The Role of Endolymphatic Hydrops in Patients with Pantonal Idiopathic Sudden Sensorineural Hearing Loss:A Cause or Secondary Reaction

The purpose of this study was to investigate the presence of endolymphatic hydrops(EH)in both affected and unaffected ears of patients with pantonal unilateral idiopathic sudden sensorineural hearing loss(ISSNHL)using three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging(3D-FLAIR MRI)and further evaluate the significance of EH in this disorder.Twenty-seven ISSHL patients were enrolled in this study.3D-FLAIR MRI was performed 24 h after intratympanic injection of gadolinium-diethylenetriaminepentaacetic acid(Gd-DPTA).The incidences of EH in the affected ears and contralateral unaffected ears were compared and the correlations of EH with vertigo or prognosis were analyzed using the Chi-square test.The results showed that the incidence of EH was 68.0%(17/25)in the affected ears and 34.8%(8/23)in the unaffected ears.There was a statistically significant difference between affected ears and unaffected ears in regard to the incidence of EH(P<0.05).There were no significant correlations of EH with vertigo(P=1.000)or with prognosis(P=0.359)in the affected ears.In conclusion,there is EH in the inner ear of patients with pantonal ISSNHL;EH is not related to vertigo,a concomitant symptom of ISSNHL,and the prognosis of this condition.The presence of EH may be a secondary reaction following the impairment of the inner ears with pantonal ISSNHL.

Dexamethasone does not affect endolymphatic hydrops(EH)in patients with Meniere's disease within 24 h,and intratympanic administration of gadolinium plus dexamethasone simplifies high-quality imaging of EH using a novel protocol of 7 min

Background:Intratympanic administration of gadolinium chelate allows for a better visualization of endolymphatic hydrops(EH)using MRI than intravenous injection and was recently further improved to facilitate high-quality imaging of EH in 7 min.The aim of the present study was to simplify the intratympanic administration protocol by mixing gadolinium chelate with therapeutic dexamethasone and to evaluate the effects of this mixture on the visualization of EH in MRI.Materials and methods:In an in vitro study,the potential impact of gadolinium-diethylenetriamine pentaacetic acid(Gd-DTPA)on the stability of dexamethasone was evaluated by analyzing dynamic changes in dexamethasone with high-performance liquid chromatography(HPLC)after mixing with GdDTPA.Ten patients with definite Meniere's disease(MD)were recruited to study the potential interference of dexamethasone on MRI visualization of EH,and 49 patients with MD were recruited to evaluate the effect of intratympanic injection of Gd-DTPA mixed with dexamethasone on MRI of EH using a 3T MR machine and a novel heavily T2-weighted 3-dimensional fluid-attenuated inversion recovery reconstructed using a magnitude plus zero-filled interpolation(hT2FLAIR-MZFI)sequence.Results:The retention times and peak area of dexamethasone in HPLC were not modified by the addition of Gd-DTPA.EH grading in the cochlea and vestibule was not influenced in any ear by intratympanic injection of dexamethasone.Excellent inner ear images were obtained from all patients,and EHs with various grades were displayed.There were significant correlations between diagnosis and cochlear EH(p<0.01,Spearman's Rho),between diagnosis and vestibular EH(p<0.01,Spearman's Rho),and between cochlear and vestibular EH(p<0.01,Spearman's Rho).The distribution of Gd-DTPA plus dexamethasone negatively correlated with the grade of vestibular EH.Injury of the endolymph-perilymph barrier was detected in one cochlea and three vestibules of 59 inner ears with MD.Conclusions:Intratympanic administration of Gd-DTPA plus dexamethasone yielded high-quality MRI images of EH in patients with MD using a novel 7-min protocol and simplified the clinical application.Intratympanic administration of Gd-DTPA plus dexamethasone might be used to test its therapeutic effect in future work.

Transcanal endoscopic assisted skull base endolymphatic sac tumor resection: A rare disease with advanced technology

Endolymphatic sac tumors(ELSTs) are rare, papillary adenomatous tumors that arise from the endothelium of the endolymphatic sac. We demonstrate a difficult case of endolymphatic sac tumor and how it is managed via transcanal endoscopic assisted technique, with discussion of feasibility of transcanal approach to lateral skull base tumor.

