1/21. cochlear implantation for auditory rehabilitation in Camurati-Engelmann disease.Camurati-Engelmann disease (progressive hereditary diaphyseal dysplasia) is a rare sclerotic bone disease involving the diaphyses of the long bones, skull base, and clavicles. Progressive sclerosis of cranial nerve foramina has been implicated in cranial nerve deficits. including facial nerve palsy, vestibular disturbances, and hearing loss. Two patients with Camurati-Engelmann disease and concomitant sensorineural hearing loss are presented. Both patients were evaluated for cochlear implantation. One patient was successfully implanted after preoperative imaging revealed no involvement of the internal auditory canals. The porous nature of the affected bone, however. necessitated the inactivation of 1 electrode to prevent facial nerve stimulation. A second patient was rejected as a potential implant recipient due, in part, to narrow internal auditory canals and rapidly progressive disease. The otologic manifestations of Camurati-Engelmann disease are reviewed, and issues related to cochlear implantation in this rare disease are discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/21. Conservative facial nerve management in jugular foramen schwannomas.OBJECTIVE: Although transposition of the facial nerve is crucial in infiltrative vascular lesions involving the jugular foramen, the objective was to show that a conservative approach to management of the facial nerve is sufficient with jugular foramen neuromas because of their noninfiltrative, less vascular nature and medial location in the jugular foramen. STUDY DESIGN: Retrospective case review. SETTING: Tertiary, private, multiphysician, otologic practice. patients: Sixteen patients with jugular foramen schwannoma (18 procedures) treated between January 1975 and October 1995. The 8 male and 8 female patients ranged in age from 13 to 66 years (mean age 47.7 years). INTERVENTION: One-stage, total jugular foramen neuroma removal without transposition of the facial nerve, using a variety of surgical approaches. MAIN OUTCOME MEASURES: facial nerve transposition (yes or no), House-Brackmann facial nerve grade, lower cranial nerve status, complications. RESULTS: One-stage total tumor removal was accomplished in all the cases. In 13 (72%) of the neuromas, removal was accomplished without facial nerve transposition. Transposition was performed in 2 revision cases in which scar tissue from a previous operation prevented complete control of the carotid artery and safe removal, 2 cases with large tumor extension anteriorly to the petrous apex, and 1 case with extensive involvement of the middle ear. A House-Brackmann facial nerve Grade I or II was obtained in 16 of the 18 procedures, with 1 Grade III and 1 case that remained Grade V, as it was preoperatively. CONCLUSIONS: One-stage, total tumor removal can be achieved with excellent control of the important vascular structures and without transposition of the facial nerve in a majority of jugular foramen schwannomas.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/21. Cochlear deafness in a Chinese family with Fechtner's syndrome.OBJECTIVE: To identify the nature of the hearing impairment in the members of a Chinese family with Fechtner's syndrome. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. patients: A Chinese family with a variant of Alport's syndrome: high-tone sensorineural hearing loss, proteinuria, macrothrombocytopenia, and ocular disease. INTERVENTIONS: The diagnosis of Fechtner's syndrome was confirmed by the characteristic ultrastructure of the Dohle-like inclusion bodies in the neutrophils of the mother and her three children. Pure-tone audiometry, evoked response audiometry (ERA), and distortion product otoacoustic emissions (DPOAE) were performed in two subjects to investigate the hearing impairment. MAIN OUTCOME MEASURE: The parameters of the ERA and DPOAEs were correlated. RESULTS: In both subjects, the ERA was within normal limits, and there were no measurable DPOAEs in frequencies >2 kHz. CONCLUSION: The hearing loss in Fechtner's syndrome is cochlear rather than neural.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/21. Cogan's syndrome: organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature.Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms. Typically, young adults suffer from interstitial keratitis and sudden onset of tinnitus and hearing loss. Few cases (around 150) have been published and thus it is difficult to determine the percentage of patients with underlying systemic disorders such as systemic vasculitis. The variety of systemic manifestations is large and includes fever, splenomegaly, lymphadenopath, and musculoskeletal complaints. systemic vasculitis can be seen in around 10% of cases and may involve the large vessels, appearing as Takayasu-like vasculitis with affection of the aortic valve but also the coronary arteries and the small kidney vasculature. Evaluating the exact extension of the systemic features determines the choice of treatment. While corticosteroids have proved to be of short-term benefit, long-term treatment with immunosuppressive drugs is controversial. Auditory function in deaf patients has often been restored successfully with cochlear implants. To illustrate the nature of the syndrome, we present two patients with a wide clinical spectrum of symptoms from local disease restricted to the eyes and ears to a widespread vasculitis affecting arteries of the brain, kidney and the upper and lower extremities. We then review the typical aspects as well as the etiology of the disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/21. Vascular occlusion in the endolymphatic sac in Meniere's disease.In 2 patients with severe Meniere's disease (MD), there was histologic evidence of occlusion of the vein of the vestibular aqueduct (VVA). This finding coincided with total or partial occlusion of numerous small vessels around the endolymphatic sac (ES), flattening of epithelium, extensive perisaccular fibrosis, and signs of new bone formation. Ultrastructural analysis of the occluding material showed foci with dense connective tissue, calcification, lipid deposits, and layers of basement membrane, sometimes concentrically arranged. The exact nature of the occluding material was unknown. In another 2 MD patients, the VVA was not visualized, and the ES vessels showed no signs of occlusion. Seven controls with acoustic schwannoma or meningioma had normal vasculature. The presence of vascular impairment in the ES in MD patients indicated that altered hemodynamics may contribute to the pathogenesis of endolymphatic hydrops and MD.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/21. CT and MR imaging characteristics of intravestibular lipoma.BACKGROUND AND PURPOSE: Intracranial lipomas are uncommon congenital malformations that most often are asymptomatic. A rare subset of intracranial lipomas arises within the vestibule of the inner ear, which can cause sensorineural hearing loss. We present the CT and MR imaging characteristics of lipomas within the vestibule of the inner ear and propose a pathogenetic mechanism for this malformation. methods: We retrospectively reviewed five cases of vestibular lipoma. T1-weighted axial conventional spin-echo and T2-weighted axial fast spin-echo sequences were performed in all five cases. Four patients underwent T1-weighted fat-saturated imaging, and one underwent CT scanning with Hounsfield units measured. RESULTS: Four female patients ranging in age from 10 to 40 years and one 26-year-old male patient had sensorineural hearing loss. Two patients had progressive high-frequency loss; one, sudden-onset high-frequency loss; and two, congenital high-frequency loss. All had lesions within the vestibule that displayed hyperintensity on T1-weighted images, isointensity to slight hypointensity on T2-weighted fast spin-echo images, hypointensity with fat saturation, and fat attenuation on CT scans. Three of the cases were associated with lipoma of the cerebellopontine angle. In none of these cases were the cerebellopontine angle and labyrinthine lipomas contiguous. CONCLUSION: Intravestibular lipoma is considered when a focal hyperintense lesion is identified within the vestibule of the inner ear on T1-weighted MR images. Lesion hypointensity on fat-saturated T1-weighted MR images verifies its lipomatous nature and separates it from intralabyrinthine hemorrhage or highly proteinaceous fluid.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/21. Audiologic profiles of children with Kawasaki disease.Kawasaki disease (KD) is an idiopathic vasculitis associated with systemic inflammation and profound immunoregulatory changes. Recent reports from japan and the united states have documented the association of sensorineural hearing loss (SNHL) with acute KD. To further characterize the nature and prevalence of this complication, we prospectively evaluated the hearing of 40 consecutive patients with acute KD at a single institution. Standard audiometric procedures were used, including visual reinforcement audiometry and play audiometry. Auditory brainstem response (ABR) testing using clicks and tone pips (1000-4000 Hz) was performed in patients with abnormal or unreliable results on behavioral audiometry. Acoustic immittance measurements were obtained on all patients. Of the 