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Deafness with labyrinthine aplasia, microtia, and microdontia

1 OMIM reference -
1 associated gene
24 signs/symptoms

COMMON GENES: 1
COMMON SIGNS: 6

Otodental syndrome

1 OMIM reference -
1 associated gene
25 signs/symptoms

Deafness with labyrinthine aplasia, microtia, and microdontia

Otodental syndrome

FGF3

(UniProt - OMIM)

FGF3

(UniProt - OMIM)


COMMON GENES	FGF3

Citations in the biomedical literature:

Deafness with labyrinthine aplasia, microtia, and microdontia		Otodental syndrome
	Synonym(s): - LAMM syndrome - Microdontia - type I microtia - deafness		Synonym(s): - Globodontia - Otodental dysplasia

	Classification (Orphanet): - Rare developmental defect during embryogenesis - Rare genetic disease - Rare otorhinolaryngologic disease		Classification (Orphanet): - Rare developmental defect during embryogenesis - Rare genetic disease - Rare odontologic disease

	Classification (ICD10): (no data available)		Classification (ICD10): - Diseases of the digestive system -

	Epidemiological data: Class of prevalence: <1 / 1 000 000 Average age onset: neonatal/infancy Average age of death: - Type of inheritance: autosomal recessive		Epidemiological data: Class of prevalence: <1 / 1 000 000 Average age onset: childhood Average age of death: - Type of inheritance: autosomal dominant

	External references: 1 OMIM reference - 1 MeSH reference: C548011		External references: 1 OMIM reference - No MeSH references


COMMON SIGNS	- Anodontia / oligodontia / hypodontia - Long face - Micrognathia / retrognathia / micrognathism / retrognathism - Sensorineural deafness / hearing loss - Supernumerary teeth / polyodontia - Tooth shape anomaly

Deafness with labyrinthine aplasia, microtia, and microdontia		Otodental syndrome
	Very frequent - Abnormal dentition / dental position / implantation / unerupted / dental ankylosis - Autosomal recessive inheritance - Complete / partial microdontia - Cranial nerve anomalies - Microtia / cryptomicrotia / anotia / external auditory canal / pinnae aplasia / hypoplasia - Structural anomalies of inner ear / cochlea / vestible / semicircular canals Frequent - Broad nasal root - Downslanted palpebral fissures / anti-mongoloid slanting palpebral fissures - Pointed chin - Small / triangular nares / nostrils Occasional - Bifid / cleft ear lobe / ear lobe pits - Hypermetropia - Hypertelorism - Long / large / bulbous nose - Preauricular / branchial tags / appendages - Strabismus / squint - Synophris / synophrys - Tall stature / gigantism / growth acceleration		Very frequent - Autosomal dominant inheritance Frequent - Broad cheeks / cherub-like / cherubin face - Delayed dentition / eruption of teeth / lack of eruption of teeth - Dental malocclusion - Enamel anomaly - Taurodontia - Thickened / hypertrophic / fibromatous gingivae Occasional - Anophthalmos / anophthalmia / microphthalmos / microphthalmia - Anteverted nares / nostrils - Cataract / lens opacification - Coloboma of iris - Coloboma of the lens - Heterochromia / mixed colouring of iris - High vaulted / narrow palate - Long philtrum - Microcornea - Pigmented naevi / naevus pigmentosus / lentigo - Prominent / bat ears - Retinoschisis / retinal / chorioretinal coloboma