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The mean pure-tone air conduction (AC) and bone conduction (BC) hearing thresholds (HT) of 534 randomly selected, caucasian, white, urban children with normal otoscopy, otomicroscopy and impedance audiometry, i.e. normal middle ear function, are presented here. Children with pathological middle ear findings or abnormal impedance audiometry were excluded. The average age was 13.8 years, SD 0.5, at the date of examination. The mean air conduction thresholds varied between 0.6 dB at 1 kHz and 9.9 dB at 6 kHz, and the bone conduction thresholds varied between -1.1 dB at 0.5 kHz and 1.1 dB at 4 kHz. The pure-tone average (PTA) (the average of AC hearing thresholds of 0.5, 1 and 2 kHz) of all ears was 1.5 dB. Ninety to ninety-eight per cent of pure-tone AC hearing thresholds at frequencies of 0.5-4 kHz were between -5 dB and 10 dB. The distributions are presented and compared.

A total of 500 patients with cholesteatoma diagnosed and operated during 1982-91 in the region of Tampere University Hospital and Mikkeli Central Hospital in Finland were analysed retrospectively. The mean annual incidence was 9.2 per 100,000 inhabitants (range 3.7-13.9) and during the study period the annual incidence decreased significantly. The incidence was higher among males under the age of 50 years. There was no accumulation of cholesteatoma diseases in lower social groups. The majority (72.4%) of cholesteatoma patients had suffered from otitis media episodes. Tympanostomy was carried out in 10.2% and adenoidectomy or adenotonsillectomy in 15.9% of all cholesteatoma ears prior to cholesteatoma surgery. Cholesteatoma behind an intact tympanic membrane with no history of otitis media was verified in 0.6% of patients and in cleft palate patients in 8%. In this study, 13.2% of patients had ear trauma or ear operation in anamnes.

Lethal congenital contracture syndrome (LCCS) is an autosomal recessive disease leading to death before the 32d gestational week. It is characterized by the fetal akinesia phenotype, with highly focused degeneration of motoneurons in the spinal cord as the main neuropathological finding. We report here the assignment of the LCCS locus to a defined region of chromosome 9q34, between markers D9S1825 and D9S1830. The initial genome scan was performed with the DNA samples of only five affected individuals from two unrelated LCCS families. The conventional linkage analysis performed with 20 affected individuals and their families was focused on those chromosomal regions in which the affected siblings were identical by descent in the initial scan. One core haplotype of 3 cM was observed in LCCS alleles, supporting the assumption of one major mutation underlying LCCS, and linkage disequilibrium analysis restricted the critical chromosomal region to <100 kb in the vicinity of marker D9S61. Two genes, NGAL (neutrophil gelatinase-associated lipocalin and NOTCH 1, were excluded as causative genes for LCCS

In order to study the variation within and between endoscopy and otomicroscopy a Gage repeatability and reproducibility design was created, with which the middle ears were studied of eight cadaver temporal bone blocks through the ear canal three times in random order using both methods. A Zeiss OPMT-1 operating microscope and Olympus endoscopes were used. The data were analysed in accordance with the analysis of variance principle, where the total variation was divided into different components. The anatomical areas were counted and registered via quadrants. There was a distinct difference between the results of the two methods in favour of the endoscopes. Furthermore, the variation between the methods and between the trials was analogous.

Cefetamet, an oral 3rd-generation cephalosporin, was investigated in 40 children with acute otitis media in a comparative randomized trial. The efficacy of 20 mg/kg cefetamet syrup in 20 patients was compared with that of 20 mg/kg cefaclor in another 20, both drugs being given orally twice daily for 7 days. Tympanocentesis was performed for every child before the initiation of antimicrobial treatment. After 7 days treatment with cefetamet pivoxil, clinical cure was obtained in 12 patients, 3 were failures and 5 could not be assessed. In the cefaclor group, 10 patients were cured, 1 improved and 9 were failures. No severe adverse events were observed with either drug.

A total of 3,340 infants, 95 per cent of them 7 to 9 months old, were randomly vaccinated in a double-blind fashion with either the 14-valent pneumococcal (Pn) polysaccharide vaccine or a saline placebo in three urban areas in Finland. The second dose of the vaccine was given 5 months later. Age and sex distribution, recruitment of infants, and their otitis-related treatment and follow-up were similar in the study areas. Side effects after vaccination were mild and fewer than among older children. Antibody responses to vaccine polysaccharides varied from type to type, but were generally poor, especially to types most prevalent in otitis media. After the first dose of vaccine, the occurrence of otitis visits among the Pn-vaccinated, as compared with controls, showed inter-area differences, but ranged from not more than a 30 per cent reduction at its best to an increase in some areas and in some clinical categories. The respective figures for children with acute otitis media were similar between the vaccination groups and the study areas. The effect of the vaccine on acute otitis media caused by specific Pn types/groups represented in the vaccine was variable but generally poor. Group 6 attacks especially seemed to behave problematically. The second dose of the vaccine did not give additional benefit serologically or clinically. The efficacy of currently available pneumococcal vaccine against otitis media seemed poor in infants.

