Ágnes Szirmai

Cochlear Implantation Influences Contralateral Hearing and Vestibular Responsiveness

A cochlear implant program has been carried out at the ORL Clinic of Semmelweis University in Budapest since 1985. Different devices and techniques have been used in pre- and postlingual children and adults. Over the last 6 years contralateral hearing improvement has been observed in 18 patients. This phenomenon can usually be demonstrated 6 months after the operation. Since our first observation several other authors have confirmed this phenomenon. However, the underlying mechanism is still obscure; both the efferent innervation and the plasticity of the brain may be important factors. The vestibular function of cochlear implant candidates was checked routinely pre- and postoperatively and changes in vestibular responsiveness were observed. Over the last 3 years air-caloric stimulation has been performed by means of a computer-based ENG system. In most patients the vestibular function was unchanged after the implantation. but in some cases a significant improvement in vestibular responsiveness was noted. Here we briefly describe cases of re-implantation carried out at the clinic. All the patients obtained better speech discrimination scores after having been implanted with intracochlear devices. None of the patients suffered any inner ear abnormality due to the first implant.

Air caloric computer system application in monitoring vestibular function changes after cochlear implantation.

INTRODUCTION: The computer-based ENG systems analytical routine refinements are available only in research laboratories. The computer-based system contains a stimulator for saccadic eye-movement and an air caloric stimulator interconnected to the registration program and analysis software. Several authors have reported the preoperative and postoperative vestibular functions in cochlear implant patients. The safe examination of the operated ear and comparison of the preoperative and postoperative average slow phase velocities of the caloric nystagmus is possible using the air caloric computer system. METHODS: The authors have used a computer-based ENG system with caloric air stimulation, which is very useful for examination of the operated ear. The vestibular system of patients with total deafness was examined before and after the cochlear implantation. The results of 60 vestibular examinations of 64 patients are reported. RESULTS: The vestibular function in the operated ear was found unchanged in 20 patients. In 14 patients the vestibular function was worse. In 16 patients the postoperative vestibular responsiveness improved. The detailed data analysis of 10 patients in whom preoperative and postoperative vestibular examination was similar was performed by the authors. The preoperative and postoperative average slow phase velocity values and the relative canal paresis was observed. The values demonstrated that the caloric responsiveness of the operated ear improved in few cases. CONCLUSIONS: The computer-based air caloric system is a useful and safe method in evaluation of the vestibular system changes after ear surgery. The reason of the improvement of vestibular responsiveness is not clear. The results need further investigation to solve the problem of vestibular responsiveness improvement after cochlear implantation.

Vestibular disorders in patients with migraine

Vestibular symptoms frequently occur in patients with migraine headache. The common migraine is defined in neurology as a unilateral, pulsating headache, which may be associated with nausea and vomiting, and lasts one or several days. In the classic form patients have visual prodromal symptoms. Focal neurological signs in the migraine complique include, for example, oculomotor palsy and vestibular abnormalities. This so-called vestibular migraine is different from basilar migraine, which involves the irritation of the cervical sympathetic system, and can cause symptoms that resemble transient brainstem ischemia. In order to evaluate vestibular dysfunction electronystagmography (ENG) was used. Patients frequently had abnormal caloric test responses, especially with a directional preponderance, and most had a spontaneous nystagmus. In the migraine attack the patients are presumed to have hypersensitivity of the labyrinth with nausea and vomiting, while in the headache-free period the ENG was almost normal. At present, we have had a high success rate in treating patients with pyracetam. Diazepam was used to treat basilar migraine and flunarizine to prevent vestibular migraine.

Vestibular disorders in migrainous children and adolescents

Abstract Recurrent vertigo is a special form of migraine in childhood. It is a periodic syndrome of childhood which was previously called migraine equivalent. Thirty young patients (less than 18 years of age) with migraine and vertigo were examined by the authors. The vestibular system of the patients was examined by computer-based electronystagmography. All patients had migraine-related vestibular dysfunction. Most had spontaneous nystagmus and 86.7% had an abnormal bithermal caloric test. Other forms of migraine-associated periodic syndromes – especially abdominal pain – were found in about 25% of the patients. Approximately one-third of the patients had a family history of migraine, and about half of them had motion sickness. The cause of migraine and migraine-related vestibular disorders is still unidentified, but the origin of the attacks is believed to be located in the brainstem, especially in the pons. This fact is congruent with our results indicating that a central vestibular dysfunction can be found in patients with migraine.

