Biomedical subjects

József Jóri

Publications and source records attributed to József Jóri.

13 recordsLinked to original sources

PubMed2006

Hearing disturbances in hypertrophic cardiomyopathy. Is the sensorineural disorder neurogenic or myogenic?

BACKGROUND: In a previous study, we demonstrated that a hidden hearing defect is present in about 50% of patients with hypertrophic cardiomyopathy (HCM). Such defects were found significantly less frequently in age and clinical stage-matched patients with dilated cardiomyopathy (DCM), and were practically absent in patients with valvular aortic stenosis, and in patients taking beta-receptor blockers for different reasons (such as hypertension, ischemic heart disease, etc.). The hearing disturbances were first examined by means of brain-stem evoked response audiometry (BAEP). This method permitted only a rough differentiation between the origins of cochlear (myogenic) and retrocochlear (neurogenic) hearing disturbances, and did not allow us to establish their myogenic or neurogenic nature with certainty. AIMS: Our present aim was to determine whether the hearing disturbances present in HCM and DCM patients are myogenic or neurogenic in origin. METHODS: The neurogenic function of the inner ear was examined by BAEP as before, and the myogenic function by the distortion product otoacoustic emission technique. RESULTS: Myogenic abnormalities were found in 39/69 ears (57%) and neurogenic abnormalities in 19/69 (28%) ears among the HCM cases, as compared with 14/39 (36%) and 8/39 (21%) ears respectively among the DCM cases (p<0.005). Healthy controls displayed the lowest incidence of both types of hearing abnormalities. CONCLUSION: Our results lead us to conclude that myogenic lesions are more frequent than neurogenic lesions in patients with HCM. Both myogenic and neurogenic lesions are more frequent in HCM patients than in DCM patients or healthy controls. It may be hypothesized that abnormal sarcomeric proteins present in the muscular structures of the inner ear in HCM are possibly responsible for the hearing disorders in these patients, and that this is not merely a neurological defect.

Adolescent↗

PubMed2006

Antimeasles immunoglobulin g for serologic diagnosis of otosclerotic hearing loss.

HYPOTHESIS: Persistent measles virus infection of the otic capsule is suggested to be an etiologic factor in otosclerosis. Otosclerosis is a disease of complex unknown etiology causing progressive conductive and/or sensorineural hearing loss (HL). BACKGROUND: Diagnostic methods of otosclerosis are sensitive to ossicular chain fixation with low specificity for otosclerotic stapes ankylosis. METHODS: Nucleic acid was extracted from stapes foot plates of clinically stapes fixation patients (N = 213). Measles virus nucleoprotein RNA was amplified by reverse-transcriptase polymerase chain reaction. Amplification results were correlated to histologic findings in 49 cases. Antimeasles IgG levels of all clinically stapes fixation as well as control sera specimens were measured by enzyme-linked immunosorbent assay. RESULTS: Among clinically stapes fixation patients, 141 stapes foot plates contained measles virus RNA. Among 49 histologic specimens, viral RNA was detectable only in histologically otosclerotic stapes foot plates (n = 35). Histology for virus-negative foot plates (n = 14) excluded otosclerosis. Antimeasles IgG levels were significantly lower in the sera of patients with virus-positive stapes than in control sera. CONCLUSIONS: Combination of decreased antimeasles IgG serum level and conductive HL has a great specificity and sensitivity as a diagnostic method in the preoperative evaluation of ossicular chain fixations otosclerosis. Low antimeasles IgG level indicates otosclerosis, whereas high level suggests non-otosclerotic ossicular chain fixations. Preoperative elucidation of the cause of a conductive HL may suggest optional medical treatment in preference to surgical methods.

Adult↗

PubMed2006

Detection of osteoprotegerin and TNF-alpha mRNA in ankylotic Stapes footplates in connection with measles virus positivity.

