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Spontaneous vertigo and headache: endolymphatic hydrops or migraine?

We undertook a study to assess whether patients who had both spontaneous vertigo and headache would respond to treatment for endolymphatic hydrops rather than treatment for migraine. We also attempted to discover if there were any individual characteristics that might predict which patients with an equivocal history and symptoms would be more likely to have either of the two conditions as opposed to the other. All patients were initially treated for endolymphatic hydrops with sodium restriction and increased water intake. Response to treatment was assessed by three tools: the Dizziness Handicap Inventory, a symptom severity scale, and a disability scale. Patients who did not respond to treatment for endolymphatic hydrops were switched to treatment for migraine. At the first follow-up period, 23 patients were available for analysis by telephone survey. According to data obtained by the three assessment tools, 14 patients (60.9%) improved after initial therapy and six (26.1%) improved following subsequent migraine treatment; the remaining three patients (13.0%) did not comply with their initial treatment regimen, and they showed no improvement. Although no statistically significant conclusions can be drawn because of the small sample size, there were trends to suggest that certain demographic, clinical, and objective-testing characteristics might predict which patients are likely to have one of these two conditions as opposed to the other. We conclude that a stepwise approach to treatment, beginning with therapy for endolymphatic hydrops, is an appropriate strategy. Also, the fact that no improvement was seen among the three patients who did not comply suggests that improvement is indeed the result of treatment rather than simply a function of the passage of time.

Adolescent↗

Autoimmune endolymphatic hydrops: five-year review.

This article reviews the authors' experience with autoimmune endolymphatic hydrops over the past 5 years. Twenty-seven of 52 patients with diagnosed autoimmune inner ear disease (52%) manifested endolymphatic hydrops, usually bilateral. Treatment consisted of low salt diet, diuretic, vestibular suppressant, and usually prednisone. Cytotoxic drugs and lymphocytoplasmapheresis were reserved for refractory cases. Audiometric followup (average, 23 months) was available in 12 patients. Using American Academy of Otolaryngology--Head and Neck Surgery guidelines, hearing improved or stabilized in at least one ear in nine patients and deteriorated bilaterally in three patients. Vestibular treatment results paralleled auditory treatment results. Autoimmune endolymphatic hydrops should be considered in the differential diagnosis when symptoms are bilateral and do not respond to conventional therapy or when immune laboratory tests are positive. In suspect patients, medical therapy with prednisone, and rarely cytotoxic drugs and lymphocytoplasmapheresis, should be recommended. Further research is needed to determine whether surgery should be performed in medically unresponsive cases.

Adolescent↗

Electrocochleography in the diagnosis of delayed endolymphatic hydrops.

It is known that delayed endolymphatic hydrops (DEH) occurs in patients with unilateral and/or bilateral profound hearing loss. In the ipsilateral and bilateral types of DEH, no audiological tests can be performed on the affected ear because of profound deafness, although these tests can be done in the contralateral type. Electrocochleography (ECochG) was recorded on better hearing ears in both types of DEH. The results showed an increased negative summating potential (SP) in 6 out of 10 contralateral DEH patients whereas it could be measured in only one of 10 ipsilateral DEH patients. The mean SP/AP ratio of the contralateral type was 0.42 which was significantly larger than that of the ipsilateral type. The results suggest that contralateral-type DEH has the same pathology in the cochlea as Meniere's disease, and that ECochG can distinguish between these two types of DEH.

Action Potentials↗

Time-related changes in cochlear potentials in guinea pigs with experimentally induced endolymphatic hydrops.

In order to examine the changes in cochlear function occurring in hydropic ears over time, endolymphatic hydrops was provoked by obliterating the endolymphatic sac in 62 albino guinea pigs. Cochlear potentials (EP, CM, SP and AP) were recorded in the 1st, 2nd, 4th and 12th postoperative weeks, respectively. A significant reduction in the level of EP was already observed in the 2nd week and the potential was further suppressed by the 4th week. There was no significant difference between the levels of the 4th and 12th weeks. The amplitudes of CM and AP decreased progressively as time elapsed after the surgery. In sharp contrast to the other potentials, the abnormality in SP was most frequently observed in the 1st week. These results are discussed in the light of the available literature.

