Surgical aspects of paediatric cochlear implantation.
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Biomedical subjects
Publications and source records attributed to D Proops.
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AIMS: To estimate the prevalence of Usher syndrome in the city of Birmingham, and to establish a database of patients who have been classified into different clinical subtypes essential for future gene mutation analysis. METHODS: Symptomatic cases of Usher syndrome (US) resident in the city of Birmingham in June 1994 were ascertained through multiple sources. Ophthalmic and audiological reassessment together with examination of medical records and patient questionnaires allowed classification of three subtypes, US 1, US 2, and US 3. In addition, family pedigrees were examined and blood was taken from index patients for DNA extraction. RESULTS: In the population aged over 15 years the prevalence was 6.2 per 100 000 population for all US subtypes. The prevalence for US 1 and US 2 was 5.3 per 100 000 population. This is greater than previously reported. In the age group 30-49 years the prevalence approached 1 in 10 000. Clinical classification found 33% US 1, 47% US 2, and 20% US 3. CONCLUSION: This higher prevalence rate and greater frequency of US 2 and US 3 may reflect a more complete ascertainment.
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Screening and follow-up blood lead measurements in a 7-year-old child of a US Embassy official in Mexico City revealed an increase in blood lead concentration from 1.10 to 4.60 mumol/L in less than 4 weeks. The cause was traced to fruit punch contaminated with lead leached from traditional ceramic pottery urns. Consumption of the contaminated punch at a picnic was associated with a 20% increase in blood lead concentrations among embassy staff and dependants who were tested 6 weeks after the exposure. This episode highlights the continued health risk, even from brief exposure, posed by traditional pottery in Mexico.
Ewing's sarcoma arising in the head and neck region is very rare. A case arising from C2 in a young child is presented. The unusual presentation, differential diagnosis and pathological features are discussed.
Congenital deformities of the labyrinth of the inner ear can be associated with a fistulous communication between the intracranial subarachnoid space and the middle ear cavity. We describe seven such cases, six confirmed by high resolution CT and one by postmortem histological section. The seven patients all presented with meningitis although a cerebrospinal fluid fistula was demonstrated at subsequent surgery or postmortem. The lesions were bilateral in three patients, unilateral in three and probably bilateral in the postmortem case although only one temporal bone was obtained. In every case there was a dilated sac instead of the normal two and a half turn cochlea on the affected side and this was confirmed at surgery. The demonstration of the basal cochlear turn is of paramount importance in any deaf child presenting with meningitis. A true Mondini deformity with a normal basal turn and some hearing is not at risk of developing a fistula.
Benign tracheal tumours are uncommon in adults and rare in children. According to several extensive reviews, fibromas are the second most common benign tumour to occur in the trachea. (Gilbert et al., 1949, 1953). In our review of the literature, 197 adult cases of fibromas have been reported and 23 in children. However only one previous report of a congenital subglottic fibroma exists. (Tsui and Lore 1975). No previous case with a late onset vocal cord palsy has been reported.
The study of the topographical and three dimensional anatomy of the human external auditory canal and tympanic membrane has been simplified by applying a dental impression technique to the cadaver ear. A light silicone resin was catalyzed and syringed into the anterior recess and then into the entire external canal. Analysis of these impressions showed them to compare well with earlier morphological techniques, and to provide a durable and reliable replica of the surface of the tympanic membrane, the interior of the external auditory canal, and the lateral surface of the pinna. The resulting impressions displayed the anatomical features of the external auditory canal in a unique three-dimensional fashion which was also suitable for measurement of the anatomical features of the canal.
Twelve human laryngeal carcinomas and 14 normal vocal cord epithelia were studied in vitro by the method of tissue culture incorporating video time-lapse microscopy. Different events were photographed by taking 'stills' from the television monitor during play-back. Outgrowths from both tumour and normal explants formed only as monolayers. Mitoses were mainly found to be localized approximately mid-way between explant and the edge of the outgrowth. Some cells were seen to become suddenly active producing oscillating blebs and sometimes they detached themselves from the monolayer culture and changed appearance. This was more prevalent in the tumour cultures. In both tumour and normal cell cultures lymphocytes were observed and their normal chemokinetic movement was changed into a rapid chemotactic attraction by the presence of a mitotic epithelial cell. These lymphocytes appear to represent T-suppressor cells. The present study has shown that primary tissue culture can be a valuable tool in the study of laryngeal carcinoma. The interaction between mitotic tumour cells and lymphocytes may represent a transformation of T-lymphocytes into LAK cells.
The histopathologic findings in 8 temporal bones from 5 patients with osteogenesis imperfecta congenita are reported. The otic capsule, bony walls of middle ear, and ossicles showed evidence of both deficient and abnormal ossification. Microfractures were found in the otic capsule and in the anterior process and handle of the malleus, in addition to their common location at the crura of the stapes. The cochlear and vestibular end-organs appeared normal. Pathologic changes compatible with otosclerosis were not seen. The possible implication of these changes on hearing and balance is discussed.
Paget's disease of bone occurs more commonly in the elderly and has been reported to involve the temporal bone in 30% of those afflicted. The clinical and histopathological features of six temporal bones from three patients with this disease are reported and the relevant literature reviewed. The effect of Paget's disease on the middle ear structures was more variable than its extension into the otic capsule. Pagetic involvement of the otic capsule was observed in five temporal bones. One patient had bilateral asymptomatic neurofibromas in the eighth cranial nerve. the potential mechanisms responsible for the conductive deafness, the sensorineural deafness and vestibular dysfunction associated with Paget's disease are discussed.
The anterior process of the malleus has been recognized and its unique embryological development described for some time. However, most authorities, while describing it in the fetus and the child, claim that it atrophies with age and offer no theory as to its function. This study examined the horizontal serial sections of whole temporal bones of 50 ears, from donors of various ages from a 24 week fetus to extreme old age. Measurements indicate that, while the anterior process of the malleus seems to get smaller with increasing age, its anatomical consistency would indicate a physiological function. It is proposed that the anterior malleal process, in conjunction with the anterior malleal ligament, acts as the fulcrum of the axis of rotation of the malleus/incus complex and also acts as a damper to resonant oscillations of the complex at low frequencies.
OBJECTIVES: To test the hypothesis that noise exposure may cause hearing loss in classical musicians. DESIGN: Comparison of hearing levels between two risk groups identified during the study by measuring sound levels. SETTING: Symphony orchestra and occupational health department in the west Midlands. MAIN OUTCOME MEASURES: Hearing level as measured by clinical pure tone audiometry. RESULTS: Trumpet and piccolo players received a noise dose of 160% and 124%, respectively, over mean levels during part of the study. Comparison of the hearing levels of 18 woodwind and brass musicians with 18 string musicians matched for age and sex did not show a significant difference in hearing, the mean difference in the hearing levels at the high (2, 4, and 8 KHz) audiometric frequencies being 1.02 dB (95% confidence interval -2.39 to 4.43). CONCLUSIONS: This study showed that there is a potential for occupational hearing loss in classical orchestral musicians.