Abstract
Objective: Hypertrophic pachymeningitis (HP) is a rare disorder that causes localized or diffuse
inflammatory fibrosis and thickening of the dura mater. Antineutrophil cytoplasmic
antibody (ANCA)-associated vasculitis (AAV)-related HP is the most frequent form of
HP. Otitis media with AAV (OMAAV) patients with HP are associated with higher rates
of both ANCA-negative phenotypes and disease-related mortality. However, few studies
have reported the imaging characteristics of HP due to AAV/OMAAV. Therefore, we investigated
this issue in the present study.
Methods: This retrospective study included patients diagnosed with HP between 2011 and 2020
at our hospital. Age, sex, causative disease, serum C-reactive protein (CRP) level,
and MRI data were collected from medical records. We compared the locations of MRI
enhancement depending on the causative diseases.
Results: Of the 18 included patients with HP (mean age, 64.1 ± 2.6 years; range, 33–77 years),
10 (55.6%) were female, 12 (66.7%) were diagnosed with AAV/OMAAV, four (22.2%) were
diagnosed as idiopathic, two (11.1%) were diagnosed with invasive Aspergillus mastoiditis. Eleven (61.1%) had cranial neuropathies. Facial nerve paralysis was
common in AAV/OMAAV, while abducent nerve paralysis was common in idiopathic HP. Cranial
fossa enhancement was most common presentation in patients with HP, whereas inner
acoustic canal (IAC) enhancement was seen only in patients with AAV/OMAAV, while HP
involving the cavernous sinus was seen only in patients with idiopathic and mastoiditis.
Conclusion: HP involving the IAC may be a key factor in diagnosing AAV/OMAAV.
Keywords
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Article info
Publication history
Published online: January 21, 2022
Accepted:
January 12,
2022
Received:
November 15,
2021
Identification
Copyright
© 2022 Japanese Society of Otorhinolaryngology-Head and Neck Surgery, Inc. Published by Elsevier B.V. All rights reserved.
