Delayed endolymphatic hydrops and its relationship to Meniére's disease

Delayed endolymphatic hydrops (EH) can be characterized as having ipsilateral and contralateral types. They are similar in that both have early and late phases of otologic symptoms and that the early phase is a profound hearing loss in one ear. The late phases differ, however, in that the ipsilateral type develops the symptoms of EH (episodic vertigo) in the deaf ear and the contralateral type develops the symptoms of EH (fluctuating hearing loss and/or episodic vertigo) in the hearing ear. In more than half the cases of both types of delayed EH, the profound hearing losses in the early phase are simply discovered to be present in early childhood without a known time of onset. The temporal bones of two patients with contralateral delayed EH show pathologic changes in the deaf ears that are similar to those known to occur in mumps and measles labyrinthitis, whereas the pathologic changes in the hearing ears are similar to those known to occur in Meniere's disease. These observations support the proposition that Meniere's disease may occur as a delayed sequela of inner ear damage sustained during an attack of subclinical viral labyrinthitis occurring in childhood.     

Autoimmune reactivity in Ménière's disease: a preliminary report

Autoimmune inner ear disease was first described by McCabe in 1979. The diagnosis is supported by cell mediated immune responses to inner ear membrane antigen stimulation. Meniere's disease consists of episodic vertigo, fluctuating sensorineural hearing loss, tinnitus and often aural pressure. Although most clinicians feel that Meniere's disease has no known cause, recently several researchers have suggested that some cases of Meniere's disease may be autoimmune in origin. In the present study, 10 patients with bilateral Meniere's disease were studied prospectively for autoimmune inner ear disease using both cellular and humoral immune tests. Results were compared with normal control subjects. One additional patient with Meniere's disease was identified retrospectively to have positive autoimmune test results. Preliminary conclusions suggest that at least some cases of presumed idiopathic Meniere's disease are autoimmune in origin. Therapeutic implications are significant: the addition of steroids, cytotoxic drugs and/or apheresis to conventional treatment may help prevent total deafness which otherwise might be inevitable.     

Clinical diagnosis of immune inner-ear disease

The purpose of this study was to develop a high-risk clinical profile of patients with autoimmune (immune-mediated) inner-ear disease. The records of 52 patients diagnosed over the past 5 years were reviewed. Age, sex, bilateral versus unilateral involvement, otologic symptoms, concomitant systemic immune disease, and presenting clinical diagnoses were recorded. The presenting diagnoses were Cogan's syndrome, Meniere's syndrome, Dandy's syndrome without hearing loss, or progressive sensorineural hearing loss without dizziness. Because Cogan's and Dandy's syndromes were relatively uncommon, the typical high-risk clinical profile was a middle-aged patient (often female) with bilateral, asymmetric, progressive sensorineural hearing loss, with or without dizziness, and occasional systemic immune disease such as rheumatoid arthritis. When a more common clinical diagnosis cannot be reached in suspicious patients, immune laboratory tests should be obtained and a trial of immunotherapy offered. Positive test results and beneficial response to therapy support a presumptive diagnosis of immune inner-ear disease.     

Validity of the Western blot immunoassay for heat shock protein-70 in associated and isolated immunorelated inner ear disease

OBJECTIVE: To assess the validity of the Western blot immunoassay for heat shock protein-70 (hsp-70) for diagnosis of autoimmune inner ear disease. STUDY DESIGN: Retrospective study of 53 patients affected by sudden deafness (n = 19), idiopathic progressive sensorineural hearing loss (n = 24), and Meniere's disease (n = 10) who were treated from 1995 to 1999. The clinical course and response to corticosteroid were evaluated. METHODS: A purified hsp-70 antigen from bovine kidney cell line was used for the Western blot immunoassay. RESULTS: Only five patients (9.4%) showed anti--hsp-70 antibodies: Two presented a sudden sensorineural hearing loss (sudden deafness group), two showed an idiopathic progressive sensorineural hearing loss (idiopathic progressive sensorineural hearing loss group), and one was affected by fluctuating hearing loss (Meniere's disease group). A systemic autoimmune condition was observed in 29.1% of patients with idiopathic progressive sensorineural hearing loss. CONCLUSIONS: The low sensitivity of Western blot immunoassay for patients affected by idiopathic progressive sensorineural hearing loss and Meniere's disease may result from either the long time elapsed from the hearing loss and vertigo to the initial examination or from the increased percentage of cases of systemic autoimmune disease present in patients with idiopathic progressive sensorineural hearing loss. More studies to detect the immune-mediated inner ear disease in Western blot immunoassay-negative patients are required.     

