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Hearing Loss and Chiari Malformation Type I: A Scoping Review
Andrea Migliorelli1, Marianna Manuelli1, Chiara Bianchini1
1ENT & Audiology Unit, Department of Neurosciences, University Hospital of Ferrara, 44124 Ferrara, Italy.
Diseases (Basel, Switzerland)
|October 28, 2025
Summary
Chiari malformation (CM) type I is linked to hearing loss. Further research is needed to understand the connection and guide patient management for this rare condition.
Area of Science:
- Neurology
- Otolaryngology
- Genetics
Background:
- Chiari malformation (CM) type I is a rare neurological condition.
- It can manifest with diverse neurological and otoneurological symptoms.
- Sensorineural hearing loss is a recognized, though uncommon, associated symptom.
Purpose of the Study:
- To investigate the association between Chiari malformation type I and hearing loss.
- To synthesize current literature on this specific CM type and auditory dysfunction.
Main Methods:
- A comprehensive literature review was conducted.
- Databases searched include PubMed/MEDLINE, EMBASE, and Cochrane Library.
- PRISMA guidelines for scoping reviews were followed, covering literature from 2000 to April 2025.
Main Results:
- Eight articles comprising 139 patients with CM type I were analyzed.
- The patient cohort predominantly consisted of women, with a mean age of 38.5 years.
- Surgical intervention was required in two cases to restore normal hearing thresholds.
Conclusions:
- The precise pathophysiological mechanisms linking CM type I and hearing loss remain unclear.
- Additional research is essential to elucidate these mechanisms.
- Further studies are needed to establish optimal patient management strategies.
