Immune-mediated pathogenesis has been suggested for idiopathic
sensorineural hearing loss. Recent studies have investigated the relationship between idiopathic
sensorineural hearing loss and
autoantibodies against inner ear
antigens. We conducted a prospective, observational study in a series of pediatric patients affected by idiopathic
sensorineural hearing loss.
Autoantibodies against inner ear (anti-Cogan
peptide, anti-
connexin 26, anti-DEP1/CD148 and anti-reovirus), previously described in the serum of patients with
Cogan's syndrome, were detected in our population. The characteristics of children whose results were positive were also evaluated to verify if clinical data,
disease progression and response to treatment could confirm an immune-mediated pathogenesis. Eleven patients were enrolled and 9 of them were positive for inner ear
antibodies. Non-organ specific
autoantibodies were present in 5 children out of 9. An immune-mediated condition was diagnosed in 2 cases and minor immune manifestations were found in 2 additional patients. In 5 cases
hearing loss remained stable without
therapy, while 4 children developed progression. Two subjects were treated with
corticosteroids and
methotrexate, achieving hearing improvement. Another subject showed stabilization on
methotrexate. Inner ear
autoantibodies can be positive in children with autoimmune
sensorineural hearing loss, and in conjunction with clinical data may assist the clinician in identifying a subset amenable for immune modulation
therapy. Large prospective studies are needed to investigate usefulness, diagnostic and prognostic role of these
autoantibodies.