Degree Of Hydrops Research Articles

Evaluation of the relationship between endolymphatic hydrops and hearing loss in Meniere’s disease based on three-dimensional real inversion recovery sequence

ObjectivesThe degree of endolymphatic hydrops in Meniere's disease may be related to hearing loss. However, the results of prior studies have been inconsistent. We aimed to investigate the relationship between endolymphatic hydrops and hearing loss characteristics in Meniere’s disease. MethodsThis study included 54 patients (62 ears) with Meniere’s disease. Patients underwent three-dimensional real inversion recovery sequences for magnetic resonance imaging and pure-tone audiometry. Endolymphatic hydrops were assessed according to Gurkov’s criteria (2011). Correlations between different degrees of endolymphatic hydrops and pure-tone audiometry, as well as staging, were analysed. ResultsPure tone audiometry and staging were higher for vestibular endolymphatic hydrops complicated by cochlear ones than isolated cochlear or vestibular hydrops (both p < 0.05). There was no significant correlation between vestibular endolymphatic hydrops and pure-tonal audiometry or staging (all p > 0.05). The degree of hydrops in the middle turn was correlated with the mid-frequency hearing threshold (p < 0.05). The degree of cochlear hydrops was correlated with the audiometry, low-frequency hearing thresholds, mid-frequency hearing thresholds and staging (p < 0.01). ConclusionThe types and sites of endolymphatic hydrops based on a 3D-real inversion recovery sequence can be used to indicate the degree of hearing loss in patients with Meniere’s disease. Level of evidenceLevel III.

Endolymphatic hydrops imaging and correlation with clinical characteristics, audiovestibular function and mental impairment in patients with Meniere’s disease

PurposeMR imaging was used to visualize the vestibular and cochlear endolymphatic hydrops in patients with Meniere’s disease (MD). The relationship between the degree of hydrops and clinical characteristics, audiovestibular function, anxiety and depression state in MD patients.Methods70 patients with definitely or probably unilateral Meniere’s disease received bilateral intratympanic gadolinium administration and MR scanning. The degree of bilateral vestibular and cochlea hydrops were analyzed and evaluated by three-dimensional real inversion recovery (3D-real IR) sequence, and the correlation between the grades of endolymphatic hydrops (EH) and disease course, vertigo grading assessment, the duration of vertigo, hearing loss level, caloric test, vestibular myogenic evoked potential (VEMP), electrocochleogram (EcoG), vertigo disability scale (physical, emotional, functional), anxiety and depression scale were studied.ResultsIt was found that the vestibule and cochlea EH of the affected and the contralateral ear had different degrees of hydrops and there was no statistical difference between the left and right vestibules. The degree of vestibule EH (V-EH) was significantly positively correlated with the degree of cochlear EH (C-EH). C-EH and hearing loss level were positively correlated with EcoG. There was positive correlation between vestibular EH and hearing loss level, VEMP, caloric test, disease course or vertigo duration. There was a negative relationship between Dizziness Handicap Inventory (Emotion) (DHI(E)) and VEMP. Self-rating Anxiety Scale (SAS) and Self-rating Depression Scale (SDS) scores were positive correlated with DHI(E) and DHI total scores in MD patients.ConclusionEndolymph-enhancing MRI was used as an important imaging method for the diagnosis of labyrinthine hydrops in Meniere’s disease. There were certain correlation between EH and the degree of vertigo attack, hearing loss level, vestibular function, and further changes in anxiety and depression emotion.

Magnetic resonance imaging of endolymphatic hydrops in Ménière's disease: A comparison of the diagnostic value of multiple scoring methods.

