Estimation of the prevalence of Ménière's disease is important not only for its etiology but also for making medical plans for the number of beds in regional hospitals. In this paper, past epidemiological studies carried out in order to estimate the prevalence of Ménière's disease in Japan are compared and discussed. New methods of estimation of the prevalence of Ménière's disease and vestibular dysfunction are also discussed on the basis of theories of Statistics and Epidemiology.
The characteristics of Meniere's disease (M.D.) in Toyama Prefecture were investigated by a survey of the Medical Association of Toyama Prefecture, survey A, and by a survey of our department, survey B. In survey A, the name, address, sex and onset age of M.D. patients were recorded from 1974 for 3 or 4 year intervals in all hospitals in Toyama prefecture. In survey B, 277 definite M.D. patients treated in our department were investigated epidemiologi-cally. In survey B, the accuracy of diagnosis of M.D. in survey A was included. From the results of survey A, the incidence of M.D. in Toyama prefecture was almost constant in every survey : about 17 per 100, 000. However, as some suspected M.D. patients or those with vertigo without inner ear disorders were included in survey A, the incidence of definite M.D. might be lower than that reported in survey A. In survey A, females with M.D. were significantly more numerous than males in every survey. The sex ratio of M.D. in nation-wide survey was almost even in the 1st survey, 1975-76, however, became female predominant in the 2 nd and 3 rd survey. Therefore, the sex ratio of M.D. in Toyama 10 years ago was different from that reported in the nation-wide survey. In survey B, M.D. was diagnosed in about 5% of all vertigo disease with no difference between rural and urban area in Toyama. In survey B, first attacks of M.D. occurred more often in winter than in other seasons.
In order to clarify the characteristics of the severe Meniere's disease we analyzed various epidemiological factors, such as sex ratio, duration of illness, etc., in a series of 958 patients with a definite Meniere's disease. The data were obtained from the three Japan-wide surveys of Meniere's disease conducted by the Meniere's Disease Research Committee of Japan (1975-76) and the Vestibular Disorders Research Committee of Japan (1982-84 & 1990). All subjects were classified into two groups : severe and non-severe. The former contained patients with bilateral and unilateral disease. Those with unilateral severe disease had suffered from markedly disabling attacks of paroxysmal vertigo for more than 3 years. 1) There were 95 patients with bilateral disease, 238 with unilateral severe disease and 625 with non-severe vertigo. About 40% of the subjects had severe disease. 2) Over half of the patients with definite Meniere's disease were females. (bilateral disease 64% ; unilateral severe disease 52% ; non-severe disease 53%) 3) The average duration of illness in the severe group (bilateral, 7.1 years ; unilateral, 8.5 years) was longer than that in the non-severe group (3.3 years). 4) The incidence of otitis media in the past history was higher in the unilateral severe group than in non-severe group. 5) Changes in the atmospheric pressure and in the emotional state were more likely to trigger vertigo attacks in the severe group than in the non-severe group.
An epidemiological survey of vestibular neuronitis was conducted in Japan with questionnaires. From 1988 through 1990 531 patients in 71 hospitals collected. The results were as follows. 1) There was little difference in the number of patients in each of the three years. The incidence of vestibular nouronictis was a little higher in males than in females. 2) The average age at onset was 44 years for males and 46 years for females. This study has shown the need to discuss the following three points in detail : 1) diagnostic criteria, especially regarding recurrent attacks of vertigo, 2) the advisable diagnostic level of canal paresis, and 3) the advisable length of clinical observation. A further problem is to unify the diagnostic criteria used in Japan with those used in other countries.
In this study we attempt to determine whether the dominant negative summating potential (DNSP) is present during the earlier stages of induced hydrops development. Electrocohleographs (ECoG) were taken 3 days, 4 days, 1 week, 2 weeks, 3 weeks, and 10-20 weeks after obliteration of the endolymphatic sac in guinea pigs with electrodes in the basal turn scala vestibuli. The action potential (AP) thresholds rose and the AP amplitudes decreased with time following surgery. The increases in the summative potential (SP) amplitudes and in the rises in the SP/AP ratios at 95 dB pe SPL in response to clicks tended to be greater 3 and 4 days postoperatively than subsequently. These results indicate that the potential for DNSP exists during the early stages of endolymphatic hydrops formation, before the hydrops is fully developed.