Endolymphatic hydrops in Meniere's disease secondary to otitis media and visualized by gadolinium-enhanced magnetic resonance imaging

Aimed to test the hypothesis that endolymphatic hydrops in Meniere's disease(MD) may be secondary to otitis media, history of a patient who developed MD as a complication of otitis media was reviewed. The inner ear was imaged using a 3.0 Tesla MR system post-intravenous injection of gadolinium-tetraazacyclododecane-tetraacetic acid(Gd-DOTA) in a standard single dosage(0.1 mmol/kg). Both t2-spc-rst-tra-iso(T2-weighted) and heavily T2-weighted 3-dimensional fluid-attenuated inversion recovery magnetic resonance imaging [h T(2)W-3D-FLAIR] sequences were applied. As a result, in the T2-weighted images, the perilymph and endolymph, cerebrospinal fluid surrounding the eighth nerve(N8), and middle ear granulation tissue showed intense signals. In the h T(2)W-3D-FLAIR images, evident enhancement by Gd-DOTA was observed in the middle ear cavity and the perilymphatic compartments of the cochlea. Cochlear endolymphatic hydrops was implicated by the enlarged scala media in the basalturn. In general, the Gd-DOTA uptake in the vestibule was weak, and signs of vestibular endolymphatic hydrops were obvious. The N8 on the diseased side was also significantly enhanced. To conclude, endolymphatic hydrops in MD may be induced by otitis media. Cochlear endolymphatic hydrops in MD secondary to otitis media may not follow the classical pattern.

MEFV,IRF8,ADA,PEPD,and NBAS gene variants and elevated serum cytokines in a patient with unilateral sporadic Meniere's disease and vascular congestion over the endolymphatic sac

The etiology and underlying mechanism of Meniere's disease(MD)development are still unknown,although inflammation and autoimmunity have been implicated as underlying mechanisms.The human endolymphatic sac(ES)has been reported to have innate and adaptive immune capacity in local immune reactions.In vivo demonstration of inflammation of the ES in patients with MD is missing in the literature.We report the case of a 47-year-old female patient diagnosed with unilateral MD with genetic variants and cytokine markers indicating inflammation and vascular congestion of the ES.Endolymphatic hydrops in the right cochlea(grade 2)and vestibulum(grade 3)were detected using MRI.She carried heterozygous variants in MEFV(c.442G>C),IRF8(c.1157G>T),ADA(c.445C>T),PEPD(c.151G>A),NBAS(c.4049T>C),CSF2RB(c.2222C>T),HPS6(c.277G>T),IL2RB(c.1109C>T),IL12RB1(c.1384G>T),IL17RC(c.260_271del GCAAGAGC TGGG),LIG1(c.746G>A),RAG1(c.650C>A),and SLX4(c.1258G>C,c.5072A>G).In the serum,the levels of granulocyte colony-stimulating factor(G-CSF),macrophage inflammatory protein 1a,and IL7 were significantly elevated,and the level of IL2Ra was reduced.Intratympanic administration of dexamethasone temporarily alleviated her hearing loss.Her vertigo was significantly relieved but remained slight after ES administration of corticosteroids.

Correlation of semi-quantitative findings of endolymphatic hydrops in MRI with the audiometric findings in patients with Meniere's disease