ObjectivesTo compare three methods of scoring endolymphatic hydrops in patients with Ménière's disease in order to assess the correlation between endolymphatic hydrops and auditory characteristics.MethodsA retrospective study of 97 patients with unilateral definite Ménière's disease (DMD) who underwent contrast-enhanced three-dimensional fluid attenuated inversion recovery (3D FLAIR) MRI. Each patient was scored by the Inner Ear Structural Assignment Method (IESAM), the Saccule to utricle area ratio (SURI), and the Four Stage Vestibular Hydrops Grading (FSVH), according to their corresponding axial images. Cohen's Kappa and intra-class correlation coefficient were used for consistency testing, combined with binary logistic regression analysis, to compare the sensitivity and specificity of the three methods. The degree of hydrops in different stages of MD was compared. The correlation between endolymphatic hydrops in the inner ear sub-units and hearing thresholds was further analyzed.ResultsThe intra- and inter-reader reliability for the scoring of endolymphatic hydrops were excellent. The IESAM had a high diagnostic value for identifying definite Ménière's disease (sensitivity: 86.6%, specificity: 97.9%). The hearing thresholds were correlated with the degree of endolymphatic hydrops. Stages 3 and 4 were more significant for the severity of hydrops than stage 1. Within the subgroups of the Ménière's disease patients, compared with the non-hydrops group and the pure vestibular hydrops (V group), the cochlear combined vestibular hydrops group (CV group) had significantly higher auditory thresholds. The amplitude ratio of electrocochleogram was significantly higher in the affected ear than in the healthy ear.ConclusionThe IESAM is a more sensitive and specific diagnostic scoring method for the diagnosis of DMD. Diagnostic imaging may improve the detection of inner ear hydrops which is correlated with severity of hearing loss. A comprehensive evaluation of the inner ear sub-unit structures maybe necessary.

Endolymphatic hydrops in the unaffected ear of patients with unilateral Ménière’s disease

PurposeCurrent studies show that frequency tuning modification is a good marker for the detection of endolymphatic hydrops (EH) employing magnetic resonance imaging (MRI) in patients with Ménière’s disease (MD). The purpose of the present study is to analyze the auditory and vestibular function with audiometric and vestibular-evoked myogenic potentials (VEMP) responses, respectively, in both the affected and unaffected ears of patients with unilateral MD using MRI as diagnostic support for the degree of EH.MethodsWe retrospectively reviewed the medical records of 76 consecutive patients with unilateral definite MD (age 55 (28–75); 39 women, 37 men). MRI was used through intravenous gadolinium administration, audiometry, and VEMPs. Functional tests were performed up to a week after the MRI. All were followed up one year after imaging utilizing clinical, auditory, and vestibular testing to rule out bilateral involvement.ResultsIn the unaffected ear, the mean pure-tone average is normal even in cases with hydrops and, for a similar severity of hydrops is significantly lower than in the affected ear. Significant differences for the amplitude of the response at 0.5 kHz, at 1 kHz between the affected and unaffected ears were found to be lower in the affected ears. The relative amplitude ratio (1 Kz–0.5 kHz) was significantly lower in the affected ear and in the case of the oVEMP response depends on the degree of EH. The response in the unaffected ear was not modified by the presence or the degree of hydrops.ConclusionIn the unaffected ear, hydrops is not associated with hearing deterioration. For a similar degree of hydrops, hearing loss is significantly greater in the affected ear. The endolymphatic hydrops in the vestibule induces a frequency bias in the VEMP response only in the affected ear and not in the unaffected ear. Because of these findings we consider that hydrops does not represent an active disorder in the unaffected ear.

Magnetic resonance imaging study of endolymphatic hydrops based on 3D-FLAIR sequence after single-dose intravenous gadolinium injection

