1/247. Multisystem neuronal involvement and sicca complex: broadening the spectrum of complications.We report two patients with multisystem neuronal involvement associated with sicca complex. One had a lower motor neuron syndrome combined with a flaccid bladder and rectum. The second patient had unilateral hearing loss, sensory neuronopathy, Adie's pupils, upper motor neuron signs, and autopsy-proven anterior horn cell degeneration. Our cases lead us to propose that the spectrum of neuronal involvement occurring with sicca syndrome may be wider than is currently appreciated.- - - - - - - - - - ranking = 1keywords = hearing, hearing loss (Clic here for more details about this article) |
2/247. suggestion audiometry for non-organic hearing loss (pseudohypoacusis) in children.Pertaining to non-organic hearing loss in children, three goals should be attained: detection of this disease, determination of true hearing levels and information about the possible cause. Recently, objective tests have been used principally for children with non-organic hearing loss; however, these lack the simplicity and convenience of traditional audiometry. A new method, which is referred to as suggestion audiometry, since it is suggested to the patient that hearing will be improved as a result of the test procedure, was developed for the purpose of simultaneously achieving the above-stated three goals. The subjects were 20 patients aged 8-16 years suspected of demonstrating non-organic hearing loss and whose apparent hearing loss had been identified by school hearing examinations. suggestion pure tone audiometry was useful for the detection of non-organic hearing loss and suggestion speech audiometry was valuable for the determination of true hearing levels. The subjects were classified into four groups according to the test results. We discuss causes of the disease based on the classification of the subjects obtained from this test procedure.- - - - - - - - - - ranking = 12.303321390158keywords = hearing, hearing loss (Clic here for more details about this article) |
3/247. Use of patient-specific estimates in patient evaluation and rehabilitation.For a number of years we have been using open-ended questionnaires in the assessment of hearing disability and handicap, tinnitus complaint behaviour together with shortcomings and benefits of various rehabilitative interventions. More recently we have applied this approach to positive experiences reported by adults who have developed a hearing impairment and to the use of silence or social withdrawal as a communication tactic. In addition, we have asked individuals to rate the magnitude of each of their listed disabilities, handicaps, shortcomings, etc., on a scale to provide a qualitative assessment of each of these elements important to the individual. Such a procedure can be repeated after the intervention to measure changes in such estimates. This approach can thus highlight for the therapist, those elements of the patients' problems which are being well or poorly addressed and hence help them to concentrate their effort on the latter. Results obtained using this technique are presented and discussed.- - - - - - - - - - ranking = 1.6516606950789keywords = hearing (Clic here for more details about this article) |
4/247. Congenital malformation of the inner ear associated with recurrent meningitis.Congenital deformities of the labyrinth of the inner ear can be associated with meningitis and varying degrees of hearing loss or deafness. A recurrence of meningitis is due to the development of a fistulous communication between the subarachnoid space and the middle ear cavity, and can prove lethal. An illustrative case of a 4-year-old Japanese girl with bilateral severe hearing loss, recurrent meningitis and malformations of the inner ear and stapes footplate is presented. Removal of the stapes during tympanotomy provoked a gush of cerebrospinal fluid. The defect was repaired successfully, and there has been no further episodes of meningitis to date.- - - - - - - - - - ranking = 2keywords = hearing, hearing loss (Clic here for more details about this article) |
5/247. Clinical findings for a group of infants and young children with auditory neuropathy.OBJECTIVE: To examine the prevalence of auditory neuropathy in a group of infants at risk for hearing impairment and to present an overview of the clinical findings for affected children. DESIGN: Results for 20 subjects who showed repeatable cochlear microphonic potentials in the absence of click-evoked auditory brain stem responses are included in this study. Behavioral and steady state evoked potential thresholds were established in each case. Where possible, otoacoustic emission and speech perception results (unaided and aided) also were obtained. RESULTS: One in 433 (0.23%) of the children in our series had evidence of auditory neuropathy. The audiometric findings for these subjects varied significantly, with behavioral thresholds ranging from normal to profound levels. Discrimination skills were also variable. Approximately half of the subjects showed little understanding, or even awareness, of speech inputs in both the unaided and aided conditions. There were, however, a number of children who could score at significant levels on speech discrimination tasks and who benefited from the provision of amplification. CONCLUSION: The results suggest that auditory neuropathy is more common in the infant population than previously suspected. The effects of neuropathy on auditory function appear to be idiosyncratic, producing significant variations in both the detection and discrimination of auditory signals. As such, the management of children with this disorder must allow for individual differences.- - - - - - - - - - ranking = 0.82583034753944keywords = hearing (Clic here for more details about this article) |
6/247. Atypical Wegener's granulomatosis with positive cytoplasmic antineutrophil cytoplasmic antibodies, ophthalmologic manifestations, and slowly progressive renal failure without respiratory tract involvement.A 68-year-old woman had microscopic hematuria and proteinuria since the age of 50. She also had hearing impairment, arthralgia, retinal embolism, peripheral arterial occlusion of the right foot and chronic renal failure during the course. At the age of 68, she had progressive renal failure and nephrotic syndrome with high titers of serum cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA). No evidence of respiratory tract involvement was found. methylprednisolone pulse therapy and low dose cyclophosphamide therapy ameliorated the renal failure and reduced the serum c-ANCA level. She, however, died on July 19, 1998 due to pulmonary fungal and pneumocystis carinii infection.- - - - - - - - - - ranking = 0.82583034753944keywords = hearing (Clic here for more details about this article) |
