Autoimmune aberration in sudden sensorineural hearing loss: association with anti-cardiolipin antibodies

Abstract

In view of the presence of autoantibodies against inner ear antigens, the pathogenesis of sudden deafness (SD) and progressive sensorineural hearing loss (PSNHL) is suggested to be of an autoimmune nature. However, microthrombosis of the inner ear may result from pathogenic anti-cardiolipin antibody (aCL) activity. We studied 30 patients (17 females and 13 males, age range 20-52 y), of whom 11 suffered from SD and 19 from PSNHL. All were clinically and serologically evaluated for association with autoimmune disorders (serological examination included: aCL, ANA, ENA, ANCA, proteinelectrophoresis, and complement levels). Twenty healthy matched subjects served as controls. None of the control group were aCL positive, whereas 8 out of 30 (27%) patients demonstrated low-moderate titers (P < 0.02), of whom 5 out of 8 suffered from SD. In addition, 2 aCL negative patients with PSNHL demonstrated hypergammaglubolinemia accompanied by hypocomplementemia, whereas none with SD had such abnormalities. Our data suggests that aCL is detected in patients with sudden sensorineural hearing loss and therefore may play an important role in the pathogenesis of this disability. If sustained by additional studies, these findings would warrant the consideration of anticoagulant therapy.

Surdez súbita causada por hemorragia intralabiríntica

“…[2][3] It is typically unilateral, 4 and may be partial or complete, and sudden or rapidly progressive. 1 Causes of SSHL may be infectious (viral or bacterial), vascular, immunomediated (within the inner ear or systemically), or resulting from neurological disease (migraine, multiple sclerosis), neoplasms or ototoxicity.…”

The autoimmune characteristics of progressive or sudden sensorineural hearing loss

“…In 1997 we reported our results on the presence of aCL in the sera of patients with SNHL [43]. Low to moderate positive aCL to one or both IgG/IgM isotypes were found in 8/30 (27%) patients, whereas none were detected in the healthy controls.…”

Unusual Clinical Manifestations of the Antiphospholipid Syndrome

“…Less frequently described manifestations of APS include chorea [4,[64][65][66], transverse myelopathy [5,67,68], Guillain-Barre syndrome [6], sensorineural hearing loss [69,70] and ocular syndromes with retinal and choroidal ischaemia [71][72][73][74].…”

“…A large series of 30 patients [69] showed that 27% of patients with sensorineural hearing loss had low-to-moderate titers of aCL, as opposed to none from the control group. Naarendorp et al [70] described 6 patients who developed sudden sensorineural hearing loss and had elevated serum levels of aCL or LA.…”