Some thoughts on peripheral vestibular disorders.
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This is an analysis of 1,024 primary cases of mastoid surgery for cholesteatoma operated upon during a ten-year period at the Otologic Medical Group, Inc. Our philosophy of management of the mastoid in these cases has been as follows: 1) avoid an open mastoid cavity when possible; 2) perform the operation in two stages if necessary; 3) reexplore the mastoid and middle ear for residual cholesteatoma when indicated. One-third of 380 revised cases had residual cholesteatoma, disease left by the surgeon. In 260 cases in which the surgeon felt it unlikely that there was residual disease he found it in 23%. In 4% this residual cholesteatoma was found in the mastoid. The incidence of residual cholesteatoma was higher in children and in planned, as opposed to unplanned, revisions. Residual cholesteatoma was detected in the middle ear more frequently than in the epitympanum, and in the epitympanum more frequently than the mastoid. Indications for, and timing of, the reexploration are discussed. Recurrent cholesteatoma refers to a retraction pocket and must be differentiated from residual cholesteatoma; the causes, prevention and treatment are different. Recurrent cholesteatoma was detected in 5%. The most common complication of the disease was a labyrinthine fistula (10%). Operative facial nerve damage occurred in one case. The most common postoperative complication was graft failure (3%). Intact canal wall tympanoplasty with mastoidectomy should be performed as a two-stage procedure in most cases when used in the treatment of aural cholesteatoma.
The etiology of poststapedectomy reparative granuloma remains obscure. To evaluate the possibility of inadvertently introduced mucous membrane into the vestibule as a possible cause of granuloma formation, this animal study was devised. The stapes was removed from the right ear of four squirrel monkeys and middle ear mucosa placed in the vistibule, the stapes then being replaced. In the left ear, a control operation was done in which only the stapes was removed, then replaced. The animals were sacrificed between the 2nd and 17th day and their temporal bones were prepared and studied. The mucous membrane was identified within the vestibule of all animals. It was well-tolerated and did not evoke the inflammatory response, which would be expected if mucosa was a cause of granuloma formation. The mucosa was still present at 17 days, eventually becoming surrounded by the endosteal lining of the vestibule.
This study compares the results from simultaneous binaural bithermal and alternate binaural bithermal caloric testing of 652 patients with dizziness. Also compared are the abilities of these tests to distinguish between a population of healthy asymptomatic subjects and our patient population. Caloric testing used a closed-loop irrigation system. Eye movements were monitored with electro-oculography. The agreement between the results from the two tests was low. Also, while the simultaneous test was more sensitive, the alternate test was more specific. Using receiver-operator characteristic methodology, it was found that the ability of the alternate test to distinguish between a healthy population and a patient population was superior to that of the simultaneous test. There was no improvement in the ability to distinguish between a healthy population and a patient population when the results from the two tests were combined as compared to using the alternate caloric test alone.
To improve understanding of the pathophysiology of perilymph fistulas, a predictable animal model of a chronic fistula was developed. Our findings suggest that guinea pig fistulas do not remain patent for prolonged periods. By extrapolating these findings to humans, we postulate that the symptoms and signs of perilymph fistula are possibly due not to one prolonged constant fistula, but rather a series of "blowouts" from an inherent congenital or posttraumatic weak spot in either the round or oval window. We feel that a diagnosis of perilymph fistula must be considered in any patient presenting with a Meniere's-like symptom set concomitant with a congenital inner ear deformity or a history of inner ear trauma.
Twenty of 46 guinea pigs with experimental perilymph fistula that showed canal paresis on an ice water caloric test during the first week after operation were studied on the following days to observe the progression and final outcome of the caloric test. Two weeks after operation, 12 animals showed persistent canal paresis, whereas 8 animals developed a normal caloric response. Resolution of the caloric response progressed from canal paresis, via caloric irregularity, to a final return of normal caloric response. Morphological study of animals with recovery of caloric function shows healing of the previously ruptured membrane, with preservation of the contour of the membranous labyrinth in the lateral semicircular duct, and intact sensorineural elements. Recovery of caloric function may serve not only as an important diagnostic sign in patients with healed perilymph fistula, even without surgical proof, but also as an expectation for the recovery of hearing loss.
The authors report two patients with cerebellar infarctions in the territory of the medial branch of the posterior inferior cerebellar artery who had vertigo, spontaneous ipsilesional nystagmus, and contralesional truncal lateropulsion. Although one of the two patients had slight dysmetria, overall signs closely mimicked those of acute peripheral vestibulopathy. The authors suggest that interruption of nodulouvular inhibitory projections to vestibular nuclei may account for the vestibular signs.
