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Autoimmune Inner Ear Disease ( AIED )

Autoimmune Inner Ear Disease ( AIED ). Bastaninejad , Shahin , MD Assistant Professor of ORL-HNS, TUMS, AmirAlam Hospital. Case Presentation. 45yrs female R ight sided hearing loss and aural fullness, dysequilibrium progressive over 2 months Normal Px , except Weber test

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Autoimmune Inner Ear Disease ( AIED )

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  1. Autoimmune Inner Ear Disease (AIED) Bastaninejad, Shahin, MD Assistant Professor of ORL-HNS, TUMS, AmirAlam Hospital

  2. Case Presentation 45yrs female Right sided hearing loss and aural fullness, dysequilibriumprogressive over 2 months Normal Px, except Weber test CBC, chemistries, TFTs, RPR, ESR were normal MRI  Normal

  3. Low salt diet, Thiazide diuretics At follow-up, Left hearing worsed Prednisone 60 mg Daily Serum anti-68kDa protein positive

  4. On steroids, Hearing improved While steroid taper down, one relapse occured, again with improvement on steroids

  5. Presentation Outlines • Definition • Epidemiology& Pathogenesis • Clinical Presentation • Differential Diagnosis • Laboratory and paraclinical work up • Treatment • Secondary AIED

  6. Definition • AIED(McCabe 1979): • Rapidly progressive SNHL (over a course of weeks to months) • Bilateral • Responds to the administration of corticosteroids • Importance: • AIED is one of the few medically reversible causes of sensorineural hearing loss

  7. Definition • Classification: • Primary AIED  AIED • Secondary AIED: • SLE • Ulcerative Colitis • Cogan’s Syndrome • Multiple Sclerosis • Wegener’s Granulomatosis • Various systemic vasculitides • Sjogren’s Syndrome • Celiac Disease

  8. Presentation Outlines • Definition • Epidemiology& Pathogenesis • Clinical Presentation • Differential Diagnosis • Laboratory and paraclinical work up • Treatment • Secondary AIED

  9. Epidemiology & Pathogenesis • Primary AIED is a rare disorder • True incidence is not clear. More common in middle aged woman • Pathogenesis: • Although this disorder is likely immune mediated, there is no direct evidence that Primary AIED is autoimmune in etiology … • inflammation lead to the fibrosis and osteoneogenesis of the cochlea • Vasculopathy  necrosis associated with vasculitis in the absence of inflammation Secondary AIED

  10. Presentation Outlines • Definition • Epidemiology& Pathogenesis • Clinical Presentation • Differential Diagnosis • Laboratory and paraclinical work up • Treatment • Secondary AIED

  11. Clinical Presentation • The hearing loss may initially be unilateral, and it may take months for the bilateralityto emerge • Fluctuations in hearing may occur, but the overall course is one of a relentless deterioration in auditory function • 50% have Vestibular symptoms, in 20% of the cases Vertigo is like Meniere’s disease

  12. Presentation Outlines • Definition • Epidemiology& Pathogenesis • Clinical Presentation • Differential Diagnosis • Laboratory and paraclinical work up • Treatment • Secondary AIED

  13. Differential Diagnosis • Sudden deafness • It’s mainly unilateral  while AIED is bilateral • It is an emergency  while AIED is not • It develops in less than 72hr  but AIED occurs in weeks to months • In SSNHL treatment window is 2 to 4 weeks, but in a patient with AIED who develop SNHL in 6 to 12mo, we can still achieve significant recovery with corticosteroids

  14. Differential Diagnosis • Meniere’s Disease • Very difficult to differentiate these two entities during first months of evaluation • The only difference is: more aggressive course of AIED (regarding haring loss) • Otosyphilis • Acoustic neuroma • Meningitis • MS • Malignancy(e.g., metastatic disease, lymphoma)

  15. Presentation Outlines • Definition • Epidemiology& Pathogenesis • Clinical Presentation • Differential Diagnosis • Laboratory and paraclinical work up • Treatment • Secondary AIED

