<unk> , also known as<unk>  dysplasia, is an exceptionally rare disease that is distinguished by a specific phenotype known as globodontia, that in rare cases can be associated with eye coloboma and high frequency hearing loss. Globodontia is an abnormal condition that can occur in both the primary and secondary dentition, except for the incisors which are normal in shape and size. This is demonstrated by significant enlargement of the canine and molar teeth. The premolars are either reduced in size or are absent. In some cases, the defects affecting the teeth, eye and ear can be either individual or combined. When these conditions are combined with eye coloboma, the condition is also known as oculo-otodental syndrome. The first known case of<unk>  was found in Hungary in a mother and her son by Denes and Csiba in 1969. Prevalence is less than 1 out of every 1 million individuals. The cause of<unk>  is considered to be genetic. It is an autosomal dominant inheritance and is variable in its expressivity. Haploinsufficiency in the fibroblast growth factor 3 (FGF3) gene (11q13) has been reported in patients with<unk>  and is thought to cause the phenotype. Both males and females are equally affected. Individuals diagnosed with<unk>  can be of any age; age is not a relevant factor. Currently there are no specific genetic treatments for<unk> . Dental and orthodontic management are the recommended course of action.
Otodental syndrome
Diencephalic syndrome
Exploding head syndrome
Austrian syndrome
Perisylvian syndrome
Organic brain syndrome
Dejerine–Roussy syndrome
Sudden wealth syndrome