Otodental Syndrome Overview
Author Information
Author(s): Agnès Bloch-Zupan, Jane R Goodman
Primary Institution: Faculté de Chirurgie Dentaire de Strasbourg, Université Louis Pasteur
Hypothesis
The otodental syndrome is inherited in an autosomal dominant manner and is characterized by specific dental and auditory anomalies.
Conclusion
Otodental syndrome is a rare condition that leads to distinctive dental features and associated hearing loss.
Supporting Evidence
- The syndrome is characterized by globodontia, which affects both primary and permanent teeth.
- Sensorineural high frequency hearing loss is commonly associated with the syndrome.
- Eye abnormalities such as coloboma are also reported in some cases.
Takeaway
Otodental syndrome is a rare disease that affects teeth and hearing, making teeth look funny and causing trouble hearing.
Limitations
The study is based on a limited number of families and may not represent the full spectrum of the syndrome.
Participant Demographics
Described in at least 9 families from various countries including Hungary, the UK, Brazil, China, Poland, Austria, Italy, and Belgium.
Digital Object Identifier (DOI)
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