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1/53. Sensorineural hearing loss associated with otitis media with effusion in children.

    Sensorineural hearing loss (SNHL) is known to occur in various types of otitis media. Although the mechanism by which SNHL develops in association with otitis media with effusion (OME) is unknown, several hypotheses have been advocated up to now. We reviewed the clinical records of children with otitis media with effusion (OME) to reveal the association with sensorineural hearing loss. The material consisted of 71 children (119 ears) who were diagnosed as having OME and gave reliable audiograms in our clinic during an 11 month period from February 1997 through January 1998. From these cases those which showed bone conduction loss of 25 dB or higher at any one of the frequencies of 250 through 4 kHz were selected and considered to be cases of SNHL. Eight cases (9%) which had temporary threshold shift (TTS) or permanent threshold shift (PTS) were considered to be etiologically related to OME. The clinical course in each of these cases with SNHL was reviewed and evaluated in detail. We noted that all children with TTS improved completely. The result of this study indicates that we have to be aware of a possible development of SNHL during the course of OME.
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ranking = 1
keywords = otitis
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2/53. Unilateral sensorineural hearing loss and its aetiology in childhood: the contribution of computerised tomography in aetiological diagnosis and management.

    OBJECTIVES: The objective of this study was to identify factors correlated with the CT outcome and to examine the contribution of the CT scan in the aetiological diagnosis and management of unilateral sensorineural hearing loss in childhood. methods: The records of 35 consecutively investigated patients by the audiology Department of Great Ormond Street Hospital between January 1996 and June 1998 were reviewed. The CT results, population sample characteristics, initiation of further investigations after the CT results and management decisions based on the CT results were tabulated and analysed. RESULTS: In a series of 35 consecutively investigated children with unilateral sensorineural hearing loss, 11 CT scans were identified as abnormal. The CT findings were: labyrinthitis ossificans (3), unilaterally dilated vestibular aqueduct (2), bilaterally dilated vestibular aqueduct (2), unilateral deformity of the cochlea ('Mondini') (1), unilateral severe labyrinthine dysplasia (1), unilateral markedly narrow internal acoustic meatus (1), bilaterally dilated lateral semicircular canals (1). The presence of progressive hearing loss was a significant predictor of abnormal CT outcome, while the severity of hearing loss was not. The CT scans offered valuable information regarding the aetiological diagnosis in all cases and, in addition, prompted the appropriate vestibular rehabilitation in three cases, further investigations in four (with dilated vestibular aqueduct) and hearing preservation counselling in two (bilateral DVA) (seven out of 35 = 20%). CONCLUSION: All children with unilateral sensorineural hearing loss should have a CT scan of the petrous pyramids/IAMs performed at some stage, as not only aetiology but also prognosis and management of these cases may be significantly influenced by the CT outcome.
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ranking = 4.3933074803876
keywords = labyrinthitis
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3/53. williams syndrome and the elastin gene in Thai patients.

    williams syndrome (WS) has long been known as a complex disorder of dysmorphic facial features, described as elfin face, mental retardation or learning disability, loquacious personality, and supravalvular aortic stenosis. The etiology is now known to be due to deletion of the elastin gene (ELN) on long arm of chromosome 7. Thai patients were previously reported by clinical diagnosis. This study reports the first two cases of WS with ELN deletion diagnosed by fluorescent in situ hybridization (FISH) technique. Clinically, hyperacusis is a common finding in WS associated with otitis media. Neither of the patients had hyperacusis, but one of them had bilateral sensorineural hearing loss, which to our knowledge, has never been reported.
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ranking = 0.14285714285714
keywords = otitis
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4/53. Wegener's granulomatosis: diagnostic dilemma.

    Wegener's granulomatosis is a systemic disease characterized by a granulomatous lesion that can affect any organ throughout the body. This case-report illustrates the problem posed by a patient presenting with bilateral serous otitis media with marked sensorineural hearing loss, facial nerve paralysis and an atypical serological picture. The importance of early diagnosis and the protocol for the management of a patient with an uncertain diagnosis is discussed. Due to atypical presentations, only a high index of suspicion will ensure an early diagnosis.
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ranking = 0.14285714285714
keywords = otitis
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5/53. facial paralysis in Wegener's granulomatosis of the middle ear.

    A case of Wegener's granulomatosis, which presented as chronic otitis media with facial nerve palsy, is described. early diagnosis is vital if unnecessary surgical exploration is to be avoided. A false negative cANCA may delay the diagnosis, especially in cases of locoregional disease, and a policy of repeated titres should be adopted, if clinical suspicion is high.
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ranking = 0.14285714285714
keywords = otitis
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6/53. Severe audiovestibular loss following ear syringing for wax removal.

