Bilateral vestibulopathy revisited

Bilateral vestibular failure (BVF) is an often undetected disorder of the peripheral labyrinths or the eighth nerves. Key symptoms are oscillopsia during locomotion or head movements and unsteadiness, particularly in the dark. Diagnosis is made by a bedside test for defective vestibulo-ocular reflex and the absence of nystagmic reaction to both caloric and rotatory pendular testing. Most frequent etiologies include ototoxicity, cerebellar degeneration, meningitis, neuropathies, sequential vestibular neuritis, autoimmune disorders, tumors, and miscellaneous otological diseases. Idiopathic BVF is found in more than twenty percent of the patients. Recovery is possible but mostly incomplete. Somatosensory and visual input largely substitute the vestibular deficit for spatial orientation, postural balance and ocular motor control.     

Antiphospholipid syndrome associated with progressive systemic sclerosis

Hemifacial spasm is a neurological disorder due to abnormal hyperactivity of the facial nerve. The most common cause of hemifacial spasm is a neuro-vascular conflict in the cerebellopontine angle between a vascular loop and the root of the facial nerve (96% of cases). Tumors are the cause of hemifacial spasm in only 1% of cases). The authors present their results in 100 patients who underwent microvascular decompression for essential hemifacial spasm between 1990 and 1995. They used microsurgical and endoscopic procedures by a minimal retrosigmoid approach in all cases. The most common offending vessels were the posterior inferior cerebellar artery (70%), the vertebral artery (41%) and the anterior inferior cerebellar artery (28%). An aberrant vein was found in 2 cases. There were 38% of multiple artery-nerve conflicts. Physiopathology of hemifacial spasm is explained by two principal theories: in the ephaptic theory, hyperactivity and an abnormal nervous impulse pathway are due to a short demyelinated area on the nerve trunk caused by the offending vessel, inducing short circuiting between adjacent nerve fibers. In the nuclear theory, hyperactivity of the facial nerve is due to an abnormal and automatic activity of the facial nerve nucleus itself, induced by the vessel. The authors used pre and postoperative electromyographic tests and intraoperative electromyographic tests. Their results tend to prove the nuclear theory. Ninety per cent of the patients had a good result, with a mean follow-up time of 30 months in 60 cases. In 82% of the cases, there was a total recovery after a single procedure. There was no mortality and no facial palsy. Hearing loss occurred in less than 5%.     

Sudden hearing loss: frequency of abnormal findings on contrast-enhanced MR studies

BACKGROUND AND PURPOSE: Our purpose was to determine the frequency of abnormal findings on contrast-enhanced high-resolution MR imaging studies in patients with sudden hearing loss. METHODS: Seventy-eight consecutive patients with sudden hearing loss underwent contrast-enhanced MR imaging of the temporal bone, cerebellopontine angle, and brain. Additional tests included audiologic examination, electrocochleography, fistula tests, and serologic tests for viral agents and autoimmune disorders. RESULTS: Probable causes of the sudden hearing loss in these patients included viral or immune-mediated disease, Meniere disease, vascular disorder, syphilis, neoplasm, multiple sclerosis, and perilymphatic fistula. Twenty-four (31%) of the 78 patients were found to have abnormal imaging results early in the course of their work up and treatment. CONCLUSION: The prevalence of abnormal findings on contrast-enhanced MR studies is higher than previously reported in patients with sudden hearing loss.     

Sudden deafness and Barlow disease

From January 1993 to December 1994 twelve patients were evaluated for sudden hearing loss. The median age was 49 years with a range of 18 to 71. All had severe or profound initial hearing loss. The incidence of bilateral disease was 25%. Total deafness occurred in five (33%) ears. Nine (75%) patients had vestibular symptoms and eight (67%) admitted experiencing tinnitus. Two-dimensional echocardiography revealed mitral prolapse in eight (67%) patients; another patient showed moderate to severe ischemic left ventricular dysfunction with apical aneurysm.     

