A longitudinal study of unilateral Ménière's disease and clinical evolutionary models.

OBJECTIVE: The heterogeneity of Ménière's disease is presently defined by a variety of subtypes. This study introduced three different subtypes of unilateral Ménière's disease based on the evolution of vertigo crises from their inception. METHOD: A longitudinal descriptive study of 327 unilateral Ménière's disease patients was performed. In a subgroup of patients followed from the onset of the disease, 3 subtypes of unilateral Ménière's disease were defined according to the vertiginous crises suffered during the first 10 years of the disorder. RESULTS: Data was available for 87 patients with unilateral Ménière's disease from the start of their disease (26.6 per cent of the original sample). These patients were grouped into three models according to their symptomatic evolution. Model 3 was associated with a worse hearing prognosis, a greater number of Tumarkin's otolithic crises and the need for surgery. Model 1 presented less hearing loss. CONCLUSION: Unilateral Ménière's disease models based on the evolution of vertiginous crises present differences according to aspects such as hearing loss, vertiginous crisis, Tumarkin's otolithic crisis and the need for surgery.

Bilateral Ménière's disease according to its form of debut: synchronous and metachronous disease.

OBJECTIVE: Bilateral Ménière's disease is classified according to the time of appearance of symptoms in each ear into synchronous and metachronous types. A descriptive longitudinal study, involving 59 bilateral Ménière's disease patients, was carried out to assess the two forms of bilateral Ménière's disease. METHOD: Data on symptomatic chronology in each ear, auditory evolution and evolution of vertiginous crisis, among other aspects, were obtained, analysed and compared. Possible risk factors for Ménière's disease becoming bilateral were analysed after conducting nested case-control studies in a cohort. RESULTS: The metachronous form was seen in 76.3 per cent of cases, and the time it took for the disease to become bilateral took a median time of seven years. The symptomatic triad was the most frequent symptomatic debut for the first ear in both forms. Synchronous debut presented a greater average hearing loss. Suffering from migraine and a symptomatic onset with a greater number of symptoms appear to be possible predictors of conversion to bilateral Ménière's disease. CONCLUSION: Bilateral Ménière's disease temporal models presented differences. The study of them helps to better understand, prevent and predict the behaviour of these patients.

Extended phenotype and clinical subgroups in unilateral Meniere disease: A cross-sectional study with cluster analysis.

OBJECTIVES: To define clinical subgroups by cluster analysis in patients with unilateral Meniere disease (MD) and to compare them with the clinical subgroups found in bilateral MD. DESIGN: A cross-sectional study with a two-step cluster analysis. SETTINGS: A tertiary referral multicenter study. PARTICIPANTS: Nine hundred and eighty-eight adult patients with unilateral MD. MAIN OUTCOME MEASURES: best predictors to define clinical subgroups with potential different aetiologies. RESULTS: We established five clusters in unilateral MD. Group 1 is the most frequently found, includes 53% of patients, and it is defined as the sporadic, classic MD without migraine and without autoimmune disorder (AD). Group 2 is found in 8% of patients, and it is defined by hearing loss, which antedates the vertigo episodes by months or years (delayed MD), without migraine or AD in most of cases. Group 3 involves 13% of patients, and it is considered familial MD, while group 4, which includes 15% of patients, is linked to the presence of migraine in all cases. Group 5 is found in 11% of patients and is defined by a comorbid AD. We found significant differences in the distribution of AD in clusters 3, 4 and 5 between patients with uni- and bilateral MD. CONCLUSIONS: Cluster analysis defines clinical subgroups in MD, and it extends the phenotype beyond audiovestibular symptoms. This classification will help to improve the phenotyping in MD and facilitate the selection of patients for randomised clinical trials.

Patient baseline characteristics in an open-label multinational study of betahistine in recurrent peripheral vestibular vertigo: the OSVaLD study.