  16. Laboratory and Paraclinical work up • Lab: • CBC/diff • ESR • RF • ANA, Anti-dsDNA, Anti-phospholipid • Anti-SSA/B • C3, C4 complement level • FTA-ABS • HIV • Western blotting for antibody against 68-kD Ag, or Anti-HSP70  positive result may support the diagnosis of AIED, but can neither confirm nor rule out the diagnosis

  17. Laboratory and Paraclinical work up • Paraclinics: • MRI, Brain and Skull base, +/-Gd • ENG • MonthlyAudiometericassesment • About Anti-HSP70: • It is positive in 89% of AIED • Further studies have failed to show that this test has sufficient sensitivity and specificity to rule in or rule out the diagnosis of AIED

  18. In summary • The diagnosis of primary AIED is based on clinical evaluation, the demonstration of progressive sensorineuralhearing loss on audiometric assessment done at monthly intervals, and most importantlyapositive response to the administration of corticosteroids

  19. Presentation Outlines • Definition • Epidemiology& Pathogenesis • Clinical Presentation • Differential Diagnosis • Laboratory and paraclinical work up • Treatment • Secondary AIED

  20. Treatment • Corticosteroid therapy • In Adult patients start with 60mg/day for 4weeks (children 1mg/kg/day) • Taper down in 10-12 days in nonresponders • In responders (15dB Improvement in one freq. or 10dB at two freq. or significant SDS improvement)…

  21. Treatment • …Continue full-dose therapy until monthly audiograms reached a plateau of recovery • Then taper it down slowly over 8 weeks to a maintenance dose of 10 to 20mg every other day • Continue maintenance dose for a variable time (total treatment duration more than 6mo)

  22. Treatment • Other therapies: • MTX (?)  Steroid sparing (?) • CTX (Cyclophosphamide)  severe side effects • Etanercept (?)  inhibitor of TNF-alpha • IT Steroid therapy • IT TNF-alpha • Systemic IgG injection • Plasmapheresis • Cochlear implantation

  23. Treatment • The only drug of proven utility in the management of AIED is Corticosteroid

  24. Presentation Outlines • Definition • Epidemiology& Pathogenesis • Clinical Presentation • Differential Diagnosis • Laboratory and paraclinical work up • Treatment • Secondary AIED

  25. Cogan’s Syndrome Typical Cogan • Interstitial keratitis • vertigo, tinnitus, SNHL • archetypal autoimmune inner ear disease

  26. Labyrinthine pathology may be coincident with the ocular manifestations or may occur up to 6 months before or after the onset of eye disease

  27. Vogt-Koyanagi-Harada (VKH) Syndrome • SNHL, Vestibular signs, Uveitis, … • Periorbital hair loss and skin depigmentation • Aseptic meningitis • It may be autoimmunity to melanocytes

  28. Wegener’s Granulomatosis • Necrotizing granulomata • Vasculitis • Respiratory tract and kidneys involvement • Serous OM • C-ANCA 90% specific • 30-50% ear involvement, usually middle ear, concomitant inner ear involvement  30%  SNHL

  29. PolyarteritisNodosa • Vasculitis of small and medium-sized arteries • Renal and visceral involvement • Rarely rapidly progressive hearing loss

  30. Behçet’s Disease

  31. Relapsing Polychondritis • Recurrent inflammation of ear, nose, trachea, larynx • Autoantibodies to cartilage • NSAIDs, steroids, dapsone

  32. Systemic Lupus Erythematosus • Anti-nuclear, anti-DNA antibodies • Numerous systemic manifestations • COM with vasculitis, SNHL (58%), dysequilibrium

  33. Rheumatoid Arthritis • Small joints of hands and feet • Vasculitis, muscle atrophy, subcutaneous nodules, splenomegaly • 44% bilateral SNHL

  34. Thank You!

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