Acute inner ear deafness]

Sudden inner ear hearing loss initially might suggest a psychogenic disorder of hearing, particularly when it is bilateral and simultaneous. The differential diagnosis includes disseminated encephalitis, syphilitic labyrinthitis and Cogan's syndrome. The history and cause of acute bilateral deafness in meningitis are easy to recognise. Furthermore, unilateral acute inner ear deafness should not be regarded as idiopathic without further consideration. A acoustic neuroma is a possible cause even of a low-tone hearing loss. More controversial is rupture of the round window membrane as a cause of sudden deafness. The deafness after epidemic parotitis obviously leads to a total unilateral hearing loss in every case. Even labyrinthine apoplexy with loss of hearing and vestibular function can be caused by a tumour of the cerebellopontine angle. Idiopathic sudden deafness should be defined as an acute sensory hearing loss whose anatomical basis in an acute vascular endolymphatic hydrops of unknown cause. The sudden deafness affects only one ear; tinnitus and brief vertigo can be accompanying symptoms. A sudden hearing disorder due to other causes should be distinguished from idiopathic lesions.     

Cogan's syndrome: a cause of progressive hearing deafness

In 1934 Morgan and Baumgartner first described a nonsyphilitic interstitial keratitis (IK) associated with vestibuloauditory dysfunction (Morgan RF, Baumgartner CF, Menier's disease complicated by recurrent interstitial keratitis. Excellent result following cervial ganglionectomy. West J Surg 1934;42:628). Cogan was the first to describe this syndrome as a clinical entity with the report of 5 additional cases in 1945 (Cogan DA: Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol 1945;33:144-9). Since that time, more than 100 cases of Cogan's syndrome (CS) have been in the literature. Cogan's syndrome is a rare clinical disease, which primarily affects young adults; however, published reports range from 2.5 to 60 years for age of onset (Kundell Sp, HD Ochs: Cogan's syndrome in childhood. J Pediatr 1980;97:96-8). This disease primarily affects whites and is believed not to be hereditary. Typical CS is characterized by IK and vestibuloauditory dysfunction. The IK usually occurs with sudden onset and is characterized by photophobia, lacrimation, and eye pain. The vestibuloauditory dysfunction is usually bilateral, presenting with tinnitus, sensorineural hearing loss, and acute episodes of vertigo. Atypical CS presents with significant inflammatory eye disease (ie, scleritis, episcleritis, retinal artery occlusion, choroiditis, retinal hemorrhages, papilloedema, exophthalmos, or tenonitis) with or without IK (Laryngoscope 1960;70:447-9). In this report, we describe a typical case of CS, in which the hearing loss was unresponsive to corticosteroid therapy.     

Is recurrent loss of low frequency tone perception--without vertigo--a precursor of Meniere disease?

PURPOSE: Recurrent low-frequency sensory hearing loss is one characteristic sign of Meniere's disease. It is therefore often assumed to be a prodromal sign of Meniere's disease. METHODS: We report on 81 patients with recurrent low-frequency sensory hearing loss who did not previously suffer from vertigo. All patients underwent a follow-up examination after at least 1 year up to more than 10 years after the onset of the disease [average: 64.65 months (SD 56.33)]. CONCLUSIONS: Only 3.7% of these patients developed the typical signs of Meniere's disease with labyrinthine vertigo, whereas 25.9% suffered from recurrent hearing loss and an unspecific vertigo, which could be diagnosed and treated as psychogenic vertigo; 3.7% showed a typical benign positional vertigo. We can thus conclude that although almost every patient with Meniere's disease suffers from recurrent hearing loss, only a few patients with recurrent hearing loss will develop Meniere's disease. However, many patients with low-frequency sensory hearing loss develop anxiety and psychogenic dizziness in expectation of "imminent" Meniere's disease. Therefore, it is important to counsel the patients carefully that the probability of this occurring is not very high.     

