Cases reported "Ossification, Heterotopic"

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1/11. 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 = 1
keywords = labyrinthitis
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2/11. Radiologic diagnosis of labyrinthitis ossificans.

    labyrinthitis ossificans is the pathological ossification of the membranous labyrinthine spaces in response to processes which are destructive of the membranous labyrinth or the endosteum of the otic capsule. It has been primarily a histopathologic diagnosis. Complex motion tomography however, allows a detailed view of the osseous labyrinth and permits the diagnosis in the living state. Radiologic documentation of labyrinthitis ossificans is objective evidence of a process destructive of the membranous labyrinth. It supports the likelihood of an absence of cochlear and vestibular function. It alerts the surgeon to the possible obliteration of key inner ear anatomical landmarks.
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ranking = 5
keywords = labyrinthitis
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3/11. External auditory canal closure in cochlear implant surgery.

    OBJECTIVE: To evaluate surgical techniques and complications associated with external auditory canal (EAC) closure in cochlear implant surgery. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center with a large cochlear implant program. patients: Twenty-eight patients (8 adults and 20 children) underwent multichannel cochlear implantation with EAC closure. The follow-up periods ranged between 1 and 10 years. Closure of the EAC was performed in conjunction with implantation of ears with chronic suppurative otitis media or cochlear drill-out procedures for ossification, or for access to the cochlea in patients with abnormal temporal bone anatomy. A modified Rambo technique was used for EAC closure in all but one case. INTERVENTIONS: cochlear implantation with EAC closure and subsequent clinical and radiologic follow-up. MAIN OUTCOME MEASURES: postoperative complications or the development of cholesteatoma in the implanted ear. RESULTS: cholesteatoma developed in the implanted ear in two patients. Breakdown of EAC closure occurred in one of these patients. The details of these patients are reviewed. CONCLUSION: Closure of EAC can be done with relatively low risk. Close and careful follow-up is required for early detection of a developing cholesteatoma.
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ranking = 0.0010535837285133
keywords = otitis
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4/11. Nontypeable haemophilus influenzae meningitis complicated by hearing loss in a 9-year-old hiv-infected boy.

    A 9-year old boy with perinatal hiv infection developed meningitis due to nontypeable haemophilus influenzae. His course was complicated by progressive hearing loss due to labyrinthitis ossificans. Placement of cochlear implant improved hearing thresholds. Nontypeable H. influenzae meningitis and use of cochlear implants have not previously been in hiv-infected children.
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ranking = 1
keywords = labyrinthitis
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5/11. Multichannel cochlear implant and electrically evoked auditory brainstem responses in a child with labyrinthitis ossificans.

    Ossification of the cochlea following meningitis presents a surgical challenge. Electrode mapping, especially in the young child, is difficult given the uncertainty of electrode contact with viable neural elements. This paper reviews surgical technique and the use of auditory brainstem responses to map the electrodes. A 4-year-old child deafened by meningitis at age 20 months had bilateral cochlear ossification by computed tomography. At surgery, a canal wall-down mastoidectomy and closure of the ear canal were performed. A trough around the modiolus was drilled, and the electrode array was placed in it. Post-operatively, the patient gave aversive or no responses to electrode stimulation. To assess electrode function, auditory brainstem responses to individual electrode activation were obtained under general anesthesia. Functioning electrodes could thus be selected for mapping. The patient now responds well to sound.
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ranking = 4
keywords = labyrinthitis
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6/11. Keutel syndrome: a report of four cases.

    We report four new cases of Keutel syndrome. Clinical manifestation included abnormal cartilage ossification, multiple peripheral pulmonary stenosis (PPS), brachytelephalangism, subnormal IQ, repeated respiratory infections, otitis media and hearing loss. All four children have a typical facial appearance and are of consanguineous parents. Father and the fifth offspring exhibit a normal phenotype; the mother has pulmonary stenosis. This observation confirms Keutel syndrome as a distinct autosomal recessive syndrome.
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ranking = 0.0010535837285133
keywords = otitis
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7/11. Labyrinthine ossification: etiologies and CT findings.

    Ossification of the membranous labyrinth (labyrinthitis ossificans) develops as the final result of many inflammatory processes, for example, meningitis, blood-borne septic emboli, middle ear infection, and cholesteatoma. Labyrinthine ossification may also occur as a result of previous labyrinthectomy or secondary to trauma. Seven cases of labyrinthine ossification accompanied by severe vertigo and total hearing loss in the affected ear are discussed. The computed tomographic appearance of varying degrees of ossification, the clinical and surgical circumstances from which this disorder may develop, and the various approaches to labyrinthectomy are described.
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ranking = 1
keywords = labyrinthitis
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8/11. labyrinthitis ossificans.

    Three cases with postinflammatory inner ear sequelae are presented to illustrate unusual histopathologic changes. endolymphatic hydrops without changes in the perilymphatic system was present in one ear following "influenza" meningitis and labyrinthitis ossificans in the contralateral ear. The characteristic histopathological changes of the temporal bones with hematogenic bacterial infection were an extensive labyrinthine ossification associated with a generalized sclerotic change of the whole periotic bone. Bony fixation of the stapedial footplate occurred with the generalized inflammatory process of the otic capsule. Severe and diffuse labyrinthitis ossificans occurred in one case due to tympanogenic inflammation spreading through the round window membrane in the course of suppurative otitis media. A general immunosuppression leading to fatal termination was the apparent factor predisposing to the inner ear complication.
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ranking = 2.0010535837285
keywords = labyrinthitis, otitis
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9/11. Labyrinthine ossificans and cochlear implants.

    We report two clinical and five histopathological examples of labyrinthitis ossificans and discuss the possible cause of this condition. diagnosis can be established by polytomography. The advent of the cochlear implant makes ossification of the cochlea (labyrinthitis ossificans) of more than academic interest, since it requires alteration of the insertion of an electrode into the scala tympani and minimizes the chances of successful stimulation. Hearing losses due to meningitis, advanced cochlear otosclerosis, and trauma are likely to be due to cochlear ossification. Therefore, it is important to rule out this condition by means of polytomography in individuals being considered for cochlear implant surgery.
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ranking = 2
keywords = labyrinthitis
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10/11. pathology of prelingual profound deafness: magnitude of labyrinthitis fibro-ossificans.

    Quantitative histologic studies were performed on 15 temporal bones from eight adult persons who were known to have prelingual bilateral profound hearing loss. The pathologic changes are characterized by severe degeneration of the structures of the cochlear duct, often with degeneration of the vestibular sense organs, causing a reparative host response that features osteoneogenesis and fibrous proliferation followed by retrograde neuronal degeneration. The pathology is consistent with meningogenic bacterial or viral labyrinthitis that occurred subclinically or went undiagnosed. Bone and fibrous tissue are present in varying extent in the scala tympani of 12 of the 15 temporal bones. Six cochleae from four subjects with fibro-osseous proliferation in the scala tympani extending as far as the ascending part of the basal turn have neuronal populations ranging from 963 to 5,355 (mean 2,826, 8% of neonatal normal, 35,500). In three cochleae from two subjects with no fibro-osseous proliferation in any area of the scala tympani the neuronal population ranges from 11,322 to 20,484 (mean 15,438, 43% of neonatal normal). Relative to cochlear implantation, computed tomographic imaging provides a means for determining the extent of fibro-osseous proliferation in the scala tympani, which in turn alerts the surgeon to surgical obstacles to optimal implantation as well as providing a basis for judging the extent of loss of cochlear neuronal population.
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ranking = 5
keywords = labyrinthitis
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