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Living with Dentinogenesis imperfecta type 2

A rare, severe form of dentinogenesis imperfecta in which teeth are weak and discolored.

Understanding Dentinogenesis imperfecta type 2

Dentinogenesis imperfecta type 2 is a rare and severe form of dentinogenesis imperfecta, a condition that affects tooth development.

People may have weak, discolored teeth. Both baby and permanent teeth are affected, and people with this type have no normal teeth. Hearing loss from inner ear changes has been found in some people. GARD lists onset in childhood.

Signs & symptoms

GARD doesn't list a symptom list. Based on its summary, features include:

  • Weak teeth
  • Discolored teeth
  • Hearing loss from inner ear changes (in some people)

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. Consider genetic testing

    Changes in DSPP are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.

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 rare disease patient organizations for support
  • 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
How can we protect and restore these teeth?
Should hearing be tested?
Can genetic testing of the DSPP gene help our family?

Quick facts

Symptoms usually startChild
InheritanceAutosomal Dominant
GenesDSPP

Changes in the DSPP gene cause this condition. It is autosomal dominant, so a changed copy of the gene from one parent can be enough.

Genetic

Also known as: capdepont teeth; dentinogenesis imperfecta - shield's type ii; dentinogenesis imperfecta 1; dentinogenesis imperfecta type 1; dentinogenesis imperfecta, shields type 2; dentinogenesis imperfecta, shields type ii

Patient organizations

Rare-disease groups that can help

Source: NIH GARD · Dentinogenesis imperfecta type 2GARD ID 12796 · 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 Dentinogenesis imperfecta type 2.

Explore the Dentinogenesis imperfecta type 2 blog

Explore research for Dentinogenesis imperfecta type 2

Explore actively recruiting Dentinogenesis imperfecta type 2 studies with HealthSpark.

HealthSpark

Find actively recruiting studies for Dentinogenesis imperfecta type 2 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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