autoimmune inner ear disease (aied) bastaninejad, shahin, md assistant professor of orl-hns, tums,...

Autoimmune Inner Ear Autoimmune Inner Ear Disease Disease ((AIEDAIED))

Bastaninejad, ShahinBastaninejad, Shahin, , MDMD

Assistant Professor of ORL-HNS, TUMS, AmirAlam Assistant Professor of ORL-HNS, TUMS, AmirAlam HospitalHospital

Case PresentationCase Presentation

45yrs female45yrs female

Right sided hearing Right sided hearing loss and aural loss and aural fullness, fullness, dysequilibrium dysequilibrium progressive over 2 progressive over 2 months months

Normal Px, except Normal Px, except Weber testWeber test

CBC, chemistries, CBC, chemistries, TFTs, RPR, ESR were TFTs, RPR, ESR were normalnormal

MRI MRI Normal Normal

Low salt diet,

Thiazide diuretics

At follow-up, Left At follow-up, Left

hearing worsedhearing worsed

Prednisone 60 mg

Daily

Serum anti-68kDa

protein positive

On steroids, Hearing On steroids, Hearing

improvedimproved

While steroid taper While steroid taper

down, one relapse down, one relapse

occuredoccured, again with , again with

improvement on improvement on

steroidssteroids

Presentation OutlinesPresentation Outlines

DefinitionDefinition

Epidemiology & PathogenesisEpidemiology & Pathogenesis

Clinical PresentationClinical Presentation

Differential DiagnosisDifferential Diagnosis

Laboratory and paraclinical work upLaboratory and paraclinical work up

TreatmentTreatment

Secondary AIEDSecondary AIED


• AIEDAIED (McCabe 1979):– Rapidly progressive SNHL (over a course of

weeks to months)– Bilateral– Responds to the administration of

corticosteroids

• ImportanceImportance: – AIED is one of the few medically reversible

causes of sensorineural hearing loss

DefinitionDefinition• Classification:

– PrimaryPrimary AIED AIED– SecondarySecondary AIED:

• SLE

• Ulcerative Colitis

• Cogan’s Syndrome

• Multiple Sclerosis

• Wegener’s Granulomatosis

• Various systemic vasculitides

• Sjogren’s Syndrome

• Celiac Disease







TreatmentTreatment



• 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 there is no direct evidencedirect evidence that Primary AIED is that Primary AIED is autoimmune in etiology …autoimmune in etiology …

– inflammationinflammation lead to the fibrosis and osteoneogenesis of the cochlea

– Vasculopathy necrosis associated with vasculitis in the absence of inflammation







TreatmentTreatment


Clinical Presentation

• The hearing loss may initially be unilateralmay initially be unilateral, and it may take months for the bilaterality to emerge

• Fluctuations in hearing may occurFluctuations in hearing may occur, but the overall course is one of a relentless deterioration in auditory function

• 50% 50% have Vestibular symptoms, in 20%20% of the cases Vertigo is like Meniere’s disease







TreatmentTreatment


Differential Diagnosis

• Sudden deafnessSudden 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 DiseaseMeniere’s Disease

– Very difficult to differentiate these two entities during Very difficult to differentiate these two entities during first months of evaluationfirst months of evaluation

– The only difference isThe only difference is: more aggressive course of : more aggressive course of AIED (regarding haring loss)AIED (regarding haring loss)

• OtosyphilisOtosyphilis• Acoustic neuromaAcoustic neuroma• MeningitisMeningitis• MSMS• MalignancyMalignancy (e.g., metastatic disease, lymphoma)







TreatmentTreatment


Laboratory and Paraclinical work upLaboratory 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 diagnosisbut can neither confirm nor rule out the diagnosis

Laboratory and Paraclinical work upLaboratory and Paraclinical work up

• Paraclinics:Paraclinics:– MRI, Brain and Skull base, +/-Gd– ENG– MonthlyMonthly Audiometeric assesment

• About Anti-HSP70: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 sensorineuralprogressive sensorineural hearing loss on

audiometric assessment done at monthly

intervals, and most importantly aa positive positive

response to the administration of response to the administration of

corticosteroidscorticosteroids

In summary

In summary







TreatmentTreatment


TreatmentTreatment

• Corticosteroid therapy– In Adult patients start with 60mg/day for

4weeks (children 1mg/kg/day)– Taper down in 10-12 days in nonrespondersnonresponders– In respondersresponders (15dB Improvement in one freq.

or 10dB at two freq. or significant SDS improvement)…

TreatmentTreatment• …Continue full-dose therapy until monthly

audiograms reached a plateau of recovery

• Then taper it down slowly over 8 weeks to a maintenance dose maintenance dose of 10 to 20mg every other day

• Continue maintenance dose for a variable time (total treatment duration more than 6mo)(total treatment duration more than 6mo)

TreatmentTreatment• Other therapies: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 implantationCochlear implantation

TreatmentTreatment

• The onlyThe only drug of proven utility in the drug of proven utility in the

management of AIED is management of AIED is CorticosteroidCorticosteroid







TreatmentTreatment


Cogan’s SyndromeCogan’s Syndrome

• Interstitial keratitis

• vertigo, tinnitus,

SNHL

• archetypal

autoimmune inner

ear disease

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

Vogt-Koyanagi-Harada (VKH) Vogt-Koyanagi-Harada (VKH) SyndromeSyndrome

• SNHL, Vestibular signs, Uveitis, …

• Periorbital hair loss and skin depigmentationPeriorbital hair loss and skin depigmentation

• Aseptic meningitis

• It may be autoimmunity to melanocytes

Wegener’s GranulomatosisWegener’s Granulomatosis• Necrotizing granulomata

• Vasculitis

• Respiratory tract and kidneys

involvement

• Serous OM

• C-ANCA 90% specific

• 30-50% ear involvement, 30-50% ear involvement,

usually middle ear, usually middle ear,

concomitant inner ear concomitant inner ear

involvement involvement 30% 30% SNHL SNHL

Polyarteritis NodosaPolyarteritis Nodosa

• Vasculitis of small and medium-sized

arteries

• Renal and visceral involvement

• Rarely rapidly progressive hearing loss

Behçet’s DiseaseBehçet’s Disease

Relapsing PolychondritisRelapsing Polychondritis

• Recurrent

inflammation of ear,

nose, trachea, larynx

• Autoantibodies to

cartilage

• NSAIDs, steroids,

dapsone

Systemic Lupus Systemic Lupus ErythematosusErythematosus

• Anti-nuclear, anti-DNA

antibodies

• Numerous systemic

manifestations

• COM with vasculitis,

SNHL (58%58%),

dysequilibrium

Rheumatoid ArthritisRheumatoid Arthritis

• Small joints of hands and feet

• Vasculitis, muscle atrophy, subcutaneous

nodules, splenomegaly

• 44% bilateral SNHL

autoimmune inner ear disease (aied) bastaninejad, shahin, md assistant professor of orl-hns, tums,...

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paraclinical work uplab

course of weeks

left hearing worsedprednisone

dailyserum anti

progressive snhl

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overall course