23 males and 17 females (mean age 3.2 /- 2.3 years, range 0.6-11.1 years), all but three were evaluated and treated with aspirin and intravenous gama globulin within 1 month of onset of fever. Seven children had test results suggesting sensorineural threshold shifts, 16 had normal hearing, and 14 had inconclusive hearing evaluations. Laboratory data in patients with hearing threshold shifts revealed significantly longer duration of fever (4.1 /- 1.0 versus 1.9 /- 0.5 days), and a tendency for higher temperatures and white blood cell counts at diagnosis compared to those with normal hearing. Results suggest that transient as well as persistent SNHL may be associated with the acute vasculitis of KD, and may be associated with laboratory markers indicating more severe systemic inflammation. Audiologic screening should be considered for all patients following KD.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/21. Evolution of lesions in susac syndrome at serial MR imaging with diffusion-weighted imaging and apparent diffusion coefficient values.BACKGROUND AND PURPOSE: susac syndrome is a rare disorder consisting of encephalopathy, hearing loss, and retinal arteriolar occlusions. The purpose of this study was to evaluate the evolution of lesions in this disease by using serial MR imaging with diffusion-weighted imaging (DWI) and apparent diffusion coefficients (ADCs). Abnormalities in the nonlesional white matter (NLWM) were also analyzed. methods: Serial MR and DWI findings in two patients with susac syndrome were reviewed retrospectively. ADCs of the lesions and the NLWM were compared with values of the corresponding anatomical regions in 16 control subjects. RESULTS: T2-weighted images, DWIs, and fluid-attenuated inversion-recovery (FLAIR) images demonstrated diffuse small hyperintense lesions predominantly involving the corpus callosum, white matter, cerebral cortex, and deep gray structures. During the whole course in the two patients, 437, 295, and 113 lesions were depicted on FLAIR images, T2-weighted images, and DWIs, respectively. With the aggravation and mitigation of the clinical symptoms, the size and number of the lesions changed over time. Of 65 lesions with measured ADCs, six had restricted ADCs (5.29-6.91 x 10(-4) mm(2)/s), and 29 had elevated ADCs (8.02-13.5 x 10(-4) mm(2)/s). With disease progression, ADCs in the NLWM changed from normal to elevated; this corresponded to the diffuse signal-intensity change seen in the white matter. CONCLUSION: FLAIR imaging is the most sensitive sequence for detecting lesions of susac syndrome. DWI is useful in demonstrating the heterogeneous nature of lesions, depicting occult abnormalities in the white matter, elucidating underlying pathologic processes, and conducting patient follow-up.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/21. Tympanic electrocochleography: normal and abnormal patterns of response.Electrocochleography has been widely used in human and animal studies of endolymphatic hydrops. A variety of response patterns have been reported in normal and hydropic ears. Recent clinical studies have focused almost exclusively on the amplitude ratio of the summating potential (SP) and action potential (AP) derived from alternating polarity click responses. In this report normal response patterns are described with a tympanic membrane electrode employing condensation, rarefaction and alternating polarity clicks and tone burst stimulation. A variety of response abnormalities are described in patients with suspected endolymphatic hydrops. The exclusive use of alternating polarity clicks is not adequate to reveal the nature of these abnormalities.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/21. endolymphatic sac tumor located around semicircular canals.We report a case of endolymphatic sac tumor (ELST). A 48-year-old female had recurrent and slowly progressive hearing loss, accompanied with dizziness like Meniere's disease. A tumor was located around the semicircular canals, and was detected on CT and MRI. The patient underwent total removal of the tumor using a transmastoid approach. Histopathological examinations agreed with features of an ELST. The tumor was highly suspected to have originated from the rugose portion of the endolymphatic sac or the endolymphatic duct, based on surgical and imaging studies. Structure of the membranous labyrinth was preserved regardless of the existence of the tumor around semicircular canals with bone destruction. ELSTs seem to have an osteolytic or osteophilic nature, by examining patterns of tumor infiltration.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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