Guinea pigs were exposed to a noise of con-stant intensity 4 months after the experimental production of endolymphatic hydrops in one ear and were examined morphometrically for the degree of atrophy and vascularity of the stria vascularis 1 to 3 months after noise ex-posure. In the animals assessed 1 and 2 months after noise exposure, there was no significant atrophy or diminished vascularity of the stria vascularis on the hydrops side in comparison with the control side. However, 3 months after noise exposure there was significant atrophy and decreased vascu-larity of the stria vascularis on the hydrops side. Animals examined 3 months after noise exposure showed significantly more severe atro-phy of the stria vascularis than did those ex-amined 7 months after the production of endo-lymphatic hydrops but without prior noise ex-posure. In this study, we could produce ab-normalities in guinea pigs-i.e. atrophy and de-creased vascularity of the stria vascularis-simi-lar to those seen in the late stage of Meniere's disease by exposing to noise guinea pigs with endolymphatic hydrops. Microcirculatory dis-turbance of the stria vascularis might be one of the pathophysiological features of Meniere's disease.
We describe the morphological changes of the endolymphatic sac following inner ear immune reactions, and we discuss the mechanisms of endolymphatic hydrops. Hartley guinea pigs were immunized with bovine type II collagen (BIIn), Keyhole limpet hemocyanin (KLH) or horseradish peroxidase (HRP), boosters and challenging antigens were injected through the stylomastoid foramen. Animals were sacrificed every few days up to 56 days after the antigen challenge. Polyethyleneimine (PEI) was used as a cationic tracer to demonstrate the location of anionic sites on the basement membrane. In the animals immunized with BIIn or HRP, mild to moderate endolymphatic hydrops, an infiltra-tion of mononuclear cells and a significant decrease of anionic charge in the sac tissue were observed in the early postimmunization stage. However, the KLH immunization group did not show remarkable tissue changes. The blood-labyrinth barrier descriminates according to size and charge barrier may control antigen transfer and macromolecular transport into the inner ear. Immune reactions in the inner ear may lead to impairment of barrier function, hyperpermeability of the vessels and malab-sorption of endolymph, and subsequently the development of endolymphatic hydrops.
Click-evoked otoacoustic emissions were recorded from 18 patients with Meniere's disease (MD), most of whom had low-frequency hearing loss (LFHL). The main frequencies obtained from patients with MD, where the maximum peak power of the emissions existed on the frequency spectra, were examined and compared with those obtained from patients with LFHL without MD and from subjects with normal hearing. The main frequencies of the emissions in patients with MD, ranged mainly from 0.8kHz to 1.1kHz, -somewhat lower than those in subjects with normal hearing (mainly from 1.0kHz to 1.4kHz). And in MD patients with LFHL, the main frequencies were lower than in patients with LFHL without MD. Furthermore, in 2 patients with MD, the main frequencies became higher when the osmotic diuretics, glycerol or isosorbide were administrated. These findings suggest that in ears with MD, low-frequency dominant emissions are elicited because of a modification of the oscillations of the basilar membrane in endolymphatic hydrops, which is considered to be the main pathological finding in MD. It is possible that the main frequencies of the emissions might rise when endolymphatic hydrops is reduced by treatment with osmotic diuretics.
We examined the sera of patients with Meni-ere's disease or sudden deafness for inner ear autoantibodies using western blotting technique. The SDS soluble extract was prepared as the inner ear antigen from bovine inner ear tissues. Inner ear autoantibodies were found in 60% of the patients with Meniere's disease and in 59% of those with sudden deafness. Six patients had inner ear specific autoantibodies which reacted with 33-35kD determinants. Some patients wiht idiopathic sensorineural hearing loss, such as Meniere's disease and sudden deafness, may have autoimmune reactions directed against proteins derived from the inner ear. The presence of inner ear specific autoanti-bodies may enable the detection and treatment of autoimmune mediated inner ear disorders. It is now necessary to purify the inner ear specific antigens for the accurate diagnosis of autoimmune activity against the inner ear in patients with idiopathic inner ear disorders.
Body sway was examined in 76 patients with Meniere's disease. Power spectra were analyzed by autoregressive models. Power density distributions showed large amplitudes in the whole range in the early stage of vertigo attacks, and they gradually declined from the high frequency levels. Low frequency sway of about 0.2-0.5Hz was manifest in left-right and anterior-posterior body sway. Furthermore, relatively high frequency sway of about 0.8-1.6Hz was present in left-right body sway.