Mutations in the COCH gene are a frequent cause of autosomal dominant progressive cochleo-vestibular dysfunction, but not of Meniere's disease

The COCH gene is the only gene identified in man that causes autosomal dominantly inherited hearing loss associated with vestibular dysfunction. The condition is rare and only five mutations have been reported worldwide. All affected families showed a similar progressive hearing loss and vestibular dysfunction. Since Meniere's disease-like symptoms have also been described in some families, it was suggested that COCH mutations might be present in some patients diagnosed with Meniere's disease. In this study, using a Japanese population, we performed a COCH mutation analysis in 23 patients from independent families with autosomal dominant hearing impairment, four of whom reported vestibular symptoms, and also in 20 Meniere's patients. While a new point mutation, A119 T, was found in a patient with autosomal dominant hearing loss and vestibular symptoms, no mutations were found in the Meniere's patients. Like all other previously identified COCH mutations, the mutation identified here is a missense mutation located in the FCH domain of the protein. The current mutation is located in close spatial proximity to W117, in which a mutation (W117R) had previously been associated with autosomal dominant hearing loss. Model building suggests that, like the W117R mutation, the A119 T mutation does not affect the structural integrity of the FCH domain, but may interfere with the interaction with a yet unknown binding partner. We conclude that mutations in the COCH gene are responsible for a significant fraction of patients with autosomal dominantly inherited hearing loss accompanied by vestibular symptoms, but not for dominant hearing loss without vestibular dysfunction, or sporadic Meniere's disease.     

Diencephalic and cerebellar pathology in alcoholic and nonalcoholic patients with end-stage liver disease

Formalin-fixed sections from the brains of 36 patients (30 alcoholic and 6 nonalcoholic) with autopsy-proven cirrhosis who died while in a hepatic coma were stained with hematoxylin and eosin, and examined for the presence of diencephalic, cerebellar, pontine, and basal ganglia lesions. Significant neuropathology was identified in 23 of 36 cases consisting of mammillary body and thalamic lesions characteristic of Wernicke encephalopathy (WE) (9 cases, all alcoholic patients) and cerebellar degeneration (20 cases, 17 alcoholic and 3 nonalcoholic patients). Clinical diagnosis of WE had been entertained during life in only 2 of these patients. All cases, alcoholic and nonalcoholic, manifested mild to severe Alzheimer's type II astrocytosis. No cases of central pontine myelinolysis nor acquired (non-Wilsonian) hepatocerebral degeneration were found. These findings show that the brains of a high proportion of cirrhotic patients with end-stage liver disease manifest concomitant unsuspected diencephalic and cerebellar pathology. The high incidence of WE underscores the need for early sustained treatment of alcoholic cirrhotic patients with vitamin B1. Evaluation of the neurological sequelae of liver transplantation, particularly of alcoholic patients with end-stage liver disease, may require a careful neurological and radiological assessment both before and after surgery.     

Acoustic neuroma: correlations between morphology and otoneurological manifestations

Forty-two patients with acoustic neuroma (AN) were studied to determine whether different types of neuroma could be correlated with specific signs and symptoms of the disease. Based on gadolinium-enhanced TI-weighted MRI sequences, the 42 cases of AN could be divided into three groups, either by size (small: 11.9%, medium: 50%, and large: 38.1%) or by site of origin of the tumour (lateral: 16.7%, intermediate: 69%, and medial: 14.3%). Relations were found between the size and the site of origin of the neuromas and certain clinical, audiological and vestibular findings. The clinical presentation seemed to vary with the site of origin and the size of the tumour: patients with lateral neuromas generally had small tumours, sometimes only located in the internal auditory canal (IAC), and presented early subjective hearing loss while patients with medial neuromas had larger tumours which grew without causing significant audiological symptoms. Normal hearing function was seen only in the patients with medial ANs; however, a significant relation between the size or the site of origin of the AN and the average hearing threshold was not demonstrated. The sensitivity of the stapedial reflex test (SR) was higher for lateral ANs. Anomalies in the brainstem auditory evoked potentials (BAEPs) did not seem to be related to either the size or the site of origin of the AN. The vestibular tests demonstrated a higher frequency of central vestibular involvement in the large tumours, while normal function was more frequent in the lateral tumours. In the group studied the combination of BAEPs and vestibular tests allowed us to identify all the ANs with an optimal level of sensitivity.     

