Histopathologic Study of Temporal Bone and Eustachian Tube in Oculoauriculovertebral Spectrum

Bilateral temporal bone specimens from a 21-month-old girl and a left temporal bone-eustachian tube (ET) specimen from a full-term female newborn, both with oculoauriculovertebral spectrum, were studied histopathologically. The external and middle ears demonstrated severe anomalies, similar to those of previous reports describing the histopathologic findings of this syndrome. In addition, despite having a normal auricle, the 21-month-old child had bilateral hypoplastic cochleas as seen in Mondini dysplasia. The newborn had several anomalies of the ET, including a widely opened cartilaginous portion of the ET lumen and absence of the lateral lamina of the ET cartilage. We discuss the implications of the observed anomalies with regard to developmental and clinical issues.