Cases reported "Hearing Disorders"

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1/13. cholesteatoma: an unusual presentation.

    A case is described of an extensive cholesteatoma presenting as a lump on the side of a patient's head overlying the squamous temporal bone, with erosion of the underlying bone and intracranial extension. The patient was otherwise asymptomatic. This case highlights the bone-eroding capacity of cholesteatoma and the vigilance required in assessing lumps on the head.
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keywords = cholesteatoma
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2/13. Inner ear cholesteatoma and the preservation of cochlear function.

    Labyrinthine destruction by direct cholesteatoma invasion has always been considered a serious threat to the inner ear function. A number of reports in the literature have cited both patients who had preservation of hearing despite widespread erosion of the labyrinth by cholesteatoma and patients who had retained auditory function despite surgical removal of the matrix from the labyrinth. In most cases the vestibular portion of the inner ear was invaded but cases of cochlear involvement have been described as well. Twelve cases with pre-operative auditory function preservation despite extensive labyrinthine destruction treated at our Institution are reported. Seven cases retained cochlear function post-operatively. Possible explanations of this occurrence and implications of related with hearing preservation in the presence of widespread inner ear destruction by chronic inflammatory disease are discussed.
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keywords = cholesteatoma
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3/13. Congenital cholesteatoma.

    Congenital cholesteatoma of the temporal bone may be encountered by the otologist in the cerebellopontine angle, petrous bone, or middle ear cleft; the latter occurrence seems most likely. The etiology, epidemiologic factors, location of intracranial occurrence, clinical features, methods of diagnosis, differential diagnosis, gross and microscopic pathologic features, and surgical treatment of congenital cholesteatoma are presented. Our personal experience is described. The advantages and complications of various operative approaches are discussed.
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ranking = 3
keywords = cholesteatoma
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4/13. cholesterol granuloma of the petrous apex.

    cholesterol granuloma of the petrous apex is a newly recognized clinical entity distinct from cholesteatoma. cholesterol granuloma usually produces symptoms of trigeminal, facial, and abducens nerve palsy and may not affect the auditory nerve. The characteristic computerized cranial tomographic (CCT) finding is a nonenhancing, smooth-walled, expansile lesion that is isodense with brain tissue in a patient with bilaterally well pneumatized mastoids and petrous apices. Treatment is limited to drainage of the cyst and insertion of a stent for permanent aeration of the petrous apex. Of ten cases of cholesterol granuloma of the petrous apex recently diagnosed at the Otologic Medical Group, the correct preoperative diagnosis was made in nine patients on the basis of CCT findings. Six of the nine patients had serviceable hearing preoperatively; serviceable hearing was maintained postoperatively in five patients.
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ranking = 0.5
keywords = cholesteatoma
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5/13. cholesteatoma with extension to the cochlea.

    cholesteatoma invading the membranous labyrinth has been regarded as a serious threat to inner ear function. Thus, a so-called dead ear might be created either by labyrinthine invasion of the cholesteatoma itself or by surgical intervention. A number of reports, however, have contradicted these "established facts," citing patients who have had some degree of hearing in the affected ear before as well as after surgical removal of the cholesteatoma. In the majority of these cases, however, only the vestibular labyrinth was involved, leaving the cochlea unaffected. In the few case reports describing actual invasion of the cochlea, hearing was lost in the affected ear either as a direct consequence of the operation or after an interval of some months. These findings might indicate that the two parts of the inner ear, to some degree, may be functionally separate so that cochlear function could be retained in spite of partial vestibular ablation. We report three cases of intracochlear cholesteatoma. All affected ears had some degree of auditory function before surgical treatment. All three patients presented with partial or total facial nerve palsy on the affected side. After surgery none of the patients had any hearing in the operated ear. The pathophysiologic significance of a functioning cochlea that has been proved to be invaded by cholesteatoma will be discussed. The criteria for correct preoperative management as well as for choice of treatment will also be discussed.
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ranking = 2
keywords = cholesteatoma
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6/13. Fibrous dysplasia of the temporal bone: the use of computerized tomography.

    Fibrous dysplasia of the temporal bone is a rare condition characterized histologically by proliferation of fibrous tissue with scattered trabeculae of immature bone. Eighteen cases of monostotic fibrous dysplasia of the temporal bone have been reported in the literature. The clinical course of temporal bone fibrous dysplasia is unpredictable. Potential complications include cholesteatoma, recurrence, and malignant transformation. Surgery has been the recommended treatment, but the indications, approach, and extent have not been clearly established. The introduction of computerized tomography with high-resolution bone reconstruction is a significant advance in the therapeutic approach to temporal bone fibrous dysplasia. It accurately defines the extent of the disease within the temporal bone, and periodic scanning will reveal any progression. This information can be used to resolve many surgical dilemmas and to minimize secondary complications. This article includes a comprehensive review of the literature on temporal bone fibrous dysplasia and summarizes a case in which computerized tomography was used.
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keywords = cholesteatoma
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7/13. cerebrospinal fluid otorrhea.