Purpose:To investigate the correlation between vestibular hydrops(VH),cochlearhydrops(CH),vestibular aqueduct non-visibility(VANV),and visually increased perilymphatic enhancement(VIPE)with the findings of pure-tone audiometry(PTA)in Meniere’s disease(MD)patients.Methods:In this cross-sectional study,53 ears belonging to 48 patients were divided into two groups and evaluated.In group“MD patients,”there were 24 ears of 19 patients diagnosed with the definite MD(14 patients with unilateral and 5 patients withbilateral involvements).The“control group”consisted of 29 non-symptomatic ears belonging to patients diagnosed with unilateral sudden sensory-neural hearing loss or unilateral schwannoma.All the patients underwent 2 sessions of temporal bone MRI using the same 3T system:an unenhanced axial T1,T2,and 3D-FLAIR MRI,an intravenous gadoliniumenhanced axial T1 fat-sat,and 4 h after the injection,an axial 3D-T2 cube and 3D-FLAIR session.VH,CH,VANV,and VIPE were assessed.Subsequently,the correlation between EH indices and PTA findings(in three frequency domains of low,middle,and high)were evaluated,and the predictive value of MRI was calculated.Results:VH was significantly correlated with the hearing threshold in the low,middle,and highfrequency domains.CH was also correlated with the hearing threshold in the low and middle domains.Contrarily,VIPE was not associated with hearing thresholds,and VANV was only correlated with the hearing threshold in low frequencies.Conclusion:The grade of VH,CH,and VANV were significantly correlated with the hearing thresholds in PTA.

Contralateral delayed endolymphatic hydrops: Clinical features and long term outcome

Background:Contralateral delayed endolymphatic hydrops(CDEH)is a clinical entity characterized by fluctuating low frequency hearing loss and/or vertigo,mimicking Meniere’s disease(MD),that manifests after the appearance of severe non-hydropic hearing loss(NHHL)at the other ear.Objectives:to describe the clinical features and the course of 57 patients affected by CDEH.Method:this is a retrospective study;57 patients affected by CDEH,out of 1065 patients seen in the same period and affected by MD,were subjected to otoscopy,PTA threshold evaluation,impedance testing,ABR,research of positioning nystagmus,vestibular function evaluated by means of bithermal caloric test under video-oculographic,and MRI with gadolinium.Results:the CDEH was definite in 24 cases(42%),probable in 2(4%)and possible in 31(54%).The mean PTA threshold at the hydropic ear was 41 dB.At the last follow-up,40 patients(70%)did not report vertigo or fluctuating hearing loss.Among the 17 patients who still reported symptomatology,11(64%)were affected by fluctuating hearing loss alone,4(23%)reported a subjective worsening of hearing loss and 2(12%)an acute vertigo crisis.Conclusions:contralateral delayed endolymphatic hydrops is a relatively rare form of Meniere disease that manifests more frequently as a definite form or with fluctuating low-frequency hearing loss.The prognosis at a long term follow-up is relatively good in terms of vertigo resolution.Contralateral delayed endolymphatic hydrops rarely determines a severe hearing loss in the better ear.

Cell proliferation in the endolymphatic sac in situ during the immune response of inner ear

Background Normally, few immunocompetent cell are present in the endolymphatic sac (ES). During an active immune response in the inner ear, large amount of inflammatory cells, including immunocompetent cells, are seen in the ES. The current study aimed at assessing cellular proliferation within the ES during induced immune response in the inner ear. Methods Fifteen healthy, female SD rats were sensitized systemically with keyhole limpet hemocyanin (KLH), followed by local inoculation in the cochlea through basal turn fenestration with the same antigen. On Days 3, 7 and 14 following inoculation, the animal was sacrificed after intraperitoneal administration of 5-bromo-2'-deoxyuridine (BrdUrd), and the temporal bone harvested. Following decalcification, infiltration by BrdUrd- and IgG-positive cells in the ES was studied on frozen sections with H & E and immunohistochemical staining. Results During the secondary immune response in the inner ear against T-dependent antigens, there is increased cellular proliferation in the ES. The proliferated cells may differentiate into immunocompetent cells at the same location. Conclusions These findings indicate that the ES plays an important role in immune response of inner ear.

