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Conductive Hearing Loss (conductive + hearing_loss)
Selected AbstractsHearing loss in Fabry disease: data from the Fabry Outcome SurveyEUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 9 2006S. Hegemann Abstract Background, Hearing loss is a common symptom in Fabry disease, but neither its natural course nor its aetiology has been defined precisely. The aim of this study was to provide a detailed epidemiological description of hearing impairment in patients in the Fabry Outcome Survey (FOS), which is the largest available database of Fabry patients. Materials and methods, Questionnaires were completed by 566 Fabry patients, of whom 316 reported ear-related symptoms. Pure-tone audiograms from 86 patients, performed before starting enzyme replacement therapy, were analysed and compared with age- and sex-specific normal values (International Organization for Standardization, ISO 7029). Results, When compared to an age-matched population (ISO 7029), 74% of patients had a threshold elevated above the 95th centile in at least one tested frequency. All frequencies were affected to a similar degree. However, only 14 patients (16%) were clinically affected by hearing impairment according to the age-independent World Health Organization (WHO) classification (mean threshold at 0·5, 1 and 2 kHz worse than 25 dB). Hearing loss was sensorineural in 63 patients (73%) of whom 7 patients (8%) had also a conductive component. One patient had a purely conductive hearing loss. Episodes of sudden hearing loss seemed to occur more frequently than in the general population. Men were affected earlier and more severely than women. Conclusions, Hearing in Fabry disease is significantly worse than in an age-matched general population but leads to clinically relevant hearing impairment in only 16% of cases. It resembles accelerated presbycusis with an additional Fabry-specific strial-type hearing loss. [source] Two Subgroups of Stapes Fixation: Otosclerosis and Pseudo-Otosclerosis,THE LARYNGOSCOPE, Issue 11 2005Tamás Karosi MD Abstract 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 cap-sule. [source] Eliminating the Limitations of Manual Crimping in Stapes Surgery?THE LARYNGOSCOPE, Issue 2 2005A Preliminary Trial with the Shape Memory Nitinol Stapes Piston Abstract Objective: Manual piston malcrimping in stapedotomy may be the major cause of the occurrence of the significant, interindividual variations of postoperative air-bone gap (ABG), air-bone gap closures (ABGC), and postoperative recurrences of conductive hearing loss. To eliminate the effects of manual crimping on stapedotomy outcomes, the self-crimping, shape memory alloy Nitinol stapes piston was investigated and hearing evaluated. Study design: Prospective, preliminary case-control study in a tertiary care referral center. Methods: Sixteen patients with otosclerosis undergoing reversed stapedotomy using the Nitinol stapes piston were matched to reference patients out of our conventional titanium piston database. The effects of the self-crimping Nitinol piston on the postoperative ABGC, the postoperative air-bone gap (ABG) variations, and the postoperative short-term hearing results were investigated 3, 6 and 9 months postoperatively. These data were statistically compared with the results of the control patients in our titanium stapes piston database. Results: The mean postoperative ABG and the interindividual variations of the postoperative ABG were significantly smaller in the Nitinol group, the extent of ABGC greater in the Nitinol piston group, but not significant. The postoperative short-term stability of ABGC was similar in both groups. No infections or adverse reactions occurred during follow-up. Conclusion: Our preliminary results suggest that the self-crimping shape memory alloy Nitinol stapes piston eliminates the limitations of manual malcrimping in stapedotomy, thus optimising the surgical procedure. This allows reliable, safe, and consistent air-bone-gap closure in patients with otosclerosis up to 1 year after surgery. [source] Lateralization During the Weber Test: Animal ExperimentsTHE LARYNGOSCOPE, Issue 3 2002Jean-Yves Sichel MD Abstract Objectives/Hypothesis The objective of this study were to present an assessment of a new theory to explain lateralization during the Weber test using an animal model. This theory is based on the discovery that a major pathway in bone conduction stimulation to the inner ear is through the skull contents (probably the cerebrospinal fluid [CSF]). The placement of a bone vibrator or tuning fork on the skull excites the inner ear by the classic osseous pathway and by the suggested CSF pathway. We assume that there is a phase difference between the stimulation mediated by the ossicular chain (inertial and occlusion mechanisms) and the one mediated by the CSF. The presence of a conductive pathology will decrease the magnitude of the sound energy mediated by the ossicular chain. Thus, the out-of-phase signal arriving through the bony pathways will be decreased, hence increasing the resultant sound intensity stimulating the cochlea. Study Design Prospective animal study. Methods The experiment was performed on 10 fat sand rats, which had undergone unilateral cochleostomy and a small craniotomy. The auditory nerve brainstem response (ABR) thresholds were measured to air-conducted stimulation, to stimulation with the bone vibrator applied to the skull, and to stimulation with the bone vibrator applied directly to the brain through the craniotomy. The ossicular chain of the second ear was then fixed to the middle ear walls with cyanoacrylate glue to induce a conductive hearing loss. The ABR thresholds to the same three stimuli were then measured