Objective: To investigate the visualization of endolymph in patients with otogenic vertigo by intravenous administration of single dose of gadolinium contrast agents and magnetic resonance three-dimensional fluid-attenuated inversion recovery sequence (3D-FLAIR MRI), and further assess the extent of endolymphatic hydrops. Methods: From Beijing Tongren Hospital of Capital Medical University between October 2017 and June 2018, 30 patients (16 males, 14 females) with unilateral otogenic vertigo were involved in this study, with the age of 30 to 68 years, mean age of (53±10) years. Eight hours after intravenous administration of single dose (0.1 mmol/kg, body weight) of gadopentetate (Gd-DTPA), 3D-FLAIR sequence was performed in 30 patients. The location of endolymphatic hydrops was observed and then the degree of hydrops was quantitatively elevated by two radiologists. The consistency test was used to analyze the location and degree of endolymphatic hydrops in the two radiologists and the paired t-test was used to compare the difference between the affected and healthy side of endolymphatic spaces of the patients with otogenic vertigo. Results: In 30 patients, the gadolinium distributed in all parts of the perilymph inside the inner ear, and can accurately outline the boundaries of the peri-and endolymph. Twenty-six patients (26/30, 86.7%) were found to have unilateral endolymphatic hydrops, including 18 mild hydrops, 8 significant hydrops. The two radiologists had a very good agreement on the assessment of endolymphatic hydrops(kappa=0.864, ICC=0.959). In the 3D-FLAIR MR images of 26 patients with endolymphatic hydrops, the saccule (26/26, 100%) had a higher rate of hydrops than the cochlea and utricle(16/26, 61.5%; 14/26, 53.8%), and two radiologists had a very good agreement on the location of endolymphatic hydrops(kappa=0.820). Moreover, there was a significantly statistical difference between the affected and healthy area of the endolymphatic space in this study (P<0.01). Conclusion: The technique of 3D-FLAIR MR imaging through single dose intravenous gadolinium injection is feasible, which can estimate endolymphatic hydrops in patients with otogenic vertigo, and accurately classify the degree of hydrops.

Endolymphatic hydrops severity in magnetic resonance imaging evidences disparate vestibular test results

ObjectivesIt has been suggested that in Ménière’s disease (MD) a dissociated result in the caloric test (abnormal result) and video head-impulse test (normal result) probably indicates that hydrops affects the membranous labyrinth in the horizontal semicircular canal (HSC). The hypothesis in this study is that based on endolymphatic hydrops’ cochleocentric progression, hydrops should also be more severe in the vestibule of these patients than in those for whom both tests are normal. Methods22 consecutive patients with unilateral definite MD were included and classified as NN if both tests were normal or AN if the caloric test was abnormal. MRI evaluation of endolymphatic hydrops was carried out with a T2-FLAIR sequence performed 4h after intravenous gadolinium administration. The laterality and degree of vestibular endolymphatic hydrops and the presence or absence of cochlear endolymphatic hydrops were recorded. Demographic data, audiometric and vestibular evoked myogenic potentials were collected, and video head-impulse and caloric tests were performed. ResultsPatients in both groups (NN and AN) were similar in terms of demographic data and hearing loss. The interaural asymmetry ratio was significantly higher for ocular and cervical VEMP in patients in the AN group. There was a significantly higher degree of hydrops in the vestibule of the affected ear of AN patients (χ2; p=0.028). ConclusionSignificant canal paresis in the caloric test is associated with more severe endolymphatic hydrops in the vestibule as detected with gadolinium-enhanced MRI and with a more severe vestibular deficit.Level of evidence: 2a

Clinical features and imaging characteristics of Meniere's disease verified by intravenous gadolinium contrast-enhanced magnetic resonance imaging

Objective: To analyze the clinical features and imaging characteristics of Meniere's disease (MD)verified by intravenous gadolinium contrast-enhanced magnetic resonance imaging(MRI). Methods: A total of 174 patients with Meniere's disease were involved in this study between March 2016 and October 2017. All of them underwent 3D-fluid attenuated inversion recovery (FLAIR) MRI and presented endolymphatic hydrops and clinical characteristics of suspected Meniere's disease. The clinical characteristics, the grades of endolymphatic hydrops (EH) using Nakashima grading standard were analyzed, and the correlation between grade and clinical features was evaluated. Results: There were totally 174 patients (88 males, 86 females), with the age of 7-83 years [mean age of (51.0±15.0)] years. Age of onset and disease course was 7-83 years [mean age of (44.6±16.6) years], 36.0 (12.0, 111.0) months (from 2 days to 70 years), respectively. EH was found in all the patients, and 169 cases (97.1%) manifested as modest or severe hydrops. Among the 174 patients, 139 cases (79.9%) presented unilateral EH, and 35 cases (20.1%) presented bilateral EH. The degree of EH in the latter was more serious than the former. For 174 patients, clinical characteristics were inconsistent, which was not in accord with the existing diagnostic criteria. Furthermore, there was a correlation between degree of hydrops and degree of hearing loss (P<0.05). Conclusions: EH in Meniere's disease can be measured objectively by intravenous gadolinium contrast-enhanced MRI. Clinical characteristics in MD verified by EH are not accordant with the existing criteria, which means the significance of EH in diagnosis of MD. Moreover, there's a correlation between degree and distribution of hydrops and degree of hearing loss.

