Hearing Loss and Chiari Malformation Type I: A Scoping Review

Background/objectives: Chiari malformation (CM) type I is an uncommon condition that can be associated with a variety of neurological and otoneurological symptoms, including sensorineural hearing loss. The aim of this paper is to analyze the association between type I CM and hearing loss. Methods: A review of the literature was performed using PubMed/MEDLINE, EMBASE, and Cochrane Library databases, according to PRISMA criteria for scoping review (from 2000 to April 2025). Results: A total of 8 articles and 139 patients with type I CM have been included; the majority of studies focused on women, with a mean age of 38.5 years (range: 10-44 years). In two cases, surgery was necessary for restoring normal hearing thresholds. Conclusions: To date, the pathophysiological mechanisms related to type I CM and hearing loss are not fully understood yet; further studies are necessary to clarify these features and to evaluate the correct management of these patients.