Endolymphatic sac obliteration for large vestibular aqueduct syndrome

The objective of this study was to investigate the effects of endolymphatic sac obliteration for stabilization of progressive hearing loss in patients with the large vestibular aqueduct syndrome. This was a retrospective case review conducted at a private neurootologic office in a metropolitan area. Seven ears in six patients were subjected to surgery for obliteration of the endolymphatic sac in an effort to stabilize progressive hearing loss associated with the large vestibular aqueduct syndrome. The study population was composed of four boys and two girls 4-17 years of age. The large vestibular aqueduct was unilateral in two patients and bilateral in four patients. All seven ears demonstrated progressive sensorineural hearing loss preoperatively. Surgical tissue obliteration was performed via a transmastoid approach in seven ears. The main outcome measure was comparison of pre- and postoperative hearing levels and stability. Magnetic resonance imaging also was performed in all cases at least 6 months postoperatively to determine patency of the endolymphatic sac and vestibular aqueduct. Six of seven ears maintained stable hearing during the follow-up period, which ranged from 6 months to 6 years (mean 3.2 years). One patient showed continued progression of hearing loss postoperatively. All seven ears demonstrated continued obliteration on postoperative imaging studies. Surgical obliteration of the endolymphatic sac may stabilize hearing in patients with the large vestibular aqueduct syndrome and progressive hearing loss. These results support the theory of pressure or fluid reflux into the labyrinth as a cause of progressive hearing loss in these patients.     

Cross-sectional epidemiological survey of rheumatoid arthritis patients seen in private practice in France. Descriptive results (1629 cases)

OBJECTIVE: To conduct an epidemiological study of rheumatoid arthritis patients seen by office-based rheumatologists in France (first semester of 1996). METHODS: Cross-sectional study of 1629 rheumatoid arthritis patients conducted by 373 office-based rheumatologists who volunteered for the study (one visit per patient). Each rheumatologist was to complete a 200-variable questionnaire for the first four rheumatoid arthritis patients who came to their office. RESULTS: Women contributed 81% of the sample (mean age, 57 years); 19% of patients were seen in the Paris area, 20% in the North East, 20% in the North West, 22% in the South East and 19% in the South West. Twenty-nine per cent of patients had a paid job and 21.1% (all women) were homemakers. Among the patients with a paid job, 44% were on sick leave, with the reason for the sick leave being the rheumatoid arthritis in 36% of cases. Nineteen per cent of patients had stopped working permanently because of their rheumatoid arthritis, after a mean disease duration of six years. Mean disease duration in the overall sample was eight years. The diagnosis was established within six months of symptom onset in 75% of cases. A family history of rheumatoid arthritis was found in 11% of patients and a family history of other autoimmune diseases in 2%. The disease was precipitated by a stressful life event in 17% of cases. Follow-up was being provided only by the study rheumatologist in 59% of cases and also by a general practitioner in 39%. The disease was quiescent in 9% of cases, minimally active in 32%, moderately active in 46% and severely active in 13%. Eighty-four per cent of patients were on one (78%) or more (6%) second-line drugs including methotrexate (45%), an antimalarial (17%), intramuscular gold (14%), tiopronin (9%), D-penicillamine (6%) and sulfasalazine (12%). Fifty-two per cent of patients were on steroid therapy (mean dose, 7.5 +/- 5.7 mg/d). Other drugs included nonsteroidal antiinflammatory agents (61%), analgesics (61%), gastroduodenal protective agents (45%) and anxiety-relieving agents (10%). Twenty-four per cent of patients had had one or more surgical procedures (mean, 3/patient) for their joint disease. CONCLUSION: This nation-wide epidemiological survey conducted in France provides a database on the socioeconomic and demographic characteristics of rheumatoid arthritis patients followed in private practice.     