Action Potentials↗

Endolymphatic hydrops with absence of vein in paravestibular canaliculus.

Unilateral endolymphatic hydrops is described associated with absence of the vein in the paravestibular canaliculus (PVC), and with decreased vascularity of the vestibular aqueduct and endolymphatic sac. The venous return from the vestibule was normal as far as the junction of the branches forming the PVC vein. At this junction, a blind venous loop was formed with no continuation of venous drainage through the PVC. This probably represents a developmental anomaly. The decreased vascularity of the endolymphatic sac may be related to the absence of the PVC vein. However, anatomical and functional relationships of these vessels are not clear and need further study. Perisac fibrosis and endosteal bone formation are possibly secondary to the decrease in vascularity. A large chronic rupture of the inferior saccule wall probably accounts for the absence of vertigo and the relatively mild degree of cochlear endolymphatic hydrops.

Aged↗

Vestibular evoked myogenic potentials in ipsilateral delayed endolymphatic hydrops.

We recorded vestibular evoked myogenic potentials (VEMPs) in 12 patients diagnosed as having ipsilateral delayed endolymphatic hydrops (DEH). Seventy-five percent (9/12) of the patients showed decreased or absent VEMPs in the affected ears. Almost all patients had normal VEMPs in the unaffected ears. In addition, in 4 patients, VEMPs were recorded before and 3 h after oral glycerol administration (1.3 g/kg body weight). VEMPs improved after glycerol administration in 2 of the 3 patients whose VEMPs had been abnormal in the affected ears before glycerol administration. None of the 4 patients presented significant changes in VEMPs in the unaffected ears after glycerol administration. Improvement of VEMPs after glycerol administration confirmed the existence of endolymphatic hydrops in patients with ipsilateral DEH. In conclusion, VEMPs are useful to evaluate the function of otolith organs in patients with ipsilateral DEH, and the glycerol test using VEMPs may be useful to detect endolymphatic hydrops in ipsilateral DEH.

Administration, Oral↗

Delayed endolymphatic hydrops.

Eighteen patients with delayed endolymphatic hydrops are presented. This distinctive entity typically affects young adults and is characterized by episodic vertigo of Ménière's type occurring years after a sudden hearing loss. Measles or mumps were thought to have caused the hearing loss in eight patients. In the entire group of 18 patients the delay between identification of hearing loss and vertigo averaged 22.6 years. The diagnosis is aided by electronystagmography and labyrinthotomy in selected cases. Medical treatment may be effective and surgical treatment is curative.

Adolescent↗

Effects of endolymphatic-perilymphatic fistula on endolymphatic hydrops in guinea pig.

The endolymphatic-perilymphatic shunt operation between the scala media and scala tympani was performed in 22 guinea pigs with endolymphatic hydrops induced by the silver nitrate injection method 2 months before the operation. Two (n = 10), 7 (n = 6), and 28 (n = 6) days after the operation, the condition of the fistula and the inner ear pathology were studied histopathologically. In half of the animals, the fistula was open, whereas in the other half, the fistula was closed. In the group examined after 2 postoperative days, the animals with open fistulas showed a slight collapse of Reissner's membrane, which might indicate that excess endolymph escaped through the fistula into the scala tympani. In the groups examined later, especially in the group examined after 28 postoperative days, the animals showed distention of hydrops despite preservation of the fistula. Moderate-to-severe degenerative changes of the organ of Corti, macula sacculi, and stria vascularis were observed in the vicinity of the fistula. We conclude from this study that the endolymphatic-perilymphatic fistula created inside the cochlea apparently was not sufficient to reduce the hydrops. Rather, it caused degenerative changes of the inner ear structure. This type of surgery is not suitable for Meniere's disease.