    Cerebrospinal fluid (CSF) otorrhea is a dangerous and potentially life threatening occurrence for which the otolaryngologist is often consulted. CSF otorrhea occurs on the basis of skull fracture, tumor, infections, congenital anomalies, and operative trauma. Forty-three patients with CSF otorrhea of varied etiology are reviewed in this paper. Eight cases are of congenital or labyrinthine origin confirming at surgery the probable connection between the subarachnoid and perilymphatic spaces. Eleven cases had spinal fluid otorrhea due to infection. All cases presented with symptoms of chronic infection: 4 cases had a history of previous surgery for chronic ear disease; 7 cases had temporal lobe abscess; 1 case had a cerebellar abscess; 8 had tegmen defects secondary to cholesteatoma; in 1 case the tegmen defect was due to previous surgery for chronic infection. Nine of 11 cases have serviceable hearing postoperatively. Fourteen cases of spinal fluid otorrhea resulted from trauma: 1 case was due to traumatic stapes footplate fracture in a congenitally malformed ear; 4 were due to transverse temporal bone fracture; and 9 were due to longitudinal temporal bone fractures. All transverse fractures resulted in nonhearing ears. Three cases were due to a combination of temporal bone fracture and infection. In 2 of these cases chronic infection preceded the fracture; in 1 case the fracture led to chronic ear disease with spinal fluid leakage. One patient required 1 surgical procedure for closure of the otorrhea, 1 patient 2 procedures, and 1 patient 3 procedures. Ten cases are due to translabyrinthine acoustic neuroma removal: 7 cases had resolution of the spinal fluid leakage after conservative nonsurgical treatment; and 3 required surgical intervention using muscle, fat and fascia obliteration of the spinal fluid pathway.
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ranking = 0.5
keywords = cholesteatoma
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8/13. Treatment of labyrinthine fistula with interruption of the semicircular canals.

    Evaluation of postoperative hearing acuity and equilibrium was performed in eight patients with labyrinthine fistula caused by cholesteatoma, in which at least one of the semicircular canals (five cases, lateral; one case, superior; one case, posterior; and one case, both lateral and superior) was interrupted during eradication of the matrix and granulations from the semicircular canals. The interrupted semicircular canals were obliterated firmly with autologous materials such as fascia, perichondrium, bone chips, and cartilage. The observation period ranged from 9 months to 3.3 years. Postoperative hearing was unaltered or improved in seven patients, and decreased by 12 dB in one patient. Postoperative disequilibrium lasting more than 2 weeks was experienced in two patients and disappeared at the second and fifth postoperative months, respectively. Relief from fistula symptoms was complete after surgery, indicating adequacy of this procedure in one-stage open-method tympanoplasty. The present study indicates that manipulation of the semicircular canal with awareness can be conducted without damaging the cochlear function, and that the treatment of labyrinthine fistulas should be performed very carefully but not so conservatively as to lead to future problems. In some cases of deep fistulas of the semicircular canals, interruption and/or obliteration of the semicircular canals can be the most proper procedure.
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ranking = 0.5
keywords = cholesteatoma
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9/13. How a labyrinthine fistula may sometimes be useful for surgery.

    The author reports a case of chronic otitis with cholesteatoma complicated by a fistula of the lateral semicircular canal and a stapedo-ovalar ankylosis, probably of otosclerotic origin, in which the decision to take advantage of the fistula to create conditions similar to those generally produced by labyrinthine fenestration allowed virtual functional recovery of the patient's hearing. The case illustrates how, with a ploy of this kind, certain intrinsically negative features may actually turn out to be a blessing in disguise.
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ranking = 0.5
keywords = cholesteatoma
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10/13. Congenital cholesteatoma: review of twelve cases.

    This report reviews 12 cases of congenital cholesteatoma of the middle ear, mastoid, and petrous pyramid seen mostly in adult and older juvenile patients between 1984 and 1992. Featured herein are two unique instances of purulent discharge external to an intact tympanic membrane via a fistula caused by extensive mastoidal erosion, three cases that illustrate the importance in Oriental populations of the use of high resolution computed tomography to obviate misdiagnosis when all other diagnostic techniques suggest unilateral otosclerosis, and two instances of multicentric cholesteatomas in well-separated portions of the same temporal bone associated with ossicular anomalies of possible congenital origin. The predilective sites and extent of involvement in this series reinforces the pattern for this age group noted by others. That is, although most of the lesions were too diffusely invasive to define possible formative sites, those that were relatively small and discretely located were found at locations other than the anterior tympanum. This would suggest that their sites of origin and pathogeneses may be different from those commonly found in the anterosuperior tympanum of very young children.
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ranking = 3
keywords = cholesteatoma
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