The role of barium enema in colostomy closure in trauma patients

Recently, the routine use of barium enema preceding colostomy closure in trauma patients has been challenged. It has been argued that the nature of the injury should be apparent from the initial laparotomy and that the likelihood of finding an unsuspected colonic lesion in the young, previously healthy patients who constitute the majority of trauma patients is very small. We retrospectively reviewed 124 consecutive cases of patients who received colostomy takedowns for trauma. One hundred six of the patients had preoperative barium enema evaluation. 87.1 per cent of the examinations were negative, with a subsequent stoma closure complication rate of 20.4 per cent. Of the 13 positive barium enemas, 9 were falsely positive. These patients had a higher stoma closure complication rate of 39 per cent, a fact that could not be explained on the basis of their abnormal studies. The 18 patients who did not have barium enema performed did not have an increase in complications (17.6%). Barium enema failed to uncover unsuspected pertinent diagnoses, often added unnecessary delays and expense, and in no case changed the operative management. Contrast studies were found to be useful in defining anatomy in cases of known fistulas and when the takedowns were performed without the benefit of operative reports from the previous surgery.     

Perilymphatic fistula and its interest to audiologists

Forty-one patients who had sudden hearing loss associated with spontaneous perilymphatic fistllas were reviewed in this investigation. A history of physical exertion or an upper respiratory infection prior to the onset of hearing loss strongly suggests the presence of spontaneous perilymphatic fistula. However, absence of such a history does not necessarily preclude the possibility of membrane rupture. A great variation in audiologic and vestibular findings was observed. Tinnitus was present for most patients and vertigo or disequilibrium was reported by some. Findings support the need for surgical exploration in patients with sudden hearing loss when a perilymphatic fistula is suspected. Postoperative success in restoring hearing function in cases with perilymphatic fistula is good if the fistula is closed within two weeks after the initial insult. Delay in closure of such fistulas can cause irreversible loss of hearing.     

Immunological abnormalities in patients with etiology unknown sensorineural hearing loss

An immunological study was carried out in 50 patients with etiology unknown sensorineural hearing loss, including the following: 12 low tone deafness, 7 sudden deafness, 8 unilateral deafness, 7 idiopathic bilateral progressive sensorineural hearing loss and 14 other bilateral sensorineural hearing loss. Twenty-five out of 50 of the cases demonstrated immunological abnormalities. A full array immunological tests were performed with the following results. High immnunoglobulin titers showed in 18 out of 50 cases. Six cases were positive for antinuclear antibody. The anti-DNA antibody assay revealed high titers in 2 cases. Also, anti-rheumatoid factor antibody assay showed high titers in 6 cases while abnormal values of complements in serum were detected in 6 cases, though an immune complex was demonstrated in only one case. Also, it was noted that six patients with immunological abnormalities have autoimmune diseases. The above results indicated that some cases of etiology unknown sensorineural hearing loss may have an immunological link.     

Disorders of granulocytes induced by toxic agents

In 152 consecutively selected patients with detrusor hyperreflexia (DH) 96 (63 per cent) had neurological disorders. Thirty-two patients did not show any primary neurological or urological cause for DH. This group was chosen to elucidate the evaluation of possible neurological symptoms in relation to the urological symptomatology. Nine had died and one failed to appear to the neurological examination. Twelve (63 per cent) of the 22 examined patients showed signs of lesions in different parts of the central nervous system, particularly cerebrovascular diseases and myeloneuropathy. Six had had neurological symptoms for years. In two the urological symptoms were first to appear. In the group with no neurological complaints the urological symptoms had existed for 2-30 years. No essential difference was found in the degree of voiding disturbance whether or not neurological signs were disclosed. It is concluded that the discovery of DH should be followed by a neurological examination to disclose further signs of lesions in CNS. Likewise, an extended urological examination with demonstration of DH might help in the evaluation of an obscure neurological disease.     

