Histopathologic study of temporal bone and eustachian tube in oculoauriculovertebral spectrum

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