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Arnold-Chiari malformation type I is a central nervous system malformation characterized by caudal displacement of the cerebellar tonsils exceeding 5mm below the foramen magnum with or without syringomyelia. Symptoms vary in onset and severity and include suboccipital headache, neck pain, vertigo, tinnitus, ocular symptoms (diplopia, blurred vision, photofobia, nystagmus), lower cranial nerve signs, cerebellar ataxia, and spasticity. Some affected individuals can be asymptomatic.
Features include: Hearing loss (hearing impairment), Vertigo, Syringomyelia, and Gait ataxia and 21 more.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Brain and nerves | 9 | Gait ataxia, Lower limb spasticity, Unsteady gait |
Arms and legs |
Biomarker and diagnostic research for Chiari malformation type I has been reported in the published literature.
Estimated prevalence: Unknown (Unknown prevalence).
No clinical trials have been registered for Chiari malformation type I.
178 publications have been identified in PubMed for Chiari malformation type I. Research spans Case Report / Case Series (30%), Clinical Trial Publication (24%), and Review / Meta-Analysis (16%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 54 | 30% |
Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 12:49 AM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about Chiari malformation type I
4
Lower limb spasticity, Limb muscle weakness, Lower limb hyperreflexia |
Ears | 3 | Hearing loss (hearing impairment), Vertigo, Tinnitus |
Eyes | 2 | Nystagmus, Diplopia |
Kidneys and urinary system | 1 | Urinary incontinence |
Muscles | 1 | Limb muscle weakness |
Digestive system | 1 | Difficulty swallowing (dysphagia) |
Bones and joints | 1 | Sideways curvature of the spine (scoliosis) |
42 |
24% |
Research summaries | 29 | 16% |
Disease patterns and progression | 16 | 9% |
Testing and diagnosis research | 14 | 8% |
Laboratory research | 13 | 7% |
Other research | 9 | 5% |
New treatment approaches | 1 | 1% |
Dang H (2026). [PMID: 42116881](https://pubmed.ncbi.nlm.nih.gov/42116881/). *Surg Neurol Int*. [Clinical Trial Publication]
Davidson L (2026). [PMID: 41489686](https://pubmed.ncbi.nlm.nih.gov/41489686/). *Childs Nerv Syst*. [Clinical Trial Publication]
Dinc S (2026). [PMID: 41808980](https://pubmed.ncbi.nlm.nih.gov/41808980/). *J Neurol Surg B Skull Base*. [Clinical Trial Publication]
Woiciechowsky C (2026). [PMID: 41781756](https://pubmed.ncbi.nlm.nih.gov/41781756/). *Childs Nerv Syst*. [Basic Science / Preclinical]
Møen EN (2026). [PMID: 41541404](https://pubmed.ncbi.nlm.nih.gov/41541404/). *Brain & spine*. [Clinical Trial Publication]
Zhao J (2026). [PMID: 41950723](https://pubmed.ncbi.nlm.nih.gov/41950723/). *Clin Neurol Neurosurg*. [Epidemiology / Natural History]
Sharif MS (2026). [PMID: 42125261](https://pubmed.ncbi.nlm.nih.gov/42125261/). *Radiol Case Rep*. [Case Report / Case Series]
Habibi P (2026). [PMID: 41654330](https://pubmed.ncbi.nlm.nih.gov/41654330/). *AJNR Am J Neuroradiol*. [Clinical Trial Publication]
Fedorova J (2026). [PMID: 40432397](https://pubmed.ncbi.nlm.nih.gov/40432397/). *Cleft Palate Craniofac J*. [Case Report / Case Series]
Adolfsson T (2026). [PMID: 41276079](https://pubmed.ncbi.nlm.nih.gov/41276079/). *World Neurosurg*. [Basic Science / Preclinical]