Animals↗

Posttraumatic endolymphatic hydrops.

A case of posttraumatic endolymphatic hydrops that was confirmed by a positive glycerin test is reported. Although head trauma has been implicated as a cause of hydrops, few such cases are reported in the literature. Careful consideration of perilymphatic fistula must be accorded before a diagnosis of posttraumatic hydrops is made.

Adult↗

Endolymphatic hydrops. An overview and classification.

Endolymphatic hydrops (EH) is a pathological condition which is the final common manifestation of a variety of otologic insults. In this paper we develop a classification which, on the basis of clinical and pathological data, distinguishes symptomatic and asymptomatic forms. Clinical case histories and temporal bone studies are presented to illustrate and substantiate this classification. The symptomatic form becomes evident by the hallmark symptoms of episodic vertigo and fluctuating hearing loss. The asymptomatic form is silent. Interconversion from one form to another may occur over time. Each of the forms can be subdivided, according to etiology, into embryopathic, acquired, and idiopathic types. The embryopathic type comprises those cases in which some noxious influence interferes with prenatal labyrinthine development. The acquired type includes those cases in which a documented insult, be it inflammatory or traumatic, is suffered by a previously normal labyrinth. The inflammation may be viral, bacterial, or spirochetal in nature, while the traumatic event may be either accidentally or surgically sustained. The idiopathic type includes cases in which the instigating event precipitating the EH is unknown. Menière's disease represents merely one example of the EH group of disorders, namely symptomatic idiopathic EH.

Adolescent↗

Pre and post-glycerol special audiometric tests battery results in endolymphatic hydrops.

The glycerol test for endolymphatic hydrops was administered to 17 patients exhibiting classical symptoms of Meniere's disease. A complete battery of audiometric tests including the SISI, TTD (Rosenberg variation). ABLB, Sweep Frequency Bekesy, two tests of speech discrimination (Rush Hughes and Northwestern), and air and bone conduction thresholds were administered pre and post-glycerol ingestion in order to determine the effect of osmotic diuresis. Improvement in pure-tone sensitivity and speech discrimination scores were most often observed. Among the special audiometric tests only the SISI scores exhibited changes, however, these could be related to the threshold changes. There were no significant differences observed in the threshold tone decay tests nor in the ABLB. The sweep frequency Bekesy test revealed only the changes which were observed in pure-tone sensitivity.

Acoustic Stimulation↗

Luetic endolymphatic hydrops: diagnosis and treatment.

Luetic endolymphatic hydrops (LEH) is an effectively treatable disorder that requires astute clinical judgment to suspect, confirm, and treat. We provide a review of the literature as well as recommendations regarding the clinical management of LEH with discussion of several illustrative cases. We also give an overview of laboratory testing for the diagnosis and monitoring of the infectious process. Ultimately, the control of the otologic symptoms in LEH requires acumen on the part of the clinician.

Aged↗

Rupture of Reissner's membrane during acute endolymphatic hydrops in the guinea pig: a model for Ménière's disease?

CONCLUSION: The changes in cochlear function during a destructive acute endolymphatic hydrops were relatively small. This might be consistent with the hypothesis that an endolymphatic hydrops is a marker of disordered inner ear homeostasis rather than the cause of the clinical symptoms of Ménière's disease. OBJECTIVE: Assessment of cochlear function during induction of a destructive acute endolymphatic hydrops. MATERIALS AND METHODS: During repetitive microinjections of 0.5 microl of artificial endolymph at a rate of 50 nl/s the 2f1-f2 and f2-f1 cochlear microphonics distortion products (CMDP) and 2f1-f2 distortion products otoacoustic emissions (DPOAE) were recorded in the guinea pig. RESULTS: A 'catastrophe' occurred in the inner ear when 2.5-3.5 microl of artificial endolymph was injected. A rupture of Reissner's membrane was then found, most often in the apical turn of the cochlea. This rupture had only minor effects on the endocochlear potential, whereas it caused a marked decrease in 2f1-f2 DPOAE amplitude. The 2f1-f2 and f2-f1 CMDP amplitude increased during each injection prior to the rupture. After the rupture the f2-f1 CMDP amplitude decreased during each injection, possibly due to a shift of the cochlear transducer operating point position.