Focal Endolymphatic Hydrops as Seen in the Pars Inferior of the Human Inner Ear.

Endolymphatic hydrops of the human inner ear may be localized focally in the pars inferior of the human inner ear. Endolymphatic hydrops may be found in the human inner ear in patients who in life had suffered from Ménière's syndrome or a variety of other disorders. The degree of endolymphatic hydrops may differ based on location in the inner ear. A computer-assisted search of all cases in the collection of the Massachusetts Eye and Ear Infirmary in which endolymphatic hydrops was found in the inner ear yielded 13 specimens in which there was good evidence for focal endolymphatic hydrops in the pars inferior. Temporal bones were prepared for light microscopy. Semi-serial sections were reviewed to generate localization data for endolymphatic hydrops and also to search for evidence of a previous inflammatory process, including fibrosis or new bone formation. Endolymphatic hydrops was present in the saccule in 10 of 13 specimens. In the cochlear duct, there were segments of the cochlea in which there was no cochlear hydrops juxtaposed to other regions in which there was severe endolymphatic hydrops. Transition between hydropic and non-hydropic status in the cochlear duct was often abrupt.Evidence for a previous inflammation process was found in 6 of 13 specimens including fibrosis because of temporal bone fracture, or traumatic stapedectomy and in those cases in which the cause of hearing loss was idiopathic, fibrosis, and osteoid between the labyrinthine surface of the footplate and the hydropic saccular wall, and/or osteoid in the scala vestibuli, or in the proximate Rosenthal's canal. Evidence of a previous inflammatory process was uniformly seen in the perilymphatic compartment. Endolymphatic hydrops of the pars inferior in the human may have a focal distribution. This study suggests that the pathogenesis of endolymphatic hydrops is unlikely to be because of distal obstruction of longitudinal flow and was more consistent with the hypothesis that homeostasis of the endolymphatic and perilymphatic volumes occurs all along the cochlear duct. Other factors including variable distensibility of Reissner's membrane or disturbance of local solute homeostatic mechanisms may be responsible for wide variations in the degree of hydrops. A focal inflammatory process during life may be one cause of focal endolymphatic hydrops as seen histopathologically.

The reclassification of sudden deafness

Objective:Reclassified the total deafness and flat type of sudden deafness,identified the relationship between new classification and hearing prognosis.Method:To analyze 192 cases of patients with sudden deafness,especially for 159 cases of flat type and total deafness patients for further curve type classification,classified as rise,decline and consistent,analysis the possible factors with hearing in multiple factors using regression analysis.Result:According to age group of 45 years old,P>0.05,the difference has not statistically significant;Course of the disease according to the 0-7 days and 14 days more,P<0.01,OR=4.291;8-14 days and 14 days,P<0.05,OR=2.983;According to the flat type and total deafness grouping,P<0.05,OR=0.409;According to the curve type of deafness,the difference between rise type and total deafness was significant,P<0.01,OR=9.692;Decline type,consistent type compared to total deafness type,there is no statistically significant difference,P>0.05.Conclusion:According to the pathogenesis of classification,sudden deafness can be divided into rise type,decline type and consistent type.The pathogenesis of rise type may be different degree of hydrops of inner ear.Decline type may be hair cell damage,and consistent type may be related to the damage of vascular stria and potential labyrinthitis,prognosis is poorer.

