Bilateral electrocochleographic findings in unilateral Menière's disease.

At present, electrocochleography is the only proven investigation that can demonstrate objectively the presence of endolymphatic hydrops. The electrophysiologic recordings in response to sound stimuli show an enhancement of the negative summating potential in these cases. It is well established that patients with unilateral Meniere's disease have a high likelihood of development of the disease bilaterally in the fullness of time. Using transtympanic electrocochleography in 40 patients who manifested unilateral clinical Meniere's disease, we have recorded bilateral abnormalities indicative of endolymphatic hydrops in 35% of cases. The early recognition of incipient Meniere's disease in the asymptomatic contralateral ear of a patient with known unilateral disease has obvious profound implications for patient management.     

The Prevalence of High-Riding Jugular Bulb in Patients with Suspected Endolymphatic Hydrops

Background To determine the prevalence of a high-riding jugular bulb (HRJB) in the endolymphatic hydrops population. Methods This was a retrospective chart and radiology review of patients seen at a tertiary care medical center. Patients were identified using the International Classification of Diseases, 9th edition, code 386.xx (Meniere disease–unspecified), and were required to have undergone an imaging study that included views of the jugular bulb that were available for review. A radiologist then evaluated all of the imaging studies for evidence of HRJB or inner ear dehiscence with a jugular bulb abnormality. Results The prevalence of a HRJB in all endolymphatic hydrops patients was 9.0% (7 of 78), and it was 4.5% (7 of 156) in all ears. The prevalence of HRJB ipsilateral to an ear with endolymphatic hydrops was 4.6% (4 of 88 ears); it was 4.4% (3 of 68 ears) in ears without endolymphatic hydrops. The incidence of inner ear dehiscence with a HRJB was 1.3% (1 of 78). Electrocochleography results were not correlated with jugular bulb volume. Discussion The results of this study indicate that a small subset of patients treated for endolymphatic hydrops patients have a HRJB. Overall, these results suggest that HRJB does not play a major role in endolymphatic hydrops, although it may play a role in a few isolated patients.     

Treatment of endolymphatic hydrops with cochleosacculotomy. Clinical results and experimental findings

Cochleosacculotomy was performed on 25 patients with Meniere's syndrome. Long-term relief of vertigo was obtained in 19 out of 23 (82%). Postoperatively dead ears occurred in three cases. The same operation was performed on 12 guinea pigs in which hydrops had been surgically induced by blockage of the endolymphatic duct and sac. All fistulas were healed and cochleosacculotomy did not decrease or prevent the induced endolymphatic hydrops in these animals. Although histologic confirmation of persistent fistulas in human ears is lacking, the relief of vertigo in patients may not be caused by "drainage" but, rather, by a nonspecific effect on the inner ear. Cochleosacculotomy gives results comparable with other nondestructive surgical procedures performed to suppress vertigo in Meniere's syndrome.     

Effectiveness and safety of everolimus in the treatment of autoimmune hepatitis related to anti-hepatitis C virus therapy after liver transplant: three case reports

Treatment of recurrent hepatitis C after liver transplantation is indicated in selected cases. During the combined treatment with pegylated interferon and ribavirin, some patients develop immune-mediated liver dysfunction similar to the previously described “de novo” autoimmune hepatitis. Herein we have presented three liver transplant patients who during or after combined antiviral treatment were diagnosed as autoimmune hepatitis related to interferon based on their clinical, biochemical, and liver histology features. There were two women and one man, of ages 49, 52, and 49 years who were transplanted due to cirrhosis related to hepatitis C virus genotype 1. In two patients, elevated liver enzymes occurred during antiviral therapy and in the third, after the therapy. The diagnosis of autoimmune hepatitis was reached after excluding other possible causes. One patient had a sustained viral response; and two cases were nonresponders. Antinuclear antibodies were present in two subjects and antinuclear antibodies and anti-smooth muscle antibodies in the other case. First-line treatment of autoimmune hepatitis with prednisone and azathioprine stabilized clinical and biochemical parameters'. In order to avoid the long-term use of prednisone, everolimus was introduced in the three patients. Interestingly, hepatitis C did not progress and clinical, biochemical, as well as histological parameters stabilized. In one patient, the liver fibrosis stage as assessed by histology showed improvement. However, one subject experienced repeated cerebral hemorrhage and died. Although this is heterogeneous population with partially known characteristics, with a difficult differential diagnosis, the objectives of preserving liver function and avoiding recurrent progressive hepatitis C seemed to be achieved by adding everolimus. In addition, we totally stopped prednisone therapy. In conclusion, treatment with everolimus in combination with cyclosporine achieved a partial remission in two liver transplan cases of autoimmune hepatitis related to interferon therapy.     