Definition of fluctuant hearing loss.

In summary, fluctuant hearing loss is defined as a disorder of the inner ear characterized by fullness, roaring tinnitus, and fluctuations in hearing. It is believed to be caused by an inadequate absorption of endolymph from the endolymphatic sac, with or without one or more metabolic disorders, that interferes with the delicate balance between the production and absorption of endolymph and thus produces cochlear hydrops. This triad of fullness, roaring tinnitus, and fluctuant hearing loss resulting from cochlear hydrops is much more common than the quadrad of true turning vertigo, fullness, roaring tinnitus, and fluctuant hearing loss due to vestibular and cochlear hydrops known as Meniere's disease. Although patients with fluctuant hearing loss only may eventually develop vertigo as the chief complaint and then be said to have Meniere's disease, it is remarkable how many patients continue to suffer mainly from cochlear symptoms at all times. It would appear, because of the greater frequency of fluctuant hearing loss than in Meniere's disease, that the cochlear labyrinth is more susceptible to hydrops than the vestibular labyrinth. For the purposes of diagnosis and treatment it is very useful to separate patients into those with fluctuant hearing loss and those with Meniere's disease.     

Hearing loss and tinnitus in Meniere's disease

OBJECTIVES: To characterize hearing loss, tinnitus and associative factors in Meniere's disease. METHODS: From our vertigo database consisting of 1356 patients, we retrieved 243 patients with Meniere's disease (MD). RESULTS: Hearing loss was the initial symptom in 13% of cases. Altogether 64% (n=133) of the patients stated that their hearing was reduced during the vertigo attack. The hearing deteriorated more likely during the vertigo attack if the vertigo attack was very intense [r(206)=0.19]. Tinnitus was the initial symptom in 5% of cases. The tinnitus was mild in 38% (n=90), moderate in 32% (n=76) and severe in 30% (n=72) of patients. The intensity of tinnitus correlated with the occurrence of drop attacks [r(237)=0.29], vertigo provoked by head positioning [r(235)=0.25], by physical activity [r(230)=0.33], or by pressure changes [r(239)=0.27]. CONCLUSION: Prolonged disease causes deterioration of hearing. Intense tinnitus is common in MD and is more often seen in late stage of the disease.     

Vestibular type of Mondini anomalies with BPPV and Meniere's disease-like symptoms

An extremely rare case with labyrinthine anomaly with normal hearing was demonstrated. This case firstly showed transient positioning vertigo like benign paroxysmal positional vertigo (BPPV) and subsequently episodic vertigo like Meniere's disease. A 55-year-old male attended our hospital, complaining of transient but persistent positioning vertigo in 2004. The apogeotropic positioning nystagmus was observed in spine position. In 2005, he came to feel episodic vertigo continuously for more than 30min like Meniere's disease. We examined CT scan and 3D-MRI, indicating the hypoplastic lateral semicircular canal (L-SCC) fused together with enlarged vestibule on the left side. We finally diagnosed this case as Mondini anomaly on the left side. The imaging analysis is the most useful for differential diagnosis of Mondini anomalies, because symptoms in these patients are actually various due to the individual inner ear condition. The mechanism of generation of BPPV-like vertigo: the otolith in deformed utricle might be transferred into the fused space with vestibule and L-SCC, resulting in the irrigation of the hypoplastic cupula. The mechanism of generation of Meniere's disease-like vertigo: neuro-otologic findings with furosemide test implied endolymphatic hydrops on the left side. Meniere's disease-like episodic vertigo could be due to subsequently generated endolymphatic hydrops.     

