Otodental Dysplasia

Also Known As:

Chromosome 11q13 Deletion Syndrome; Oculootodental Syndrome; Otodental Syndrome; Otodental Syndrome With Coloboma

Networked: 5 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Signs and Symptoms Pathological Conditions
- Pathologic Processes: 10023
  - Chromosome Aberrations: 11361
    - Aneuploidy: 8932
      - Monosomy: 435
        Chromosome Deletion: 104
        Otodental Dysplasia: 5
- Signs and Symptoms
  - Neurologic Manifestations: 7102
    - Sensation Disorders: 117
      - Hearing Disorders: 325
        Hearing Loss: 12265
        Sensorineural Hearing Loss: 3974
        Otodental Dysplasia: 5
Genetic Phenomena
Cells
- Cellular Structures
  - Chromosomes
    - Mammalian Chromosomes
      - Human Chromosomes
        6-12 and X Human Chromosomes
        Pair 11 Human Chromosomes
        Otodental Dysplasia: 5
Eye Diseases: 3522
- Eye Abnormalities: 95
  - Coloboma: 478
    - Otodental Dysplasia: 5
- Hereditary Eye Diseases: 18
  - Coloboma: 478
    - Otodental Dysplasia: 5
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Congenital Abnormalities: 25723
- Inborn Genetic Diseases: 11939
  - Chromosome Disorders: 136
    - Otodental Dysplasia: 5
Otorhinolaryngologic Diseases: 203
- Ear Diseases: 261
  - Hearing Disorders: 325
    - Hearing Loss: 12265
      - Sensorineural Hearing Loss: 3974
        Otodental Dysplasia: 5
Nervous System Diseases: 14178
- Neurologic Manifestations: 7102
  - Sensation Disorders: 117
    - Hearing Disorders: 325
      - Hearing Loss: 12265
        Sensorineural Hearing Loss: 3974
        Otodental Dysplasia: 5
Stomatognathic Diseases: 705
- Tooth Diseases: 33
  - Tooth Abnormalities: 33
    - Otodental Dysplasia: 5
- Stomatognathic System Abnormalities
  - Tooth Abnormalities: 33
    - Otodental Dysplasia: 5

Related Diseases

1.	Sensorineural Hearing Loss
2.	Facies
3.	Rickets (Rachitis)
4.	Hypophosphatasia
5.	Ventricular Heart Septal Defects (Ventricular Septal Defect)

Experts

1.	Giani, G: 1 article (03/2022)
2.	Paglia, L: 1 article (03/2022)
3.	Paglia, M: 1 article (03/2022)
4.	Pisoni, L: 1 article (03/2022)
5.	Enright, Sinead: 1 article (12/2015)
6.	Humphrys, A Karen: 1 article (12/2015)
7.	James, Jacqueline A: 1 article (12/2015)
8.	Rea, Gillian: 1 article (12/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Otodental Dysplasia:

1.	Vitamin D FDA LinkGeneric 12/01/1976 - "Such cases include hypophosphatemic vitamin D-resistant and dependent rickets, pseudo-hypoparthyroidism otodental syndrome and hypophosphatasia. "

Therapies and Procedures

1.	Crowns (Crown, Dental) 09/01/1996 - "Otodental dysplasia, a form of ectodermal dysplasia, is characterized by abnormal dental crown morphology and sensorineural hearing loss. " 10/01/1998 - "Three unusual dental anomalies are presented which should be of interest to the pediatric radiologist: otodental syndrome - an autosomal dominant syndrome of bizarre, greatly enlarged teeth with bulbous crowns (globodontia) that spares the incisors, in combination with sensorineural hearing loss; oculo-facio-cardio-dental syndrome - an X-linked dominant syndrome that is lethal in males, characterized by congenital cataracts, unusual facies, atrial septal defect (ASD), ventricular septal defect (VSD), and canine teeth that cease to grow only when they cut off their own blood supply by growing through the orbit and lower border of mandible; and lobodontia - a dominant disorder characterized by teeth whose crowns resemble those of a wolf."
2.	Oral Pathology 12/01/2015 - "Referral to Clinical Genetics and Oral Pathology was instrumental in establishing a diagnosis of otodental syndrome for this young patient and her mother, who had similar dental defects. "
3.	Oral Diagnosis (Oral Examination) 03/01/2022 - "After extra- and intra-oral examination and radiographic exams, a clinical diagnosis of otodental syndrome was made, and a genetic testing was requested to confirm the diagnosis. "