Management of endolymphatic sac tumors:a case series report

Objective To study clinical characteristics of endolymphatic sac tumor (ELST) and its diagnosis and treatment. Methods ELST was diagnosed in 6 cases based on surgical and histological findings. These cases were reviewed for their clinical manifestations, differential diagnosis and surgical treatment techniques. Results There were 1 male and 5 females in this group, aged from 28 to 59 years (mean age=38.7 years). The tumor was in left ear in 4 cases and in right ear in the other 2 cases. Disease courses ranged from 5 to 30 years (mean duration=12.6 years). Clinical presentations included sensorineural hearing loss (n=4), otorrhea and tinnitus (n=2), tinnitus and facial spasm(n=1), otorrhea with facial paralysis(n=1), and hearing loss with tinnitus (n=2). None of the cases was diagnosed as ELST preoperatively. Two cases were misdiagnosed as glomus jugulare tumor, 2 as chronic suppurative otitis media, 1 as sweat gland adenoma on biopsy and 1 as temporal bone tumor. Tumors were surgically resected in all 6 cases via the mastoidectomy (n=2) or combined oto-cervical or cranio-oto-cervical approaches(n=4). Postoperative cerebrospinal fluid otorrhea occurred in 1 case. The tumors were confirmed on histological examination to be a low -grade adenocarcinoma. All patients have survived at the time of this paper. Conclusion ELST is rare and commonly misdiagnosed and inadequately treated. Its prognosis is relatively favorable because of its slow growth rate.

Effects of electroacupuncture on cochlear morphology and expression of aquaporins in rats with AVP-induced endolymphatic hydrops

Objective: The objective of this study was to investigate the effects of EA on EH and the regulation of AQP2 and AQP7 protein expression in rats.Methods: Twenty-four rats were allocated randomly to four groups of blank group, EH group,EH+tolvaptan group and EH + EA group(n = 6 per group). EH rat model was established by intraperitoneally injection of arginine vasopressin(AVP). EA was administered at acupoints "Baihui(百会 GV 20)"and "Tinggong(听宫 SI 19)". Rats in the EH + tolvaptan group and EH+ EA group were treated with tolvaptan and EA, respectively, after EH establishment. Hematoxylin-eosin staining was used to measure the cochlear hydrops degree, and then the ratio of scala media(SM) area to SM + scala vestibuli(SV) area(R value) was calculated. Immunohistochemical method was used to observe AQP2/AQP7 protein expression in the rat cochlear lateral wall after treatment.Results: ①There was no endolymphatic hydrops in the blank group. Reissner' s membrane was extended markedly and bulged into SV in cochleae of the EH group and endolymphatic hydrops was noted. Statistical analysis revealed that R value in the EH group showed a significant increase compared with that in the blank group(0.42 ± 0.02 vs. 0.31 ± 0.05, P=0.000). The distension of Reissner's membrane was less obvious in the EH + tolvaptan group and EH + EA group when compared with the EH group. R value in the EH + tolvaptan group and the EH + EA group was significantly less than that in EH group(0.32±0.04 vs. 0.42 ± 0.02, =0.001;0.35 ± 0.05 vs. 0.42 ± 0.02, P=0.012). The degree of the hydrops in the EH + EA group was not different from that in the EH + tolvaptan group(0.35 ± 0.05 vs. 0.32 ±0.04,P= 1.000). ②The AQP2 protein expression in the rat cochlear lateral wall of EH group was significantly increased when compared with the blank group(12.74 ± 5.18 vs. 5.92 ± 1.52, P = 0.014). The AQP2 protein expression in the rat cochlear lateral wall of EH + tolvaptan group and EH + EA group were all lower than that of the EH group(6.52 ± 2.73 vs. 12.74 ± 5.18. P = 0.029;6.95 ± 3.10 vs. 12.74 ± 5.18, P = 0.047).The AQP2 protein expression in the rat cochlear lateral wall of EH + EA group was not different from that in the EH + tolvaptan group(6.95 ± 3.10 vs. 6.52 ± 2.73, P= 1.000).③The AQP7 protein expression in the rat cochlear lateral wall of EH group was significantly increased when compared with the blank group(30.32 ± 6.39 vs 16.64 ± 3.21, P=0.000). The AQP7 protein expression in the rat cochlear lateral wall of EH + tolvaptan group and EH + EA group were all lower than that of the EH group(18.32 ± 2.45 vs.30.32 ± 6.39, P= 0.001;19.54 ± 4.61 vs. 30.32 ± 6.39, P= 0.003). The AQP7 protein expression in the rat cochlear lateral wall of EH + EA group was not different from that in the EH + tolvaptan group(19.54 ±4.61 vs. 18.32 ± 2.45, P= 1.000).Conclusions: These results indicate that repeated EA stimulation exerted the same effects as tolvaptan application on AQPs levels and subsequent aquaretic effects. And dehydrating effect of EA on the inner ear might be associated with its down-regulation of both AQP2 and AQP7 protein expression, thereby provide a potential molecular mechanism involved in the treatment of Meniere's disease by EA.