OBJECTIVES AND METHODS: OSVaLD (Observational Study in patients suffering from recurrent peripheral vestibular Vertigo to Assess the effect of betahistine 48 mg/day on quality of Life and Dizziness symptoms) is a 3-month, open-label, multi-national post-marketing surveillance study of betahistine 48 mg/day in the management of patients with vertigo of less than 5 years in duration. The aim of the study is to examine the burden of disease associated with vertigo, as determined by scores on the Dizziness Handicap Inventory (DHI), Short Form-36 (SF-36) questionnaire and the Hospital Anxiety and Depression Scale (HADS). Changes in DHI, SF-36 and HADS scores between baseline and 3 months are used to assess the therapeutic effects of betahistine. RESULTS: Participants (n = 2037) have been recruited from 13 countries in four continents (North and South America, Asia and Europe), representing a wide range of cultural and linguistic traditions. Approximately two-thirds of the patients are women. Sixty per cent of patients have diagnoses of peripheral vestibular vertigo of unknown pathology or benign paroxysmal positional vertigo; 13% have a diagnosis of Ménière's disease. All three of the instruments used characterize this as a population with extensive vertigo-attributable morbidity at baseline. The mean DHI score of the population is 63.7 +/- 15.7 (DHI scale: 0 = no handicap; 100 = major self-perceived handicap), SF-36 scores in all domains are below the population average for the USA and the HADS indicated that > 50% of patients exhibit symptoms of anxiety or depression or both, including 9% who have severe manifestations of either or both conditions. CONCLUSIONS: This report describes the design and implementation of OSVaLD and presents baseline demographic and clinical features of the patients. Full results of the study, anticipated in 2007, will provide more details about the manifestations of vertigo in routine practice and the response to betahistine.

[The vestibulocollic reflex: assessment and characteristics of vestibular-evoked myogenic potentials analysed by age groups]. / Reflejo vestibulocervical. Valoracion y caracteristicas de los potenciales vestibulares miogenicos evocados analizados por tramos de edad.

INTRODUCTION: Obtaining vestibular-evoked myogenic potentials (VEMP) by means of the vestibulocollic reflex is a readily available technique that provides an image of vestibular functioning and is useful for evaluating the pathologies that involve compromise of the anatomical pathway of the reflex. Although normal patterns do exist, responses vary at different ages. AIM: To obtain reference values of the vestibulocollic reflex according to different age groups. SUBJECTS AND METHODS: We studied 40 volunteers with no symptoms of auditory or vestibular compromise. Each ear was stimulated separately by a series of clicks (sounds lasting 0.1 s; 3 pps; intensities of 100 dB nHL and 85 dB nHL) and recordings were made in the sternocleidomastoid muscles by means of surface electrodes as patients who were lying on their backs contracted these muscles as they lifted their heads. We studied the latency of the initial p13-n23 positive-negative potential and the peak-to-peak amplitude. The existence of later n34-p44 potentials was evaluated. RESULTS: No statistically significant differences were found between genders or between the two ears. We did not find any differences between the latencies of the waves according to the intensity of the stimulus, but there is a relationship between the amplitude of the p13-n23 potential and the intensity of the stimulus. The latencies of the responses in children under 10 years of age differ from those of the other groups, but no differences were found among those over the age of 11. CONCLUSIONS: The VEMP display steady and easily identifiable latencies. We obtained different reference values for latency in children under the age of 10 and those over 11 years old. The amplitude decreases with the intensity of the stimulus.

Identification of 14 novel mutations in the long isoform of USH2A in Spanish patients with Usher syndrome type II.

Mutations in USH2A gene have been shown to be responsible for Usher syndrome type II, an autosomal recessive disorder characterised by hearing loss and retinitis pigmentosa. USH2A was firstly described as consisting of 21 exons, but 52 novel exons at the 3' end of the gene were recently identified. In this report, a mutation analysis of the new 52 exons of USH2A gene was carried out in 32 unrelated patients in which both disease-causing mutations could not be found after the screening of the first 21 exons of the USH2A gene. On analysing the new 52 exons, fourteen novel mutations were identified in 14 out of the 32 cases studied, including 7 missense, 5 frameshift, 1 duplication and a putative splice-site mutation.

