Review Article

Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Aspects, and Therapeutic Modalities of Cogan’s Syndrome

Table 2

Evidence for treatment of Cogan’s syndrome with biological agents.

Biological agentImmune mechanismNumber of CS patients reported to be treated with biotherapyAge (years)/gender/clinical presentationBiologic dosage and regimenNumber of CS patients that responded to biotherapyStudy designCountry of publicationRef

InfliximabChimeric anti-TNF-α monoclonal antibody233/M/AVD, IK300 mg × 1/month2CRSwitzerland[42]
49/M/SNHL300 mg × 6/week
330/F/AVD, IK3 mg/Kg at weeks 0, 2, 6, 8, and then every 8 weeks3CRItaly[40]
29/M/AVD, IKNA
35/M/SNHL, scleritisNA
237/F/AVD, IK3 mg/kg at 0, 2, and 6 weeks1CRUSA[39]
36/F/AVD, IK3 mg/kg for 4 months
116/M/TINU, SNHL, BRAO, glaucoma, and uveitis900 mg at 0, 3, and 5 weeks1CRUSA[41]
148/F/AVD3 mg/kg at 0 and 3 weeks and then every 8 weeks1CRSpain[43]
151/F/SNHL3 mg/kg every 8 weeks for 3 years1CRIsrael[6]
1NA/NA/AVD, scleritisNA1CRSwitzerland[74]
167/F/AVDNA1CRGreece[45]

EtanerceptTNF-α receptor fusion protein3NA25 mg × 2/week for 24 weeks2CRUSA[46]

AdalimumabAnti-TNF-α monoclonal antibody169/M/SNHL, iritis, aortitis, meningitis, panniculitis, and seronegative arthritis40 mg × 1/week for 2 weeks0CRJapan[50]
125/F/AVD, conjunctivitis, IK40 mg × 1/week for 6 months0CRItaly[48]

RituximabAnti-CD20 monoclonal antibody125/F/AVD, conjunctivitis, IK500 mg × 1/week for 4 weeks1CRItaly[48]
167/F/AVDNA0CRGreece[45]
143/F/AVD, IK375 mg/m2 × 1/week for 4 weeks0CRUSA[49]

TocilizumabHumanized anti-IL-6 receptor monoclonal antibody169/M/SNHL, iritis, aortitis, meningitis, panniculitis, and seronegative arthritis8 mg/kg × 1/month1CRJapan[50]
159/M/SNHL, anterior uveitis162 mg × 1/week for 2 weeks0CRUSA[47]

CS: Cogan’s syndrome; Ref: references; M: male; F: female; AVD: audiovestibulary dysfunction; SNHL: sensorineural hearing loss; IK: interstitial keratitis; TNF-α: tumor necrosis factor-α; CD: cluster of differentiation; IL: interleukin; CR: case report; NA: data is not available; TINU: tubulointerstitial nephritis and uveitis syndrome; BRAO: branch retinal artery occlusion. Dosage is noted as given according to “international protocol.” Unpublished data.