梅尼埃病内耳内淋巴磁共振成像研究进展

梅尼埃病（MD）是一种以膜迷路积水为主要病理特征的原发性内耳疾病,目前临床诊断仅能依靠典型症状及相关耳科检查。MRI具有良好的软组织分辨力,结合多种序列,能够观察活体膜迷路积水,为诊断MD提供直观可靠的影像学依据。     

Profound hypokalemia and hypochloremic metabolic alkalosis during thiazide therapy in a child with Pendred syndrome

Pendred syndrome is a recessive autosomal disorder characterized by thyroid goiter and sensorineural hearing loss. The Pendred syndrome gene (SLC26A4) encodes a new anion exchanger named pendrin which mediates iodide transport by thyrocytes and regulates ion and fluid transport by the endolymphatic sac epithelium. Pendrin defects result in inner ear malformations, with enlargement of the endolymphatic sac and duct in association with a large vestibular aqueduct. Furthermore, patients may develop endolymphatic hydrops requiring diuretic therapy, mainly in the form of thiazides. Pendrin could also account for apical Cl(-)/ HCO3(-) exchange at level of intercalated cells of the cortical collecting duct in the kidneys, however, humans with Pendred syndrome have no symptoms attributable to renal pendrin abnormalities in basal conditions. We report the case of a child with Pendred syndrome and intercurrent endolymphatic hydrops, who developed profound hypokalemia and severe hypochloremic metabolic alkalosis (potassium 1.7, chloride 70, sodium 129, HCO3 43.8, base excess +17.8 mmol/l, pH 7.52) following thiazide therapy. In subjects with Pendred syndrome thiazide therapy seems to provoke more severe Cl(-) and extracellular volume depletion. A possible explanation could be the defective action of the disrupted pendrin, which exacerbates the effects of the inhibition of C1(-) reabsorption mediated by the thiazide-sensitive NaCl cotransporter (SLC12A3).     

Unilateral sensorineural hearing loss after spine surgery: Case report and review of the literature

BACKGROUND: Sudden sensorineural hearing loss is infrequent, with an estimated incidence of 5 to 20 cases yearly per 100,000 people. Although multiple etiologies have been identified, infection and idiopathic SNHL are most common. Sudden sensorineural hearing loss after nonotologic noncardiac surgery is highly unusual, particularly after spinal surgery. CASE DESCRIPTION: We report a case of unilateral sudden SNHL after lumbar spinal fusion with review of the literature. Potential etiologies, treatment, and prognosis are summarized. CONCLUSION: Multiple etiologies have been proposed for SNHL after nonotologic noncardiac surgery. Excessive positive upper airway pressure during induction of anesthesia or Valsalva maneuvers can result in SNHL from LMR. Aberrant NO accumulation in the middle ear cavity during general anesthesia with subsequent LMR is also a potential etiology, as is excessive leakage of CSF causing a form of endolymphatic hydrops. There is no definitive treatment of postoperative SNHL, although middle ear exploration may be beneficial for suspected overpressure injury to the middle ear, causing a perilymphatic fistula. Corticosteroids have been beneficial in certain cases of idiopathic SNHL; however, its effectiveness for postoperative SNHL is undocumented.     