[Ménière's disease and migraine]. / Enfermedad de Ménière y migraña.

INTRODUCTION: The possible link between vertigo and migraine has been known for a long time as well as its association with Ménière's disease. OBJECTIVE: To determine the lifetime prevalence of migraine in EM patients and to compare it with the prevalence of migraine in general population according to the International Headache Society (IHS) criteria. METHODS: We interviewed 65 EM patients. Our results have been compared with a sex- and age-matched control group with no otological antecedent. RESULTS: The lifetime prevalence of migraine was significantly higher in Ménière group compared to the age- and sex-matched control group (35.4% versus 15.4%, p < or = 0.05). Nine patients with EM and migraine related an evident relationship between their vertigo episodes and the migraine. CONCLUSIONS: The results obtained in our study suggest the possibility of common pathophysiological mechanisms to both pathologies and it may lead to new therapeutic options for treatment of EM and migraine patients.

Mutation profile of the MYO7A gene in Spanish patients with Usher syndrome type I.

Usher syndrome type I is the most severe form of Usher syndrome. It is an autosomal recessive disorder characterized by profound congenital sensorineural deafness, retinitis pigmentosa, and vestibular abnormalities. Mutations in the myosin VIIA gene (MYO7A) are responsible for Usher syndrome type 1B (USH1B). This gene is thought to bear greatest responsibility for USH1 and, depending on the study, has been reported to account for between 24% and 59% of USH1 cases. In this report a mutation screening of the MYO7A gene was carried out in a series of 48 unrelated USH1 families using single strand conformation polymorphism analysis (SSCP) and direct sequencing of those fragments showed an abnormal electrophoretic pattern. Twenty-five mutations were identified in 23 out of the 48 families studied (47.9%). Twelve of these mutations were novel, including five missense mutations, three premature stop codons, three frameshift, and one putative splice-site mutation. Based on our results we can conclude there is an absence of hot spot mutations in the MYO7A gene and that this gene plays a major role in Usher syndrome.

[Vestibular evoked myogenic potential: a contribution to the vestibular physiology and pathology knowledge. Quantitative patterns in healthy subjects]. / Potencial vestibular miogénico evocado: un aporte al conocimiento de la fisiología y patología vestibular. Patrones cuantitativos en la población normal.

INTRODUCTION: Vestibulocollic reflex is a muscular reflex which is activated by acoustic stimulation. It shows two components: the first one, the vestibular evoked myogenic potential (VMEP) related to the vestibular pathway and the second, to the auditive pathway. The VMEP potential could be useful for vestibular physiology and pathology knowledge. OBJECTIVE: To determine the VMEP's parameters of normality and to evaluate influential factors such as age, sex or stimulation intensity. MATERIAL AND METHODS: Prospective study of the VCR in 40 healthy individuals, distributed in 4 groups according to their age. Stimulation was carried out at 100db and 85db on each ear independently. RESULTS: There are not differences between both ears and sexes. Normal latency levels must be separated between two groups of age: older and younger than 11 years old. Normal absolute values of amplitude response differ depending on muscular contraction, age and stimulation intensity. It is more representative to consider the difference between the values obtained in the two ears of the same individual than to consider the absolute values.

[Kinetic tests representativity in vestibular function]. / Representatividad de las pruebas cinéticas en la función vestibular.

INTRODUCTION: The most frecuently tests used to study the vestibular system, with videonystagmographic register, are the caloric and rotatory ones. MATERIAL AND METHODS: We have carried out a comparative study of the kinetic tests through a sample of 41 patients without vestibular pathology, stratified by age and gender. The performed rotational tests have been: Constant/cycles, postrrotatory test, increasing, decreasing and constant test. RESULTS: We have not observed age or gender influence on the results. We give the numeric results of these tests. CONCLUSION: The obtained data allow to conclude that the information given by the increasing and decreasing pendulous test is similar, phenomenon that doesn't happen in the rest of the practiced tests. We also study the habituation phenomenon to a know stimuli. We point out the importance of each laboratory to have their tests done previously in a normal control group in order to compare the results when performing them in pathological patients.

