Cochleo-vestibular malformations and large vestibularaqueduct are the 2 most frequent deformities.
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Results suggest that all ears with a large vestibularaqueduct have associated cochlear modiolar deficiencies.
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Furthermore, shorter relaxation times were measured for the contents of the vestibularaqueduct than for other labyrinthine structures.
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Loss-of-function and hypo-functional mutations cause an enlargement of the vestibularaqueduct (EVA) and sensorineural hearing loss.
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Objective: Defects of the cochlear modiolus have been found to be associated with most cases of large vestibularaqueduct.
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Thus, a large vestibularaqueduct may be only occasionally, if ever, an isolated developmental anomaly of the inner ear.
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However, the thickness and morphology of the vestibularaqueduct may simply be markers for more subtle cochlear dysplasia manifest by modiolar deficiency.
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Mutations of SLC26A4 are a common cause of human hearing loss associated with enlargement of the vestibularaqueduct.
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SLC26A4 mutations cause fluctuating and progressive hearing loss associated with enlargement of the vestibularaqueduct (EVA).
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Enlargement of the vestibularaqueduct (EVA) is the most common inner ear anomaly detected in ears of children with sensorineural hearing loss.
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Conclusions: These observations support the hypothesis that large vestibularaqueduct-related hearing loss may be caused by transmission of subarachnoid pressure forces into the inner ear.
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Vestibularaqueduct morphology and thickness correlated very strongly with the severity of hearing loss.
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Patients: Thirty consecutive patients with large vestibularaqueducts participated.