superficial siderosis
Findings
No curated finding names superficial siderosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Superficial siderosis is a rare neurologic disease characterized by progressive sensorineural hearing loss, cerebellar ataxia, pyramidal signs, and neuroimaging findings revealing hemosiderin deposits in the spinal and cranial leptomeninges and subpial layer. The disease progresses slowly and patients may present with mild cognitive impairment, nystagmus, dysmetria, spasticity, dysdiadochokinesia, dysarthria, hyperreflexia, and Babinski signs. Additional features reported include dementia, urinary incontinence, anosmia, ageusia, and anisocoria.
Definition from the Mondo Disease Ontology (MONDO:0016594), read 2026-09-29. CC BY 4.0.
Features
49 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal vestibulocochlear nerve morphologyHPOHP:0009591
- Very frequent (80% to 99% of cases)
- AtaxiaHPOHP:0001251
- Very frequent (80% to 99% of cases)
- Bilateral sensorineural hearing impairmentHPOHP:0008619
- Very frequent (80% to 99% of cases)
- Abnormal bleedingHPOHP:0001892
- Frequent (30% to 79% of cases)
- Abnormal corpus callosum morphologyHPOHP:0001273
- Frequent (30% to 79% of cases)
- Cerebellar atrophyHPOHP:0001272
- Frequent (30% to 79% of cases)
Show the remaining 37
- Abnormal pyramidal signHPOHP:0007256
- Occasional (5% to 29% of cases)
- Abnormal spinal cord morphologyHPOHP:0002143
- Occasional (5% to 29% of cases)
- AnisocoriaHPOHP:0009916
- Occasional (5% to 29% of cases)
- AnosmiaHPOHP:0000458
- Occasional (5% to 29% of cases)
- Arteriovenous malformationHPOHP:0100026
- Occasional (5% to 29% of cases)
- Atrophy of the spinal cordHPOHP:0006827
- Occasional (5% to 29% of cases)
Where it sits
- A kind of
Other names
5 names
Resolves to: superficial siderosis
- Also called
- hemosiderosis of the central nervous systemsuperficial hemosiderosis of the central nervous systemsuperficial hemosiderosis of the CNSsuperficial siderosis of the central nervous systemsuperficial siderosis of the CNS