Arginine Vasopressin-Aquaporin-2 Pathway-Mediated Dehydration Effects of Electroacupuncture in Guinea Pig Model of AVP-Induced Endolymphatic Hydrops

Objective: To investigate the effects of electroacupuncture(EA) on endolymphatic hydrops(EH) and the regulation of arginine vasopressin(AVP)-aquaporin-2(AQP2) pathway in guinea pigs. Methods: EH was induced in male guinea pigs by an intraperitoneal injection of AVP. For the treatment, EA was delivered to Baihui(GV 20) and Tinggong(SI 19) acupoints, once per day for 10 consecutive days. In histomorphological studies, cochlear hydrops degree was evaluated by hematoxylin-eosin(HE) staining, and then the ratio of scala media(SM) area to SM + scala vestibuli(SV) area(R value) was calculated. In mechanical studies, a comparison of plasma AVP(p-AVP) concentrations, cyclic adenosine monophosphate(cAMP) levels, vasopressin type 2 receptor(V2R) and AQP2 mRNA expressions in the cochlea were compared among groups. Results: EA significantly reduced cochlear hydrops in guinea pigs(P=0.001). EA significantly attenuated the AVPinduced up-regulation of p-AVP concentrations(P=0.006), cochlear c AMP levels(P=0.003) and AQP2 mRNA expression(P=0.016), and up-regulated the expression of V2R mRNA(P=0.004) in the cochlea. Conclusion: The dehydrating effect of EA might be associated with its inhibition of AVP-AQP2 pathway activation.

Effect of electroacupuncture on arginine vasopressin-induced endolymphatic hydrops

OBJECTIVE: To investigate the influence of electroacupuncture(EA) on experimentally induced endolymphatic hydrops(EH) in guinea pigs, and elucidate the association between the dehydrating effect of EA and changes in stria vascularis ultrastructure and expression of vasopressin type 2 receptor(V2R), cyclic adenosine monophosphate(cAMP),and aquaporin 2(AQP2) in the endolymphatic sac(ES).METHODS: The EH model was established by intraperitoneal injection of arginine vasopressin(AVP).As a treatment, EA was delivered to Baihui(GV 20)and Tinggong(SI 19) acupoints, once daily for 10 consecutive days. For histomorphological studies,degree of cochlear hydrops was evaluated by hematoxylin-eosin staining, and the ratio of scala media(SM) area to SM + scala vestibuli area was calculated. In mechanical studies, ultrastructural changes in stria vascularis tissue were examined by transmission electron microscopy. In addition, cAMP levels and mRNA expression levels of V2 R and AQP2 in the ES were compared among groups.RESULTS: EA treatment significantly reduced cochlear hydrops compared with hydropic guinea pigs(P = 0.015). Furthermore, EA attenuated ultrastructural changes in the stria vascularis tissue following EH, significantly upregulated the expression of V2 R(P = 0.016), and attenuated AVP-induced upregulation of both cAMP(P = 0.038) and AQP2 expression(P = 0.017) in the ES.CONCLUSION: Collectively, the results of the present study suggest that the dehydrating effect of EA is associated with improvement of stria vascularis ultrastructure and V2 R-cAMP-AQP2 signaling pathway regulation in the ES.