The threshold shifts in AP after exposure to an intense pure tone were investigated in hydropic guinea pigs. The endolymphatic duct and sac were obliterated to induce endolympatic hydrops experimentally in 32 albino guinea pigs. Electrophysiological recording and acoustic overstimulation were performed in the 6th, 12th or 24th postoperative week. A pair of AP thresholds was measured at each frequency from 2 to 16kHz before and after acoustic overstimulation (2kHz, 110 or 120dB SPL, 30minutes), and the threshold shift caused by acoustic overstimulation was determined at each frequency. The following results were obtained : 1) threshold shifts in hydropic guinea pigs were smaller than those reproted by us in 1990 in normal guines pigs ; 2) the frequency at the maximal threshold shift was much higher in hydropic guinea pigs than in normal ones. Our results can be explained on the basis of cochlear hydrodynamics ; namely, the movement of the ba-silar membrne is damped earlier, and the maximal amplitude point of the travelling wave is shifted toward the stapes in hydropic cochlea. This change in the hydrodynamics of the cochlea may explain the mechanism of the development of diplacusis in Ménière's disease.
Guinea pigs with experimental endolymphatic hydrops were treated with glycerol, bifemelane hydrochloride or diphenidol hydrochloride and observed for morphological changes in the affected inner ear. 1. Treatment with any of the three drugs im-proved inner ear circulation. 2. The degree of reduction of endolymphatic hydrops tended to be associated with that of strial atrophy. These results suggest that reduction of endo-lymphatic hydrops depends on strial function.
Acute endolymphatic hydrops was produced by the injection of artificial endolymph into the cochlear duct of guinea pigs. The endocochlear potential (EP) of the basal turn was measured during endolymph volume increase and anoxia. The ears were grouped into untreated, 0.5, 1.0, and 2.0, μl volume increase. The +EP increased only slightly as the volume increased. Anoxic changes in the EP occurred more slowly in acute hydropic ears than in untreated ears. In contrast, the lowest -EPs were -41.2mV in untreated ears, 31.0mV in 0.5μl ears, -23.3mV in 1.0μl ears, and -22.2mV in 2.0μl ears. There were significant differences in the lowest -EP between the untreated group and the acute volume increased groups (p<0.01 ; t-test). The results of this study show that the causes of changes in +EP and -EP are different.
The standing body sway of normal subjects and of patients with unilateral vestibular disorders was measured. The patients were not able to stand with toes and heels together, so the upright position with toes and heels apart was applied. Visual stimulation consisted of clockwise and counter-clockwise concentrically-moving spotlights at constant velocities of 60 and 90 deg/sec. The body sway of the patients was significantly larger during contralateral stimulation by moving spotlights than during ipsilateral moving stimulation, while the sway of normal subjects was symmetrical, increasing with increasing velocity.
The changes in angulation of the shoulders while stepping in patients with unilateral peripheral vestibular disorders were mesured by POLGON and relationship between them and deviations in the Fukuda's stepping test were analyzed. The ratio of average angular change of the shoulder on the affected side to that of the contralateral shoulder was increased in patients with marked deviations in Fukuda's test. There were a significant correlation between the abovementioned ratio and the angle of rotation in Fukuda's stepping test. The coefficient of variation (C.V.) of the changes of shoulder angulation tended to be increased on the affected side, while no correlation was obtained between the ratio of the C.V. on the affected side to that on the intact side one and the angle of deviation. These results suggest that the differences bet-ween the angulation of the shoulders while stepping are closely related to the results of Fukuda's test.