In Vivo Visualized Endolymphatic Hydrops and Inner Ear Functions in Patients With Electrocochleographically Confirmed Ménière’s Disease

To study the correlation between the degree of endolymphatic hydrops as detected in vivo by magnetic resonance (MR) imaging and the auditory and vestibular function in patients with definite Ménière's disease. Prospective observational study. Tertiary referral center for balance disorders. In this prospective study, 41 patients who fulfilled the criteria for definite unilateral Ménière's disease according to the American Association of Otolaryngology-Head and Neck Surgery and who showed a summating potential-to-action potential ratio of greater than 0.4 on electrocochleography were included. Intratympanic contrast-enhanced 3 Tesla MR imaging of the inner ear was performed using a 3D Inversion Recovery Turbo Spin Echo sequence. The degree of endolymphatic hydrops was graded on a Likert scale (0-3) in the cochlea and by vestibular endolymph space ratio in the vestibulum. The degree of hydrops was then analyzed with respect to its correlation with audiometric hearing levels, interaural amplitude ratios of vestibular evoked myogenic potentials, degree of horizontal semicircular canal paresis on caloric irrigation, and disease duration. The degree of hearing loss and the disease duration correlated significantly with cochlear hydrops (r = 0.85; r = 0.34). Sacculus dysfunction was significantly correlated with vestibular hydrops (r = -0.47). There was no significant correlation between horizontal semicircular canal paresis and vestibular hydrops. In patients with clinically and electrocochleographically confirmed definite Ménière's disease, the degree of MR morphological hydrops severity correlates significantly with impairment of hearing function and sacculus function.

Visualization of Endolyphatic Hydrops in Patients with Ménière’s Disease by Locally Enhanced Inner Ear MRI: Correlation with Audiovestibular Function

Objective: The pathophysiologic role of endolymphatic hydrops in Ménière’s disease is still unresolved. The aim of this study was to determine whether the degree of endolymphatic hydrops as it is detected in vivo in patients with definite Ménière’s disease correlates with audiovestibular function. Method: In this prospective study, 37 patients with definite Ménière’s disease were examined with intratympanic contrast enhanced temporal bone MRI. The degree of hydrops was then compared with hearing levels, SP/AP ratios, interaural amplitude ratios of vestibular evoked myogenic potentials and horizontal semicircular canal paresis. Results: There was a significant correlation between the degree of hydrops on the one hand and the averaged hearing level at 0.25 to 1 kHz and 0.5 to 3 kHz (r = 0.71, P &lt; .01) and the vestibular evoked myogenic potential interaural amplitude ratio (r = -0.44, P &lt; .05) on the other hand. A trend toward a correlation was found between the hydrops and the caloric response; no correlation was found between the hydrops and the SP/AP ratio. Conclusion: The degree of endolymphatic hydrops correlates with a progressive loss of auditory and sacculus function in patients with Ménière’s disease.

B106 Identification of factors which promote hearing preservation

The aim of this study was to investigate the audiological and histopathologic effects of dexamethasone in the treatment of experimentally induced endolymphatic hydrops.Thirty mature, male guinea pigs weighing 400 ± 50 g were operated on to induce experimental endolymphatic hydrops in their right ear. Left ear served as control. Subjects were separated into control and dexamethasone groups, with the latter receiving dexamethasone 5 mg/(kg d) intraperitoneally for 10 days. Electrocochleography and auditory brainstem response were applied to all subjects at preoperation, on the second postoperative day and also on the 15th postoperative day in animals that lived for a long time. The histopathologic examination of the inner ear in all animals was done at the end of the study.The summating potential and the ratio of the summating potential to the action potential measured on the second postoperative day were found to be increased in both groups, but more significantly in the control one. When the left and right ears were compared, significant difference was found in the control group; however, no significant difference was found between the ears in the dexamethasone group. Histopathologic examination revealed varying degrees of hydrops in the control group, but showed only normal findings or minor changes in the dexamethasone group.Dexamethasone can prevent the audiological and histopathologic findings of experimentally induced endolymphatic hydrops. However, these results must be supported by clinical and experimental studies designed with a large number of subjects.