ECoG results in perilymphatic fistula: clinical and experimental studies

Patients with perilymphatic fistula have been described as having symptoms similar to Meniere's disease and endolymphatic hydrops. Direct clinical or experimental evidence linking the two inner ear disorders has been lacking. An enhancement of the summating potential observed with electrocochleography suggests a diagnosis of ELH in both of these inner ear disorders. In this study, ECoG results of 27 patients with surgically confirmed PLF are reported. Fourteen patients with surgically confirmed spontaneous PLF had abnormal ECoG. Six of these 14 patients had normal hearing. The ECoG changes in patients with Meniere's disease and those with surgically confirmed PLF are identical, indicating the underlying pathologic change in both is hydrops. But there is no specific diagnostic abnormality on ECoG that differentiates these two inner ear disorders. Also, an experimental model of PLF was developed and studied in guinea pigs. "Inactive" PLF is defined as "an opening was made into the cochlea, but if no perilymph moved out through the fistula, it was defined as inactive" An "active" PLF occurs when perilymph actually moves from the inner ear out to the middle ear. ECoGs were recorded before and after creation of an "active" PLF. ECoG abnormalities were seen in "active" PLF and correlated with histologic data demonstrating ELH. An abnormally enhanced summating potential was demonstrated after active removal of perilymph through the experimentally created fistula. Cochlear duct histology showed hydropic distention of Reissner's membrane in the experimental ears and no changes in the membranous labyrinths of the unoperated, control ears.(ABSTRACT TRUNCATED AT 250 WORDS)     

Vascular mechanisms in Meniere's disease

Normal venous drainage of the vestibular organs through the vein of the paravestibular canaliculus (PVC) may be crucial to inner ear fluid mechanics. It is proposed that increased venous pressure, with resultant venous insufficiency of the vestibular organs, may result in endolymphatic hydrops unless collateral venous circulation develops. Certain variations in pattern of venous drainage where the vestibular organs drain predominantly through the PVC vein may be a predisposing factor. In patients with Meniere's disease, different mechanisms can cause venous insufficiency. One suggested mechanism is morphologic change in the microcirculation of the intermediate portion of the endolymphatic sac. Microcirculation changes may be associated with fibrosis of the perisac tissues or shortening of the intermediate sac region or might be physiologically determined. Venous insufficiency may also result from anomalies of the PVC vein.     

Otosclerosis involving the vestibular aqueduct and Menière's disease

The coexistence of otosclerosis and endolymphatic hydrops in the temporal bone have been described; however, the mechanism for the development of endolymphatic hydrops in otosclerosis remains unknown. Among 128 temporal bones with otosclerosis, involvement of the vestibular aqueduct by otosclerosis was observed in four temporal bones from two patients. In all four, the vestibular aqueduct was filled with active otosclerotic foci; the lumen of the endolymphatic duct and sac was narrowed as a result of fibrosis, and endolymphatic hydrops, more severe in the pars inferior than the pars superior, was observed. Collapse of the ductus reuniens and dilated saccule was seen in three temporal bones. Our study indicates that otosclerotic obstruction of the vestibular aqueduct may create a disturbance of the outflow and/or absorption of endolymph, leading to the development of endolymphatic hydrops and Meniere's disease, thus supporting the theory of longitudinal flow of endolymph.     

Diagnosis and management of drop attacks of vestibular origin: Tumarkin's otolithic crisis

Erroneous signals of vestibular origin can cause sudden falls without warning. Drop attacks of vestibular origin in our experience most commonly occurred in patients with late or end-stage endolymphatic hydrops (usually idiopathic, ie, Meniere's disease). Eponymically termed Tumarkin's otolithic crisis, drop attacks of vestibular origin must be distinguished from drop attacks due to cardiovascular abnormalities, seizure disorders, vertebral basilar arterial insufficiency (transitory brain stem ischemia), multiple sclerosis, and drug-induced motor control disturbances. The treatment of choice for drop attacks of vestibular origin is either surgical ablation (labyrinthectomy) in the absence of serviceable hearing or middle fossa vestibular nerve section in order to preserve serviceable hearing.     

Use of streptomycin sulfate in the treatment of Meniere's disease

Streptomycin sulfate has been known to be ototoxic since its use in the treatment of tuberculosis. This report describes 10 years of experience in the treatment of Meniere's disease with streptomycin. Streptomycin has been used in classical Schuknecht ablation of the vestibular system in bilateral Meniere's disease; classical Schuknecht ablation of the vestibular system in unilateral Meniere's disease in the only hearing ear; intratympanic streptomycin in the treatment of unilateral Meniere's disease; and low-dose intramuscular streptomycin as outpatient treatment in unilateral Meniere's disease. The results of bilateral vestibular ablation were similar to Schuknecht's and others. Patients developed profound ataxia with a wide-based gait and oscillopsia, which improved rapidly over a period of months. Approximately 30% experienced significant improvement in hearing, which usually deteriorated again after several months. All patients were relieved of vertigo. Patients with unilateral Meniere's disease in the only hearing ear responded as did the bilateral cases. Hearing in the only hearing ear was preserved in all cases. Low-dose subototoxic streptomycin as outpatient treatment offers promise in some cases for relieving attacks of Meniere's disease while improving hearing without producing the temporary disabling effects of ataxia and oscillopsia. Streptomycin and similar drugs that may reduce the production of endolymph may eventually be the treatment of choice in Meniere's disease.     

