Cases reported "Ear Diseases"

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1/56. Salivary gland choristoma of the middle ear: case treated with KTP laser.

    Salivary gland choristoma of the middle ear is rare. It consists of non-malignant, non-growing, normal salivary gland tissue in the middle ear. It is a developmental abnormality that occurs around the proximal part of the second branchial arch before the fourth month of intrauterine life. The authors found the 25th recorded case in our centre and another 24 reported cases from a review of the literature between 1961 and 1999. Intratympanic salivary gland choristoma frequently occurs during the first and second decades of life and with a female preponderance (56 per cent). Nearly all the patients (96 per cent) in our review presented with a hearing loss, that had begun since birth, in infancy, or during childhood. tinnitus (28 per cent), and serous otitis media (24 per cent) were also commonly present. One case complained of otorrhoea. Intratympanic and extratympanic anomalies were found in 96.2 per cent and 34.6 per cent of cases respectively. Of these anomalies, ossicular chain (88.5 per cent), facial nerve (65.4 per cent), middle-ear muscles (30.8 per cent) and labyrinthine windows (23 per cent) were the four most common sites. Therefore, salivary gland choristoma may represent a manifestation of a congenital ear anomaly. Diagnosis of salivary gland choristoma is generally not documented pre-operatively, but is based on surgical biopsy and histopathological investigations. Treatment of this rare lesion depends on the size, location and extent of the mass, degree of anatomical abnormality and expertise of the surgeon. In difficult cases where the mass is attached to the dehiscent or inferiorly placed facial nerve, only biopsy is recommended. However, complete surgical removal is advocated for a mass that is easy to remove. KTP laser use via a 200 micron fibre-optic light carrier can facilitate removal especially in cases with ossicular chain involvement.
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ranking = 1
keywords = otitis
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2/56. Acute mastoiditis and cholesteatoma.

    Acute coalescent mastoiditis is an uncommon sequela of acute otitis media. It occurs principally in the well-pneumatized temporal bone. The findings of fever, pain, postauricular swelling, and otorrhea are classic. cholesteatoma, on the other hand, being associated with chronic infection, usually occurs in the sclerotic temporal bone. The signs and symptoms are isidious in nature and consist of chronic discharge and hearing loss which result from its mass, bone erosion, and secondary infection. Of 17 consecutive cases of acute mastoiditis over a six-year period, four were atypical because they were complications of chronic otitis media and cholesteatoma, yet they had the physical findings of acute mastoiditis-subperiosteal abscess and purulent otorrhea, plus radiographic evidence of mastoid coalescence.
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ranking = 2
keywords = otitis
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3/56. Wegener's granulomatosis: presentation as an otologic problem.

    Presentation of Wegener's granulomatosis as an otologic problem is unusual and leads to incorrect diagnosis and unsuccessful therapy. In seven years, five patients with a diagnosis of otitis media failed to respond to the usual therapeutic regimens. All had surgical manipulations on the middle ear or mastoid, or both. Wegener's granulomatosis was suspected because of persistence of the otologic problem. All patients developed pulmonary lesions and had positive diagnoses by biopsy of either the lung, kidney, or upper respiratory mucosa. Withe timely treatment, all patients have survived.
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keywords = otitis
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4/56. Wegener's granulomatosis presenting with bilateral facial nerve palsy.

    Primary manifestation of Wegener's granulomatosis in the mucosa of the middle ear is rather rare, and has been reported as presenting with serous otitis media, chronic otitis media, sensorineural hearing loss, and, in rare instances, unilateral facial palsy. Bilateral facial palsy has never been reported. This last fact constitutes the interest in our report of a 23-year-old female patient who presented with symptoms of recurrent bilateral otitis media, eventually developing sensorineural hearing loss and bilateral facial palsy. Soon thereafter neurological symptoms appeared and lung extension was noted. Histological examination of repeated biopsies taken from the nasal and middle ear mucosa was not conclusive for the suspected disease, and c-ANCA titers were also initially repeatedly negative. Eventually, positive lung biopsy and elevated c-ANCA titers when the patient had developed pulmonary granulomas confirmed the diagnosis of Wegener's granulomatosis. Mastoid surgery with facial nerve decompression of the most severely afflicted side did not result in the recovery of facial nerve function. Medical therapy with corticosteroids and cyclophosphamide improved the clinical picture but were ineffective in improving the bilateral sensorineural hearing loss and the facial paralysis on the operated side. We would contribute to the literature a unique case of bilateral facial nerve palsy due to Wegener's granulomatosis.
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ranking = 3
keywords = otitis
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5/56. Tuberculous otitis media -- a diagnostic dilemma.