[Menière's disease: diagnostic criteria, criteria to establish stages, and standards for treatment evaluation. Bibliographic review and update]. / Enfermedad de menière: criterios diagnósticos, criterios para establecer estadios y normas para la evaluación de tratamientos. Revisión bibliográfica y actualización.

If all doctors would use the same concepts to define Meniere's Disease (MD) and the same scale to either establish the different steps of the disease or to evaluate a specific treatment, it would be easy to compare our results. This would be beneficial for patients because of the possibility of giving them a more accurate information about the follow-up of their disease and the advantages of the different possibilities of treatment. Since the introduction of informatic systems a new goal appeared: large databases could be achieved, as a result of being able to gather the different database that are independently used in each hospital, provided that rules to establish data would be previously standardized. The purpose of this paper is to evaluate the proposal of the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology and those proposed by other authors according to the stated courses of action.

[Benign paroxysmal positional vertigo secondary to surgical maneuvers on superior maxilla]. / Vértigo posicional paroxístico benigno secundario a maniobras odontológicas sobre maxilar superior.

The benign paroxysmal positional vertigo (BPPV) is a common vestibular end organ disorder. BPPV may be idiopathic or secondary to head traumas, viral labyrinthitis, otology surgery and many others conditions. We report two cases of BPPV secondary to surgical maneuvers on superior maxillary. We have not found this etiology in scientific literature but, the increase of this kind of surgery, can produce an increase of its frequency. For this reason the otoneurologists and oral surgeons must bear in mind this possibility to make a right diagnostic and treatment of the syndrome.

[Medium and large acoustic neurinomas with normal tonal audiometry at the moment of diagnosis]. / Neurinomas acústicos medianos y grandes con audiometría tonal normal en el momento del diagnóstico.

INTRODUCTION: Early diagnosis of an acoustic neurinoma for any possible treatment is important, especially in view of the relationship between surgical morbidity and the size of the tumour. The commonest presenting symptom of these tumors is unilateral perceptive deafness. However, between 4 and 12% are diagnosed before deafness occurs, although they may be large in size. Therefore one should remember other symptoms which lead to suspicion of these tumors, and have diagnostic guidelines available to allow the earliest possible detection. PATIENTS AND METHODS: We report four cases of acoustic neurinoma or vestibular schwannoma of medium or large size in which audiological studies did not at first give rise to suspicion of the presence of this tumour, since audition was considered to be normal at conversation frequencies (500 to 3,000 Hertz) for the age of the patient or who had an audiometry curve with symmetrical deafness in both ears. CONCLUSION: We evaluated the diagnostic importance of the tests done and compared the results with data published in the literature.

[Syndromic hereditary deafness. Usher's syndrome. Oto-neurologic and genetic factors]. / Sordera hereditaria sindrómica: síndrome de Usher. Aspectos otoneurológicos y genéticos.

Usher syndrome (USH) is an autosomal recessive hereditary disorder characterized by congenital bilateral sensorineural hearing loss and progressive loss of vision due to retinitis pigmentosa (RP). The prevalence of Usher syndrome is estimated to be 3-4.4 cases per 100.000 people. Several clinical types have been distinguished by age at onset, rate of progression, and severity of symptoms. Type I (USH1) is characterized by a congenital, severe-to-profound deafness and absent vestibular function. Type II (USH2) shows a congenital and moderate-to-severe hearing loss and normal vestibular response. It is also suggested a third type (USH3), clinically similar to USH2, but with progressive hearing loss. Genetic heterogeneity of USH is quite extensive. Up to now, seven different loci responsible for the defect are known: 14q, 11q, 11p, 10q and 21q for USH1; 1q for USH2 and 3q for USH3. Moreover, there are USH1 and USH2 families that fail to show linkage to these candidate regions demonstrating that should exist other loci causing USH, although their ubications are unknown. To date, only two genes involved in the USH pathology are known, although together they are responsibles of about the 80% of total USH cases: myosin VIIA, an unconventional myosin, involved in the USH1b phenotype and a protein similar to the laminina, responsible for the USH2a phenotype.