Autoimmune hepatitis: new diagnostic and therapeutic approach

Autoimmune hepatitis is a self-perpetuating hepatocellular inflammation. The diagnosis is established by a number of diagnostic criteria, defined by the International Autoimmune Hepatitis Group, and the exclusion of other causes of chronic hepatitis. There are two fundamental goals in therapy: induction of remission and maintenance of remission. The standard initial treatment is prednisone monotherapy or combination therapy with prednisone and azathioprine, which induce a clinical, biochemical and histologic remission in 65-87% of patients within 3 years. Other typical treatment endpoints in autoimmune hepatitis are an incomplete response, treatment failure and intolerance of the administrated drugs. If the treatment results are unsatisfactory, liver transplantation and alternative drugs such as Cyclosporin A, tacrolimus, cyclophosphamide, mycophenolate mofetil, budesonide, ursodeoxycholic acid should be considered; however, efficacy in clinical trials has not been shown. Future investigations must focus on the clarification of pathogenic mechanisms, characterization of target autoantigens, identification of host susceptibility factors, and assessment of alternative treatment strategies.     

Low-level laser therapy on the treatment of oral and cutaneous pemphigus vulgaris: case report

Pemphigus vulgaris is a chronic autoimmune mucocutaneous disease that initially is manifested by painful intraoral erosions and ulcers which spread to other mucosa and the skin, generally more than 5 months after oral lesion manifestation. The treatment consists of prednisone alone or in combination with an immunosuppressive agent, and the clinical response is perceived within 2 to 4 weeks. Low-level laser therapy has been effective in accelerating the healing of injured tissue, thus inducing cell proliferation and increasing ATP, nucleic acid, and collagen synthesis. We reported two cases of pemphigus vulgaris that received systemic treatment associated with low-level laser therapy for oral and cutaneous lesions. We observed prompt analgesic effect in oral lesions and accelerated healing of oral and cutaneous wounds. Therefore, the present report suggests LLLT as a noninvasive technique that should be considered as an adjuvant therapy in oral and skin disorders in patients with PV.     

Titration streptomycin therapy for bilateral Meniere's disease: a preliminary report

Intramuscular dosages of streptomycin sulfate were titrated in eight patients with bilateral Meniere's disease in an attempt to alleviate disabling vertigo and hearing loss. It appears possible to eliminate the vertiginous episodes, preserve or improve hearing, and avoid ataxia and oscillopsia in most of these individuals. At present we consider this treatment regimen our first choice of therapy in people with bilateral active Meniere's disease or in patients whose only hearing ear is actively fluctuating and in whom associated disabling vertigo is present. Great caution and supervision should be exercised in the use of streptomycin titration therapy in the individual with bilateral Meniere's disease, as further experience is required to determine the efficacy of this form of management.     

Management of posttraumatic vertigo.

OBJECTIVE: To evaluate patients after blunt trauma of the head, neck, and craniocervical junction (without fractures) with vertigo and to report the results of treatment after extensive diagnostics. STUDY DESIGN: Prospective study of consecutive new cases with vertigo after trauma at different periods of onset. During 2000-2002, 63 patients were examined and treated. SETTING: Regional trauma medical center for the greater Berlin Area, tertiary referral unit. RESULTS: The primary disorders included labyrinthine concussion (18), rupture of the round window membrane (6), and cervicogenic vertigo (12). The secondary disorders included otolith disorders (5), delayed endolymphatic hydrops (12), and canalolithiasis (9). The patients were free of vertigo symptoms (except cervicogenic and otolith disorder) after treatment, which consisted of habituation training, medical and surgical therapy options. The follow-up was 1 year. CONCLUSION: Posttraumatic vertigo can be treated with a high success rate once the underlying disorder has been identified. The extent of the neurotological test battery determines the precision and quality of diagnostics. Surgical measures should be an integral part of treatment modalities if conservative treatment is not effective. SIGNIFICANCE: Minor trauma of the head, neck, and craniocervical junction can have major impact on the vestibular system at different sites. Patients need to be carefully diagnosed, even if the onset of vertigo occurs a few weeks or months after the initial trauma.     

