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An abnormally low position of the middle cranial fossa in relation to the petrous pyramid was observed. The cochlea was of normal length. The modiolus was poorly developed with apparently normal population of ganglion cells and moderate diffuse hydrops of the cochlear duct and saccule. The stria vascularis was partially degenerated in the upper apical coil. Vestibular abnormalities included bilateral absence of common crus of the vertical canals and unusually high origin of endolymphatic aqueduct with no medial dilation present, the convoluted portion of the sac located beneath the dura.<\/jats:p>","DOI":"10.1177\/000348947808700103","type":"journal-article","created":{"date-parts":[[2014,10,3]],"date-time":"2014-10-03T14:29:51Z","timestamp":1412346591000},"page":"10-17","source":"Crossref","is-referenced-by-count":6,"title":["Ear Anomalies Associated with Renal Dysplasia and Immunodeficiency Disease"],"prefix":"10.1177","volume":"87","author":[{"given":"John R.","family":"Lindsay","sequence":"first","affiliation":[{"name":"Chicago, Illinois"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ra\u00fal","family":"hinojosa","sequence":"additional","affiliation":[{"name":"Chicago, Illinois"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[1978,1,1]]},"reference":[{"key":"bibr1-000348947808700103","doi-asserted-by":"publisher","DOI":"10.1177\/000348947608500212"},{"key":"bibr2-000348947808700103","unstructured":"Ascherson FM: DeFistulis Colli Congenitis. 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