HYPOTHESIS: Otosclerosis is a bone remodeling disorder of the otic capsule causing conductive and sensorineural hearing loss. Persistent measles virus infection of the temporal bone with increased tumor necrosis factor (TNF)-alpha and decreased osteoprotegerin mRNA expression is supposed to be the main etiologic factor in otosclerosis. BACKGROUND: Determinants of measles virus infection and reactive inflammation were studied in otosclerosis. The presence of measles virus was shown in otosclerotic patients by reverse transcriptase-polymerase chain reaction (RT-PCR) amplification of the viral RNA. No report is available, however, about the role and interactions of bone-specific cytokines in otosclerosis. METHODS: : Nucleic acid was extracted from stapes footplates of clinically otosclerotic patients. Measles virus nucleoprotein RNA was amplified by seminested RT-PCR. TNF-alpha and osteoprotegerin mRNA coexpression was detected by RT-PCR in otosclerotic bone and was correlated to measles virus positivity. RESULTS: Among 154 clinically stapes fixation otosclerotic patients, 99 stapedes contained measles virus RNA. TNF-alpha mRNA was detectable in 88 virus-positive and in 6 virus-negative stapes footplates. Osteoprotegerin mRNA expression was significantly lower in the TNF-alpha-positive specimens (P < .0001) that was independent from virus positivity. CONCLUSION: Detection of TNF-alpha mRNA demonstrates activated osteoclast functions and inflammatory pathways in otosclerotic stapes footplates associated with measles virus presence. Increased expression of TNF-alpha and its action on RANK production inhibits the protective functions of osteoprotegerin on normal bone turnover in the otic capsule.

Adult↗

PubMed2006

Activated osteoclasts with CD51/61 expression in otosclerosis.

HYPOTHESIS: Stapes ankylosis is supposed to be a disease with variable histopathology caused by otosclerosis or pseudo-otosclerosis. Persistent measles virus infection of the otic capsule could induce reactivation of quiescent embryonic osteoclasts in otosclerosis. BACKGROUND: Presence of measles virus RNA was demonstrated in the footplates of otosclerotic patients by reverse-transcription polymerase chain reaction (RT-PCR). Histology of active otosclerosis is featured by the presence of numerous osteoclasts with unknown phenotype. METHODS: Nucleic acid was extracted from stapes footplates of clinically otosclerotic patients (n = 261). Genomic RNA of measles virus was amplified by RT-PCR. Amplification results were correlated to postoperative histologic and CD51/61 specific immunohistologic findings. A parallel alcalic phosphatase activity assessment was performed to evaluate the metabolic activity of osteoclasts in each section. RESULTS: Among 261 stapes fixation cases, 175 otosclerotic stapes contained measles virus RNA. Histology for virus negative stapes (n = 86) represented nonotosclerotic, degenerative disorders. Histologically confirmed otosclerosis was featured by the presence of osteoclasts with renewed, embryonic phenotype. In otosclerosis, alcalic phosphatase activity was significantly higher compared with nonotosclerotic stapes ankylosis (P < .001). CONCLUSION: The presence of CD51/61 positive osteoclasts in otosclerotic bone containing viral sequences provides the basis for an inflammatory bone remodeling disorder. Otosclerosis is a disease caused by persistent measles virus infection and reactivation of resting embryonic osteoclasts in the otic capsule.

Adult↗

PubMed2005

Cholesterol granuloma of the maxillary sinus resembling an invasive, destructive tumor.

The case of a maxillary sinus cholesterol granuloma posing as a malignant tumor is presented. The patient was referred to the authors' clinic with symptoms typical of maxillary sinusitis, but physical examination suggested the presence of neoplasm. Radiology also resulted in confusing, tumor-like pictures. Histological examination of a preoperative tissue sample identified the process as a cholesterol granuloma, which was removed by a classic Caldwell-Luc operation. The patient has been symptom free since the operation. The pathogenesis of cholesterol granuloma is described, and the problems of establishing a diagnosis without preoperative histology are discussed.

Cholesterol↗

PubMed2005

Codetection of measles virus and tumor necrosis factor-alpha mRNA in otosclerotic stapes footplates.

