PubMed · 6962302
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A S Scott, R J Jamsky. 1982. Oculoauriculovertebral dysplasia.. https://pubmed.ncbi.nlm.nih.gov/6962302/
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We investigated the vestibular endorgans in three children using 3-D reconstructions from histological sections. The right temporal bone of a newborn child without peripheral vestibular pathology was used as reference model and the temporal bones from a child with Goldenhar syndrome and a child with Pierre Robin sequence with known peripheral vestibular pathology were studied. All five temporal bones were prepared by the celloidin technique and sectioned at 20 microm. Each available section was digitized with a slide scanner. The imaging data were layered anatomically correctly and rendered in a 3-D software. With this technique all vestibular endorgans were reconstructed and measured. The standard deviations in distances ranged between 0.5 and 1.2% and in angles between 0.1 and 2.9 degrees. Both maculae were curved in the longitudinal and transverse axes which described a curve of approximately 35 degrees. The angles between the semicircular ducts varied between 97 and 110 degrees. The pathological models demonstrated a distorted configuration of the semicircular canals and differed substantially from the reference model in most of the measured distances and angles. The method presented is capable of generating 3-D models of the vestibular system from histological sections with an acceptable precision without previously inserted reference marks. Archival celloidin sections are widely available and will be an important resource in understanding the detailed 3-D geometry of the vestibular system which has not yet been accomplished.
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BACKGROUND: Oculoauriculovertebral dysplasia (OAVD) (Goldenhar's syndrome) is a congenital syndrome with ipsilateral deformity of the ear and face, epibulbar lipodermoids, coloboma, and vertebral anomalies. Goldenhar's anomaly has often been associated with a degree of congenital hearing deficits, almost always of a conductive origin, but a sensorineural component is also suspected in some cases, evident through malformations of the inner ear. PATIENTS AND METHODS: Both temporal bones of a 10-day-old deceased patient with oculoauriculovertebral dysplasia were examined by light microscopy. RESULTS: The ear deformities included deformity of the auricle, atresia of the external auditory canal, and malformation of the tympanic cavity and ossicles. Abnormalities of the stria vascularis and the semicircular canals were also demonstrated. Further inner ear deformities were not identified in this case. CONCLUSION: These histopathologic findings appear to confirm the conductive component of the congenital hearing deficit, but a sensorineural component could not be omitted. The ear alterations favor early developmental field defects. The causes of this condition are controversial. Recent results in genetic research pertaining to the MSX class genes permit better understanding of the variety, variability, and different degrees of severity of the anomalies described here.