1/33. Study of systemic lupus erythematosus in temporal bones. Despite some reports of sensorineural hearing loss with systemic lupus erythematosus (SLE), its pathologic correlate has remained unidentified due to the scarcity of human temporal bone studies. We here present findings in 14 temporal bones from 7 patients with SLE, examined histologically and immunohistochemically for pathologic conditions in the cochlea that might relate to their otologic histories. Blue-staining concretions were seen in the stria vascularis of 6 ears. Most of the cases showed a loss of spiral ganglion cells, with various degrees of hair cell loss and atrophy of the stria vascularis. One ear demonstrated formation of fibrous tissue and bone throughout the cochlea, with complete loss of the membranous labyrinth. Cochlear hydrops was found in only 1 ear. These findings in temporal bones from patients with SLE are discussed in relation to autoimmune disease of the inner ear. ( info) |
| The vestibular-evoked myogenic potential (VEMP) is expected to be one of the examinations for saccular function. We report a case of dysequilibrium possibly due to a saccular disorder. A 27-year-old female came to our clinic complaining of severe recurrent dysequilibrium spells for few hours on September 21, 1998. The first attack was only vertigo with bilateral tinnitus for 8 days before. She did not show possible vestibular findings on a routine vestibular test. An electrochochleogram showed the dominant negative summating potential (SP) on both sides, and the furosemide test showed negative findings on both sides. A VEMP test indicated a low amplitude in the right ear, which improved to the normal response after furosemide injection. The amplitude was increased by furosemide administration even in the left ear. These findings suggested bilateral endolymphatic hydrops in the cochlea and saccule especially on the right side. The VEMP test may reveal saccular hydrops, which differs from the conventional Meniere type vertigo. ( info) |
| We compared auditory and vestibular function between a patient with typical Cogan's syndrome and a patient with atypical Cogan's syndrome. Repeat audiograms demonstrated fluctuating sensorineural hearing loss in the affected ear. Brainstem auditory evoked response testing revealed no abnormalities. Hallpike caloric testing showed a decreased response in the affected ear in the typical case and responses within normal limits in the atypical case. Sinusoidal harmonic acceleration was normal in the typical case, and there was an abnormal phase at 0.01 Hz in the atypical case. endolymphatic hydrops was investigated by electrocochleography. In both patients, there was an enhancement of the summating potential, with an increased ratio of summating potential to action potential amplitude. This finding is consistent with endolymphatic hydrops. ( info) |
4/33. Clinical study of elderly patients with Meniere's and related diseases. In order to study the characteristics of elderly patients (70 or over at onset) with endolymphatic hydrops disorders, we selected six patients with Meniere's disease (MD), five patients with delayed endolymphatic hydrops (DEH), and two patients with syphilis of the labyrinth (SL) from 7112 patients who had visited the Neurotological Clinic of the Toyama Medical and Pharmaceutical University Hospital during 1979-1998. Clinical features were characterized as follows: (1) Since 1990, the proportion of elderly patients increased as lifespans lengthened. (2) A narrow fluctuation in hearing, low positive rate on glycerol test, high positive rate of dominant-SP in EcochG, and the moderate abnormal rate of the oculomotor system tests were found. (3) The prognosis was relatively good. From these neurotological observations, it could be concluded that elderly patients with endolymphatic hydrops disorders were characterized by the multipathological finding of the endolymphatic hydrops and the complications of CNS disorders. ( info) |
5/33. Delayed endolymphatic hydrops: study and review of clinical implications and surgical treatment. Delayed endolymphatic hydrops (DEH) differs from Meniere's disease in that it occurs in pre-existing ear pathology in patients who have a profound unilateral or total deafness that was caused by infection, trauma, or unknown causes during childhood or adulthood. We performed a retrospective review of 160 patients with ipsilateral, contralateral, or bilateral DEH. Eighty-seven patients who did not respond to medical therapy underwent surgical treatment. Our findings indicate that the more conservative surgical procedures--endolymphatic sac surgery, cochleosacculotomy, and streptomycin perfusion--are all as effective as and less destructive than labyrinthectomy for controlling vertigo. The clinical results of this study would seem to support the observations of others that DEH and Meniere's disease are related disorders caused predominantly by cases of viral labyrinthitis with unknown etiology. ( info) |
