2012
DOI: 10.1177/197140091202500412
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Congenital X-linked Stapes Gusher Syndrome

Abstract: X-Linked sensorineuronal hearing loss (SNHL) is a rare but recognized cause of bilateral SNHL associated with CSF gusher. We report a case presenting a varied imaging spectrum and clinical scenario of a one year old boy with unilateral profound hearing loss and recurrent meningitis due to spontaneous oval window gusher. Imaging showed a unilateral abnormality, with the fistula leaking from the oval window into the middle ear cleft.

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Cited by 4 publications
(2 citation statements)
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“…The condition is rare. A thorough search of the literature yielded circa 89 patients since 1971 (29,47,(49)(50)(51)(52)(53)(54)(55)(56)(57)(58). These children present with progressive mixed hearing losses and varying degrees of vestibular problems as was found in our study.…”
Section: Discussionsupporting
confidence: 68%
“…The condition is rare. A thorough search of the literature yielded circa 89 patients since 1971 (29,47,(49)(50)(51)(52)(53)(54)(55)(56)(57)(58). These children present with progressive mixed hearing losses and varying degrees of vestibular problems as was found in our study.…”
Section: Discussionsupporting
confidence: 68%
“…Some other minor fatty acids may be present in the same vegetable sources, including a small amount of branched-chain, cyclic and odd number straight-chain acids. The unsaturated fatty acids may have one double bond (monosaturated) or have more than one cis-methylene interrupted double bond [5].…”
Section: Background Of the Studymentioning
confidence: 99%