MRI detection of endolymphatic hydrops in Meniere's disease in 8 minutes using MIIRMR and a 20-channel coil after targeted gadolinium delivery

Background:Endolymphatic hydrops(EH)become visible in vertigo patients,particularly in those with Meniere's disease(MD),in vivo using gadolinium-enhanced MRI.However,the image quality is not satisfying after intravenous injection of gadolinium chelate(GdC),and occasional failure in GdC uptake has been noticed after traditional intratympanic injection.In the present report,targeted delivery of GdC and using a cost-effective MRI system to obtain high quality images of EH in only 8 min will be fntroduced.Methods:39 MD patients were recruited in the study.First,0.1 ml of 20-fold diluted gadolinium-diethylenetriamine acid(Gd-DTPA)was delivered onto the posterior upper part of the tympanic medial wall using a soft-tipped micro-irrigation catheter through an artificially perforated tympanic membrane.Inner ear MRI was performed 24 h after Gd-DTPA administration using a 3T MR machine and a 20-channel head/neck coil with an 8 min sequence of medium inversion time inversion recovery imaging with magnitude reconstruction(MIIRMR).The parameters were as follows:TR 16000 ms,TE 663 ms,inversion time 2700 ms,flip angle 180°,slices per slab 60.Results:Efficient inner ear uptake of Gd-DTPA was detected 24 h after delivery and it created excellent contrast in the inner ear of all cases.High quality images demonstrating EH in the vestibule and cochlea were obtained.Conclusion:Targeted delivery of minimum Gd-DTPA(0.1 ml,20-fold dilution)onto the posterior upper portion of the tympanic medial wall and MRI with MIIRMR in a 3T machine and 20-channel head/neck coil are clinically practical to obtain high quality images displaying EH.

Endolymphatic sac tumor: case report and literature review

Background:Endolymphatic sac tumor(ELST)is one of neuroectodermal tumor which arising from endolymphatic sac and duct.It is actually quite rare,with less than 200 cases reported.Although ELST presents benign appearance in histopathology,it can present aggressive destructive behavior in clinical.The cornerstone of treatment for ELST is complete surgical excision.However,it is almost impossible to completely resect the advanced stage tumor.There is still controversy about other treatments,such as radiotherapy and gamma knife surgery.Case presentation:A 47-year-old man was admitted in The First Affiliated Hospital of Fujian Medical University with a 7-year history of progressive hearing loss and near 6-month repeated attacks of headache.Preoperative CT revealed a massive intracranial lesion and associated hydrocephalus.MR scanning demonstrated a 7.2 cm×4.6 cm×4.2 cm bulky mass located in left-sided posterior cranial fossa and temporo-occipital region which showed hyperintensity on T1-weighted images and mixed signal intensity on T2-weighted images.There was no neither clinical manifestation nor family history of Von Hippel–Lindau syndrome(VHL).Due to the mass that was large and invading the bone of skull base,it was difficult to extirpate surgically,so the ventriculoperitoneal shunt combined with local biopsy was performed.The postoperative pathology and immunohistochemical findings confirmed the lesion was an endolymphatic sac tumor.After operation,the patient regularly received radiotherapy.Conclusion:The widely accepted management of ELST is complete surgical resection.However,it is difficult for surgeons to achieve radical resection with late-stage ELST.Currently,there is much dispute about the role of radiotherapy for the management of ELST in academic circles.In this case where the mass cannot be surgical removed,radiotherapy has the curative effect for ELST in terms of disease control and quality of life.

内淋巴囊手术

梅尼埃病是以发作性眩晕、波动性耳聋、耳鸣和(或)耳胀满感为特征的特发性内耳病。目前梅尼埃病的治疗尚无国际统一规范,主要是经验治疗。对于药物治疗无效的患者,可选择手术治疗。内淋巴囊手术属于保存听力的功能性手术,适应于早中期梅尼埃病患者,尤其是听力尚存在波动的患者。内淋巴囊手术主要分为内淋巴囊减压术和内淋巴囊引流术,其总体眩晕控制率在60%~80%。

内淋巴囊低度恶性腺癌1例并文献复习

内淋巴囊肿瘤（endolymphatic sac tumor,ELST）是来自内淋巴囊和内淋巴管上皮的肿瘤,又称内淋巴囊低度恶性腺癌。因其发病率低、发展慢,症状缺乏特异性,肿瘤又位于侧颅底、位置深在、周围解剖复杂且瘤体血供丰富,因此该病术前诊断困难,术中切除难度大,术后并发症多且重。为促进对该病的认识并给以恰当的治疗,我们结合近来收治的1例ELST，并复习相关的文献，报道如下。

