New prospects in the diagnosis and treatment of immune-mediated inner ear disease

Table 1 Specific and non-specific autoantibodies present in autoimmune inner ear disease patients and/or animal models of sterile labyrinthitis

Specific autoantibodies

Collagen type II

Collagen type IX

Cochlin

DEP-1/CD 148

KHRI-3

Myelin protein P0

Raf-1

Beta-tectorin

Beta-actin

Connexin 26

Non-specific autoantibodies

Antinuclear antibodies

Anti-neutrophil cytoplasmic antibodies

Anti-endothelial cell antibodies

Rheumatoid factor

Heat shock protein of 70 kDa

Anti-phospholipids/anticardiolipin antibodies

Antithyroid antibodies

Table 2 Diagnostic profile for autoimmune inner ear disease

Major criteria

Bilateral hearing loss

Systemic autoimmune disease

ANA > 1:80

Decrease of native T cells (CD4CD45RA)

Hearing recovery rate > 80%

Minor criteria

Unilateral hearing loss

Young or middle aged

Woman

Hearing recovery rate < 80%

Hearing recovery rate (after immunosuppressive therapy) is obtained: (initial hearing levels - final hearing levels)/initial hearing level – opposite ear hearing levels) × 100 (%). An autoimmune inner ear disease is suspected when three major criteria or two major and two minor criteria are met.

Table 3 Biological therapy agents

Drug	Anti	Dosage	Licensed indications	EMA approval	FDA approval
Etanercept	TNF-α	25 mg × 2/wk or 50 mg/wk sc	RA, JRA, PsA, AS, Ps	2000	1998
Infliximab	TNF-α	3 mg/kg at 0, 2 and 6 wk followed by 3.5-7.5 mg/8 wk iv	RA, PsA, AS, Ps, UC, CD	1999	1998
Adalimumab	TNF-α	40 mg/1-2 wk sc	RA, JRA, PsA, AS, Ps, CD	2003	2002
Anakinra	IL-1	100 mg/d sc	NHL, CLL, RA	2002	2001
Rituximab	B-cell CD20R	1 g/wk × 2 iv	RA	1998	1997

Dosage and indications approved by the European Medicines Agency (EMA) and the United States Food and Drug Administration (FDA). RA: Rheumatoid arthritis; JRA: Juvenile rheumatoid arthritis; PsA: Psoriatic arthritis; AS: Ankylosing spondylitis; Ps: Psoriasis; UC: Ulcerative colitis; CD: Crohn´s disease; NHL: Non-Hodgkin lymphoma; CLL: Chronic lymphocytic leukemia.