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Living with Otodental syndrome

A very rare inherited condition with very large canine and molar teeth and hearing loss.

Understanding Otodental syndrome

Otodental syndrome is a very rare inherited condition with grossly enlarged canine and molar teeth (globodontia) and hearing loss from the inner ear.

Teeth can have enamel and shape differences, and some may be missing or come in late. Other features include enlarged gums and full cheeks. Hearing loss, especially of high-pitched sounds, tends to get worse over time. GARD lists childhood as the usual onset.

Signs & symptoms

Symptoms vary from person to person. GARD lists these among the most common:

  • Very large canine and molar teeth
  • Enamel differences, cavities, and gum disease
  • Late-erupting or missing teeth
  • Overgrown gums
  • Hearing loss from the inner ear, especially high pitches
  • Full cheeks and a long face

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include genetic specialists and gum, teeth and mouth specialists.

  3. Ask which tests can help

    Your care team will decide which exams and tests can confirm the diagnosis.

Care & treatment

Care team
Genetic specialists (geneticists); Gum, teeth and mouth specialists (odontologists)
Expert care
Ask about multidisciplinary care centers for rare conditions
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with Chromosome Disorder Outreach
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
Which dental care plan fits my tooth differences?
How often should my hearing be tested?
Would genetic testing help my relatives?

Quick facts

Symptoms usually startChild
InheritanceAutosomal Dominant

GARD says changes in genes cause this condition. It is inherited in an autosomal dominant way, meaning one changed copy of the gene can be enough.

Genetic

Also known as: chromosome 11q13 deletion syndrome; globodontia; otodental dysplasia; otodental dysplasia chromosome deletion syndrome

Patient organizations

Broader rare-disease groups

Source: NIH GARD · Otodental syndromeGARD ID 4168 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Otodental syndrome.

Explore the Otodental syndrome blog

Explore research for Otodental syndrome

Explore actively recruiting Otodental syndrome studies with HealthSpark.

HealthSpark

Find actively recruiting studies for Otodental syndrome with HealthSpark. Share your city to explore research near you.

  • Recruiting studies from ClinicalTrials.gov
  • Share your city to look for research near you
  • Eligibility is confirmed by the study team

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Powered by HealthSpark, our AI chat helper · Study information from ClinicalTrials.gov · Eligibility is confirmed by the study team.

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