[Audiometric threshold shift in patients with cochlear implantation]. / Variación de los umbrales audiométricos en pacientes implantados.

A great number of studies have been carried out during many years in order to evaluate the histological changes that take place after the insertion of a cochlear implant, having been experimentally and histologically, in temporal bones, demonstrated its relative harmlessness. We have focused our functional investigation induced by the implants through the shift of pre- and postoperative tonal audiometric thresholds, in 13 carriers on an C. I. Nucleus 22. Most of them suffered minimal or even showed no shift of thresholds. Therefore we consider the functional alterations due to the insertion of electrodes as practically nonexistent.

[Petrous bone approach for the surgery of petroclival meningiomas]. / La vía transpetrosa para el abordaje de meningiomas petroclivales.

Four patients, 3 woman and 1 man, with giant petroclival meningiomata are presented. In all cases the tumor size exceed 44 mm in its greater diametre. Main clinical symptoms were endocranial hypertension and cranial nerves deficit. A modified transcochlear approach in two stages was used. A total petrosectomy was performed, using the petrosygmoidal via for opening the dura. Transversus and sygmoid sinus were spared. Total removal was achieved in 2 cases, subtotal and partly in each of the other 2. No operative mortality occurred (hospital exitus) and neurologic morbility related to V-VI-VII and VIII cranial nerves and 1 hemyparesia was recorded. The AA. explain and comment the fundamentals of the followed procedure and compare, as well, the own outcomes with those reported by more classic alternatives. Finally, the conclusion is drawn out that retroclival meningiomata are conditions treatable, although always are difficulty approached and demand meticulous microsurgical techniques.

Linkage analysis in Usher syndrome type I (USH1) families from Spain.

Usher syndrome (USH) is an autosomal recessive hereditary disorder characterised by congenital sensorineural hearing loss and gradual visual impairment secondary to retinitis pigmentosa (RP). The disorder is clinically and genetically heterogeneous. With regard to Usher type I (USH1), several subtypes have been described, the most frequent being USH1B located on chromosome 11q13.5. Of 18 USH1 families studied by linkage analysis, 12 (67%) showed significant lod score values for locus D11S527 (Zmax=14.032, theta=0.000) situated on chromosome 11q. Our findings suggest considerable genetic heterogeneity in the Spanish USH1 population. It is important to note that one of our families linked to the USH1B locus shows interesting intrafamilial clinical variability. As regards the remaining six USH1 families, the linkage analysis did not provide conclusive data, although two of them show slight linkage to markers located on chromosome 3q (Zmax=1.880, theta=0.000 for D3S1279), the same location that had previously been assigned to some USH3 families.

[Vertigos in childhood. Characteristics according to our results]. / Vértigos de la infancia. Características según nuestros resultados.

The paper deals with a review of 25 cases of infantile dizziness done for a term of 10 years. The protocol employed in the study, the characteristics and the follow-up of the crises are contemplated.

[Meningioma with otological etiology]. / Meningiomas de inicio otológico.

Meningiomas are tumours which first symptomatology are related to its sitting. When localized in the posterior fossa present as otological complaints and may mimic other otological conditions. The AA. report 3 meningioma cases starting with audiovestibular pictures. All were both electrophysiologically and radioimaging studied and underwent an otoneurologic procedure and the histopathological verification. The paper underlines the importance of these cases for ENT-specialist under both the clinical and surgical point of view.