Two temporal bone-eustachian tube (ET) specimens; one from a 1-day-old female newborn with Townes Syndrome (Case 1) and the other from a 15-year-old female with oral-facial-digital syndrome (Case 2) were studied histopathologically and morphometrically. Both specimens had anomalies of the ET and its associated structures as compared with eight age-matched control cases without anomaly (six cases for Case 1 and two cases for Case 2, respectively). Case 1 had a weak attachment of the tensor veli palatini muscle (TVPM) to a poorly developed lateral lamina (LL) of the ET cartilage, a large voluminous medial lamina (ML) of the ET cartilage, a small voluminous ET lumen with a few ridges of mucosal folds, poorly developed ET glands and a poorly developed levator veli palatini muscle (LVPM). Case 2 had a short and longitudinally elongated ET lumen that was insufficiently covered with a poorly developed ML and LL and an aberrant course of the LVPM. Both cases were accompanied by a mild inner ear anomaly (slightly shortened cochlea). We discuss the implications of the observed anomalies with regard to functional and clinical issues. In particular, we speculate that these ET anomalies may closely be related to potential ET dysfunction with high susceptibility to otitis media.