HYPOTHESIS: Otosclerosis is a disease of unknown etiology causing conductive or sensorineural hearing loss. Persistent measles virus infection of the otic capsule is considered to be one of the etiologic factors in otosclerosis. BACKGROUND: Determinants of measles virus infection and reactive inflammation were studied in otosclerosis. The presence of measles virus was shown in otosclerotic patients by reverse-transcription polymerase chain reaction (RT-PCR) amplification of the viral RNA. No report is available, however, on the types and features of paracrine cytokines in otosclerosis. METHODS: Nucleic acid was extracted from stapes footplate samples of clinically otosclerotic patients. Measles virus nucleoprotein RNA was amplified by seminested RT-PCR. Tumor necrosis factor (TNF)-alpha mRNA expression was detected by RT-PCR in otosclerotic bone and was correlated with preoperative audiologic findings and measles virus positivity. RESULTS: Among 154 clinically otosclerotic patients, 99 stapes footplate specimens contained measles virus RNA. TNF-alpha mRNA was detectable in 88 virus-positive and in 6 virus-negative stapes footplates. There was no detectable TNF-alpha mRNA expression in virus negative cases. CONCLUSION: The etiologic role of measles virus in the pathogenesis of otosclerosis should be considered. Detection of TNF-alpha mRNA demonstrates activated osteoclast functions and inflammatory pathways in otosclerotic stapes footplates associated with measles virus presence. Virus-associated and virus-negative pathomechanisms of otosclerosis should be distinguished.

Adult↗

PubMed2005

Two subgroups of stapes fixation: otosclerosis and pseudo-otosclerosis.

HYPOTHESIS: Stapes ankylosis is a disease with variable histopathology and can be caused by otosclerosis or pseudo-otosclerosis. Viral pathogenesis of otosclerosis could be established only by correlative analysis: histologic examination of the stapes footplate and reverse-transcriptase polymerase chain reaction (RT-PCR) amplification of the viral RNA. BACKGROUND: Presence of the RNA genome of measles virus was demonstrated in the footplates of clinically otosclerotic patients by RT-PCR, and also viral proteins were detected by immunohistochemistry. METHODS: Nucleic acids were extracted from ankylotic stapes footplates of clinically stapes fixation patients (n = 104). Measles virus genomic nucleoprotein (NP) RNA was amplified by seminested RT-PCR. Amplification results were correlated to postoperative histologic and audiologic findings. RESULTS: Measles virus RNA was detectable only in histologically otosclerotic stapes footplates (n = 67). Histology for virus negative footplates (n = 37) excluded otosclerosis. Virus negative stapes footplates showed nonotosclerotic, degenerative disorders. CONCLUSIONS: Stapes ankylosis is a heterogeneous disease causing conductive hearing loss with different etiologies. Nonotosclerotic stapes fixations could be established as pseudo-otosclerosis and may belong to nonspecific, degenerative disorders with variable and noncharacteristic histopathology. Otosclerosis is an inflammatory disease caused by persisting measles virus infection of the otic capsule.

Adult↗

PubMed2004

Cochlear implantation for treatment-induced ototoxic deafness in Langerhans cell histiocytosis. A case report.

A case is presented where the authors carried out a cochlear implantation on a girl whose deafness was induced by cytostatic agents. The normally hearing child was diagnosed with Langerhans cell histiocytosis at the age of 3 years and received polychemotherapy for almost 2 years. By her 5th year, she started to develop a bilateral, progressive sensorineural hearing loss, necessitating a hearing aid in both ears. While her histiocytosis was cured, her hearing ultimately deteriorated to total deafness on the right and profound hearing loss on the left ear. After 3 years, her hearing aids no longer provided adequate hearing for the postlingually deafened girl. At the age of 14, a cochlear implantation was performed on her right ear with excellent results.

Adolescent↗

PubMed2003

ALA (5-aminolevulinic acid)-induced protoporphyrin IX fluorescence in the endoscopic diagnostic and control of pharyngo-laryngeal cancer.

Photodynamic diagnosis is a modern method for the fluorescence imaging of superficial pharyngeal and laryngeal tumours. 5-aminolevulinic acid (ALA)-induced protoporphyrin IX fluorescence benefits the tumour selective accumulation of protoporphyrin; therefore, tumours can be differentiated from healthy tissue. ALA-induced fluorescence of laryngo-pharyngeal tumours and precancerous and benign lesions were evaluated by endoscopy. At the Department of Otorhinolaryngology, Head and Neck Surgery, Szeged, Hungary, 31 patients underwent ALA-induced protoporphyrin fluorescence imaging. After topical application of ALA, mesopharyngeal tumours were visualised by direct fluorescence endoscopy. Laryngeal and hypopharyngeal tumours were examined with a laryngomicroscope with the patients under general anaesthesia after inhalation of an ALA solution. Intensity of PPIX fluorescence was classified and compared with pathological findings. The examination of all 13 laryngeal and 12 pharyngeal tumours resulted in a middle or strong intensity of red fluorescence, but for one cancer, four precancerous and two benign lesions. Healthy tissues showed green autofluorescence. Margins of mesopharyngeal and vocal cord tumours were clearly outlined under fluorescent vision, giving a helpful contribution to diagnosis and therapy even in clinically non-visible tumours. Laryngomicroscopy combined with laser surgery also indicated PPIX fluorescence examination visualising margins of the tumour intermediately with the endoscope. The ALA-induced PPIX fluorescence imaging method revealed a sensitivity of 96%. This method is applicable for detecting early superficial tumours, margins of tumours and follow-up after surgery/radiation therapy in the laryngo-pharynx.

