spastic paraparesis-deafness syndrome
MONDO:0010732Mondo
Findings
No curated finding names spastic paraparesis-deafness syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Spastic paraparesis-deafness syndrome is a chronic neurodegenerative disorder characterized by spastic paraparesis (beginning at about 10 years of age) and hearing deficits.
Definition from the Mondo Disease Ontology (MONDO:0010732), read 2026-09-29. CC BY 4.0.
Features
13 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.
- Gait disturbanceHPOHP:0001288
- Very frequent (80% to 99% of cases)
- Hemiplegia/hemiparesisHPOHP:0004374
- Very frequent (80% to 99% of cases)
- HyperreflexiaHPOHP:0001347
- Very frequent (80% to 99% of cases)
- Impaired pain sensationHPOHP:0007328
- Very frequent (80% to 99% of cases)
- Sensorineural hearing impairmentHPOHP:0000407
- Very frequent (80% to 99% of cases)
- Spastic paraparesisHPOHP:0002313
- Very frequent (80% to 99% of cases)
- Abnormality of movementHPOHP:0100022
- Frequent (30% to 79% of cases)
- CataractHPOHP:0000518
- Frequent (30% to 79% of cases)
- HypogonadismHPOHP:0000135
- Frequent (30% to 79% of cases)
- Short statureHPOHP:0004322
- Frequent (30% to 79% of cases)
- Visual impairmentHPOHP:0000505
- Frequent (30% to 79% of cases)
- AtaxiaHPOHP:0001251
- Occasional (5% to 29% of cases)
Show the remaining 1
- NystagmusHPOHP:0000639
- Occasional (5% to 29% of cases)
Where it sits
- A kind of
Other names
1 name
Resolves to: spastic paraparesis-deafness syndrome
- Also called
- Wells-Jankovic syndrome