Cases reported "Hearing Loss, Conductive"

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1/94. Fibrous dysplasia of the temporal bone and maxillofacial region associated with cholesteatoma of the middle ear.

    Fibrous dysplasia of the temporal bone is a rare disease which may lead to progressive stenosis of the external auditory canal and the development of cholesteatoma. We present a case in which minimal symptoms were present despite a massive temporal bone fibrous dysplasia. cholesteatoma resulted most probably secondary to external auditory canal stenosis. Retroauricular fistula developed as a result of destructive effect of cholesteatoma, that influenced previous diagnosis and treatment of this clinically silent disease.
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2/94. A patient-benefit evaluation of unilateral congenital conductive hearing loss presenting in adulthood: should it be repaired?

    OBJECTIVE: To evaluate subjective and objective outcomes of surgical therapy in congenital conductive hearing loss (HL) presenting in adulthood. The impact of hearing restoration on quality of life (QOL) for these patients may differ from those with acquired HL. STUDY DESIGN: Retrospective review of five patients treated surgically. methods: Standard audiometry was completed. patients completed a subjective questionnaire as well as subjective (open-set) questions. A seven-point Likert scale was used for closed-set responses with 1 representing the worst possible outcome and 7 being best. RESULTS: The average four-frequency improvements in pure-tone average and air-bone gap were 18 dB (range, 7-29 dB) and 16 dB (range, 2-30 dB), respectively. An average score was obtained for each closed-set question. The highest-scoring items were for overall improvement of QOL (average score, 6), followed by perceived improvement in hearing (average score, 5.6). Other positive responses were improved ability to use the telephone (four of five patients) and improved localization of sound (four of five patients). Negative subjective responses included prolonged dysequilibrium in two of five, prolonged adjustment period in three, and dysgeusia in one patient. CONCLUSION: Important components of outcome are likely to escape our review if only objective measures are used to evaluate surgical outcomes. These results suggest that improved hearing in this population provides specific improvements in lifestyle and is perceived as a significant improvement in overall QOL.
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3/94. malleus ankylosis: a clinical, audiometric, histologic, and surgical study of 123 cases.

    OBJECTIVE: malleus ankylosis is a cause of conductive hearing loss that can be difficult to assess, particularly in association with otosclerotic stapes fixation. The aim of this study is to seek the clinical and audiometrical features unique to this pathology. STUDY DESIGN: The study design was a review of malleus ankylosis cases and an analysis of functional results after ossiculoplasty. SETTING: The study was performed at Jean Causse Clinic in france. patients: The authors reviewed a series of 123 ears in 112 patients who underwent surgery for incudo-mallear ankylosis from January 1991 to September 1997. Intervention: The surgical technique depends on the type of pathology encountered. In case of associated stapedial fixation, a stapedotomy with vein graft interposition and reconstruction with a total prosthesis will be performed during a same step. MAIN OUTCOME MEASURES: Clinical evaluation, preoperative and postoperative audiometrical evaluation, operative findings, histologic examination, and postoperative functional results. RESULTS: In our series, a postoperative air-bone gap smaller than 10 dB was obtained in 77% of cases. These results confirm the possibility of managing both pathologies in a single surgical step. CONCLUSION: Incudo-mallear ankylosis remains an unusual pathology but should be systematically assessed during surgery and preferably after separation of the incudo-stapedial joint. A preoperative diagnosis is difficult to ascertain; some audiometrical features allow a suspicion. In this series, it is shown that otosclerosis can be responsible for ankylosis as seems to be confirmed by the two cases presented in this study.
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4/94. Neurotologic manifestations and treatment of multiple spontaneous tegmental defects.

