Cases reported "Endolymphatic Hydrops"

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1/3. Spontaneous intracranial hypotension: a rare cause of labyrinthine hydrops.

    Spontaneous intracranial hypotension should be considered as a possible cause of cochlear hydrops. We report a case of unilateral hearing loss attributed to spontaneous intracranial hypotension on the basis of characteristic abnormalities seen on magnetic resonance imaging. The diagnostic gold standards for intracranial hypotension are lumbar measurement of cerebrospinal fluid pressure and magnetic resonance imaging. The usual treatment is an autologous blood injection into the peridural spaces. The mechanism of hearing loss is thought to involve secondary perilymph depression due to a patent cochlear aqueduct. This perilymph depression would induce a compensatory expansion of the endolymphatic compartment, with a subsequent decrease in basilar or Reissner's membrane compliance. endolymphatic hydrops can occur in the course of intracranial hypotension, and not only because of abnormal endolymph production or resorption. Hydrops can thus be classified into 1) syndromes of endolymphatic origin and 2) syndromes of perilymphatic origin, in which loss of perilymph induces compensatory expansion of the endolymphatic space.
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keywords = membrane
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2/3. Paget disease and sensorineural hearing loss associated with spiral ligament degeneration.

    HYPOTHESIS: Previously unreported cystic degeneration of the spiral ligament in cases of Paget disease. BACKGROUND: About 70% of cases of Paget disease involve the skull, with hearing affected in approximately 50% of these. The hearing impairment may be sensorineural, mixed, or, rarely, only conductive. The etiology and pathogenesis of the hearing loss are not yet understood, and reports in the literature are inconsistent regarding the pathologic changes responsible for sensorineural hearing loss. Of six pairs of temporal bones from patients with Paget disease in the temporal bone collection of a research institution, two pairs have abnormalities not previously associated with sensorineural hearing loss or Paget disease. We report the histopathologic findings in these temporal bones. methods: The temporal bones were fixed in formalin, decalcified in ethylenediaminetetraacetic acid, embedded in celloidin, and sectioned in the horizontal plane at a thickness of 20 microm. Every 10th section was stained with hematoxylin-eosin and mounted on glass slides. The sections were examined by light microscopy. RESULTS: Cystic degeneration of the spiral ligament, primarily in the basal segment, was found in both cases. endolymphatic hydrops and a small endolymphatic sac with calcification of the perisaccular tissue were found in one case. CONCLUSIONS: Cystic degeneration of the spiral ligament has not been previously reported and may be unique to Paget disease. This is consistent with recent literature showing a previously unsuspected role of the spiral ligament in sensorineural hearing loss.
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keywords = ligament
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3/3. High-resolution MRI of the human cochlea.

    The majority of magnetic resonance imaging (MRI) studies in neurotology concern the evaluation of retrocochlear pathologies or temporal bone lesions. Recently, quite a number of investigators have attempted to use imaging diagnosis to obtain a diagnosis and manifest the pathological findings of Meniere's disease. However, there is no evidence that endolymphatic hydrops in patients with Meniere's disease can be identified by imaging techniques. In this study we could depict Reissner's membrane clearer than before with the use of image processing. At the present time, we cannot apply the 2.11T MRI machine to patients under FDA regulation. We believe that MRI diagnoses of endolymphatic hydrops and small lesions of the internal structures of the inner ear will be possible in the near future.
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