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1/10. Management of brain herniation and cerebrospinal fluid leak in revision chronic ear surgery.

    OBJECTIVES/HYPOTHESIS: brain herniation and cerebrospinal fluid (CSF) leakage into the middle ear and mastoid are rare but described complications of chronic ear disease. This paper will discuss the presentation and management of brain herniation and/or CSF leak encountered in revision chronic ear surgery. STUDY DESIGN: Retrospective chart review. methods: Twelve of 1,130 cases of revision chronic ear surgery in which brain herniation or CSF leak was diagnosed were identified and analyzed. RESULTS: Ten (83%) patients' initial diagnosis was tympanic membrane (TM) perforation with cholesteatoma and two (17%) with TM perforation without cholesteatoma. Initial revision procedures included one (8.3%) tympanoplasty with canal-wall-up mastoidectomy maintaining ossicular continuity, two (17%) tympanoplasties with canal-wall-down (CWD) mastoidectomies with ossicular chain reconstruction (OCR), and nine (75%) tympanoplasties with CWD mastoidectomies without OCR. Three (25%) required a second procedure, two (17%) a third, and one (8.3%) a fourth, finally resulting in four (33%) with an ossicular reconstruction and eight (67%) without. brain herniation and/or CSF leak were repaired by way of transmastoid and middle fossa approaches. Preoperative and postoperative pure-tone average air-bone gaps were statistically similar (33.1 and 28.1 dbHL, respectively; P = .464). CONCLUSIONS: brain herniation and/or CSF leak appear to be rare complications of surgery for revision chronic ear disease. Their management require adherence to the principles of establishing a safe ear with hearing restoration as a secondary goal.
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ranking = 1
keywords = cholesteatoma
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2/10. fistula of the cochlear labyrinth in noncholesteatomatous chronic otitis media.

    OBJECTIVE: To discuss the clinical aspects and management of promontory fistula of the cochlear labyrinth. STUDY DESIGN: Case report and review of the literature. SETTING: University hospital, tertiary referral center. PATIENT, INTERVENTION, AND RESULTS: The authors describe an unusual case of cochlear fistula localized to the promontory discovered during tympanoplasty for noncholesteatomatous chronic otitis media in a 59-year-old woman. bone conduction was slightly impaired after operation and hearing improved after a revision myringoplasty performed for reperforation. CONCLUSION: Erosion of the bone of the labyrinth can also be observed in noncholesteatomatous otitis media. The presence of a fistula is not always associated with profound hearing loss. Overlying pathologic tissue can be removed without damaging the membranous labyrinth.
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ranking = 3
keywords = cholesteatoma
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3/10. incidence of cholesteatoma with cleft palate.

    The overall incidence of cholesteatoma in iowa has been estimated to be 6.01/100,000 population, or less than 0.01%. However, in patients with cleft palate seen at the University of iowa cleft palate Clinic between 1947 and 1968 and followed for at least 10 years, the risk of developing cholesteatoma was 9.2%. In order to determine whether this high risk of cholesteatoma is still valid, a comparable retrospective study was undertaken of 153 patients with cleft palate who were born between 1969 and 1977, were enrolled in the University of iowa cleft palate Clinic within 1 year of birth, and were followed by the interdisciplinary team for a minimum of 10 consecutive years. cholesteatoma occurred in four patients (2.6%), a substantial decrease in the risk of developing cholesteatoma in a well-defined and longitudinally followed population.
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ranking = 4
keywords = cholesteatoma
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4/10. Bilateral congenital cholesteatoma: a cause or result of chronic otitis media with effusion?

    Bilateral congenital cholesteatoma in children is unusual, and our experience with a case arising in a 6-year-old boy is described. Various theories of the origin of this disease are briefly reviewed. The possibility that this entity may be more common than previously realized, and its relationship to eustachian tube obstruction and middle ear effusions, are also discussed.
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ranking = 2.5
keywords = cholesteatoma
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5/10. cholesteatoma with intact tympanic membrane: a report of 41 cases.

