Cases reported "Deafness"

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1/48. 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.
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2/48. Necrotizing otitis externa caused by staphylococcus epidermidis.

    We present a case of malignant necrotizing otitis externa (MNOE) caused by staphylococcus epidermidis, which is usually a non-pathogenic microorganism. The patient is an otherwise healthy, nondiabetic 58-year-old white man. Contributory history began in 1994 after surgery for bilateral exostoses of the external auditory canals. Between April 1994 and May 1998 persistent otalgia occurred, with progressive mixed hearing losses, purulent discharge from both ears, spontaneous perforations of the tympanic membranes and ulceration of canal wall skin. From the beginning, Staph. epidermidis was isolated in all but one culture, but was not recognized as the pathological agent because of the presence of other more frequently involved bacteria and fungi. After multiple intravenous and oral antibiotics and antifungal treatments failed, further management involved frequent debridement of both external auditory canals and tympanic membranes, right tympanoplasty, bilateral mastoidectomy, revision tympanomastoidectomies and left modified radical mastoidectomy. Antistaphylococcal therapy including ceftazidime, vancomycin, teicoplanin, clindamycin and rifampicin was tried. Following the modified radical radical mastoidectomy, normalization of the status of his ears took approximately 2 months and has since remained stable to date. His left ear is deaf because of vancomycin administration, while magnetic resonance imaging and gallium scintigraphy have shown persistent inflammation of the skull base.
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3/48. Labyrinthine involvement and multiple perforations of the tympanic membrane in acute otitis media due to group A streptococci.

    We present here three cases of acute otitis media caused by a virulent group A streptococcal infection that rapidly led to deterioration in hearing. Two of the three cases presented with severe sensorineural and mixed hearing loss with multiple tympanic membrane perforations, and the third presented with severe bilateral sensorineural hearing loss following acute otitis media involving group A streptococci. All patients were treated with systemic (piperacillin) and topical antibiotics (ofloxacin ear drops): one patient also received a systemic steroid (betamethasone). deafness persisted in one patient but in the other two, hearing gradually recovered. Severe cytotoxicity was considered to have occurred in all patients, resulting in multiple perforations of the tympanic membrane and necrosis in the middle ear.
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4/48. Mould constituents in the middle ear, a hearing-aid complication.

    During the placement of a mould for a hearing-aid by a hearing-aid dispenser, moulding material entered the middle ear through pre-existent perforations in the tympanic membrane in both ears. Besides hearing loss, there were no other symptoms. Surgical removal of the moulding material by tympanotomy was necessary, and was complicated by encirclement of the ossicles by the material. All the material could be removed and the hearing was saved. Recommendations for an improved procedure of mould-making are made including more detailed information of the otoscopic findings at the prescriptions for hearing-aid moulds.
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5/48. deafness complicating mucous membrane pemphigoid: a case report.

    A patient with moderately severe mucous membrane pemphigoid is described. The onset of deafness was associated with the appearance of postero-superior retraction prockets in both eardrums. It is postulated that this was due to mucous membrane pemphigoid involving the mucosa of the middle ear. We are not aware of this complication having been described previously.
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6/48. Can migraine damage the inner ear?

    BACKGROUND: Auditory and vestibular symptoms and signs are common in patients with migraine, yet little is known about the pathogenesis of these symptoms and signs. OBJECTIVE: To perform clinicopathological correlation in a patient with migraine, sudden deafness, and delayed endolymphatic hydrops. methods: A patient with long-standing migraine with aura developed sudden hearing loss in the left ear at the age of 50 years and meniere disease on the right side at age 73. At age 76, he had a flurry of sudden drop attacks typical of otolithic crisis. He died of unrelated causes at age 81. The brain and temporal bones were removed approximately 24 hours after death. The cochlea and vestibular end organs were dissected after the surrounding bone was carefully removed. RESULTS: The brain and cerebrovasculature were normal. The left cochlea showed prominent fibrosis consistent with an old infarction. The right inner ear showed hydrops, with relatively good preservation of the hair cells in the cochlea, saccular macule, and cristae of the semicircular canals. However, the utricular macule was denuded of hair cells. CONCLUSIONS: The sudden left-sided deafness likely resulted from ischemia, possibly due to migraine-associated vasospasm. Presumably, the right ear suffered only minimal damage when the patient was 50 years old, but this damage later led to the development of delayed endolymphatic hydrops on the right. Otolithic crises are thought to result from pressure changes across the utricular macule. We speculate that loss of hair cells in the utricular macule resulted from a collapse of the utricular membrane onto the macule.
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7/48. otitis externa sicca/fibrotising external otitis (FEO) as a complication of sjogren's syndrome.

    sjogren's syndrome (SS) is a condition characterized by sicca symptoms and by autoimmune features. We describe two SS patients with otitis externa fibroticans/sicca. One of these 2 patients developed a lesion of the tympanic membrane making it necessary to perform a tympantomy and meatoplasty. Our findings suggest firstly that the epithelial cell-mediated secretion of lamellar bodies and the production of the permeability barrier are defective in SS. Secondly, local moisturing and/or topical corticosteroid treatment in SS patients with sicca symptoms in the auditory canal could help to avoid reconstructive surgical treatment.
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8/48. Unpredictable hearing loss after intratympanic gentamicin treatment for vertigo. A new theory.

    A new hypothesis is advanced suggesting that unpredictable cases of profound hearing loss after intratympanic gentamicin treatment (IGT) may be caused by decreased patency of the communication routes between the inner ear and the cerebrospinal fluid, primarily of the cochlear aqueduct. A tympanic displacement analyzer, which can indirectly analyze inner ear and intracranial pressure changes and can also evaluate the efficiency of communication between these two compartments, was used. Two cases are presented: in the first, a patient who became deaf after IGT showed signs of decreased patency of the communication routes with the tympanic membrane displacement (TMD) test; in the second, a patient without hearing damage after IGT had efficient communication evaluated by the TMD test. These preliminary findings are in accordance with the proposed pathophysiology. If future clinical studies confirm the present theory and findings, it may prove possible to predict and prevent deafness after IGT and possibly also after systemic aminoglycoside treatment.
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9/48. Pathologic features of the inner ear in congenital deafness.

    We present the morphologic findings of the temporal bones and brain of a patient with congenital deafness. We discuss these findings in relation to pathologic observations in other reported cases of congenital deafness. Morphologic abnormalities in the patient were mainly in the pars inferior of the membranous labyrinths. The osseous labyrinths were well developed. There was severe dilation of the cochlear duct with herniation of the Reissner membrane, extensive atrophy of the stria vascularis that was associated with calcified thrombi to the strial vessels, encasement of the tectorial membrane in a syncytium, and dyspiastic or regressive degeneration of the organ of corti. Absence of spiral ganglion cells and their fibers was a prominent feature. The extensive and varied pathologic changes that were present in our patient simultaneously suggest a congenital abnormality in endolymph production and raise the possibility of anomalous development of the labyrinthine vasculature.
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10/48. rupture of the round window membrane.

    A perilymph leak into the middle ear through a ruptured round window membrane results in the symptoms of hearing loss, tinnitus and vertigo, either singly or in combination. The case histories of thirteen patients with such a fistula are described, these patients having in common a predisposing incident which had led to a rise of C.S.F. pressure. Symptomatology and the results of investigation are analysed and operative technique and results discussed. While it appears that vertigo uniformly responds very satisfactorily to operative treatment the improvement in hearing loss and tinnitus is more difficult to predict.
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