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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.
Features include very common findings: Inner ear hearing loss (sensorineural hearing impairment); and common findings: Vertigo. 6 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Ears | 3 | Congenital sensorineural hearing impairment, Inner ear hearing loss (sensorineural hearing impairment), Vertigo |
Phenotype severity distribution: 1 very common feature, 1 common feature.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
No clinical trials have been registered for deafness-oligodontia syndrome.
3 publications have been identified in PubMed for deafness-oligodontia syndrome. Research spans Review / Meta-Analysis (33%), Case Report / Case Series (33%), and Epidemiology / Natural History (33%).
Ahmadkhani A (2026). [PMID: 41486137](https://pubmed.ncbi.nlm.nih.gov/41486137/). *J Med Case Rep*. [Review / Meta-Analysis]
Ghosh U (2025). [PMID: 41035246](https://pubmed.ncbi.nlm.nih.gov/41035246/). *Clin Exp Pediatr*. [Case Report / Case Series]
Naqvi SF (2024). [PMID: 39554678](https://pubmed.ncbi.nlm.nih.gov/39554678/). *Pak J Med Sci*. [Epidemiology / Natural History]
Data assembled from 5 of 12 sources · Last updated Sep 20, 2026, 11:13 AM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
Pregnancy and birth
1 |
Congenital sensorineural hearing impairment |
AI-curated news mentioning deafness-oligodontia syndrome
Updated May 6, 2026
A new study explores the molecular basis and clinical spectrum of WNT10A-related oligodontia, shedding light on the genetic factors involved. This research enhances understanding of the condition, which affects tooth development.