In vivo visualization of endolyphatic hydrops in patients with Meniere’s disease: correlation with audiovestibular function

Our objective is to determine whether the degree of endolymphatic hydrops as it is detected in vivo in patients with definite Meniere's disease correlates with audiovestibular function. In this prospective study, 37 patients with definite Meniere's disease according to AAO-HNS criteria were included. Intratympanic contrast enhanced temporal bone MRI was performed using a 3D FLAIR protocol. The degree of endolymphatic hydrops in the cochlea and the vestibulum was graded on a Likert scale (0-3). The degree of hydrops was then analyzed with respect to its correlation with audiometric hearing levels, electrocochleographic SP/AP ratios, interaural amplitude ratios of vestibular evoked myogenic potentials and degree of horizontal semicircular canal paresis on caloric irrigation. There was a significant correlation between the degree of hydrops on the one hand and the averaged hearing level at 0.25-1 and 0.5-3kHz and the vestibular evoked myogenic potential interaural amplitude ratio on the other hand. A trend toward a correlation was noticed between the hydrops and the caloric response, no correlation was noticed between the hydrops and the SP/AP ratio. The degree of endolymphatic hydrops correlates with a progressive loss of auditory and sacculus function in patients with Meniere`s disease.

Magnetic resonance imaging of guinea pig cochlea after vasopressin-induced or surgically induced endolymphatic hydrops

Objective To investigate the ability to detect the in vivo cochlear changes associated with vasopressin-induced and surgically induced endolymphatic hydrops using MRI at 3 tesla (T). Study Design Prospective, animal model. Setting Animal laboratory. Subjects and Methods In group 1, five guinea pigs underwent post–gadolinium temporal bone MRI before and after seven and 14 days of chronic systemic administration of vasopressin by osmotic pump. In group 2, five guinea pigs underwent temporal bone MRI eight weeks after unilateral surgical ablation of the endolymphatic sac. Three-tesla high-resolution T1-weighted sequences were acquired pre- and postcontrast administration. Region of interest signal intensities of the perilymph and endolymph were analyzed manually. Quantitative evaluation of hydrops was measured histologically. Results Gadolinium preferentially concentrated in the perilymph, allowing for distinction of cochlear compartments on 3.0-T MRI. The T1-weighted contrast MRI of vasopressin-induced hydropic cochlea showed significant increases in signal intensity of the endolymph and perilymph. Surgically induced unilateral hydropic cochlea also showed increased signal intensity, compared with the control cochlea of the same animal, but less of an increase than the vasopressin group. The histological degree of hydrops induced in the vasopressin group was comparable to previous studies. Conclusions In vivo postcontrast MRI of the inner ear demonstrated cochlear changes associated with chronic systemic administration of vasopressin and surgical ablation of the endolymphatic sac. Understanding the MRI appearance of endolymphatic hydrops induced by various methods contributes to the future use of MRI as a possible tool in the diagnosis and treatment of Ménière's disease.

Spiral ganglion loss outpaces inner hair cell loss in endolymphatic hydrops

Neuronal toxicity is thought to be important in Meniere's disease and experimental endolymphatic hydrops (ELH). This study quantifies the relationship between neuronal degeneration and hair cell degeneration in ELH to evaluate the hypothesis that a primary neural insult would yield greater loss in the spiral ganglion than at the inner hair cell level. Following induction and histopathologic confirmation of endolymphatic hydrops in guinea pigs, the degree of hydrops, spiral ganglion loss, and hair cell degeneration were quantified and compared. Guinea pigs with surgically induced unilateral hydrops were sacrificed and their cochleas preserved. Hydrops severity and spiral ganglion density were quantified using automated methods. Hair cells were counted manually. Values were normalized against the contralateral ear to create loss indexes. Inner hair cell (IHC) loss at the apex is significantly lower than corresponding neuronal loss. IHC loss at the base is also lower than neuron loss, although not significantly. Regression analysis shows a significant, positive correlation between neuron loss severity and IHC loss severity at the apex, but not at the base. There is no correlation between hydrops severity and inner hair cell loss. By confirming that spiral ganglion loss is more severe than hair cell loss, and that hair cell loss appears to worsen with neuronal degeneration, this study supports the theory that neuronal toxicity is the primary insult in ELH-related disorders, such as Meniere's disease, and may provide the basis for designing treatment strategies.

