Cases reported "Deafness"

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1/29. 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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ranking = 1
keywords = otitis
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2/29. 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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ranking = 1.2
keywords = otitis
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3/29. Histopathological findings in the inner ear caused by measles.

    An otopathological analysis of three cases of viral labyrinthitis was performed. Six temporal bones cut in serial sections were available for this study. According to the degree of degenerative changes in various parts of the inner ear two types of morphologically distinct labyrinthitis after measles are presented: the first one with the port of entrance through the internal auditory meatus and characterized by, first and most significantly, changes in the spiral ganglion cells; and the second, as described previously by Lindsay, with the port of entrance of the virus in the inner ear through the stria vascularis, with degenerative changes in various structures within the endolymphatic duct.
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ranking = 21.457372811924
keywords = labyrinthitis
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4/29. 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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ranking = 1
keywords = otitis
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5/29. Delayed endolymphatic hydrops: study and review of clinical implications and surgical treatment.

    Delayed endolymphatic hydrops (DEH) differs from Meniere's disease in that it occurs in pre-existing ear pathology in patients who have a profound unilateral or total deafness that was caused by infection, trauma, or unknown causes during childhood or adulthood. We performed a retrospective review of 160 patients with ipsilateral, contralateral, or bilateral DEH. Eighty-seven patients who did not respond to medical therapy underwent surgical treatment. Our findings indicate that the more conservative surgical procedures--endolymphatic sac surgery, cochleosacculotomy, and streptomycin perfusion--are all as effective as and less destructive than labyrinthectomy for controlling vertigo. The clinical results of this study would seem to support the observations of others that DEH and Meniere's disease are related disorders caused predominantly by cases of viral labyrinthitis with unknown etiology.
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ranking = 10.728686405962
keywords = labyrinthitis
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6/29. Otorhinolaringologic manifestation of smith-magenis syndrome.

    smith-magenis syndrome (SMS) is a multiple congenital anomaly/mental retardation (MCA/MR) syndrome link to a contiguous-gene deletion syndrome, involving chromosome 1 7p 11.2,whose incidence is estimated to be 1:25,000 livebirth. SMS is characterised by a specific physical, behavioural and developmental pattern. The main clinical features consist of a broad flat midface with brachycefaly, broad nasal bridge, brachydactily, speech delay, hoarse deep voice and peripheral neuropathy. Behavioural abnormalities include hypermotility, self-mutilation and sleep disturbance. This report defines the otorhinolaryngological aspects of a new case of SMS, confirmed by cytogenetic-molecular analysis, in a 9 year old girl affected by chronic otitis media, deafness and sinusitis, who presented with typical clinical signs and symptoms.
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ranking = 0.2
keywords = otitis
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7/29. hearing loss of acquired syphilis: diagnosis confirmed by incudectomy.

    syphilis, both congenital and acquired, may cause sensori-neural hearing loss. Congenital syphilis presents in two forms: early (infantile), and late (tardive). Acquired syphilis, both secondary and tertiary, may cause deafness. The clinical course of the acquired and congenital forms are similar. Sensori-neural hearing loss with low discrimination scores may affect both ears simultaneously or sequentially, and vestibular complaints are common. A clinical diagnosis can be made on the basis of history, the presence of other stigmata of syphilis, and serological tests, including the FTA-ABS. The histopathology of acquired and congenital forms is indistinguishable and is essentially twofold: first, syphilis may cause a miningo-neurolabyrinthitis with round cell infiltration of the labyrinth and VIIIth nerve as the predominant lesion in early congenital syphilis and the acute meningitides of secondary and tertiary acquired syphilis. Second, syphilis may cause a perivascular round cell osteitis of the temporal bone with secondary involvement of the membranous labyrinth in both late congenital and acquired late latent or tertiary syphilis. endolymphatic hydrops may be seen in both the congenital and acquired forms. A case of bilateral sequential sudden deafness due to acquired syphilis is presented. A histological diagnosis of syphilitic involvement of the temporal bone was made by incudectomy. Treatment with prednisone and penicillin over a three-month period resulted in return of good cochlear function in one ear.
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ranking = 10.728686405962
keywords = labyrinthitis
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8/29. Recurrent extrusion of cochlear implant at an interval of 5 years.

    OBJECTIVE: To describe two different types of device extrusion occurring in the same patient 1 and 6 years after cochlear implantation and to discuss the relation between the two extrusions and problems of cochlear implantation in patients with chronic suppurative otitis media. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENT AND INTERVENTIONS: The patient was a 52-year-old man with profound hearing loss in the right ear caused by chronic otitis media and deafness in the left ear caused by removal of an acoustic neuroma. Surgery for cleaning the tympanic cavity and reconstruction of the bony canal after previous radical mastoidectomy was performed in the right ear first. One year later, cochlear implantation was performed in this ear. RESULTS: One year after implantation, the anterior edge of the internal device of cochlear implant was exposed along the skin incision. This site was stable after corrective surgery. Five years after this operation, however, the posterior edge of the internal device extruded, accompanied by infection around the device. CONCLUSION: The two extrusions occurred with a long interval in between and at two different sites. The first extrusion occurred along the skin incision, and the second extrusion occurred away from the skin incision with infection around the device. The authors assumed that the first and the second extrusion occurred independently; the first extrusion was caused by insufficient blood supply resulting from scar formation from repeated mastoid operations and by pressure on the skin from the internal and external devices, and the second extrusion was caused by proliferated tissue pushing the device upward and against the skin as a result of chronic middle ear infection. The clinical course indicates that sufficiently long follow-up periods are needed to determine the usefulness of the prevention techniques for cochlear implantation in patients with chronic suppurative otitis media.
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ranking = 0.6
keywords = otitis
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9/29. osteopetrosis of temporal bone with blockage of the eustachian tube. A case report.

    osteopetrosis is an inherited disorder characterized by bone sclerosis. One of the more predominant feature of the disease in the temporal bone is conductive hearing loss due to anomalous bone formation in the middle ear and to otitis media. A 55-year-old woman affected by the adult type of osteopetrosis was referred to our department with a long history of otitis media. CT study demonstrated a narrowing of eustachian tube due to abnormal deposition of sclerotic bone; this condition was confirmed during surgery. Tubotomy was performed during tympanoplastic surgery and there were no relapse of otitis after 12 months of follow up. Timpanoplastic surgery should be considered in those cases where medical treatment has failed in dealing with otitis media. In our experience an enlargement of eustachian tube shuld be performed in this kind of patients, thus helping the functional recovery and the potential well-being of the patient as we observed in our case.
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ranking = 0.8
keywords = otitis
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10/29. Niikawa-Kuroki (Kabuki) syndrome with congenital sensorineural deafness: evidence for a wide spectrum of inner ear abnormalities.

    hearing loss, mainly due to recurrent otitis media, has been reported in approximately 40% of individuals with Niikawa-Kuroki (Kabuki) syndrome (NKS). Sensorineural hearing loss leading to congenital or prelingual deafness has been described rarely. We have identified two unrelated individuals with Niikawa-Kuroki syndrome among 535 probands who have severe to profound sensorineural deafness. Bilateral absence of the cochlea with dilated dysplastic vestibule and unilateral enlarged vestibule were demonstrated in these two individuals. In conclusion, Niikawa-Kuroki syndrome should be kept in mind when evaluating an individual with congenital deafness and a wide spectrum of inner ear abnormalities occurs in this syndrome.
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ranking = 0.2
keywords = otitis
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