Therapeutic experience in renal hypertension: response treatment in 47 cases.

We report our experience in the treatment of 47 cases of renal hypertension patients with renovascular hypertension or hypertensive reno-parenchymal disease surgical therapy is essential and non-surgical therapy has its limitations. The necessity for an operation can be based on the determination of bilateral renal vein plasma renin values. Blood should be drawn from both renal veins in patients in an upright position. Surgical treatment usually produces excellent results in patients in whom renin activity on the side of the lesion is more than normal and more than 2 times that on the uninvolved side. It is advisable to perform an operation within 4 years of the onset of renal hypertension. Complete surgical cure of renal hypertension is accomplished more frequently in patients with fibromuscular dysplasia than in those atherosclerosis. The therapeutic effect of an operation in correcting hypertension is virtually the same, regardless of whether the arterial stenotic lesions are in the main renal artery or in the intrarenal arterial branches. We have found nephrectomy or dacron bypass graft to be the surgical procedure of choice in renovascular hypertension cases.     

Rapid enlargement of elastofibroma dorsi after physical therapy

Elastofibroma dorsi is an uncommon, slow-growing, ill-defined soft tissue tumor. Its most prominent symptom is back and shoulder pain. Elastofibroma dorsi is usually located beneath the scapula and bilateral involvement occurs in only 10% of patients. We report herein a case of bilateral elastofibroma dorsi who underwent physical therapy for treatment of shoulder pain. Elastofibroma was misdiagnosed and rapidly progressed after physical therapy. We conclude that elastofibroma should be kept in mind for patients with shoulder or back pain, and the patient should be evaluated carefully before initiating physical therapy.     

ENDOLYMPHATIC ISOTOPE AND BCG IN THE MANAGEMENT OF MALIGNANT MELANOMA

Endolymphatic isotope therapy had such promising early clinical results that the M.R.C. (Medical Research Council) U.K. set up a clinical trial in 1966. This was to compare the effect of endolymphatic isotope therapy with the results of standard methods in the treatment of lower limb malignant melanoma. The interim report had three groups for analysis; Standard Methods (S); Endolymphatic Satisfactory (ES); and Endolymphatic Unsatisfactory (EU). This third group was a subdivision, as a significant number of patients did not have the correct endolymphatic treatment. The five-year survival figures expressed as actuarial percentages were ES=78.8%; S=82.3%; and EU = 57.3%. Lymph node recurrence showed a significant difference: ES=2.3%; EU=12%; and S=19%. The conclusions were that endolymphatic isotope therapy was justified in specialized centres where good results could be obtained. Further animal experiments using the VX2 tumour in rabbits indicated that BCG given intracutaneously or intravenously had no therapeutic effect, whereas when applied by intralymphatic injection BCG was successful in treating lymph node metastases. Nineteen patients with poor-prognosis malignant melanoma have received endolymphatic BCG. The clinical results are recorded in this paper and are sufficiently encouraging to warrant its continued use.     

Endolymphatic isotope and BCG in the management of malignant melanoma.

Endolymphatic isotope therapy had such promising early clinical results that the M.R.C. (Medical Research Council) U.K. set up a clinical trial in 1966. This was to compare the effect of endolymphatic isotope therapy with the results of standard methods in the treatment of lower limb malignant melanoma. The interim report had three groups for analysis: Standard Methods (S); Endolymphatic Satisfactory (ES); and Endolymphatic Unsatisfactory (EU). This third group was a subdivision, as a significant number of patients did not have the correct endolymphatic treatment. The five-year survival figures expressed as actuarial percentages were ES=78.8%; S=82.3%; and EU=57.3%. Lymph node recurrence showed a significant difference: ES=2.3%; EU=12%; and S=19%. The conclusions were that endolymphatic isotope therapy was justified in specialized centres where good results could be obtained. Further animal experiments using the VX2 tumour in rabbits indicated that BCG given intracutaneously or intravenously had no therapeutic effect, whereas when applied by intralymphatic injection BCG was successful in treating lymph node metastases. Nineteen patients with poor-prognosis malignant melanoma have received endolymphatic BCG. The clinical results are recorded in this paper and are sufficiently encouraging to warrant its continued use.