Vestibular habituation and central compensation.
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Delayed endolymphatic hydrops is a disease entity that can be differentiated from Ménière's disease. Typically it occurs in patients who have sustained a profound hearing loss in one ear, usually from infection or trauma, and then after a prolonged period of time develop either episodic vertigo from the same ear (ipsilateral delayed endolymphatic hydrops) or fluctuating hearing loss, also sometimes with episodic vertigo, in the opposite ear (contralateral delayed endolymphatic hydrops). The ipsilateral form of the disease may be treated by labyrinthectomy but no satisfactory therapy is available for the contralateral form of the disease.
The search for a diagnostic assay in patients with autoimmune inner ear disease has led to the preparation of antigens from the inner ear, a presumed target in this disorder. In order to standardize the antigen preparations currently being used in the Western blot immunoassay, we have examined several distinct extraction procedures that employ well-known solutions and detergents. Results of this investigation clearly show that antigens of interest (68 kd, 33 to 35 kd, and 32 kd) are optimally extracted with a detergent (0.5% sodium dodecyl sulfate) and that bovine serum albumin, a potential contaminant, can be removed in the water-soluble fractions. Purification of inner ear antigens by these methods will lead to more reproducible results in immunoblotting, as well as a greater opportunity to identify the mechanisms involved in autoimmune inner ear disease.
Although aminoglycoside antibiotics have been successful in controlling infections, the side effects of these drugs include vestibulotoxicity that can be irreversible. In a patient with intact visual and proprioceptive systems, the effects can be compensated for, but in a patient with impairment of one of these systems, loss of vestibular function can be devastating. There is little if any agreement in the literature about how to monitor patients undergoing therapy with these drugs. A reading test is described. It uses the ability of the vestibulo-ocular reflex to stabilize an image during head movement. The test is quantified using change in visual acuity with head movement to monitor damage to the vestibular system. Patients with loss of vestibular function are incapable of performing the test. Some evidence points to the test being more sensitive than standard caloric irrigations. We propose that it be used to monitor patients receiving aminoglycoside antibiotics.
Cochleosacculotomy was performed on 25 patients with Meniere's syndrome. Long-term relief of vertigo was obtained in 19 out of 23 (82%). Postoperatively dead ears occurred in three cases. The same operation was performed on 12 guinea pigs in which hydrops had been surgically induced by blockage of the endolymphatic duct and sac. All fistulas were healed and cochleosacculotomy did not decrease or prevent the induced endolymphatic hydrops in these animals. Although histologic confirmation of persistent fistulas in human ears is lacking, the relief of vertigo in patients may not be caused by "drainage" but, rather, by a nonspecific effect on the inner ear. Cochleosacculotomy gives results comparable with other nondestructive surgical procedures performed to suppress vertigo in Meniere's syndrome.
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Temporal bone histopathology was studied in five patients (aged 51-67) who received cis-diamminedichloroplatinum (DDP) chemotherapy for head and neck squamous cell carcinoma. In each case, sensorineural hearing loss occurred during the course of treatment and temporal bones were acquired 3-5 hours postmortem for anatomical study. Scanning electron microscopy revealed acute degenerative changes in cochlear hair cells that appeared to be the result of drug treatment. However, the presence of age-related degeneration made it difficult to unequivocally identify hair cell loss due solely to ototoxicity. In one patient, a decrement in vestibular function was observed during DDP treatment. Postmortem examination showed severe degeneration of the maculae and cristae which could be correlated with the absence of caloric response seen after chemotherapy.
This paper proposes two relatively common "syndromes" seen in children with perilymph fistulas using illustrative case reports. One, a 2 1/2-year-old child with bilaterally symmetric progressive sensorineural hearing loss, was found to have bilateral oval and round window fistulas. The other was a child who presented originally with an unexplained unilateral sensorineural loss. It was only when hearing deteriorated in the opposite ear several years later that a perilymph fistula was suspected and confirmed by tympanotomy. The clinical presentations and other details of these cases diverge widely, but may represent two emerging syndromes of perilymph fistulas in children: 1. a congenital or hereditary predisposition, and the other, 2. sudden hearing loss in the "good" ear of children with an unexplained sensorineural hearing loss in the opposite ear.
The relationship between the vestibulo-ocular reflex (VOR) and gaze fixation was investigated in normal adults, normal children, patients with cerebellar lesion and patients with labyrinthine dysfunction. Large VOR gains were found in patients with cerebellar lesion and normal children, both presenting poor suppression by gaze. Pathological decline in the VOR gain with labyrinthine lesion impaired spatial gaze fixation; however, it did not affect fixation-induced suppression. Gaze effect on the VOR, whether amplifying or suppressing, decreased linearly and rapidly at higher frequencies so that VOR seemed to be no more modified in the light at 2-3 Hz in any subject group. The present study suggested that VOR is controlled even in the dark by both mechanisms to obtain spatial gaze fixation and to gaze at a spatially moving target.
The Pathologic changes in the inner ears of 5 patients with Cogan's syndrome were studied by a combined application of MR imaging and CT scanning. We used T1- and T2-weighted MR imaging sequences and a special three-dimensional MR imaging technique which allows a very detailed imaging of the inner ear structures. By the use of a CT scan simultaneously, calcified and noncalcified obliterations could be differentiated. The aim of the study was to visualize membranous labyrinth pathology in Cogan's syndrome and correlate them with the clinical findings. In one patient who recovered completely without hearing loss, the MR and CT investigations revealed no pathology. In the remaining 4 cases, narrowing or obliteration of parts of the vestibular labyrinth was seen. In 3 patients, we found aberrations in the cochlea consistent with the audiological data. The gadolinium-enhanced T1-images showed enhancement of the cochlea and vestibular labyrinth in one case, probably indicating an active process with interruption of the "blood-labyrinth barrier". The results are consistent with the hypothesis that the inner ear pathology in Cogan's syndrome is probably caused by obstructive vasculitis.
