One form of inner ear dysplasia is unique because it has been associated with delayed onset of SNHL. An enlarged vestibular aqueduct is commonly seen in combination with other inner ear dysplasias, but more recently, it has been noted as an isolated finding in many ears. These patients
may have any level of hearing from normal to a profound loss. Frequently, both ears are affected, and the losses are asymmetric. Fluctuation of hearing is common and usually affects one ear at a time; this may manifest as anacusis in one ear with fluctuation in the other. There is often evidence of a conductive component to the low-frequency portion of hearing loss. In patients who have been followed over time, a progressive stepwise loss has been noted in many. This syndrome has been found to be familial in some cases, and probably occurs much more commonly than generally appreciated. It is seen in isolation, as part of the Mondini malformation, and in patients with branchio-oto-renal syndrome and Pendred syndrome. It is well shown on high-resolution CT imaging of the temporal bone.
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