Clinical statistics of recurrent acute low-tone senseorineural hearing loss

Recently, acute low-tone senseorineural hearing loss (ALHL) has become common, and its good prognosis is known well. On the other hand, several reports have suggested that ALHL is frequently associated with Meniere's disease. We retrospectively examined the clinical course of 357 cases that were diagnosed and treated as ALHL at our hospitals. Forty-four of these cases that showed high-tone hearing loss in association with age-related changes were classified as atypical cases. The clinical futures of 49 "poor prognosis cases", who experienced recurrent hearing loss and/or profound hearing loss, are reported. Eight of the 49 cases who experienced recurrences had progressive hearing loss upto middle or high tones. Seventeen cases complained of vertiginous sensation, and 8 of these cases experienced recurrent attacks of vertigo and were diagnosed as having Meniere's disease. The former seventeen cases accounted for 34.7% of the "poor prognosis cases", and the latter eight accounted for 16.3% of these cases. Our results suggest that the hearing loss is more frequently associated with Meniere's disease in cases who experience recurrent hearing loss. Thus, cases initially diagnosed as ALHL may include some cases of progressive hearing loss and Meniere's disease. Even in cases in which hearing improvement is obtained, careful clinical observation is necessary, especially in older patients with bilateral affliction and atypical presentation. ALHL has been generally considered to have a good prognosis, however our examination revealed a relatively high frequency of recurrences, progressive hearing loss and complication by vertigo. We recommend, based on this evidence, that careful explanation of this disease is necessary at time of initial informed consent.     

Vestibular nerve section versus intratympanic gentamicin for Meniere's disease

OBJECTIVES/HYPOTHESIS:Vestibular nerve section and transtympanic gentamicin administration are procedures with proven efficacy in the treatment of vertigo associated with Meniere's disease refractory to medical management. Hearing loss is a known complication of each of these procedures; however, there has not been a report of hearing results of both treatments from a single institution. STUDY DESIGN: Retrospective review. METHODS: Review was made of 25 patients undergoing gentamicin injection and 39 patients undergoing vestibular nerve section for Meniere's disease. Rate of vertigo control and pretreatment and post-treatment pure-tone average values and speech discrimination scores were reported. RESULTS: The mean preoperative pure-tone average for patients having vestibular nerve section was 47.2 dB, with a speech discrimination score of 75.4%. In these patients, the postoperative pure-tone average was 49.1 dB and the speech discrimination score was 75%. Patients undergoing gentamicin injection had a mean pretreatment pure-tone average of 55.9 dB and a speech discrimination score of 62%. The post-treatment pure-tone average and speech discrimination score for the gentamicin group were 68.8 dB and 49.3%, respectively. Five of 25 patients (20%) in the gentamicin treatment group and 1 of 39 (3%) in the vestibular nerve section treatment group had an increase in bone-conduction threshold greater than 30 dB. The amount of postprocedure hearing loss was significantly greater in the gentamicin treatment group (P =.006). Control of vertigo was good to excellent in 95% of the patients treated with vestibular nerve section and in 80% of the patients treated with gentamicin. CONCLUSION: Although vestibular nerve section and transtympanic gentamicin are both acceptable treatment options for vertigo associated with Meniere's disease, gentamicin causes a higher level of hearing loss related to treatment and vestibular nerve section has higher vertigo control rates.     

小剂量庆大霉素鼓室内注射治疗难治性梅尼埃病眩晕的临床研究

目的：分析小剂量庆大霉素鼓室内注射治疗难治性梅尼埃病眩晕的疗效。方法：19例单侧难治性梅尼埃病患者显微镜下鼓室内注射庆大霉素,根据床旁试验（自发性眼震、摇头实验、甩头试验）,听力变化或患者主观症状来决定3周后是否需要再次注射。结果：19例患者中17例眩晕能得到控制,眩晕控制率89％,其中5例患者经1次注射后眩晕就得到控制;8例患者2次注射后眩晕达到控制,其中1例患者因不能忍受耳内肿胀感,要求进一步治疗,行内淋巴囊减压后,症状缓减;有4例患者经3次注射后眩晕得到控制;另外2例患者注射2次后眩晕无改善,要求终止治疗。2例患者注射后听力好转,14例患者注射后听力无变化,3例患者（15％）注射后听力进一步下降。结论：运用小剂量鼓室内庆大霉素注射治疗难治性梅尼埃病眩晕,1次注射后观察3周决定再次注射的必要性,结果显示这一治疗方案既能有效控制眩晕的发作,又能降低听力损伤的风险。     