Animals↗

Failure of forskolin to elevate the endocochlear potential in experimental endolymphatic hydrops of the guinea pig.

The effect of forskolin (FSK) on the endocochlear potential (EP) in scala media (SM) was examined in experimental endolymphatic hydrops of the guinea pig. Two weeks after obliteration of the endolymphatic sac the EP of hydroptic ears and that of the contralateral control ears were measured by means of microelectrodes. The perfusion of scala vestibuli (SV) with FSK (200 microM) produced EP elevation in the contralateral control ears but failed to do so in the experimental hydroptic ears. Histological examination of experimental endolymphatic hydrops showed mild hydrops with intact appearance of outer and inner hair cells, and the stria vascularis. The mechanism underlying the failure of FSK to elevate the EP in experimental endolymphatic hydrops is discussed.

Animals↗

[Effect of aminoglycosides on experimentally induced endolymphatic hydrops].

Gentamycin or kanamycin have no effect on endolymph formation in experimental induced endolymphatic hydrops. The increase of endolymphatic volume after obliteration of endolymphatic duct and sac is not influenced by aminoglycosides as well as the decrease of DC-potential and the increase of sodium-activity in endolymphatic hydrops. The way of action of intratympanal injected gentamycin seems to be in a partial chemical induced labyrinthectomy.

Animals↗

Otosclerosis and endolymphatic hydrops.

It has been postulated that otosclerosis may produce vertigo by several mechanisms. One mechanism is by causing endolymphatic hydrops. We present six temporal bones in which otosclerosis and endolymphatic hydrops coexist. We consider that there is a spectrum-like interrelationship between these two entities. At one end of the spectrum the relationship is coincidental, while at the other end of the spectrum we consider the massive amount of active otosclerosis to be a causative factor in the development of the endolymphatic hydrops.

Aged↗

Cochlear blood flow in endolymphatic hydrops.

There is no animal model for Meniere's disease but by obliteration of the endolymphatic duct, endolymphatic hydrops may be achieved in several animal species. In order to measure the cochlear blood flow in ears with endolymphatic hydrops the endolymphatic duct was obliterated in 9 guinea pigs. The blood flow was measured with the microsphere method and the cochlear histology was studied. The regional and total blood flow was determined in the serially sectioned cochleas 2, 4 and 8 months after obliteration of the endolymphatic duct. No change in regional or total cochlear blood flow was observed in the hydropic ears.

Animals↗

Electrocochleography in an experimental animal model of acute endolymphatic hydrops.

A reliable animal model of acute type of endolymphatic hydrops was made with injection of artificial endolymph into the cochlear duct. In 12 animals without Reissner's membrane rupture, the endolymphatic potentials (EP) were kept normal after injection of artificial endolymph, but their electrocochleography (ECoG) showed as: a rise of summating potentials (SP) amplitude, a decrease of action potentials (AP) amplitude, an increase of SP/AP ratio, as well as a delay of N1 latency. In 7 animals with membrane rupture, the EP significantly decreased. ECoG showed as: a decrease of hearing function in all test frequencies, a distortion of SP-AP wave and even a loss of AP. Based on the results of our experiment it can be assumed that the dominant -SP may be seen in the acute stage of endolymphatic hydrops without a rupture of Reissner's membrane. Therefore, the dominant -SP may only be of value in the clinical diagnosis during episodic vertigo and fluctuating hearing loss of Meniere's disease.

Action Potentials↗