7/247. Outcome of a baby born from a mother with acquired juvenile hypothyroidism having undetectable thyroid hormone concentrations.We report a baby born from a mother with strongly positive thyroid stimulation blocking antibody (TSBAB) and nearly undetectable T4 level. This case is a unique model of nearly complete absence of thyroid hormones during fetal and early neonatal life in humans. The infant girl was born by cesarean section, because of fetal bradycardia, after 41 weeks gestation and received mechanical ventilation for 3 days. The TSH level was more than 120 microU/mL in the neonatal thyroid screening. At age 17 days, the results of a thyroid function study showed undetectable free T3 and free T4 concentrations, TSH 550 microU/mL, and TSH receptor antibody (TRAB) 87%. thyroxine at a dose of 30 microg/day was started at age 17 days. The patient required thyroxine treatment until age 8 months. The brain magnetic resonance image at age 2 months revealed reduced brain size. Her auditory brain stem response was absent at age 2 months. The audiogram at age 4 yr revealed sensorineural deafness of 70 dB. When she was 6 yr of age, motor development remained the same as that at age 4 months. Her height was 106 cm (- 1.5 SD). The results of thyroid function study of the mother 23 days after delivery showed undetectable free T3 and free T4, TRAB 84%, and TSBAB 83%. In conclusion, the outcome of severe thyroid hormone deficiency in utero and early in human neonatal life was normal physical growth, fetal distress resulting in cesarean section, difficulty in the onset of breathing, permanent deficit in auditory function, brain atrophy, and severely impaired neuromotor development despite the start of an adequate dose of thyroxine replacement during the neonatal period.- - - - - - - - - - ranking = 0.031581328457299keywords = sensorineural (Clic here for more details about this article) |
8/247. hearing loss and automobile airbag deployments.In an on-going research program, on the causation of injuries in motor vehicle accidents, at the University of michigan transportation research Institute, crashes with airbags have been, and are continuing to be, investigated. In order to determine the occurrence, if any, of 'hearing problems' associated with airbag deployments, drivers and passengers who had been involved in 'airbag' automobile crashes were interviewed by telephone. From the crashes investigated, 225 attempts were made to contact drivers who were exposed to airbag deployments. From these telephone interviews, contacts were made with 177 car occupants. Only three reported that they had experienced any type of hearing-related problems subsequent to their crash. One other case is reported of a driver who had pre-crash hearing loss. It appears that permanent hearing deficit due to airbag deployment, both in cars with the steering wheel airbag alone, and in those with a passenger side airbag, is an infrequent event (1.7%) from the data of this study.- - - - - - - - - - ranking = 3.4774910426183keywords = hearing, hearing loss (Clic here for more details about this article) |
9/247. noise analysis of real-life listening situations for maximal speech audibility in hearing aid fitting.This study attempts to prescribe hearing aid characteristics for one listening environment selected by an individual hearing aid user in order to provide better speech intelligibility than standard insertion gain prescriptions. The procedure is based on acoustic analyses of noise recorded by the user. The main purpose is to show the feasibility of the measurement and analysis procedure as a tool to investigate the consequences of the noise characteristics of everyday hearing aid use. The speech intelligibility Index (SII) is used as a criterion to optimize the insertion gain frequency response of the hearing aid. The results indicate that realistic background noise information is essential to achieve the best possible speech audibility. Maximizing the SII required higher insertion gain above 5000 Hz than assumed in conventional fitting procedures. Different insertion gain frequency responses are required for different listening conditions. The measurement procedure could identify acoustic listening conditions where hearing instruments would probably give no benefit.- - - - - - - - - - ranking = 7.4324731278549keywords = hearing (Clic here for more details about this article) |
10/247. Familial paragangliomas: the emerging impact of molecular genetics on evaluation and management.HYPOTHESIS: Advancements in molecular genetics has direct impact on the evaluation and management of patients and family members with familial paragangliomas (FP). BACKGROUND: Familial paragangliomas. in contrast to sporadic cases, are commonly multiple, bilateral, and present at an earlier age. Familial tumors are inherited in an autosomal dominant pattern with genomic imprinting of the paternal allele. Mapping studies have identified regions on chromosome 11q as harboring the genetic defect responsible for paraganglioma formation. methods: A multigenerational family with five affected females with head and neck paragangliomas underwent clinical and genetic evaluation. Genetic mapping was performed with microsatellite markers froin chromosome 11q13 and q23. Nonaffected individuals were screened for carrying the affected haplotype. In addition, by using dna obtained from an amniotic fluid sample. in utero screening of a fetus was performed. RESULTS: The most common complaints were hearing loss and neck masses that usually manifested by age 25. Genetic mapping identified loci 11q13 and q23 as sites likely responsible for tumorogenesis. Three unaffected family members, including a fetus, were identified as carriers of the affected haplotype. The genetic findings were used to design a screening protocol for family members at risk for developing glomus tumors. CONCLUSIONS: Genetic screening of unaffected family members can identify individuals harboring the mutated allele. Identification of family members at risk for developing FP by molecular genetic techniques may lead to early detection of head and neck paragangliomas and may directly impact morbidity from glomus tumors and their treatment.- - - - - - - - - - ranking = 1keywords = hearing, hearing loss (Clic here for more details about this article) |
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