The impact of treatment of the fetus by maternal therapy on the fetal and postnatal outcomes for fetuses diagnosed with isolated complete atrioventricular block

to analyse retrospectively the data of fetuses diagnosed with isolated complete atrioventricular block and efficacy of treatment of the fetus by maternal therapy. Between 1992 and 2004, we diagnosed complete atrioventricular block in 26 singleton and 2 twins fetuses of 27 pregnant women known to have anti Ro/La antibodies, 11 with autoimmune disease, one patient analysed in 2 pregnancies. At presentation, 20 of the fetuses were compensated and non-hydropic, while 8 had hydrops. Twenty patients were treated with dexamethasone, 2 with associated salbutamol and one mother with isoproterenol. Age at presentation was not different between the hydropic and non-hydropic fetuses. The fetuses with hydrops, however, had a lower mean heart rate at presentation, 48.5 +/- 9.25 with a range from 32 to 60, compared to 59.95 +/- 7.9 beats per minute, with a range from 50 to 80, in the non-hydropic fetuses (p less than 0.002). Equally, after birth the mean heart rate in hydropic fetuses was 42.6 +/- 5.1, with a range from 38 to 50, as opposed to 56.05 +/- 11.8 beats per minute, with a range from 29 to 110, in the non-hydropic fetuses (p less than 0.015), The hydropic fetuses were delivered at 31.7 +/- 3.8 weeks' gestation, with a range from 29 to 38 weeks (p less than 0.003) compared to 35.5 weeks' gestation +/-2.04, with a range from 31 to 38, in the non-hydropic fetuses. Mortality was 37.5% in the hydropic fetuses, versus 5% of those without hydrops (p less than 0.02). Pacemakers were implanted in 22 of 26 infants born alive, at a median of 45 days, with a range from 1 day to 5 years, in those without hydrops during fetal life, and 3 days, with a range from 1 day to 8 months in those afflicted by hydrops, of whom 2 died despite the implant of the pacemaker. The presence and degree of hydrops had a significantly negative predictive value. No significant differences were observed between the treated and non treated cases, albeit that administration of steroids ameliorated rapidly the hydrops in 3 of 5 cases. The outcome in our cases was mainly dependent on the presence and degree of fetal cardiac failure. Treatment of the fetus by maternal administration of steroids did not result in any regression of the conduction disorder, but had a favourable effect on fetal hydrops.

Evaluation of the effect of dexamethasone in experimentally induced endolymphatic hydrops in guinea pigs

Purpose The aim of this study was to investigate the audiological and histopathologic effects of dexamethasone in the treatment of experimentally induced endolymphatic hydrops. Materials and methods Thirty mature, male guinea pigs weighing 400 ± 50 g were operated on to induce experimental endolymphatic hydrops in their right ear. Left ear served as control. Subjects were separated into control and dexamethasone groups, with the latter receiving dexamethasone 5 mg/(kg d) intraperitoneally for 10 days. Electrocochleography and auditory brainstem response were applied to all subjects at preoperation, on the second postoperative day and also on the 15th postoperative day in animals that lived for a long time. The histopathologic examination of the inner ear in all animals was done at the end of the study. Results The summating potential and the ratio of the summating potential to the action potential measured on the second postoperative day were found to be increased in both groups, but more significantly in the control one. When the left and right ears were compared, significant difference was found in the control group; however, no significant difference was found between the ears in the dexamethasone group. Histopathologic examination revealed varying degrees of hydrops in the control group, but showed only normal findings or minor changes in the dexamethasone group. Conclusions Dexamethasone can prevent the audiological and histopathologic findings of experimentally induced endolymphatic hydrops. However, these results must be supported by clinical and experimental studies designed with a large number of subjects.