Delayed vertigo and profound sensorineural hearing loss

Episodic vertigo, similar to that of Meniere's disease, can occasionally develop years to decades after an ear has suffered a profound sensorineural hearing loss. Although the pathogenesis of this process is unknown, it is theorized that delayed endolymphatic hydrops develops. An inflammatory reaction could cause obstruction of the endolymphatic duct or interfere with venous drainage from the region of the endolymphatic sac, leading to hydrops. This condition is reviewed, and two adult patients with severe episodic vertigo and a unilateral profound sensorineural hearing loss which occurred in childhood are presented. Their evaluation and management are discussed.     

Hearing results and control of vertigo after retrolabyrinthine vestibular nerve section

To determine the effect of retrolabyrinthine vestibular nerve section (RVNS) on hearing, vertigo, and associated symptoms, we reviewed our experience in 48 patients. Of the 48, 39 responded to a questionnaire. Although RVNS appeared to have little effect on hearing in Meniere's patients, 91% of non-Meniere's patients had significant and often delayed postoperative sensorineural hearing loss. Our results for vertigo control compared favorably to previous reports with 96% of Meniere's patients and 69% of non-Meniere's patients reporting improvement. Presently, we more frequently recommend RVNS as the primary procedure for the control of severe vertigo in Meniere's patients. Patients with vertigo from other causes must be carefully selected.     

Menière's disease in children

Fourteen children (aged 14 years or younger) with typical Ménière's triad with cochlear sensorineural hearing loss, tinnitus, and intermittent vertigo attacks lasting from minutes to hours were investigated in four different neuro-otologic centers. Nine children, labeled as having "idiopathic Ménière's disease," developed the auditory and vestibular symptoms without any detectable causative factor. Five children, labeled as suffering from "secondary Ménière's syndrome," had histories of an initial hearing loss following mumps, hemophilus influenza meningitis, temporal bone fracture, or congenital or embryopathic complications in the ear that developed into the full Ménière's triad 5 to 11 years later. The 14 children represent 1% of all cases affected with idiopathic or secondary Ménière's disease (or syndrome) that have been detected during the past five years in the four collaborating centers.     

梅尼埃病首发症状的临床分析

目的了解梅尼埃病患者的性别、首发的年龄、症状,并根据首发症状进行临床分类。方法回顾分析准确提供首发症状的145例梅尼埃病患者,按照耳聋和（或）耳鸣（耳蜗症状）、半规管（旋转性眩晕）和耳石器症状（倾到或平衡障碍）进行分类。结果首发症状依次为耳鸣45例、耳蜗加眩晕症状30例、眩晕28例、耳鸣加耳聋27例、听力下降9例、耳石器症状6例。累及耳蜗及前庭系统30例;累及耳蜗81例：累及半规管28例：累及耳石器6例。小于20岁和大于60岁年龄组患者数最少。男性高发年龄较女性高发年龄早10年,男性和女性在30-49岁年龄段发病例数最多。三种类型梅尼埃病临床首发症状与典型症状出现的间隔差异均较大。结论梅尼埃病耳蜗受累明显高于前庭,首发症状耳鸣最常见,其次是听力下降,再次为眩晕,倾倒的出现率最低。对不典型病例的早期诊断酌情选择纯音测听、耳蜗电图、前庭双温试验和前庭诱发的肌源性电位等检查。     

Acute sensorineural hearing loss in 277 outpatient cases]

