Pediatric Autoimmune Inner Ear Disease: A Rare, But Treatable Condition

Abstract

Autoimmune inner ear disease (AIED) is a rare, but treatable cause of sudden sensorineural hearing loss in children. Most cases present acutely and involve both ears. The precise mechanism of hearing loss in AIED is not known. Many suspected etiologies have been proposed including infections, vascular abnormalities, and trauma. However, 70% of cases are defined as idiopathic. There are no standardized diagnostic criteria for AIED, and the diagnostic process may be challenging. Positive auto antibodies and response to immunosuppressive therapy support the diagnosis. Treatment may include corticosteroids and steroid-sparing immunosuppressive medications. A high index of suspicion is recommended as the hearing loss may be stabilized or even reversed with early treatment. Long-term medical treatment failures generally have good outcomes with cochlear implantation. [ Pediatr Ann. 2019;48(10):e391–e394.]

Hearing restoration and the stria vascularis: evidence for the role of the immune system in hearing restoration

“…Given the evidence for cytokine signalling within the adult mouse stria vascularis and in autoinflammatory and autoimmune disorders, we will now discuss therapeutic targeting of these signalling pathways. Given that treatment with steroids represents the first-line treatment of these disorders [34,42,64,65 ▪ ,66–73,74 ▪ ,75,76 ▪ ,77,78 ▪ ], understanding cytokine signalling in patients with steroid-responsive and steroid-unresponsive disease offers an opportunity to identify critical entry points for therapeutic immunomodulation. Corticosteroids repress IL-6 expression via inhibiting the interaction between NF-IL6 and the p65 subunit of NF- κ B [79].…”

Primäre Immundefekte und Autoimmunität

“…Die Erkrankung ist durch eine homozygote oder compound heterozygote Mutationen verursacht. Auch hier können Arthritiden auftreten, welche schon im Kindesalter beschrieben sind [36]. [40].…”