Simulation of therapy in a model of a nonhydropic and hydropic recipient in twin-twin transfusion syndrome

This study was undertaken to model the sequence of events that occurs after amnioreduction, laser therapy, and digoxin administration in twin-twin transfusion syndrome (TTTS) with and without a hydropic recipient twin. We added amnioreduction, laser therapy, and digoxin administration to our mathematical TTTS model and simulated combinations of these therapies. With a nonhydropic recipient, simulated amnioreduction delays the onset of hydrops. Conversely, with a hydropic recipient, amnioreduction aggravates the degree of hydrops. Furthermore, amnioreduction increases the transplacental fluid flow and may temporarily cause a hydropic donor. Laser therapy terminates the cause of recipient hydrops. Digoxin reduces the degree of recipient hydrops, but increases arteriovenous fetofetal transfusion. Laser therapy is superior in TTTS with a hydropic recipient, because simulated amnioreduction aggravates the recipient's cardiovascular status. Digoxin benefits a hydropic recipient but slightly worsens the donor's condition. Therefore, TTTS presenting with a hydropic recipient prior to fetal viability (approximately 26 weeks) may be best treated with laser therapy, whereas more advanced gestations may be offered digoxin administration plus amnioreduction, to delay the progression of TTTS.

The Quantification of Endolymphatic Hydrops in an Experimental Animal Model with Guinea Pigs

To better quantify endolymphatic hydrops in experimental guinea pigs, endolymphatic hydrops was induced by endolymphatic sac obliteration through an extradural posterior cranial fossa approach in the right ear. The area of the scala vestibuli and scala media of each turn on both cochlear midmodiolar sections was measured, using an automated computer-aided design (AutoCAD R14) software combined with a digital camera. No endolymphatic hydrops was observed in all nonoperated ears; however, various degrees of hydrops were present in all operated ears. The average maximum SMA (scala media area) ratio in the 4-week group (2.22 ± 0.20) was greater than that in the control group (1.10 ± 0.06). The average maximum SMA ratio of the 8-week group (4.04 ± 0.52) was greater than that of the 4-week group (p < 0.05). This study provides a reliable methodologic base for the experimental study of Ménière’s disease.

Fetal parvovirus B19 infection

Parvovirus B19 infection during pregnancy causes up to 27% cases of non-immune hydrops in anatomically normal fetuses. The virus is believed to cause arrest of maturation of red blood cell precursors at the late normoblast stage and also causes a decrease in the number of platelets. Fetal anemia is presently thought to be responsible for the development of skin edema and effusions. Myocarditis leading to heart failure may contribute to the development of fetal hydrops. We reviewed the literature regarding prevalence, transmission rates, clinical presentation, diagnostic techniques, current invasive vs. conservative management options, outcome and postmortem findings in a total of 82 studies involving 230 invasively and 435 conservatively managed pregnancies. In this non-selected population, the proportion of seronegative susceptible mothers ranged from 19 to 65%, seroconversion with an incubation time of up to 20 days occurred in 5.7-12.1%, and 188/230 (82%) who were transfused infected fetuses had a normal outcome as opposed to only 239/435 (55%) in the conservatively managed group. The average time from diagnosis to resolution in both groups was 6 weeks (range, 3-12 and 2-12 weeks, respectively). The most promising diagnostic techniques were PCR of amniotic fluid or fetal blood and electron microscopy. There are some reports of fetal abnormalities occurring (probably coincidentally) in cases of parvovirus, but the majority of postmortem findings were infection-related, in particular myocarditis and hepatic abnormalities. Although management guidelines cannot be derived from this study due to the variable degree of hydrops in the analyzed studies, the present data suggest a benefit of transfusion therapy over conservative management in infected fetuses. The only study which was corrected for severity of hydrops using ultrasound criteria showed a clear benefit of intrauterine transfusion.