Subjects were 227 patients with acute sensorineural hearing loss of 17,146 patients seen at our ear, nose, and throat (E.N.T.) outpatient clinic from October 1994 through September 2000. Of these, 45 suffered from profound and moderate idiopathic sudden hearing loss diagnosed when the arithmetical mean of hearing thresholds at 0.25, 0.5, 1, 2, and 4 KHz was 40 dB or worse. Of the 30 patients whose medical treatment was started within 7 days of onset, 18 (60%) showed excellent or complete recovery of hearing, whereas none of the 15 whose treatment was started 8 days or late after onset showed satisfactory improvement. In general, the sooner treatment was started, the better hearing recovered. Idiopathic acute low-tone sensorineural hearing loss was found in 111 (49%) patients with a female preponderance at a M:F ratio of 1:2.3, but no gender difference was seen in other diseases. The most common symptom was a feeling of pressure or fullness in the affected ear. Only 16% of patients were aware of their hearing disturbance. Some 73 (66%) showed complete recovery. Meniere's disease was diagnosed in 13, in whom hearing improved in only 2 (15%). Probable Meniere's disease was diagnosed in 9 patients who had 1 definitive episode of vertigo with low-tone sensorineural hearing loss, and their prognosis was good. The prognosis in 7 patients with hearing loss due to acoustic trauma and 1 with psychogenic hearing loss was good, but normal hearing was not restored in 1 with mumps or 1 with acoustic neurinoma. A patient with diabetic nephropathy had low-tone hearing loss and nystagmus toward the affected ear. Attacks of vertigo were controlled by diuretic therapy, but hearing recovery occurred by a little less than 10 dB. Some 35 patients had mild idiopathic hearing loss and prognosis was relatively good. Hearing was disturbed after head injury in 2 and after nose blowing in 1, probably attributable to concussion of the labyrinth or the formation of a perilymphatic fistula. The diagnosis of this condition could not be made with certainty by surgical exploration of the ear.     

Lermoyez's syndrome. A follow-up study in 12 patients

Clinical studies were performed in 12 patients with the Lermoyez syndrome during a close follow-up of 2 to 31 years. The nature and temporal sequences of the symptoms of these patients are described. The incidence of Lermoyez's syndrome, as compared with Meniere's disease, is almost 18%. This is much higher than usually is assumed. In 6 out of 12 patients typical Meniere attacks were found besides their Lermoyez attacks. Almost every one of the 12 patients showed, besides the Lermoyez attacks, also hearing fluctuations without vertigo, and vertigo without hearing fluctuations. The type of vertigo was usually rotating, often with unsteadiness and in 3 patients dropping attacks were sometimes observed. The typical Lermoyez attack lasted several hours. The preceding hearing loss lasted for days to months. The hearing recovered after the attacks and remained stable for days to months. An improved hearing was noticed within some hours after the vertiginous attack in 9 out of 12 cases. Three patients noticed the hearing improvement already during the attack. In 8 out of 12 cases the disease became bilateral.     

Familial clustering of migraine, episodic vertigo, and Ménière's disease.

OBJECTIVE: To evaluate the association between migraine, episodic vertigo, and Ménière's disease in families. STUDY DESIGN: Clinical report. SETTING: University Neurotology Clinic. PATIENTS: Index patients identified with Ménière's disease and migraine and their family members. INTERVENTION: Structured interview to assess a diagnosis of migraine, episodic vertigo, and Ménière's disease in 6 families. Genotyping was performed on 3 sets of twins to analyze monozygosity or dizygosity. MAIN OUTCOME MEASURES: Clinical history of migraine, episodic vertigo, and Ménière's disease. RESULTS: Six index patients and 57 family members were interviewed either by a senior neurologist in person or over the phone by a trained study coordinator. An additional 6 family members completed questionnaires by mail. All 6 index patients had Ménière's disease and migraine. Twenty-six (41%) of the 63 relatives met International Classification of Headache Disorders II criteria for migraine headaches. Thirteen (50%) of these 26 experienced migraine with aura. Three others experienced typical aura without headache. Seventeen (27%) of 63 family members experienced recurrent spells of spontaneous episodic vertigo. There was one twin pair in each of 3 families; 2 pairs were monozygotic and one was dizygotic. In each twin pair, one twin had migraine and Ménière's disease, whereas the other experienced migraine and episodic vertigo without auditory symptoms. CONCLUSION: The frequent association of episodic vertigo, migraine, and Ménière's disease in closely related individuals, including identical twins supports the heritability of a migraine-Ménière's syndrome, with variable expression of the individual features of hearing loss, episodic vertigo, and migraine headaches.