Adult↗

PubMed2003

Neural response telemetry in cochlear implant users.

Programming of multichannel cochlear implants requires subjective responses to a series of sophisticated psychophysical percepts. It is often difficult for cochlear implant patients (especially young prelinguistically deaf children) to provide adequate responses for device fitting. However, the neural response telemetry (NRT) system renders possible the measurement of the compound action potential threshold. We performed NRT examinations in 27 cochlear implant users with Nucleus 24-channel cochlear implants. Measurements were obtained from five electrodes (3, 5, 10, 15, and 20) in each patient. Our goal was to look for correlation between behavioral subjective thresholds and compound action potentials. The action potentials could be elicited in 23 patients in all measured electrodes. The NRT threshold values were highly correlated with electrical threshold levels obtained through subjective responses. Our results suggest that the electrically elicited neural responses may yield very important information for device fitting in patients with cochlear implants.

Child↗

PubMed2003

Investigation of auditory brainstem function in diabetic patients.

We performed brainstem auditory evoked potential (BAEP) examinations in 15 patients with long-standing type 1 diabetes mellitus. We applied cardiovascular reflex tests for assessment of autonomic neuropathy. The aim of our investigation was to compare the BAEP results of this patient group with those of controls and to look for the possible correlation between alteration of the auditory brainstem function and cardiovascular autonomic neuropathy. Analysis of the latencies (waves I, II, III, and V) and the interpeak latencies (I-III and I-V) of BAEP revealed a significant difference between those of diabetics and those of healthy controls. The amplitudes of waves I, III, and V were definitely lower in comparison with amplitudes of healthy controls. We observed a positive correlation between the overall autonomic score and the latencies (waves III and V) and interpeak latencies (I-III, I-V). These data support the hypothesis that long-standing diabetes mellitus and diabetic neuropathy might be revealed as a cause of certain dysfunctions of the central auditory pathways.

Adult↗

PubMed2002

Successful cochlear implantation in a child after recovery from a head and neck malignancy: a case report.

We present the case of a successful pediatric cochlear implantation that was carried out following bilateral perilingual deafness caused by meningitis during the treatment of a childhood malignant tumor. A rhabdomyosarcoma localized in the frontobasal area was removed from the child at the age of 2 years. He then received 11 months of postoperative cytostatic treatment. A purulent meningitis developed at the end of the chemotherapy, resulting in a major-grade, bilateral sensorineural hearing loss (practically a perilingual deafness). After 6 tumor-free years and a meticulous preoperative assessment, a Nucleus 24 M cochlear implant was successfully implanted in the child's left ear. Two years after the operation, the child shows excellent hearing results and moderate speech development.

Antineoplastic Agents↗

PubMed2002

Effects of noise on the intensity of distortion product otoacoustic emissions.

Distortion product otoacoustic emission (DPOAE) is a widely used differential diagnostic method for diagnosing inner ear disorders. It provides us information on the condition of the outer hearing cells (OHCs). Otoacoustic emission occurs only when the OHCs are functioning normally. Changes in thresholds of DPOAE curves can provide us important information on the activity of the OHCs. The inner ear shows nonlinear properties if the OHCs are functioning normally. If OHCs are injured and thus function improperly, the system stops showing nonlinear properties. If we have a system with periodic excitation and, with the addition of white noise, the signal-to-noise ratio on the output increases (at least for small noise intensities), we have witnessed what is called phenomenon stochastic resonance. Our goal was to elucidate how white noise influences the intensity of DPOAE. If there is emission, that specific ear surely exhibits nonlinear behavior, which in turn is the basic property needed for stochastic resonance.

Adult↗