    OBJECTIVE: To describe the causes, histopathologic features, manifestations, and treatment of symptomatic multiple spontaneous tegmental defects. STUDY DESIGN: Retrospective review of three clinical cases and one temporal bone histopathology report. CLINICAL FEATURES: Varied, including spontaneous cerebrospinal fluid otorhinorrhea, conductive hearing loss, chronic headaches, pneumocephalus, extradural abscess, and meningitis. A notable common feature was multiple (8-15) tegmental defects, 1 to 6 millimeters in diameter. Three of the four cases also included associated dural defects and small meningoencephaloceles or arachnoid granulations. Imaging studies generally underestimated the number of defects. INTERVENTION AND OUTCOMES: Successful middle cranial fossa repair with temporalis fascia was accomplished in the three clinical cases. Extension of exposure anteriorly and medially was necessary. Closure of the defects with a bone graft or equivalent synthetic material was not always possible, given the anatomic and pathologic features. Our data suggest that there are both congenital and acquired causes of the tegmental dehiscences. CONCLUSIONS: Multiple tegmen defects constitute a special entity. Successful repair requires a middle fossa craniotomy with extended exposure.
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5/94. Congenital absence of the long process of the incus.

    OBJECTIVE/HYPOTHESIS: To review the case reports of three generations of one family, suffering from bilateral conductive hearing loss due to congenital absence of the long process of the incus. STUDY DESIGN: review of the literature regarding hereditary congenital absence of the long process of the incus to determine its mode of inheritance such as autosomal dominant or X-linked. Also document the types and results of surgical repairs performed by previous investigators of this defect and compare them with the present study. methods: Three female patients of direct lineage. Mother, daughter, and granddaughter, who were personally seen, operated on, and followed up over a period of 26 years. RESULTS: All three female patients presented with similar symptoms and findings of hearing impairment since birth, with no history of ear infections, and with normal appearing tympanic membranes. However, they had severe conductive hearing loss with normal bone conduction and discrimination. Operative findings were identical in all three patients, with the incus ending in a blind stub, and with absence of the capitulum of the stapes. The type of ossicular reconstruction varied with the changing of times and advances in otologic surgery, as well as the prosthetic materials used. All three patients received improvement in their hearing, and this improvement has been maintained over the 26-year span of the study. CONCLUSIONS: hearing loss due to congenital absence of the incus is uncommon but not rare. Interestingly, no other instance of inherited cases were reported in the literature. In this family the defect appears to be an inherited trait either due to an autosomal dominant mutation or an X-linked dominant inheritance. This abnormality is amendable to surgery, and any well-executed technique usually gives good hearing results.
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6/94. Glial lesion of the infratemporal fossa presenting as a soft tissue middle ear mass - rudimentary encephalocele or neural crest remnant?

    We report about ectopic glial tissue of the skull base and the parapharyngeal space presenting as a soft tissue mass in the middle ear. An 11-year-old boy presented with bilateral conductive hearing loss since early childhood. The history included previously removed lesions consistent with ectopic neuroglial tissue of the tongue and the parapharyngeal space soon after birth, as well as surgery for cleft palate. High resolution computed tomography of the petrous bone and magnetic resonance imaging were useful in identifying the skull base defect and in characterizing the lesion's relation to the brain. There was no clinical, radiological or surgical evidence of any associated dural defect. The lesion was removed via a modified infratemporal approach. histology revealed neuroglial tissue with calcifications without any signs of mesodermal or entodermal origin. On the basis of this case the pathogenesis and diagnosis of ectopic brain tissue and its relation to the more commonly encountered meningoencephalic herniations are reviewed. Furthermore therapeutical implications are discussed.
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7/94. Inner ear malformations and hearing loss in linear nevus sebaceous syndrome.

    OBJECTIVE: Linear nevus sebaceous syndrome (LNSS) is a rare disorder comprising of nevus sebaceous, seizures, and mental retardation. While extensive literature describe the dermatologic, neurologic, as well as ophthalmologic manifestations of this LNSS, otologic problems have not been previously described. The objective of this report is to describe the otologic manifestation of a patient with LNSS. methods: A child with LNSS was referred to the Otologic clinic for evaluation of hearing loss. Pertinent findings on history, physical findings, audiometric testing, and imaging studies are discussed. RESULTS: Audiometric testing showed bilateral conductive hearing loss. Computerized tomography of the temporal bone demonstrated widened internal auditory canals and dysplastic lateral semicircular canals. CONCLUSION: LNSS can be associated with hearing impairment and inner ear malformations. The evaluation of a child with LNSS should include otologic and audiologic testing.
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8/94. Histopathology of residual and recurrent conductive hearing loss after stapedectomy.