again. Results After ossicular chain fixation, the ABR threshold to air-conducted stimulation increased, to bone vibrator stimulation on the bone decreased (hearing improvement), and to bone vibrator stimulation directly on the brain remained unchanged. Conclusions This experiment confirms the proposed theory. During clinical bone conduction stimulation, there is a phase difference between sound energy reaching the inner ear through the middle ear ossicles and from the CSF. A middle ear conductive pathology removes one of these components, thus increasing the effective sound intensity in the affected ear. On the other hand, when the bone vibrator is applied on the brain, the inner ear is stimulated only through the CSF, so ossicular chain fixation does not change the ABR threshold. Moreover, this study proves that lateralization during the Weber phenomenon is the result, at least in part, of an intensity difference between sound energy reaching the two cochleae. [source] Temporal Approach for Resection of Juvenile Nasopharyngeal Angiofibromas,THE LARYNGOSCOPE, Issue 8 2000J. Dale Browne MD Abstract Objective To describe a lateral preauricular temporal approach for resection of juvenile nasopharyngeal angiofibroma (JNA). Study Design A retrospective review of five patients with JNA tumors that were resected by a lateral preauricular temporal approach. Methods The medical records of five patients who underwent resection of JNA tumors via a lateral preauricular temporal approach were reviewed, and the following data collected: tumor extent, blood loss, hospital stay, and surgical complications. Results Five patients with JNA tumors had resection by a lateral preauricular temporal approach. These tumors ranged from relatively limited disease to more e-tensive intracranial, e-tradural tumors. Using the staging system advocated by Andrews et al., 1 these tumors included stages II, IIIa, and IIIb. Four patients (stages II, IIIa, IIIa, and IIIb) who underwent primary surgical excision had minimal blood losses and were discharged on the first or third postoperative day with minimal transient complications (mild trismus, frontal branch paresis, serous effusion, and cheek hypesthesia). The remaining patient (stage IIIb) did well after surgery, despite having undergone preoperative radiation therapy and sustaining a significant intraoperative blood loss. There have been no permanent complications or tumor recurrences. Conclusions A lateral preauricular temporal approach to the nasopharynx and infratemporal fossa provides effective exposure for resection of extradural JNA tumors. The advantages of this approach include a straightforward route to the site of origin, the absence of facial and palatal incisions, and avoidance of a permanent ipsilateral conductive hearing loss. [source] Genetic variants in RELN are associated with otosclerosis in a non-European population from TunisiaANNALS OF HUMAN GENETICS, Issue 5 2010Ayda Khalfallah Summary Otosclerosis is a common form of conductive hearing loss, caused by an abnormal bone remodelling in the otic capsule. Both environmental and genetic factors have been implicated in the etiology of this disease. A recent genome wide association study identified two regions associated with otosclerosis, one on chr7q22.1, located in the RELN gene, and one on chr11q13.1. A second study in four European populations has replicated the association of the RELN gene with otosclerosis. To investigate the association of these loci with otosclerosis in a non-European population, we tested 11 SNPs from the two regions in 149 unrelated Tunisian patients and 152 controls. Four SNPs were significantly associated with otosclerosis. Three SNPs are located in the RELN region and the last one is located in the region on chromosome 11. We also observed a significant interaction with gender for rs3914132. This suggests an influence of sex on the association of RELN with otosclerosis. A meta-analysis showed that the disease-associated alleles in the Tunisian sample are the same as in all previously reported associations. Our study provides additional evidence implicating RELN in the development of otosclerosis. Additional functional studies should determine the role of RELN in the physiopathology of this disease. [source] The use of the weber tuning fork test and ,scratch test' in postoperative tympanomastoid surgeryCLINICAL OTOLARYNGOLOGY, Issue 6 2006J.R. Buckland Objective., To compare the use of the Weber tuning fork test and ,Scratch test' in postoperative patients following tympanomastoid surgery. Method., Prospective cohort study with control group. Southampton University Hospitals NHS Trust. Secondary care. 19 patients undergoing routine tympanomastoid surgery for middle ear disease and 18 patients undergoing translabyrinthine surgery for lateral skull base lesions. Main Outcome Measures., Localisation of Weber tuning fork. Positive or negative Scratch test: clinician applies a gentle scratch using index finger to the bandage over the operated ear. Postoperative pure tone audiometry. Results., In the context of diagnosing a hearing or non-hearing ear, the Weber test had a specificity of 78% and sensitivity of 100%. The scratch test had a specificity of 100% and sensitivity of 100%. The Weber was 93% specific and 93% sensitive for diagnosing a unilateral conductive hearing loss. Conclusions., As a means of diagnosing an acute postoperative dead ear the Scratch test is an accurate alternative to the Weber test. References 1 Miltenburg D.M. (1994) The validity of tuning fork tests in diagnosing hearing loss. J Otolaryngol. 23, 254,259 2 Behn A., Laszlo C.A., Black D., et al. (2005) Which is mightier, the tuning fork or the bone oscillator? J Otolarygol. 34, 135,139 [source] | ||||||||||