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otodental syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

otodental syndrome

MONDO:0008161

Also known as:globodontiaotodental dysplasiaotodental dysplasia chromosome deletion syndrome

Overview

Otodental syndrome is a very rare inherited condition characterized by grossly enlarged canine and molar teeth (globodontia) associated with sensorineural hearing loss.

GeneticsAutosomal dominant

ResearchLimited published research (3 articles)

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Progressive sensorineural hearing impairment, Carious teeth, Abnormal dental pulp morphology, and Abnormal molar morphology and others; and common findings: Long philtrum, Delayed eruption of teeth, Agenesis of premolar, and Long face and others. 30 total HPO annotations.

Organ System	Phenotype Count	Example Features
Ears	4	Inner ear hearing loss (sensorineural hearing impairment), Progressive sensorineural hearing impairment, High-frequency sensorineural hearing impairment

Prognosis

Phenotype severity distribution: 5 very common features, 13 common features.

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for otodental syndrome.

3 publications have been identified in PubMed for otodental syndrome. Research spans Case Report / Case Series (100%).

Recent Publications

Rahi H (2025). [PMID: 39976283](https://pubmed.ncbi.nlm.nih.gov/39976283/). *American journal of medical genetics. Part A*. [Case Report / Case Series]
Zangoei Booshehri M (2025). [PMID: 39748371](https://pubmed.ncbi.nlm.nih.gov/39748371/). *BMC oral health*. [Case Report / Case Series]
Souza DAS (2024). [PMID: 38905602](https://pubmed.ncbi.nlm.nih.gov/38905602/). *General dentistry*. [Case Report / Case Series]

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 1:06 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:166750

Orphanet

European rare disease database

Orphanet:2791

GARD

Genetic and Rare Diseases Info Center

GARD:4168

Questions for Your Care Team

→Are there specialists or centers of excellence for this condition in my area?
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otodental syndrome

Overview

Clinical Presentation

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

Orphanet

GARD

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Questions for Your Care Team

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