The immunocytochemical distribution of vari-ous cytoskeletal proteins neurofilament (NF) 68 kD, 160kD, 200kD, cytokeratin (CK) 7, 8, 13, 19, vimentin, glial fibrillary acidic protein (GFAP), and microtubule-associated protein 2 (MAP2), was investigated in vestibular endorgans and vestibular ganglia of guinea pigs. NF proteins (NF 68kD, 160kD, 200kD) were found in afferent nerve fibers and nerve terminals which may correspond to the nerve chalice. In vestibular ganglia, NF 68kD and l60kD were predominant-ly distributed in larger cells, whereas NF 200 kD was found in almost all ganglion cells, CK 8 and 19 were found in supporting cells, dark cells of vestibular endorgans. No expression of CK 7 and 13 was detected in the vestibular periphery. Vimentin was observed in the hair cells (which were distributed in the central region of the endorgans), supporting cells, most connective tissue cells, and Schwann cells of the vestibular ganglion. Although much GFAP was seen in glial cells, no immunoreactivity for GFAP was found in the vestibular periphery. MAP 2 was distributed in almost all ganglion cell bodies and their proximal axons. These highly differentiated staining patterns indicate that these cytoskeletal proteins can be utilized as specific markers for each cell type. Furthermore, they may play distinct roles in the different cell types as well. In the present study, to determine the effects of ototoxic drugs, we also examined the expression of ctyoskeletal proteins in strepto-mycin-treated guinea pigs. Immunoreactiveties for NF and vimentin (found in the hair cells) decreased after treatment, but the other cytoskeletal proteins were not affected. These results suggest that neuronal components are the primary site of damage in streptomycin-treated animals.
Viral infection has been suggested to be one of the causative agents of vestibular neuronitis. It is probable that reactivation of herpes simplex virus (HSV) in the vestibular ganglia may lead to vestibular dysfunction, causing vertigo and/or disequilibrium. In this study, rats were innoculated with HS V-I through the middle ear. We first examined the vestibular ganglia in the acute phase with an immunofluorescent (IF) method. We detected viral antigens in some vestibular ganglion cells. Second, we used the polymerase chain reaction (PCR) to detect virus DNA in vestibular gang-lia of rats in the latent phase. We found virus DNA in about 60% of vestibular ganglia on the innoculated side only. Third, we used reverse transcript-PCR (RT-PCR) to detect latency-asso-ciated transcripts (LAT), one of the mRNA of HSV-I . We found LAT in the vestibular gang-lion on the innoculated side only in the latent phase. These data indicate that HSV-I infects vesti-bular ganglia in the acute phase and establishes a latent infection. Only in the latent phase, LA T are transcripted in vestibular ganglia as in trigeminal ganglia.
This report describes the branches of the vertebral artery and the basilar artery, as well as the principal blood vessels of the vestibular nucleus. We studied 27 human brains (17 male, 10 female) using a radiographic three-dimensio-nal method to examine arteries fixed with embalming fluid in the Department of Anatomy of Kawasaki Medical School. The results were as follows: 1. The perforating branches in the brain stem were pontine branches from the basilar artery and small branches from the anterior inferior cerebellar artery, posterior inferior cerebellar artery. 2. It is thought that the vestibular nucleus is supplied by these blood vessels.
The effect of steroids on vestibular compen-sation following unilateral labyrinthectomy was studied in pigmented young rabbits. Seventeen rabbits were injected intravenously with dexa-methasone in doses of lmg/kg and 5mg/kg during the compensation process, and spontaneous nystagmus and head deviation were recorded. The frequency of nystagmus and the angle of head deviation were both dose-depen-dently reduced markedly as compared with control rabitts which were injected with saline in the early compensate stage. It is concluded that steroids accelerate vestibular compensation.
Vestibular hair cells were isolated from the saccular and utricular macula, or crista ampul-laris of the guinea pig by enzymatic and mechanical dissociation. The isolated cells were classified into three types ; flask-shaped type I cells, rod-shaped type II cells and round suppo rting cells. The cilia of type I in the crista were longer than those in the macula, while no morphological differences were noted between these two types of cells. Isolated living vestibular cells are capable of producing selfmovement. After exposure to a medium containing high concentrations of potassium, or to a hypoosmotic (290mosm) medium, the type I cells showed tiliting of the neck portion accompanied by tilting of the hair bundle of about 15 degrees. Given the tight and dense structure of the vestibular epithelium, the changes in shape of the isolated vestibular hair cells may lead in vivo to changes of siffness of the apical region of type I cells.
The temporal bones of seven aged cadavers were examined. Five had had some kind of "vertigo" and two were controls with no history of "vertigo". They had lived in some old pepole's homes. Distention of Reissner's membrane in the basal turn was present in two out of eight cochleae in the "vertigo" cases. Pigmentation granules were more numerous in the area vascularis and the spiralganglion of those two cases than in the controls. The temporal bones of thirty-one old cadavers were examined morphologically during the last six years. Distention of Reissner's membrane was present in twenty-nine out of thirty-seven cochleae of those with "vertigo" and in six out of twelve coch-leae in the control group. The degree of hydro-ps in the cochleae of those with "vertigo" was greater than in the control group. We continue to examine human temporal bones histologi-cally.