Region-specific changes in GABAA receptor delta subunit mRNA level by tolerance to and withdrawal from pentobarbital

This paper is, aiming at understanding disturbances of and correllation between colchear and vestibular functions in Meniere's disease. Auditory and vestibular functions in 121 Meniere cases (70 males and 51 females, age ranging from 19 to 63 years), diagnosed according to the AAO-HNS criteria, were analysed. The results were as follows: 1)There was positive correlation between semicircular canal paresis and hearing impairment. 2) There was statistically significant positive correlation between hearing impairment and the -SP/AP ratio. 3) There was statistically significant correlation between hearing impairment and glycerin test result, the rate of positive result being higher in those with advanced hearing loss than in those with mild loss. 4) There was correlation between the rate of positive glycerin test result and audiogram configuration. The rate with rising audiogram pattern was higher than with that falling pattern (69.0% vs 33.3%). It was also found that ECochG was far more effective than glycerin test in diagnosing Meniere's disease. The physiologic basis and significance of ECochG and glycerin test in Meniere's disease were discussed.     

Primary nonampullary/periampullary adenocarcinoma of the duodenum

Primary duodenal adenocarcinoma not involving the ampullary region is rare. Our aim was to review the outcome of these patients and determine the factors that affect survival. We performed a retrospective review of all patients with primary, nonampullary duodenal adenocarcinoma at the Cleveland Clinic Foundation from January 1986 through December 1996. Twenty-six patients with primary, nonampullary duodenal malignancies were identified. There were 16 adenocarcinomas, 3 gastrinomas, 3 stromal tumors, 3 leiomyosarcomas, and 1 carcinoid tumor. Patients with adenocarcinoma had symptoms present an average of 6.1 months. Tumors were identified by upper gastrointestinal contrast study and esophagogastroduodenoscopy in 90 per cent and 87 per cent of patients, respectively. Twelve of 13 (93%) cancers found in the third or fourth portion of the duodenum were adenocarcinomas. Seven of the 16 adenocarcinomas were resectable on exploration. Those that were contained within the serosa have not recurred (mean, 6 years); one of the two patients with locally invasive adenocarcinoma remains disease free. The average survival for patients with unresectable disease was 6.7 months. The 5-year survival rates were: all adenocarcinoma, 38 per cent; resectable, 86 per cent; and unresectable, 0 per cent. All patients presenting with weight loss or obstructive symptoms died of disease; those with melena survived long term. Patients with tumors other than adenocarcinoma had a 90 per cent 5-year survival. We conclude that patients typically present with a long history of symptoms. Distal duodenal malignancies are most frequently adenocarcinomas. Upper gastrointestinal contrast study or endoscopy is often diagnostic. Patients with weight loss and/or obstructive symptoms had invasive disease and a morbid prognosis. Aggressive surgery is warranted, and most with resectable disease (86%) had long-term survival.     

Effect of insecticides on vital activity, hepatic enzymes and red blood cell acetyl cholinesterase activity of rabbits in Makkah

Vitamin E is important in maintaining normal neurological structure and function. In this study, 100 children with protein-energy malnutrition (PEM) were studied and compared to a suitably age-matched control group. Posterior column deficits, cerebellar deficits, and problems with fine motor coordination were present to a significant degree in the PEM subjects. The presence of neurological signs was correlated with various parameters of vitamin E deficiency, including low serum alpha-tocopherol levels and a low tocopherol/total lipid ratio which was present in 92 per cent of subjects. There was good concordance between vitamin E levels and vitamin E to serum lipid ratio in assessing vitamin E deficiency. We conclude that vitamin E deficiency is prevalent, to a hitherto unsuspected degree, in children with PEM and that these malnourished children have significant neurological deficits attributable to low vitamin E levels. This observation is of clinical significance as the neurological deficits are potentially reversible with vitamin E supplementation.     

