Clinical significance of endolymphatic hydrops on MRI in Cogan's syndrome: a case series of five patients

Abstract

Autoimmune inner ear disorder (AIED) diagnosis is based on the onset of bilateral sensorineural hearing loss (SNHL) that progresses over 3 to 90 days and responds to steroid treatment. Among autoimmune conditions, Cogan's syndrome (CS) is a rare disorder characterized by the onset of inflammation in both eyes and in the inner ear, leading to progressive sensorineural hearing loss, particularly at high frequencies, often with vestibular symptoms. If left untreated, CS can cause profound bilateral hearing loss. The disease is believed to have an autoimmune etiology, although a common biomarker has not been clearly identified. Early-stage inner ear involvement in CS can mimic Menière's Disease (MD), although key differences between phenotypes, such as the pattern and speed of progression, characterized by a pousseè evolution of bilateral high frequencies hearing loss rather than low frequencies fluctuating, help distinguish the two conditions. Few is known about imaging of the inner ear in patients with CS, and most of the insight into the radiological manifestation in the inner ear of this rare pathology came from the '90s. Recent advances in imaging, particularly with 3D-FLAIR MRI sequences after gadolinium administration, have shown promise in detecting endolymphatic hydrops (EH) in MD patients. Our study reports findings in five CS patients on whom we performed a 3 Tesla MRI, revealing EH in three of them. This is the first report of such a correlation in vivo, contributing valuable new insights into the radiological manifestations of CS.

Keywords: Cogan syndrome; Endolymphatic Hydrops; Inner ear MRI; Inner ear autoimmune disease; Late 3D FLAIR; MRI; Menière’s disease.