    HYPOTHESIS: Histopathologic examination of temporal bones from patients who had undergone stapedectomy may provide information concerning the causes of both residual and recurrent conductive hearing loss (CHL). BACKGROUND: Although closure of the air-bone gap to within 10 dB occurs in approximately 90% of primary stapedectomies, a residual CHL occurs in approximately 10% and recurrent CHL may occur in up to 35% of cases. Putative causes of failure of surgery as determined during revision include erosion of the incus, bony regrowth at the oval window, and displacement of the prosthesis. Most reports on the histopathologic findings of temporal bones from such patients have focused on complications of surgery, with little attempt to correlate postoperative air-bone gap with the observed histopathology. methods: A retrospective review of the author's collection of temporal bones ascertained 22 cases with postoperative CHL of 10 dB or greater (air-bone gap averaged at 500, 1,000, 2,000, 3,000, and 4,000 Hz, using postoperative air- and bone-conduction levels) after stapedectomy. These temporal bones were prepared by standard methodology for light microscopy. RESULTS: Of the 22 cases with postoperative CHL equal to or greater than 10 dB, there were 19 with residual CHL, 2 with recurrent CHL, and 1 with both residual and recurrent CHL. The most common histopathologic correlates of residual and recurrent hearing loss included resorptive osteitis of the incus (64%); obliteration of the round window by otosclerosis (23%); the prosthesis lying on a residual footplate fragment (23%); the prosthesis abutting the bony margin of the oval window (18%); adhesions in the middle ear (14%); and new bone formation in the oval window (14%). CONCLUSIONS: Histopathologic examination of temporal bones from patients who in life had undergone stapedectomy provides useful information concerning causes of both residual and recurrent CHL. These data provide a basis for improving both surgical technique and prosthesis design.
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9/94. Mature teratoma of the middle ear.

    OBJECTIVE: The authors report a case of mature teratoma of the middle ear in a 3-year-old girl with a 1-year history of otitis media. methods AND RESULTS: Radiologic investigation revealed a partially cystic lesion of the petrous portion of the right temporal bone. It produced opacification of the middle ear as well as destruction of septal air cells. The patient underwent a subtotal petrosectomy. Histologically, the tumor was composed of an intimate admixture of mature tissues representing all three germ layers, including brain, myelinated nerve trunks, skeletal muscle, bone, immature cartilage, seromucinous glands, and respiratory epithelium. Of note within the brain tissue was choroid plexus within an ependyma-lined rudimentary ventricle. Immunohistochemical studies were also performed. Twenty months after surgery, the patient was well, with complete recovery from symptoms. CONCLUSION: Teratomas of the middle ear are rare neoplasms. Only a few examples have been reported. As a rule, they are cured by resection and do not require adjuvant therapy.
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10/94. Goldenhar's syndrome: congenital hearing deficit of conductive or sensorineural origin? temporal bone histopathologic study.

    BACKGROUND: Oculoauriculovertebral dysplasia (OAVD) (Goldenhar's syndrome) is a congenital syndrome with ipsilateral deformity of the ear and face, epibulbar lipodermoids, coloboma, and vertebral anomalies. Goldenhar's anomaly has often been associated with a degree of congenital hearing deficits, almost always of a conductive origin, but a sensorineural component is also suspected in some cases, evident through malformations of the inner ear. patients AND methods: Both temporal bones of a 10-day-old deceased patient with oculoauriculovertebral dysplasia were examined by light microscopy. RESULTS: The ear deformities included deformity of the auricle, atresia of the external auditory canal, and malformation of the tympanic cavity and ossicles. Abnormalities of the stria vascularis and the semicircular canals were also demonstrated. Further inner ear deformities were not identified in this case. CONCLUSION: These histopathologic findings appear to confirm the conductive component of the congenital hearing deficit, but a sensorineural component could not be omitted. The ear alterations favor early developmental field defects. The causes of this condition are controversial. Recent results in genetic research pertaining to the MSX class genes permit better understanding of the variety, variability, and different degrees of severity of the anomalies described here.
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