deafness-oligodontia syndrome
MONDO:0009089Mondo
Findings
No curated finding names deafness-oligodontia syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Deafness-oligodontia syndrome is characterized by sensorineural hearing loss and oligodontia/hypodontia. It has been described in two pairs of siblings and in one isolated case. Dizziness was reported in one of the pairs of siblings. Transmission appears to be autosomal recessive.
Definition from the Mondo Disease Ontology (MONDO:0009089), read 2026-09-29. CC BY 4.0.
Features
4 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.
- OligodontiaHPOHP:0000677
- Very frequent (80% to 99% of cases)
- Sensorineural hearing impairmentHPOHP:0000407
- Very frequent (80% to 99% of cases)
- VertigoHPOHP:0002321
- Frequent (30% to 79% of cases)
- Abnormality of the inner earHPOHP:0000359
- Occasional (5% to 29% of cases)