The role of proprioceptive input from cervical receptors in vestibular compensation was investi-gated. Vibratory stimulation to the dorsal neck muscle was given to patients with unilateral vestibular lesions and to normal subjects. In normal subjects, the center of gravity shifted forward after vibratory stimulation without any right-left deviation. In patients whose compensation had been achieved, the center of gravity moved to the side of the lesion. From these results, it can be speculated that cervical input plays an important role in the process of vestibular compensation.
Walking motions were analyzed from side views taken with a high speed video camera of 8 young adults and 10 aged persons. To assess postural control during walking, we placed wooden blocks of various heights (3, 5, 10, 15 and 20cm) in the subject's path and instructed him/her to step over the obstacle. 1) The step lenth was much shorter in aged persons than in young adults. In young adult subjects, the step length increased with the height of an obstacle, from 0 to about 15cm, but decreased at 20cm. It is suggested that a change of strategy of step over an obstacle occurs at a certain height of the obstacle. 2) The postural disturbance of the antero-postero sway of the trunk when stepping over an abstacle was much greater in aged subjects than in young adults.
Equilibrium functions are very important for standing, walking and running. Maintaining balance is a very basic and important in sports. The purpose of this study is to determine whether or not examinations for equilibrium functions can be used as one method of trainning in sports. Twenty-six normal subjects and 9 athletes were examined. Equilibrium function tests for body balance are very useful for qymnastics, skating, etc. E-quilibrium function tests using eye movements are useful for trainning in which use balls. Key words : Equilibrium, sports, body balance, eye movements.
Although the effect of the atmospheric pressure on Meniere's disease has been studied for over 15 years, and low pressure treatment seems to be effective, many questions are left unanswerd. The major reason is thought to be in the specifications of the pressure chamber. Most pressure chambers hitherto used are capable of generating either high or low pressure. Furthermore, since the chambers are not designed for otological examinations in most cases, ventilating with fresh air produces noise exceeding 60-70 dB in the chamber during the examination. Aiming at neurootological research, we built a new soud-proof pressure chamber in which pressure can be changed between±1, 000mmH2O at a maximum speed of 100mmH2O/sec. Noise in this chamber can be kept under 30-35 dB while pressure is maintain-ed at the same level.
Retro-labyrinthine vestibular disorder (RLV D) was studied in patients with sudden deafness with vertigo and/or vestibular dysfunction. RL VD was detected by the galvanic body sway test (GBST), which was developed and introduced for routine clinical use 8 years ago. From 1985 to 1990 we saw 38 patients with sudden deafness with vertigo and/or vestibular dysfunction. Twenty-seven patients had vertigo (group 1), and in 11 without vertigo caloric tests showed canal paresis (CP) (group 2). Ten (37%) in group 1 had RLVD. All those with RLVD also had evidence of CP in the caloric test. There were no patients with RLVD in group 2. The degree of hearing loss in those with RLVD was significantly more severe and their recovery of hearing loss was poorer then in patients without RLVD. These results indicate that sudden deafness with vertigo is sometimes due to disorders of the 8th cranial nerve and that cochlear nerve dysfunction may be an etiological factor of deeafness.
Harada's disease presenting with ear symptoms was studied. In Harada's disease presenting with vertigo, the following neuro-otologic findings were obtained. Monopedal standing was poor, and staggering was often noted in the stepping test. Nystagmus tests frequently showed rotato-horizontal or horizontal nys-tagmus, and ENG with the eyes closed or covered frequently demonstrated nystagmus with horizontal components. CP was frequently detected by the caloric test. No abnormalities were noted with the optokinetic nystagmus test or eye tracking test. These findings suggest that Harada's disease with vertigo is often caused by disorders of the peripheral vestibular system, especially the inner ear, Classification of the disease was attempted from the otologic view-point. Harada's disease with inner ear symptoms can be classified on the basis of equilibrium and auditory functions into type I (cochlear type), type II (vestibular type), and type III (cochleovestibular type).
