Optimal management of Cogan’s syndrome: a multidisciplinary approach

Abstract

Cogan’s syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. CS affects mainly young Caucasian adults, mostly during their first three decades of age, and may develop into typical and atypical variants. Typical CS manifests primarily with IK and hearing loss, whereas atypical CS usually presents with inflammatory ocular manifestations in association with audio-vestibular symptoms but mostly different Ménière-like symptoms and, more frequently, with systemic inflammation (70%), of which vasculitis is the pathogenic mechanism. CS is considered as an autoimmune- or immune-mediated disease supported mainly by the beneficial response to corticosteroids. Using well-developed assays, antibodies to inner ear antigens, anti-Hsp70, and antineutrophil cytoplasmic antibodies were found to be associated with CS. Corticosteroids represent the first line of treatment, and multiple immunosuppressive drugs have been tried with variable degrees of success. Tumor necrosis factor-alpha blockers and other biological agents are a recent novel therapeutic option in CS. Cochlear implantation is a valuable rescue surgical strategy in cases with severe sensorineural hearing loss unresponsive to intensive and/or innovative immunosuppressive regimens.

Audiovestibular Symptoms in Systemic Autoimmune Diseases

“…CS develops in young adults, mostly during their first three decades of life [40, 41]. The origin of CS is still unclear.…”

“…Cogan's syndrome (CS), first described in 1934, is a rare autoimmune disorder characterized by ocular and audiovestibular symptoms [ 39 ]. CS develops in young adults, mostly during their first three decades of life [ 40 , 41 ]. The origin of CS is still unclear.…”

“…Inner ear involvement in CS has been reported with a prevalence of between 31% and 45% [ 40 – 44 ]. The most common audiovestibular manifestations in CS are hearing loss, vertigo, tinnitus, ataxia, and oscillopsia [ 41 ].…”

Assistance Dogs for Persons with Hearing Impairment: A Review

“…aging [24][25][26] , noise exposure 27 , the use of ototoxic drugs (28), autoimmune diseases [29,30] and other conditions [31][32][33][34][35] . According to WHO data, around 466 million people worldwide have disabling hearing loss, and 34 millions of these are children 36 .…”

Hyperacusis in Children: A Preliminary Study on the Effects of Hypersensitivity to Sound on Speech and Language

“…Available evidence suggests that hyperacusis may follow functional changes within the central nervous system and may be related to increased gain in the central auditory pathways and to increased anxiety or emotional response to sound 5 . Hyperacusis has been reported in some systemic conditions and syndromes [6][7][8][9][10][11][12][13][14][15][16][17][18] , although most cases of hyperacusis have unknown cause 19,20 .…”