Studies on host-flea relationship. IV. Progesterone and cortisone do not influence the reproductive potentials of rat fleas Xenopsylla cheopis (Rothschild) and X. astia (Rothschild)

Chromosome studies were performed on 54 apparently healthy relatives of 29 scleroderma patients and on 40 controls. Increased chromosomal breakage is observed in 86 per cent of brothers and sisters and in 68 per cent of children of patients. If the findings in relatives are compared to those obtained in the 29 scleroderma patients of these families, the percentage of abnormal cells does not differ significantly between the two groups (25.8 per cent and 29.1 per cent respectively), and there is no difference for the frequencies of gaps and open breaks. However acentric fragments, "minutes" and morphologically abnormal chromosomes are significantly increased in patients as compared to their asymptomatic relatives with increased breakage. The distribution of breaks is found to be random. The presence of structural chromosome aberrations in relatives of scleroderma patients may have a special importance with regard to the concept of familial autoimmune disease.     

Status epilepticus in 37 Chinese children: aetiology and outcome

OBJECTIVE: The aetiology and clinical features of status epilepticus (SE) are described, with the aim of defining any relationship between risk factors and clinical outcome. METHODOLOGY: A retrospective review was performed of 37 Chinese children admitted to Queen Mary Hospital, Hong Kong, from 1989 to 1993 with the diagnosis of SE. RESULTS: Eighty-six per cent had onset before 5 years of age; 60% were due to an acute central nervous system (CNS) insult, 11% were idiopathic, 13% had a pre-existing CNS insult, 5% were febrile and 11% were due to progressive encephalopathy. An abnormal neurological status was present in 24% before the episode of SE, and a history of seizures before the onset of SE was present in 35% of patients. Fifty-four per cent of the episodes of SE were generalized. The mortality rate was 11% during the period of follow up but no deaths were attributed to SE. Neurological sequelae were observed in 27% of patients and recurrent SE occurred in 12%. CONCLUSIONS: In those patients with normal neurological status before an episode of SE and without acute CNS insult or progressive encephalopathy, the outcome was favourable.     

Three familial cases of hearing loss associated with enlargement of the vestibular aqueduct

The present report describes three familial cases of recessive hearing loss associated with enlargement of the vestibular aqueduct (EVA). Six siblings from three families showed EVA. The common characteristic of these patients was the presence of congenital, high-frequency, fluctuating sensorineural hearing loss. These cases suggest that EVA may be a useful discriminator between different types of recessive hearing loss.     

Sudden sensorineural hearing loss associated with anticardiolipin antibody

We experienced three female patients with sudden sensorineural hearing loss. All the patients showed elevation of anticardiolipin antibody in the serum. Immunoglobulin (Ig) M anticardiolipin antibody was present in case 1 (a 34-year old woman). IgG anticardiolipin antibodies were present in cases 2 (a 50-year-old woman) and 3 (a 9 year old girl). The patient in case 1 showed hearing disturbance of low tone in the left ear and normal vestibular function in both ears. The case 2 patient had an average hearing level of 81.7dB at 0.5. 1, and 2kHz and severe canal paresis in the right ear. In case 3 the patient showed an average hearing level of 53.3dB in the right ear and normal vestibular function in both ears. It was suggested that thrombosis which might have been induced by anticardiolipin antibody could have caused the sudden sensorineural hearing loss in these patients. Steroid therapy cured the hearing disturbance completely in case 1. Steroid and prostaglandin E1 therapy did not improve the hearing disturbance in case 2. Steroid, prostaglandin E1, and ticlopidine hydrochloride therapy improved the hearing disturbance in case 3. It appears that not only steroids but also prostaglandin E1 and ticlopidine hydrochloride therapy may be effective in patients with sensorineural hearing loss associated with anticardiolipin antibody.