Change of Vestibular Function after Cochlear Implantation

The vestibular system of patients with total deafness was examined before and after cochlear implantation. The authors have used a computer-based electronystagmography system with air caloric stimulation for 3 years. It is very useful for the examination of the operated ear. The results of 60 vestibular examinations of 64 patients are reported. Immediately after the surgery 10 patients had vertigo lasting 2–4 days. In 20 patients, the vestibular function of the operated ear was the same as preoperatively. In 14 patients, the vestibular function became worse after the cochlear implantation, in 16 patients the postoperative vestibular responsiveness became better. The preoperative labyrinthine anesthesia changed, and a normal or almost normal caloric reaction was registered in the operated ear. In 4 patients, the ear receiving the cochlear implant had a hyperreactive labyrinthine function.

Electronystagmographic analysis of caloric test parameters in vestibular disorders.

The electronystagmographical analysis of the eye movements provoked by caloric stimulation is an important method in the evaluation and topical diagnostic procedure of several vestibular lesions. The aim of the study was to compare the electronystagmographical results of caloric response in several vestibular disorders. The patients were divided into five groups: right and left unilateral and bilateral peripheral lesions, central vestibular dysfunction, and normal vestibular function. In the normal vestibular system group the average caloric nystagmus SPV in normal vestibular system was 17.4°/s. In the peripheral lesion groups the average slow phase velocities are decreased in the affected side, as we expected. In the compensated vestibular lesion the average ASPV of caloric nystagmus is also decreased on the unaffected side. This might be caused by the effect of the central adaptive mechanisms. According to our observations, in central dysfunctions the average caloric ASPV and the spontaneous nystagmus ASPV is increased (25.0°/s). This suggests that in central vestibular lesions the central inhibiting mechanisms of the caloric response are impaired. Our results show that electronystagmographical analysis of spontaneous and caloric nystagmus is very important in the evaluation of dizzy patients.

Az asszisztált egyensúlyi tréning hatékonysága krónikus vestibularis rendszeri szédülésben

Absztrakt: Bevezetes: A szedulekenyseg nepegeszsegugyi jelentőseget az adja, hogy a szedulessel orvoshoz fordulo betegek szama nagy. Az egyensulyrendszeri betegsegek kezeleseben nagy jelentősege van a fejlesztesnek, a treningnek. Celkitűzes: Egy olyan egyszerű gyakorlatsor kialakitasa volt a celunk, mely hozzatartozo felugyelete mellett vegezhető, es eredmenyessege szamszerűen ertekelhető. Modszer: A betegek korhazi kezelesuk alatt keringesjavito infuzios kezelest kaptak, es egyensulyi treninget vegeztek. Az eredmenyek ellenőrzesehez Jacobson es Newman kerdőivenek magyarra forditott valtozatat alkalmaztuk. Az objektiv javulas megitelesehez ultrahangos szamitogepes kraniokorpografias mereseket alkalmaztunk. A statisztikai analizis soran ANOVA- es Kruskal–Wallis-tesztet, a tobbszoros osszehasonlitashoz Dunn-fele „post” metodust hasznaltunk (szignifikanciaszint: p<0,05). Eredmenyek: A korhazi kezeles soran a treningcsoportban a betegek altal elert atlagos treningpontszamok javultak. A nyolcnapos trening alat...

