Hypertrophic pachymeningitis and audiovestibular involvement: A review of the literature

Abstract Hypertrophic pachymeningitis (HP) primarily arises from inflammatory and infectious conditions, including immunoglobulin G4-related disease, Sjogren’s syndrome, rheumatoid arthritis, syphilis, and tuberculosis. Key symptoms include headaches and paralysis of the cranial nerves, affecting also the cochlear-vestibular nerve, which can result in hearing loss and vertigo. Treatment typically involves corticosteroids and immunosuppressants for inflammatory cases, while therapeutic approaches for infections depend on the underlying cause. This review aimed to investigate the type and prevalence of hearing loss and vestibular disorders in this rare condition. Twenty-seven case reports were analyzed for a total of thirty patients. Hearing loss was sensorineural in 67% of cases and mixed in 13%. In 57% of cases, it was progressive, and in 13% sudden. Forty-three percent of cases presented tinnitus or vertigo. The therapy was effective in 67% of cases. This review showed that hearing loss is a common presenting symptom in HP, occurring bilaterally and unilaterally; in these cases, the risk of progression in the other ear was present. In the idiopathic form, sensorineural impairment is most frequently observed, while other causes, such as sarcoidosis or Wegener, may present mixed forms. A strict follow-up is necessary to exclude early progression of the hearing loss.