The patient was a 20-year-old man with the chief complaint of bilateral hearing loss following a cold with headache, fever and hyperemia of both eyes. Two weeks after the onset bilateral hearing loss and tinnitus appeared. Pure tone audiometry revealed moderate sensory neural hearing loss; the ABR wave V detection thres-hold was 60 dB HL in both ears in response to click stimuli at 3000Hz. No abnormal nystagmus was seen on ENG, Plain X-ray films and CT scans did not reveal any abnormality of the ears. Finally, Harada disease was diagnosed by fundoscopy. Auditory acuity was improved, except at 8000 Hz, within 6 days by steroid treatment. Two weeks were required for restoration to the normal level at 8000Hz. Inner ear function appeared to respond promptly to steroid treatment, but ophthalmological improvement was slow. Harada disease is considered to be an autoimmune disease against melanocytes, and it may cause endolymphatic hydrops.
Harada disease is an autoimmune disease which is well known as a systemic disorder involving melanocytes in the eye, ear, skin, and meninges. Thirteen patients with Harada disease were examined neurotologically from January 1987 to December 1991. Subjective cochlear and/or vestibular symptoms were noted in 6 of them. In 25 of the 26 ears sensorineural hearing loss, less than 40 dB, was observed. In 12 of the 13 patients, vestibular function tests revealed abnormal findings, which suggested peripheral or central disorder. We studied a representative of 29-year-old male who complained of sudden onset of rotatory vertigo in spite of improvement of ophthalmic symptoms.
Vogt-Koyanagi-Harada (Harada's) diseases is a systemic and autoimmune disorder consisting of uveitis, hearing loss, vertigo, vitiligo and premature graying of the hair involving melanocytes. Twenty-four patients with Harada's disease were studied clinically. Mild hearing loss was noted in 28 ears (58.3%). The short increment sensitivity index (SISI) test was positive for the recruitment phenomenon in 6 of the 10 ears tested. Three patients complained of vertigo (12.5%), but they should no rotatory vertigo or horizontal nystagmus. Prednisolone therapy was tried for one month, hearing was improved in 15 ears (53.6%). These clinical findings suggest disorder of the inner ear. Therefore, we thought that it was important to discuss Harada's disease as an autoimmune disease to elucidate the pathogenesis of the inner ear problem.
Vestibular neuronitis is a peripheral vestibular disease of unknown etiology, in which severe vertigo attacks associated with disequilibrium usually start without any cochlear signs such as tinnitus or deafness after acute upper respiratory inflammation. It is generally accepted that the disease has a good prognosis beacause both subjective sensation and objective disequilibrium gradually improve and no recurrence of attacks is noticed. However, some patients complain of a transient dizzy sensation induced by quick body motion and disequilibrium during standing or walking, especially in darkness, a long time after the onset. Follow-up studies were carried out in six patients with vestibular neuronitis for 1 to 2 years after the beginning of the disease. Canal paresis was revealed by caloric tests in all six, it was bilateral in one. Five had transient diz-ziness and or unsteadiness during quick body movements 1 to 2 years after the onset of the disease. ENG studies revealed spontaneous nys-tagmus with eyes closed in two patients more than 2 years after the onset. The residual symptoms of dizziness and disequilibrium during body motion in vestibular neuronitis were assumed to be caused by prolonged failure in the acquisition of central vestibular compensation. Physical exercises were ordered in 2 patients, but complete recovery was not achieved. Disturvance of activities of daily living (ADL) are also discussed.
Case 1 : Bilateral sensorineural deafness progressed rapidly to nearly total deafness. CT revealed soft granulation in the tympanic and mastoid cavities. Several open biopsies failed to show any specific inflammation. Laboratory studies revealed a highly probable autoimmune origin : positive RA and ANA, elevated ESR, complement, and immunoglobulins. Her deafness responded to steroid therapy, and her hearing returned to a usable conversational level. Case 2 : This case represents so-called "steroid-responsive sensorineural hearing loss". Her bilateral inner ear deafness responded several times to steroid therapy when aggravations of hearing loss occured. Her deafness also improved slowly and slightly with the administration of isosorbide. Case 3 : Bilaeral fluctuant sensorineural deafness appeared in this patient with systemic lupus erythematosus. The glycerol test was markedly positive bilateraly. All three patients maintain usable hearing for daily life, in addition the first and the second have had no steroid therapy for 3 months. The pathogenesis of autoimmune sensorineural deafness is not yet fully understood. Although vasculitis is the most common pathologic change in autoimmune diseases, the present cases suggest that endolymphatic hydrops may also play some role in sensorineural deafness of autoimmune origin.