Abstract
Autoimmune and autoinflammatory inner ear diseases (AIED/AID) are characterized by the symptom of sensorineural hearing loss (SNHL). To date, standardized diagnostic tools for AIED/AID are lacking, and clinically differentiating AIED/AID from chronic otitis media (COM) with SNHL is challenging. This retrospective study aimed to construct a magnetic resonance imaging (MRI)-based decision tree using classification and regression tree (CART) analysis to distinguish AIED/AID from COM. In total, 67 patients were enrolled between January 2004 and October 2019, comprising AIED/AID (n = 18), COM (n = 24), and control groups (n = 25). All patients underwent 3 T temporal bone MRI, including post-contrast T1-weighted images (postT1WI) and post-contrast FLAIR images (postFLAIR). Two radiologists evaluated the presence of otomastoid effusion and inner ear contrast-enhancement on MRI. A CART decision tree model was constructed using MRI features to differentiate AIED/AID from COM and control groups, and diagnostic performance was analyzed. High-intensity bilateral effusion (61.1%) and inner ear enhancement (postFLAIR, 93.8%; postT1WI, 61.1%) were the most common findings in the AIED/AID group. We constructed two CART decision tree models; the first used effusion amount as the first partitioning node and postT1WI-inner ear enhancement as the second node, whereas the second comprised two partitioning nodes with the degree of postFLAIR-enhancement of the inner ear. The first and second models enabled distinction of AIED/AID from COM with high specificity (100% and 94.3%, respectively). The amount of effusion and the degree of inner ear enhancement on MRI may facilitate the distinction between AIED/AID and COM with SNHL using decision tree models, thereby contributing to early diagnosis and intervention.
Highlights
Autoimmune and autoinflammatory inner ear diseases (AIED/Autoinflammatory inner ear disease (AID)) are characterized by the symptom of sensorineural hearing loss (SNHL)
We aimed to evaluate inner ear findings using high-resolution 3 T magnetic resonance imaging (MRI) in patients clinically diagnosed with Autoimmune inner ear disease (AIED)/AID and to harness MRI findings to accurately differentiate between AIED/AID and chronic otitis media (COM) by adopting classification and regression tree (CART) analysis
Of the four patients clinically diagnosed with CINCA syndrome/DFNA34, three were confirmed to have an NLRP3 variant based on genetic testing (Table S2)
Summary
Autoimmune and autoinflammatory inner ear diseases (AIED/AID) are characterized by the symptom of sensorineural hearing loss (SNHL). To date, standardized diagnostic tools for AIED/AID are lacking, and clinically differentiating AIED/AID from chronic otitis media (COM) with SNHL is challenging This retrospective study aimed to construct a magnetic resonance imaging (MRI)-based decision tree using classification and regression tree (CART) analysis to distinguish AIED/AID from COM. Immune-mediated inner ear disease manifesting as cochleovestibular dysfunction is diagnosed by exclusion, depending upon clinical symptoms, laboratory tests, and/or steroid responsiveness In this regard, distinguishing AIED/AID from chronic otitis media (COM) can be challenging due to considerable overlap in clinical features, especially when COM presents with infectious labyrinthitis and SNHL. We aimed to evaluate inner ear findings using high-resolution 3 T MRI in patients clinically diagnosed with AIED/AID and to harness MRI findings to accurately differentiate between AIED/AID and COM by adopting classification and regression tree (CART) analysis. The purpose of our study was to construct a decision tree based on MRI features in order to distinguish AIED/AID from COM with SNHL
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