Temporal bone pathology in congenital anomalies of the oval window and the facial nerve.
Temporal bones of six infants with congenital ear anomalies were examined for abnormalities of the oval window and facial nerve. These temporal bones were classified into two groups according to the degree of malformation: group A, those with atresia or absence of the oval window; and, group B, those with hypoplasia of the stapes and annular ligament. Group A, consisting of five ears, were associated with severe middle ear anomalies such as the abnormal course of the facial nerve and absence of the stapes. In group B, consisting of seven ears, the stapes were present and the facial nerve presented minor anomalies such as obtuse angulation at the first genu, central migration of the geniculate ganglion cells, ectopic muscles and a wide bony dehiscence of the facial canal around the oval window. Probable origin of the anomalies in group A could mainly be due to maldevelopment of the facial nerve during an earlier embryonal period while that of group B could have developed after the ninth week of the fetal period and are mostly localized along the second branchial arch.
D000015:Abnormalities, Multiple / Q000473:pathology; D002814:Chorda Tympani Nerve / Q000002:abnormalities; D004432:Ear, Middle / Q000002:abnormalities; D005154:Facial Nerve / Q000002:abnormalities* / Q000473:pathology; D005260:Female; D005830:Geniculate Ganglion / Q000002:abnormalities; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010046:Oval Window, Ear / Q000002:abnormalities* / Q000473:pathology; D013199:Stapes / Q000002:abnormalities; D013701:Temporal Bone / Q000473:pathology*; D014722:Vestibule, Labyrinth / Q000002:abnormalities*