[Temporal bone pathology in neonates with severe visceral anomalies]

It is well known that hearing loss is often associated with anomalads, syndromes involving multiple anomalies. The incidence is especially high in severe cases of facial and visceral malformations. However, otologic features remain unclear in patients with a sequence of various anomalies which can not be classified into any known syndrome. We examined 11 temporal bones from 6 patients with severe visceral anomalies, which could not be classified into any known systemic bone diseases, chromosomal abnormalities, or congenital metabolic disorders. Temporal bone pathology was compared with external and visceral anomalies in each case. The temporal bones had been removed at autopsy, fixed in 10% formaldehyde, decalcified and embedded in celloidin. Serial horizontal sections were made at 20 microns and every tenth section was stained with hematoxylin eosin. Most abnormalities in the middle and inner ear were found to have an ectodermal or mesodermal origin. Inner ear abnormalities were noted in 6 temporal bones from 3 patients; the predominant feature was hypoplasia of the semicircular canals. Middle ear abnormalities excluding residual mesenchymal tissue were noted in 5 temporal bones from 4 patients; the predominant feature was an abnormal course of the facial nerve. It was also suspected that auricular and maxillomandibular abnormalities, which are often associated with severe visceral anomalies, indicate a high incidence of disorders affecting the auditory and vestibular systems.