Autoimmune Inner Ear
Disease (AIED)
Bastaninejad, Shahin, MD
Assistant Professor of ORL-HNS, TUMS, A m i r A l a m
Hospital
Case Presentation
45yrs female
Right sided hearing
loss and aural
fullness,
dysequilibrium
progressive over 2
months
Normal Px, except
Weber test
CBC, chemistries,
TFTs, RPR, ESR
were normal
MRI  Normal
Low salt diet,
Thiazide diuretics
At follow-up, Left
hearing worsed
Prednisone 60
mg Daily
Serum anti68kDa protein
positive
On steroids, Hearing
improved
While steroid taper
down, one relapse
occured, again with
improvement on
steroids
Presentation Outlines
Definition
Epidemiology & Pathogenesis
Clinical Presentation
Differential Diagnosis
Laboratory and paraclinical work up
Treatment
Secondary AIED
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
Definition
• Classification:
– Primary AIED  AIED
– Secondary AIED:
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SLE
Ulcerative Colitis
Cogan’s Syndrome
Multiple Sclerosis
Wegener’s Granulomatosis
Various systemic vasculitides
Sjogren’s Syndrome
Celiac Disease
Presentation Outlines
Definition
Epidemiology & Pathogenesis
Clinical Presentation
Differential Diagnosis
Laboratory and paraclinical work up
Treatment
Secondary AIED
Epidemiology & Pathogenesis
Secondary AIED
• 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
Presentation Outlines
Definition
Epidemiology & Pathogenesis
Clinical Presentation
Differential Diagnosis
Laboratory and paraclinical work up
Treatment
Secondary AIED
Clinical Presentation
• The hearing loss may initially be unilateral,
and it may take months for the bilaterality
to 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
Presentation Outlines
Definition
Epidemiology & Pathogenesis
Clinical Presentation
Differential Diagnosis
Laboratory and paraclinical work up
Treatment
Secondary AIED
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
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)
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Otosyphilis
Acoustic neuroma
Meningitis
MS
Malignancy (e.g., metastatic disease, lymphoma)
Presentation Outlines
Definition
Epidemiology & Pathogenesis
Clinical Presentation
Differential Diagnosis
Laboratory and paraclinical work up
Treatment
Secondary AIED
Laboratory and Paraclinical work up
• Lab:
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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
Laboratory and Paraclinical work up
• Paraclinics:
– MRI, Brain and Skull base, +/-Gd
– ENG
– Monthly Audiometeric assesment
• 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
• The diagnosis of primary AIED is based on
clinical evaluation, the demonstration of
progressive sensorineural hearing loss on
audiometric assessment done at monthly
intervals, and most importantly  a
positive response to the administration of
corticosteroids
Presentation Outlines
Definition
Epidemiology & Pathogenesis
Clinical Presentation
Differential Diagnosis
Laboratory and paraclinical work up
Treatment
Secondary AIED
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)…
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)
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
Treatment
• The only drug of proven utility in the
management of AIED is Corticosteroid
Presentation Outlines
Definition
Epidemiology & Pathogenesis
Clinical Presentation
Differential Diagnosis
Laboratory and paraclinical work up
Treatment
Secondary AIED
Cogan’s Syndrome
Typical Cogan
• Interstitial keratitis
• vertigo, tinnitus,
SNHL
• archetypal
autoimmune inner
ear disease
Labyrinthine pathology may be coincident with the ocular
manifestations or may occur up to 6 months before or after the
onset of eye disease
Vogt-Koyanagi-Harada (VKH)
Syndrome
• SNHL, Vestibular signs, Uveitis, …
• Periorbital hair loss and skin depigmentation
• Aseptic meningitis
• It may be autoimmunity to melanocytes
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
Polyarteritis Nodosa
• Vasculitis of small and medium-sized
arteries
• Renal and visceral involvement
• Rarely rapidly progressive hearing loss
Behçet’s Disease
Relapsing Polychondritis
• Recurrent
inflammation of ear,
nose, trachea,
larynx
• Autoantibodies to
cartilage
• NSAIDs, steroids,
dapsone
Systemic Lupus
Erythematosus
• Anti-nuclear, antiDNA antibodies
• Numerous systemic
manifestations
• COM with vasculitis,
SNHL (58%),
dysequilibrium
Rheumatoid Arthritis
• Small joints of hands and feet
• Vasculitis, muscle atrophy, subcutaneous
nodules, splenomegaly
• 44% bilateral SNHL