    Syringing of the ear is one of the commonest procedures performed for cleaning cerumen from the external auditory canal. Common complications following syringing are pain, external auditory canal trauma and otitis externa. Hearing and vestibular loss are often mentioned as a complication in descriptions of this technique, but we have not been able to find a reported case of such an occurrence. We report one such a case.
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ranking = 0.14285714285714
keywords = otitis
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7/53. Spontaneous recovery of profound post-meningitic hearing loss.

    The timing of insertion of a cochlear implant (CI) in post-meningitic sensorineural hearing loss is, in spite of recent advances in the understanding of the pathogenesis of the condition, still controversial. The danger of labyrinthitis ossificans allows only a little time to decide whether to implant a CI or not. On the other hand the clinician needs to be certain that no residual hearing is present and whether or not the patient will benefit from conventional amplification. A well-documented case of early spontaneous recovery of a profound post-meningitic sensorineural hearing loss in a child is presented and the relevant literature reviewed.
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ranking = 4.3933074803876
keywords = labyrinthitis
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8/53. The value of enhanced magnetic resonance imaging in the evaluation of endocochlear disease.

    BACKGROUND: gadolinium-enhanced magnetic resonance imaging (GdMRI) is routinely used in the evaluation and management of suspected retrocochlear pathology such as vestibular schwannoma. However, its value in the evaluation and diagnosis of cochlear pathology associated with sensorineural hearing loss (SNHL) has been less clear. STUDY DESIGN: Retrospective review of case histories and imaging studies of patients with SNHL and cochlear enhancement on GdMRI diagnosed between 1998 and 2000. RESULTS: Five patients with SNHL who required gadolinium administration to establish the diagnosis of endocochlear disease were identified. Diagnosed lesions included an intralabyrinthine schwannoma, intracochlear hemorrhage, radiation-induced ischemic change, autoimmune labyrinthitis, and meningogenic labyrinthitis. In these illustrative cases, the GdMRI demonstrated intrinsic high signal or contrast enhancement within the cochlea and labyrinth in the absence of a retrocochlear mass. In one patient with meningogenic labyrinthitis, cochlear enhancement on MRI led to prompt cochlear implantation before the potential development of cochlear ossification. CONCLUSION: Our experience suggests that GdMRI plays a crucial role in the diagnosis of cochlear pathology associated with sensorineural hearing loss and may directly impact patient management.
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ranking = 13.179922441163
keywords = labyrinthitis
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9/53. Otologic manifestations of Wegener's granulomatosis.

    OBJECTIVE/HYPOTHESIS: To evaluate the clinical features, treatment, and outcomes of otologic manifestations in Wegener's granulomatosis (WG) treated at Hokkaido University Graduate School of medicine, Sapporo, japan. STUDY DESIGN: We retrospectively reviewed 15 cases of WG with ear involvement. methods: Twenty-five patients with WG were treated at Hokkaido University Graduate School of medicine between 1992 and 2001. Fifteen of these patients had otologic symptoms. We evaluated the clinical course, method of therapy, and outcomes in all cases. Diagnosis of WG was made when the patients had clinical findings and a positive titer of cytoplasmic pattern antineutrophil cytoplasmic antibodies (c-ANCA), or when there were clear histologic findings. We also present three case reports. RESULTS: In 15 cases, the most frequent finding was chronic otitis media. Sensorineural hearing loss was present in 2 patients. In 7 patients whose otologic manifestations were the primary involvement of WG, all were confirmed positive for c-ANCA and were treated with glucocorticoids and immunosuppressive drugs. Three patients who could be treated within 1 month of symptom onset showed marked improvement. CONCLUSIONS: In localized cases, biopsy specimens are often small, and it is frequently difficult to make a histologic diagnosis. The prognosis for hearing was poor when appropriate treatment was not given in the early stages of the disease. Therefore, WG should be included in the differential diagnosis in cases of atypical inflammatory states of the ear. early diagnosis and appropriate treatment are important to prevent irreversible changes in the middle ear and inner ear.
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ranking = 0.14285714285714
keywords = otitis
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10/53. Neuroepithelial cysts of the middle ear.

    Neuroepithelial cysts are lesions of the central nervous system that have previously been reported in cerebral parenchyma, in the spinal cord, and within the ventricles in association with the choroid plexus. We describe 2 cases of neuroepithelial cysts of the middle ear. One was diagnosed after surgery for a retraction pocket and chronic otitis media complicated by an intraoperative cerebrospinal fluid (CSF) leak. The other produced bilateral spontaneous CSF otorrhea and mimicked the presentation of arachnoid granulations. Both cases were verified with immunohistochemical stains. Neuroepithelial cysts, although rare, should be considered in the differential diagnosis of spontaneous CSF otorrhea or an epitympanic mass.
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ranking = 0.14285714285714
keywords = otitis
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