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The association between diving, barotrauma, and the production of perilymphatic fistula has been known for almost 20 years. Forty-eight cases of round and oval window fistulas following diving have been reviewed and essentially corroborate previous findings. Any patient with a history of diving and subsequent sensorineural hearing loss within 72 hours should be suspected of having a round or oval window perilymphatic fistula and surgical exploration and closure of the fistula should be undertaken. Patients who have a loss of hearing, vertigo, nausea, or vomiting following a decompression dive should be re-compressed and if symptoms do not clear, exploration should be performed. Surgical treatment should be executed as soon as possible after the diagnosis is suspected for the best possible results.
OBJECTIVE: To investigate the protein and mRNA expression patterns of apoptosis-related genes, together with evidence of apoptosis, in relation to experimental autoimmune inner ear disease (AIED). METHODS: Male C57BL/6 mice at 4 weeks age (n = 80) were randomly assigned to one of the five group (n = 16). The inbred mice were given a single subcutaneous injection of diluted solution of pertussis and an emulsion containing equal parts of complete Freund adjuvant (CFA) and inner ear antigens (IEAg) extracted form guinea pig. The animals were sacrificed for inner ear examination at a defined time after the immunization (7, 14, 21 or 28 days). An autoimmune inner ear diseases model was established. Apoptosis was detected by terminal deoxynucleotidyl transferase (TdT)-mediated deoxyuridine triphosphate (d-UTP) nick end-laying (TUNEL) method. Using immunohistochemical techniques and reverse transcriptase polymerase chain reaction to clarify the profile of Fas, FasL, and bcl-2. RESULTS: Under normal conditions, no TUNEL-positive cell was observed in the cochlea except for a few positive cells in the supporting cells of Corti's organ and macula sacculi. Inner ear antigens administration induced TUNEL-positive reactions in a wide variety of cells such as inner hair cells, supporting cells, stria vascularis and spiral ligament fibrocytes. No positive staining was evident in outer hair cells, spiral ganglion cells and Scarpa's ganglion cells during the whole period. Fas proteins were expressed in a wide range of cells in inner ear. The levels of Fas mRNA were no significant differences between normal and AIED mice. FasL and bcl-2 proteins could be detected in spiral ganglion cells and Scarpa's ganglion cells both in normal and AIED mice. FasL positive cells increased in number in inner ear of AIED mice. bcl-2 positive cells were not detectable in inner hair cells, stria vascularis and spiral ligament both in normal and AIED mice. The mRNA of three kinds of apoptosis-related genes was detectable in the normal and AIED mice. FasL mRNA was expressed at low levels in normal, being maximal at 14 d post inoculation and decreased gradually to steady levels by 2 weeks. The levels of bcl-2 mRNA increased significantly during the period of AIED. CONCLUSION: Apoptosis mediated by Fas/FasL signal system may play a role in the initiation and maintenance of AIED. bcl-2 has a crucial role in the regulation of the process of apoptosis in the inner ear of AIED mice.
One hundred cases of head injuries were submitted to thorough repeat neuro-otological examinations. The results were evaluated with respect to the diagnostic importance of vestibular symptoms. Positional nystagmus was found to be the most common symptom (51%). In 25% of the cases, spontaneous nystagmus could be demonstrated up to two years following head injury. Pathologic caloric responses were found in only one-third of the patients. Positional and positioning nystagmus were found to be the most important symptoms of a vestibular disorder following head injury, and seem to be the most reliable criteria for the assessment of disability and working capacity.
Otospongiosis of the otic capsule is a primary focal disease, which can be reproduced by both conventional multidirectional tomography and computed tomography. The final stage of otospongiosis, otosclerosis has a complete different appearance in radiography. When the otospongiosis focus is located at the oval window a progressive conductive hearing loss will result, which in its final stage will have the character of otosclerosis with fixation of the stapes in the oval window. The radiographic diagnosis with either conventional multidirectional tomography or CT is more or less pathognomonic and will easily confirm the audiologic examinations results. The diagnosis of otospongiosis by computed tomography is advantageous to conventional tomography. With both methods high resolution technique is mandatory for safeguarding both diagnosis and evaluation of the extent of the lesion. Minor otospongiotic or otosclerotic lesions of the oval window with fixation of the footplate are still a challenge to high resolution radiographic imaging. In most cases stapedial otosclerosis is advantageously diagnosed by otoscopy and audiometry. High resolution radiographic imaging is a valuable confirmative method to corroborate clinical diagnoses and differentiating the disease from other conditions based on other morphologic lesions.
Study is performed on 133 patients with vestibular disturbances with no data for inflammatory diseases. Their age group is from 30 to 60, divided into three clinical syndromes: peripheral, central and combined. The otoneurological examination is carried out after the classic clinical scheme; and the blood test is taken in the morning before breakfast. It was established some tendencies between the rate of the pathological changes in the blood protein formula, the duration of the disease and the age. Considerable dynamic changes are registered in young patients, tendency for relative hypoalbuminemia, relative hypergammaglobulinemia etc. The changed coefficient albumin/globulins which in our patients is under the average norm is analyzed. The low values of this coefficient respond to the observed tendency for hypoalbuminemia in patients with vestibular disturbances.