We report the clinical features of 22 patients with acquired bilateral vestibulopathy of unknown cause. All had either absent or markedly decreased responses to both caloric and rotational testing. They presented with dysequilibrium and imbalance, worse at night; most reported oscillopsia but none had associated hearing loss or other neurologic symptoms. Nine reported prior prolonged episodes of vertigo consistent with the diagnosis of bilateral sequential vestibular neuritis. Of the remaining 13, none had exposure to known ototoxins or a positive family history. Idiopathic bilateral vestibulopathy is an important cause of progressive imbalance in adults and should be considered even though hearing is normal.
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In recent years, attention has focused on the role of the endolymphatic sac (ELS) and the endolymphatic duct (ELD) in the pathogenesis of endolymphatic hydrops (ELH). Changes have been noted surgically and radiographically by others in the ELS and ELD in patients with ELH. This report summarizes the development of a shark model with which to study the pathophysiology of ELH. The background material and overall results of anatomic, histopathologic, and ultrastructural studies using the model are presented. Possible implications for the clinical handling of ELH as a result of this work is emphasized.
An attempt has been made through this study to establish a tumor suspect pool of 100 patients, each one highly suspect because of a single or usually several strong clinical indicators of a retrocochlear lesion. In turn each patient has been proven tumor negative by virtue of a normal opaque cerebellopontine cisternogram. This has given us a unique opportunity to critically reassess the clinical indications of VIIIth nerve tumor from a reverse or negative viewpoint. All data from these patients has been carefully analyzed in relation to indication for opaque cisternography, clinical symptomatology, vestibular function, final etiologic diagnosis, and the associated special auditory tests, tomograph of the internal auditory canal, the associated vestibular aqueduct findings. This paper parallels our earlier study of 121 surgically proven schwannomas providing a total tumor suspect pool of 221 patients with a comparable tumor positive-tumor negative distribution.
The presence of perilymph fistula has been difficult to determine because of the lack of efficient and reliable testing methods. The condition is suspected on the basis of history alone and confirmed by surgery. This paper details a quick, reliable procedure called the ENG fistula test, using impedance bridge for pressure change and electronystagmography to aid the establishment of nystagmus and dizziness. To evaluate this procedure, a combination of tests were performed, including Valsalva maneuver, tragal compression, and pneumatic otoscopy, which were previously considered helpful in the diagnosis of fistula. Of them, Valsalva maneuver and tragal compression proved inconclusive; pneumatic otoscopy proved to be helpful. In comparison, however, the ENG fistula test proved most valuable, with results surgically confirmed in 90.8% of cases in this series. This study involved 74 patients whose primary complaint was dizziness. Only some patients simultaneously experienced hearing lows. Included are 5 patients whose positive ENG fistula test results and 15 whose negative test results were confirmed by surgery. Selected case histories are presented.
Circulatory disorders, which are well documented in most parts of the human body, are not well documented in the inner ear, although they are expected to occur. It has been previously shown that experimental occlusion of the labyrinthine artery in animals results in severe degenerative changes, fibrosis, and new bone formation in the cochlea. Accordingly, this paper presents presumptive evidence that depriving the human cochlea of its blood supply after surgical removal of an acoustic tumor results in severe degenerative changes that progress to total ossification of the cochlear spaces. This paper also discusses similar changes seen in temporal bones of two patients with sudden sensorineural hearing impairment. Based on these observations, the author concludes that occlusive arterial disease, whether thrombotic, embolic, or spastic, plays a role in some sudden sensorineural hearing losses. Cochlea ossification, detected by polytomography, suggests a vascular etiology of the hearing impairment. Treatment with vasodilator drugs and anticoagulants is justified in these cases until a better diagnostic protocol is developed to eliminate other causes.
The experience at the Mayo Clinic with 18 patients who had Cogan's syndrome is reviewed. Typically, the illness began with systemic symptoms. The ocular and audiovestibular symptoms included ocular discomfort and redness, photophobia, and fluctuating sensorineural deafness with imbalance. Each patient had interstitial keratitis; the ocular symptoms periodically recurred but responded to therapy. Thirteen patients who had fluctuating bilateral sensorineural deafness subsequently suffered total bilateral deafness. Two patients who are unilaterally deaf have good residual hearing. Two other patients regained hearing within normal limits after treatment with corticosteroids. No hearing symptoms were observed in one patient who had severe vertigo and whose condition is stable. Seventeen patients had vertigo: 11 have no labyrinthine function, 1 had absent responses on vestibular testing initially but currently demonstrates good responses, and the 5 others have remained stable with diminished vestibular function. If Cogan's syndrome is diagnosed early and treatment with corticosteroids is initiated promptly, hearing can be stabilized; otherwise, the prognosis for auditory function is poor.
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