6/33. Spontaneous intracranial hypotension: a rare cause of labyrinthine hydrops. Spontaneous intracranial hypotension should be considered as a possible cause of cochlear hydrops. We report a case of unilateral hearing loss attributed to spontaneous intracranial hypotension on the basis of characteristic abnormalities seen on magnetic resonance imaging. The diagnostic gold standards for intracranial hypotension are lumbar measurement of cerebrospinal fluid pressure and magnetic resonance imaging. The usual treatment is an autologous blood injection into the peridural spaces. The mechanism of hearing loss is thought to involve secondary perilymph depression due to a patent cochlear aqueduct. This perilymph depression would induce a compensatory expansion of the endolymphatic compartment, with a subsequent decrease in basilar or Reissner's membrane compliance. endolymphatic hydrops can occur in the course of intracranial hypotension, and not only because of abnormal endolymph production or resorption. Hydrops can thus be classified into 1) syndromes of endolymphatic origin and 2) syndromes of perilymphatic origin, in which loss of perilymph induces compensatory expansion of the endolymphatic space. ( info) |
7/33. Changes in the Tullio phenomenon and the fistula sign in the course of endolymphatic hydrops. We performed electronystagmography with caloric stimulation and studied vestibular evoked myogenic potentials (VEMPs) associated with changes in the Tullio phenomenon and the fistula sign during the clinical course of a patient with endolymphatic hydrops. The Tullio phenomenon and the fistula sign disappeared in association with a reduction in the caloric response, which implicates the ampullary function of the lateral semicircular canal. Even when no VEMP could be detected for the affected ear, the Tullio phenomenon and fistula signs were observed; thus, either these phenomena had a lower response threshold than the VEMPs, or saccular receptors were not involved in the Tullio phenomenon. ( info) |
8/33. Three cases of cochleosaccular endolymphatic hydrops without vertigo revealed by furosemide-loading vestibular evoked myogenic potential test. OBJECTIVE: To describe possible cases of cochleosaccular endolymphatic hydrops without vertigo. STUDY DESIGN: Retrospective case report. SETTING: University hospital. patients: Three patients with possible cochleosaccular hydrops without vertigo were studied. The basis of diagnosis was positive result of the furosemide-loading vestibular evoked myogenic potential test, no canal paresis in the caloric test, and recurrent cochlear symptoms or fluctuating low-tone hearing loss. CASE REPORT: In case 1, a 47-year-old woman had recurrent left aural fullness and tinnitus and a few weeks later complained of a floating sensation and could not stand up. The furosemide-loading vestibular evoked myogenic potential test showed a positive result in the left ear. In case 2, a 24-year-old woman complained of a backward falling sensation lasting several seconds; subsequently, a severe floating sensation persisted and she could not stand up for several days. Audiography showed fluctuating low-tone hearing loss in the left ear, and the furosemide-loading vestibular evoked myogenic potential test showed a positive result. In case 3, a 41-year-old woman had a floating sensation while walking and subsequently complained of tinnitus in the left ear. She could not stand up because of a severe floating sensation and, moreover, complained of a sudden falling sensation lasting for several seconds. The furosemide-loading vestibular evoked myogenic potential test indicated a positive result in the left ear. CONCLUSIONS: The patients in cases 2 and 3 complained of a short-lasting sensation of falling down. Severe disequilibrium that prohibited standing up was noted in all cases. It was suggested that these symptoms were caused by saccular hydrops. ( info) |
9/33. noise exposure and its effect on the labyrinth, Part I. According to the author, the assumption is made that in Meniere's disease, compensation should be paid in acoustic trauma cases. Meniere's disease is an aftereffect of noise exposure, even if the time lapse exceeds 10 years. A historical review of existing literature (from 1872 to 1958) shows no convincing evidence of this matter. Ten patients were chosen at random. All were exposed to noise and presented with vestibular dysfunction at a certain time. ( info) |
10/33. Drop attacks in elderly patients secondary to otologic causes with Meniere's syndrome or non-Meniere peripheral vestibulopathy. Many neurologists are unaware of the drop attack that may occur from an inner ear dysfunction especially in elderly. We studied the clinical features and results of quantitative audiovestibular tests in six elderly patients (> or =65 years of age) who presented with drop attacks attributable to an inner ear pathology. Group was divided into Meniere's syndrome (4) or non-Meniere peripheral vestibulopathy (2). Standard dizziness questionnaire and quantitative audiovestibular function testing were performed. Episodes were described as a sudden push to the ground in four or a violent illusionary movement of environment leading to a fall in two. All cases gave a history of prior vertiginous episodes and vestibular testing revealed unilateral caloric paresis. Ipsilateral hearing loss was documented in four cases. Our results suggest that otologic causes should be considered in the differential diagnosis of the drop attack in elderly, even if the symptoms and signs were not consistent with Meniere's syndrome. ( info) |