Congenital anomalies of the inner ear.
This study investigated congenital anomalies occurring in the inner ear, with particular attention to their features, localizations, and frequencies. Seventy-seven human temporal bones obtained from 53 individuals, aged one day to 39 years, each of whom had anomalies of the external ear, middle ear, and/or inner ear, were used for this study. The temporal bones had been removed at autopsy, fixed, dehydrated, embedded in celloidin, and sectioned horizontally or vertically at 20 microns. Every tenth horizontal section or every 20th vertical section was stained with hematoxylin and eosin, mounted, and studied under a light microscope. There were 206 inner ear anomalies (117 in the vestibular system, 79 in the cochlea, 10 in the internal auditory meatus) in the 51 bones studied. Fifty-four different features of anomalies were present at 33 different locations in the inner ear. The most frequent anomalies observed were shortened cochlea (23 bones), enlarged vestibule (17 bones), wide cochlear aqueduct (16 bones), absence of lateral semicircular canal (14 bones), and large osseous lateral semicircular canal (10 bones). The most common site of inner ear anomalies was thus the lateral semicircular canal, which was involved in 46 of the 206 anomalies observed; the anomalies in this organ were variable in type. The implications of inner ear anomalies are discussed as they relate to fetal development, inner ear dysfunction, and clinical interpretation of polytomographic studies.
D000015:Abnormalities, Multiple; D000328:Adult; D002901:Chromosomes, Human, 13-15; D002902:Chromosomes, Human, 16-18; D003051:Cochlea / Q000002:abnormalities / Q000473:pathology; D007758:Ear, Inner / Q000002:abnormalities* / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012665:Semicircular Canals / Q000002:abnormalities / Q000473:pathology; D013577:Syndrome; D013701:Temporal Bone / Q000002:abnormalities* / Q000473:pathology; D014314:Trisomy; D014722:Vestibule, Labyrinth / Q000002:abnormalities / Q000473:pathology