otodental syndrome
MONDO:0008161Mondo
Findings
No curated finding names otodental syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Otodental syndrome is a very rare inherited condition characterized by grossly enlarged canine and molar teeth (globodontia) associated with sensorineural hearing loss.
Definition from the Mondo Disease Ontology (MONDO:0008161), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
HPO, annotations 2026-09-02
Features
28 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 canine tooth morphologyHPOHP:0011078
- Very frequent (80% to 99% of cases)
- Abnormal dental pulp morphologyHPOHP:0006479
- Very frequent (80% to 99% of cases)
- Abnormal molar morphologyHPOHP:0011070
- Very frequent (80% to 99% of cases)
- Carious teethHPOHP:0000670
- Very frequent (80% to 99% of cases)
- Progressive sensorineural hearing impairmentHPOHP:0000408
- Very frequent (80% to 99% of cases)
- Abnormal dental enamel morphologyHPOHP:0000682
- Frequent (30% to 79% of cases)
Show the remaining 16
- High-frequency sensorineural hearing impairmentHPOHP:0001757
- Frequent (30% to 79% of cases)
- Long faceHPOHP:0000276
- Frequent (30% to 79% of cases)
- Long philtrumHPOHP:0000343
- Frequent (30% to 79% of cases)
- PeriodontitisHPOHP:0000704
- Frequent (30% to 79% of cases)
- Pulp calcificationHPOHP:0003771
- Frequent (30% to 79% of cases)
- TaurodontiaHPOHP:0000679
- Frequent (30% to 79% of cases)
Where it sits
Other names
3 names
Resolves to: otodental syndrome
- Also called
- globodontiaotodental dysplasiaotodental dysplasia chromosome deletion syndrome