Analysis of GJB2 mutations and the clinical manifestation in a large Hungarian cohort

PurposePathogenic variants of the gap junction beta 2 (GJB2) gene are responsible for about 50% of hereditary non-syndromic sensorineural hearing loss (NSHL). In this study, we report mutation frequency and phenotype comparison of different GJB2 gene alterations in Hungarian NSHL patients.MethodsThe total coding region of the GJB2 gene was analyzed with Sanger or NGS sequencing for 239 patients with NSHL and 160 controls.ResultsHomozygous and compound heterozygous GJB2 mutations were associated with early onset serious clinical phenotype in 28 patients. In 24 patients, two deletion or nonsense mutations were detected in individuals with mainly prelingual NSHL. In compound heterozygous cases, a combination of deletion and missense mutations associated with milder postlingual NSHL. A further 25 cases harbored single heterozygous GJB2 mutations mainly associated with later onset, milder clinical phenotype. The most common mutation was the c.35delG deletion, with 12.6% allele frequency. The hearing loss was more severe in the prelingual groups.ConclusionThe mutation frequency of GJB2 in the investigated cohort is lower than in other European cohorts. The most serious cases were associated with homozygous and compound heterozygous mutations. In our cohort the hearing impairment and age of onset was not altered between in cases with only one heterozygous GJB2 mutation and wild type genotype, which may exclude the possibility of autosomal dominant inheritance. In early onset, severe to profound hearing loss cases, if the GJB2 analysis results in only one heterozygous alteration further next generation sequencing is highly recommended.

How can we Detect the Balance System Disorder andits Improvement after Rehabilitation

Physicians are found to have a great and increasing amount of patients suffering from vertigo in their everyday practice. For the treatment of the balance disorders, a vestibular training was introduced which played an influential role based on our study. Rehabilitation of patients having balance disorders is a known, major challenge for physicians. Vestibular rehabilitation therapy (VRT) is an exercise-based treatment program designed to promote vestibular adaptation and substitution. The goals of VRT are

Az akut vestibularis szindróma sürgősségi diagnosztikájával szerzett tapasztalatok

INTRODUCTION AND AIM To diagnose acute vestibular syndrome (AVS) in a prospective study by a new bedside test (providing 1A evidence) based on oculomotor analysis and assessment of hearing loss. To assess the frequency of central and peripheral causes of acute vestibular syndrome in the emergency room. To establish the diagnostic accuracy of acute cranial computed tomography as compared to oculomotor analysis done by video oculography goggles and audiometry. METHOD Between 1st March 2016 and 1st March 2017 we documented 125 patients (62 women, 63 men, average age 53 years) in the emergency room of the Petz Aladár County Teaching Hospital using the above bedside and instrumental testing. Diagnosis was verified by cranial magnetic resonance imaging. RESULTS According to the results of the instrumental examination in AVS in 67% we found a peripheral cause and in 33% a central pathology. In 62% isolated posterior circulation stroke manifested itself by isolated vertigo without additional focal signs and the acute cranial computed tomography showed negative results in 96%. The instrumental examination increased diagnostic accuracy by making the diagnosis of isolated inferior semicircular canal vestibular neuritis possible. CONCLUSIONS The new bedside oculomotor test is suitable for the diagnosis of posterior circulation stroke manifesting with isolated vertigo in early cases, when the routine neuroradiologic methods have a lower sensitivity or are not available. Orv Hetil. 2017; 158(51): 2029-2040.Absztrakt: Bevezetes es celkitűzes: A szemmozgasok analizisen es az akut hallascsokkenes detektalasan alapulo 1A evidenciaju uj diagnosztikai modszer hatekonysaganak vizsgalata akut vestibularis szindromakban. A periferias es a centralis eredetű vestibularis korkepek es az izolalt szedulessel jelentkező hatsoscala-teruleti stroke-ok előfordulasi gyakorisaganak elemzese. A stroke kizarasa celjabol vegzett surgőssegi koponya komputertomografias vizsgalat diagnosztikus ertekenek a felmerese. Egy videookulografias szemuveg es az audiologiai vizsgalat segitsegevel vegzett eszkozos es az eszkoz nelkuli, agy melletti modszer diagnosztikai hatekonysaganak az osszehasonlitasa. Modszer: Egy prospektiv tanulmany soran a győri Petz Aladar Megyei Oktato Korhaz Surgőssegi Osztalyan akut vestibularis szindromaval jelentkező 125 paciensnel (62 nő, 63 ferfi, atlageletkor 53 ev) 2016. marcius 1. es 2017. marcius 1. kozott koponya komputertomografias vizsgalatot kovetően az agy melletti fizikalis, műszer nelkuli es a kvanti...