED
- Cogan’s syndrome: ocular features
- Cogan’s syndrome: systemic disease and mimics
- Periorbital depigmentation in Vogt-Koyanagi-Harada syndrome
- Vogt-Koyanagi-Harada syndrome: clinical course
- Vogt-Koyanagi-Harada syndrome: audiovestibular findings
- Susac’s syndrome: the diagnostic triad
- Susac’s syndrome: investigation
- Susac’s syndrome: treatment
- Granulomatosis with polyangiitis: otologic presentation
- Granulomatosis with polyangiitis: systemic features
- Granulomatosis with polyangiitis: diagnosis and outcome
- Temporal arteritis (giant cell arteritis)
- Temporal arteritis: audiovestibular involvement
- Behçet disease
- Behçet disease: audiovestibular findings
- Systemic lupus erythematosus: general features
- Systemic lupus erythematosus: otologic findings
- Rheumatoid arthritis
- Relapsing polychondritis
- Systemic sclerosis (scleroderma)
- Inflammatory bowel disease
- Thyroid disease and cochleovestibular dysfunction
Treatment
- Escalation of therapy in AIED
- Corticosteroids as first-line therapy
- Intratympanic steroid injection
- Adverse effects of long-term corticosteroid therapy
- Methotrexate in AIED
- The randomised trial of methotrexate
- Cyclophosphamide
- Biologic immunomodulators
- Anakinra in corticosteroid-resistant disease
- Approach to treatment failure
- Cochlear implantation in AIED
Summary
- Key points on mechanism and diagnosis
- Key points on treatment
- References
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- References (continued)
- Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
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