Teeth, Congenital Absence Of, With Taurodontia And Sparse Hair

Clinical Features

Stenvik et al. (1972) reported 4 Norwegian sibs with congenitally missing teeth and sparse hair. Three of the 4 sibs had taurodontia. Nails and ability to perspire were not specifically mentioned. The parents were not evaluated.

Moller et al. (1973) and Gorlin et al. (1975) reported a female with a similar disorder, the third of 4 children. Sweat function was normal using orthophthalaldialdehyde. The father and one of the proband's sisters congenitally lacked permanent maxillary lateral incisors but were otherwise normal. Gorlin et al. (1975) stated that this combination of findings had previously been reported by Stoy (1960).