    Tuberculous otitis media can provide a diagnostic challenge even to the most astute and experienced clinician. The rarity of the condition and its propensity to masquerade as commoner otological conditions further delays diagnosis and treatment. We present the case of a 22-year-old female who presented with chronic aural discharge, unilateral hearing loss and recurrent hemifacial paralysis. The paper highlights the difficulty in diagnosis and stresses the need for a high index of suspicion in cases resistant to the common methods of treatment.
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ranking = 5
keywords = otitis
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6/56. Promontory dehiscence: a case report.

    Dehiscence of the bony promontory without preoperative fistula formation occurred in a patient with a history of chronic otitis media and perforation of the tympanic membrane without cholesteatoma.
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keywords = otitis
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7/56. Congenital cholesteatoma of the tympanic membrane.

    We present two cases of congenital cholesteatoma of the tympanic membrane. Congenital cholesteatoma within the tympanic membrane is a rare entity with only few cases documented. The aetiopathogenesis of this lesion is still unknown. An embryologic origin is hypothesized when cholesteatoma develops in patients without previous history of otitis as in the two cases we report. In cases with previous history of inflammatory process of the external or middle ear an acquired origin is suspected due to the proliferation of the basal cell layer of the tympanic membrane epithelium. Despite the rarity of the congenital tympanic membrane cholesteatoma, we think that its early diagnosis is of utmost importance to allow an easy removal and avoid middle ear involvement.
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ranking = 1
keywords = otitis
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8/56. Ear involvement in ligneous conjunctivitis: a rarity or an under-diagnosed condition?

    conjunctivitis lignosa, a rare affliction of the conjunctiva, is sometimes associated with other disturbances. We present two children with concurrent conjunctivitis lignosa and ear involvement. In these two cases, there were histopathologically verified ligneous changes of the middle ears. Routine haematoxylin and eosin, van Gieson, periodic acid-Schiff (PAS) and alcian blue staining of specimens from the eyes and middle ears revealed findings typical for ligneous conjunctivitis. In addition, new histochemical and immunohistochemical studies for glycosaminoglycans on specimens from the eyes and middle ears showed that the accumulations of the amorphous, cell-deficient material stained strongly but heterogeneously for hyaluronic acid and weakly but uniformly for keratin sulphate. The staining for other glycosaminoglycans, e.g. chondroitin-4-sulphate and dermatan sulphate was confined to vessels and areas rich in collagen fibres and fibroblasts. In patients with conjunctivitis lignosa, the ear involvement may remain undiagnosed due to its resemblance to secretory otitis media with effusion. Since isolated ear involvement may occur, we advocate biopsies for routine haematoxylin and eosin, and specific staining for hyaluronic acid and keratin sulphate, also in children with protracted, refractory otitis media with atypical effusion.
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ranking = 2
keywords = otitis
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9/56. Mucoepidermoid carcinoma arising from the eustachian tube and middle ear.

    We report a case of mucoepidermoid carcinoma (MEC) originating from the eustachian tube and middle ear. A 31-year-old male who presented with otorrhoea and methicillin-resistant staphylococcus aureus (MRSA) in the right ear was admitted to hospital due to cerebral infarction and deep vein thrombosis. After recovery, biopsies from a granulomatous mass found in the middle ear during operation for chronic otitis media revealed intermediate-grade MEC and a nasopharyngeal mass identified after surgery also revealed the same result. He received combined radiation therapy and chemotherapy and no residual or recurrent tumour was detected after two years of follow-up.
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ranking = 1
keywords = otitis
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10/56. 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 = 1
keywords = otitis
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