Cases reported "Deafness"

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1/1255. Sudden deafness. A histopathological study.

    Histopathological studies were performed in the temporal bones from a 77-year-old woman who had bilateral sudden deafness 31 years before her death. The common findings in both cochleas were atrophy of the organ of corti and stria vascularis, and degeneration of the tectorial membrane. pathology of the tectorial membrane consisted of atrophy and droplet formation of the middle and marginal zones. In one area, an atrophied tectorial membrane was lifted up by a mass on the limbus. The saccular wall in the left temporal bone was ruptured. There was no pathology in the vascular system in the labyrinths except for moderate degeneration of sensory epithelium. The pathogenesis of sudden deafness was discussed in the light of the present findings. Alteration of the tectorial membrane seemed to be the main factor as a possible cause of sudden deafness. Histopathological findings were reviewed from the literature. ( info)

2/1255. Acoustic trauma from the bone cutting burr.

    The amplitudes of the stapes footplate movements were determined in human temporal bones when the ossicular chain was drilled with a cutting and a diamond burr. High movements result in comparison to physiological data. The frequency distribution resembles the dB (A) curve. The intensity compared to sound pressure levels on the ear drum is higher than I30 dB. The pressure is constant over the period of contact between the burr and the ossicle. Most likely these unphysiologic movements of the stapes footplate can cause inner ear damage as we had to admit in a case of facial nerve decompression. ( info)

3/1255. Intravenous azithromycin-induced ototoxicity.

    Intravenous azithromycin is increasingly administered for treatment of hospitalized patients with community-acquired pneumonia. Macrolide antibiotics cause ototoxicity, which occurs most frequently when high serum concentrations are achieved. Current dosing guidelines for intravenous azithromycin can result in much higher serum concentrations than is seen with oral administration. We describe a 47-year-old woman who developed complete deafness after receiving 8 days of intravenous azithromycin. ( info)

4/1255. Contralateral deafness following unilateral suboccipital brain tumor surgery in a patient with large vestibular aqueduct--case report.

    A 68-year-old female developed contralateral deafness following extirpation of a left cerebellopontine angle epidermoid cyst. Computed tomography showed that large vestibular aqueduct was present. This unusual complication may have been caused by an abrupt pressure change after cerebrospinal fluid release, which was transmitted through the large vestibular aqueduct and resulted in cochlear damage. ( info)

5/1255. Intraoperative loss of auditory function relieved by microvascular decompression of the cochlear nerve.

    BACKGROUND: Brainstem auditory evoked potentials (BAEP) are useful indicators of auditory function during posterior fossa surgery. Several potential mechanisms of injury may affect the cochlear nerve, and complete loss of BAEP is often associated with postoperative hearing loss. We report two cases of intraoperative auditory loss related to vascular compression upon the cochlear nerve. methods: Intra-operative BAEP were monitored in a consecutive series of over 300 microvascular decompressions (MVD) performed in a recent twelve-month period. In two patients undergoing treatment for trigeminal neuralgia, BAEP waveforms suddenly disappeared completely during closure of the dura. RESULTS: The cerebello-pontine angle was immediately re-explored and there was no evidence of hemorrhage or cerebellar swelling. The cochlear nerve and brainstem were inspected, and prominent vascular compression was identified in both patients. A cochlear nerve MVD resulted in immediate restoration of BAEP, and both patients recovered without hearing loss. CONCLUSION: These cases illustrate that vascular compression upon the cochlear nerve may disrupt function, and is reversible with MVD. awareness of this event and recognition of BAEP changes alert the neurosurgeon to a potential reversible cause of hearing loss during posterior fossa surgery. ( info)

6/1255. Progressive ponto-bulbar palsy with deafness. A clinico-pathological study.

    Progressive ponto-bulbar palsy with deafness is a rare disease. It seems to be an abiotrophic process with autosomal recessive inheritance in most instances. Only one autopsy case had been briefly described (Lelong et al., 1941). The clinical features and the pathological findings of a new case are reported. The structures primarily involved are the grey matter of the brain stem and the spinal cord, including to some extent the optic tracts and most of the fiber tracts in the brain stem with exception of the pyramidal tracts. ( info)

7/1255. Autosomal dominant polycystic kidney disease associated with familial sensorineural deafness.

    Autosomal dominant polycystic kidney disease (ADPKD) is characterized by both renal and non-renal disorders. Extrarenal involvement includes noncystic manifestations such as cardiovascular abnormalities, colonic diverticula and intracranial aneurysms. Familial sensorineural hearing loss (SNHL) has been included in the definition of Alport's syndrome. However, other types of nephropathy have been occasionally associated with hereditary deafness. The association of ADPKD with hereditary SNHL has not been previously documented. We report a family with ADPKD associated with bilateral sensorineural deafness in a pedigree of four affected members in four generations. ( info)

8/1255. Direct measurement of cerebrospinal fluid pressure through the cochlea in a congenitally deaf child with Mondini dysplasia undergoing cochlear implantation.

    OBJECTIVE: perilymph/cerebrospinal fluid (CSF) "gushers" may occur at cochleostomy during cochlear implant surgery, particularly in patients with congenital cochlear duct malformation in which CSF in the internal auditory meatus is in direct communication with the perilymphatic space in the cochlea. The object of the study was to measure the pressure and flow of a CSF gusher at cochleostomy. STUDY DESIGN: The design was a preoperative pressure measurement. SETTING: The setting was a multidisciplinary cochlear implant program. PATIENT: A 4-year-old girl with bilateral Mondini deformity undergoing cochlear implantation was studied. INTERVENTION: A size 23 FG intravenous cannula was inserted into the cochlea and connected to a pediatric drip set to form an improvised manometer. MAIN OUTCOME MEASURE: Intracochlear fluid pressure was measured at 14 cm H2O, equivalent to the normal CSF pressure that would be recorded in a child of this age at lumbar puncture. An indirect measurement of the likely size of the CSF/perilymph defect was made. RESULTS: This technique may allow better assessment of the risk of postoperative CSF leakage and meningitis. CONCLUSION: This simple technique of measuring the pressure in a perilymph gusher can be used to assess the need for careful sealing of the cochleostomy, to measure the reduction in pressure produced by head elevation or a spinal drain, and to assess the probable size of a defect in the lamina cribrosa. ( info)

9/1255. Narrow and vacant internal auditory canal.

    A case of unilateral congenital deafness revealing a narrow vacant internal auditory canal and a more anterior and superior second canal where the facial and vestibulocochlear nerves are well visualised is presented. Having reviewed the scientific and embryological data, the authors consider the mechanism of this malformation. ( info)

10/1255. Acquisition and cross modal generalization of receptive and expressive signing skills in a retarded deaf girl.

    A transfer of stimulus control procedure was used to teach a retarded deaf girl manual signing skills. Eight manual signs were trained in the receptive modality; eight others in the expressive modality. Acquisition of receptive and expressive signing skills occurred for most signs after they had been trained in multiple baseline order. There was a marked difference in cross modal generalisation between each of the trained signing modalities. With some exception training of receptive sign discrimination had little effect on the expressive usage of these signs. In contrast, training in the expressive usage resulted in a near perfect acquisition of receptive sign discrimination. ( info)
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