    Aural cholesteatoma may present itself as an unexplained hearing impairment without either perforation or otorrhea and without radiologic evidence of bone destruction. The etiology is probably congenital. In our cholesteatoma cases, 3.7% occur in individuals with an intact tympanic membrane. Most of these patients have had the disease 5 years or less and 50% are under 20 years of age. Complications of the disease are uncommon in this group. All but 1 of our 41 cases were managed with the intact canal wall technique. Most were reexplored (planned second-stage) and residual disease was encountered in 32%. One should keep the possibility of a congentital cholesteatoma in mind wheneger encountering a unilateral serous otitis media or an unexplained unilateral conductive hearing impairment, regardless of the patient's age.
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ranking = 1.5
keywords = cholesteatoma
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6/10. Ear disease in patients with Wegener's granulomatosis.

    From a group of 60 patients with histologically-proven Wegener's granulomatosis managed at the National Institute of Allergy and Infectious disease, approximately 45% were found to have disease that involved the ears. The majority of these patients had either recurrent or persistent serous otitis, resulting from eustachian tube dysfunction as a consequence of nasopharyngeal inflammations. Other pathologies included suppurative otitis, cholesteatoma, facial nerve paralyses, temporal bone granulomata, and sensory hearing losses. The presentation and management of these changes and their relationships to underlying disease are described in selected case reports, and a general philosophy of patient management is presented.
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ranking = 0.5
keywords = cholesteatoma
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7/10. Tubotympanoplasty.

    Prognostic value of the eustachian tube function test remains controversial. To prevent ear drum adhesion or the recurrence of cholesteatoma it is important to select cases with severe organic obstruction of the eustachian tube and to use surgical procedures to restore function. We summarize here the indications and the surgical procedure for organic obstruction of the eustachian tube, especially in the region of the tympanic orifice. Adequate preoperative evaluation of eustachian tube function and pertinent surgical procedures will pave the way for a better prognosis for patients subjected to ear surgery.
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ranking = 0.5
keywords = cholesteatoma
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8/10. neuroma of the facial nerve masked by chronic otitis media.

    The absence of facial twitching, weakness, or palsy makes the diagnosis of facial neuroma difficult. The authors report a case of neuroma of the horizontal portion of the facial nerve masked by the presence of a chronic ear. A woman with a long history of discharge and hypoacousia in her left ear presented with acute dizziness. Examination revealed grade 3 horizontal right nystagmus, left anacousia, and the appearance of an epitympanic cholesteatoma. Computed tomography (CT) was performed after the vestibular condition improved. The clinical diagnosis of chronic otitis media with cholesteatoma together with the radiologic finding of the mastoid and tympanic cavity completely occupied by soft tissue were enough to send the radiologist astray. The radiologic diagnosis confirmed that the bony destruction of the vestibule and lateral semicircular canal could be caused by a cholesteatoma. A neuroma of the horizontal portion of the facial nerve was discovered during surgery performed for the chronic ear. The postoperative study of the CT scans showed that there was no erosion of the malleus or incus, despite wide erosion of the vestibule and lateral semicircular canal. This finding would be enough to suggest the presence of pathology other than cholesteatoma. The patient refused exeresis of the neuroma. The authors recognize the difficulty in urging a patient to an operation that surely will result in worsening of the facial function. Follow-up in this case has revealed no change in tumor dimension or facial function over 3 years.
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ranking = 2
keywords = cholesteatoma
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9/10. lateral sinus thrombosis and cervical abscess complicating cholesteatoma in children: case report and review.

    To call attention to complications of chronic otitis media, the case of a patient who simultaneously developed lateral sinus thrombosis and Bezold's abscess is reported. A 7 year old boy presented with fever, drowsiness, cervical mass and otorrhea not responding to medical management. work-up revealed lateral sinus thrombosis and cervical abscess secondary to right ear cholesteatoma. After treatment with surgery and antibiotics, he had a favorable outcome. We review the literature regarding the diagnosis and management of these complications and concluded that although less frequent they remain a clinical challenge.
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ranking = 2.5
keywords = cholesteatoma
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10/10. osteoma with cholesteatoma in the external auditory canal.

    We report an unusual case of a 13-year-old girl with a benign osteoma associated with a cholesteatoma in the external auditory canal and serous otitis media. The osteoma was located in the antero-inferior wall of the right external auditory canal. A cholesteatoma was present between the osteoma and the tympanic membrane. Computed tomography revealed a soft tissue density within the external auditory canal and in the middle ear cleft. The shadow in the middle ear cleft was considered to represent the serous otitis media. Surgical removal of the osteoma and cholesteatoma proved successful, and no recurrences or complications have occurred in the first year postoperatively.
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ranking = 3.5
keywords = cholesteatoma
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