Diuretic Agents in Treatment of Sudden Deafness

Objective To study the role of diuretic agents in treating sudden deafness (SD) and explore the possibility of endolymphatic hydrops as a potential cause of SD. Methods Twenty-eight SD cases were reviewed. In 23 cases, treatment was initiated with routine agents. Diuretic agents were later added in 8 of these cases that failed to respond to routine treatment agents. Diuretic agents were included in the initial treatment in the rest 5 cases. In total, 13 cases received diuretics in addition to routine treatment agents and 15 cases received conventional treatment only. Results In the 8 cases who received diuretics after failed conventional treatments, 4 showed hearing improvement, whereas all 5 cases in which diuretics were included in the initial treatment demonstrated hearing improvement. Conclusion These results suggest a possible role of endolymphatic hydrops in the pathophysiologic course of SD. Diuretics should be considered when clear indications exist with no conflicts to other medical conditions.

Morbus Menière: Were the last 50 years of molecular biological research fruitless for Menière's disease?

After discovering an inner ear hemorrhage, Prosper Menière ascribed disease to the inner ear for the first time. Since that time, a lot of efforts have been made to determine the pathophysiologic causes of the classical symptoms sensorineural hearing loss, vertigo attacks, tinnitus and ear fullness. According to its express pattern Menière's disease may appear as classical andatypical disease. In the last decades, huge advances have taken place in biochemical and physiological research and in pathophysiological understanding of the inner ear and its diseases. This encloses stimulus perception and conduction, regulation of inner-ear fluid homeostasis and inner ear diseases with underlying genetics. Menière's disease pathophysiologic correlate is an endolymphatic hydrops which is characterized by changes of inner ear homeostasis with its parameters volume, concentration, osmolarity and pressure of the endolymph. Hormones, autonomous system and the immunsystem together with purinergic, adrenergic and muscarinic receptors, steroids, vasopressin, atrial natriuretic peptide and aquaporin channels regulate inner ear homeostasis. Consequently, general diagnostics comprise a magnetic resonance imaging with gadolinium, vestibular diagnostics and tone audiometry. Standard therapy for acute inner ear symptoms is limited to cortisone infusions together with a rheologic agent or a radical scavenger. For acute vertigo attacks and for the mainstay therapy antivertiginous pharmaceuticals are given. In severe cases destruction of the vestibular hair cells by ototoxic antibiotics, endolymphatic sac surgery or neurectomy of the vestibular nerve might be necessary. Certainly, in research there is a move from simple pharmaceutical therapy forward to nanoparticlebased, genetic-based and stem cell therapy.

Comparison of Transtympanic and Extratympanic Electrocochleography

Electrocochleography (ECoG) has been an important tool in the diagnosis of Meniere’s disease or endolymphatic hydrops. There are two methods employed, transtympanic and extratympanic. Many have regarded the results of these methods as being equally reliable. The purpose of this study is to determine any differences in sensitivity between the two methods. In this study patients with known endolymphatic hydrops or Meniere’s disease underwent ECoG testing with both the extratympanic method and the transtympanic method on the same day in the same ear. The results show a significant difference between the two methods, with the transtympanic wave values being smaller and therefore more sensitive than the extratympanic method. In addition, transtympanic ECoG resulted in better waveform morphology and better correlation with the audiometric findings in endolymphatic hydrops and Meniere’s disease. The results emphasize the superior role of transtympanic ECoG over extratympanic ECoG as a valuable component in the confirmation of Meniere’s disease or endolymphatic hydrops along with history and audiometric findings characteristic of the disease. Implications of the study promote the use of transtympanic ECoG rather than extratympanic ECoG in patients with symptoms suggestive of Meniere’s disease or endolymphatic hydrops.

突发性聋与内淋巴积水

内淋巴积水一直被看作梅尼埃病的病理基础,但目前的有关研究表明某些突发性聋患者中也存在内淋巴积水,且所占比例不容忽视。本文从特发性突聋的病因、病理、耳蜗电图改变、积水的比率和意义以及突聋的治疗等方面来综述突发性聋与内淋巴积水关系的研究进展。