The occurrence of acute otitis media was studied over a one-year period in a total population of 146822 persons living in different parts of Finland. Of these 146822 persons, 4583 experienced a total of 6518 otitis attacks giving an annual incidence rate of 4.44% (4.48% in males and 4.07% in females, P less than 0.001). Acute otitis media was strikingly concentrated in the youngest age groups, with the highest annual incidence rates, in the order of 50%, found in infants younger than 2 years. Half of all the episodes occurred before the age of 33 months. The relative risk of getting acute otitis media was about 200 times higher during the first 2 years of life than as an adult. The occurrence of otitic episodes followed seasonal variations, and in urban areas the incidence was significantly (P less than 0.001) higher than in the countryside.

The prerequisites for preventing a considerable proportion of acute otitis media (AOME) of children are theoretically good--pneumococcal types/groups present in the available 14-valent pneumococcal capsular polysaccharide vaccine are the most common bacteria in AOME, and a good serum antibody response is followed by type-specific protection from infection. However, the four most common types/groups (= 2/3 of all pneumococci in AOME) have very poor immunizing capacity in infants, and therefore the vaccine offers little protection from AOME for children under the age of 2 years. This information should help towards an improved new vaccine for AOME.

Serum C-reactive protein (CRP) levels were studied in 79 children with acute otitis media (AOM), aged from 4 months to 5 years. The CRP was less than 10 mg/l in 27 children, greater than or equal to 20 mg/l in 34, and greater than or equal to 40 mg/l in 17 children, 25 of the 41 attacks caused by S. pneumoniae or H. influenzae showed a CRP of greater than or equal to 20 mg/l and 15 CRP greater than or equal to 40 mg/l, in 38 cases without major otitis pathogens, the respective figures were 9 (p less than 0.01) and 2 (p less than 0.001). Although statistically significant correlations between otitis-related clinical parameters and CRP levels were rare, there was a tendency toward higher CRP values among those with a more severe clinical picture. All five attacks with CRP greater than or equal to 100 mg/l were bilateral, caused by major pathogens, and preceded by a respiratory infection. They also tended to have high fever and a large amount of fluid in myringotomy. However, even in these the general course of AOM and other morbidity was not different from the others.

We studied the occurrence of Haemophilus influenzae (Hi) in 2625 middle ear fluid (MEF) specimens obtained from 523 children with otitis media, aged 3 months to 6 years, between October 1977 and May 1979. In clinically acute cases 12.3% of the MEFs grew Hi; 6.3% (13/206) of the strains were of type b and 4.9% (10/206) were beta-lactamase producing. In the very first acute case of a child 8.0% (21/263) of the MEFs grew Hi, but in acute recurrences it was cultured in 17.0% (184/1082) (p less than 0.001). In non-acute persistent MEFs obtained during control visits Hi was found in no less than 21.7% (198/913); as many as 12.6% (25/198) of them were beta-lactamase producing. Both of these figures, but not the proportion of type b strains (8.6%), were significantly (p less than 0.001 and p less than 0.01, respectively) greater than in clinically acute MEFs. Hi was grown in 13.1% of the acute MEFs taken from children less than 4 years old, but in only 8.6% of the older children (p less than 0.05). The annual survey of all the 1816 Hi strains isolated from the MEFs of acute or subacute cases of otitis media in one laboratory in 1976-81 showed the proportion of beta-lactamase producing strains of all Hi strains to be gradually increasing from 8.0% (17/212) in 1976 to 15.2% (16/105) in 1981 (p less than 0.05).

To determine the value of different pneumotoscopic findings in diagnosing the middle ear effusion (MEE) of acute (AOM) and non-acute otitis media, 11,804 ear-related visits of 2,911 unselected children at ages 0.5-2.5 years were analysed. About half of these were examined by an otolaryngologist in one, and half by a pediatrician in another, urban area. Myringotomy was always performed when MEE was suspected, and it confirmed the presence of MEE in 85% (otolaryngologist) and 82% (pediatrician) of altogether 5,462 acute and in 69% (both doctors) of 1,092 non-acute cases suspected. Redness of the tympanic membrane (TM) was found in only 18% and 27% of the visits with AOM, and it predicted MEE with only 60% and 51% probability, if seen in acute visits. Cloudiness of the TM was noticed in 81% and 67% of the visits with AOM; its specificity and the other calculated variables were good in regard to the diagnosing of MEE, especially in acute cases in both groups. Distinctly impaired mobility of the TM was of about the same diagnostic value, but its position reliably indicated MEE only when bulging. In AOM the colour or mobility of the TM was normal very rarely, but the position was normal in a third of the cases. Thus, although there were differences in the incidences of different otoscopic findings in the two study groups, the diagnostic value of certain pneumatic otoscopic findings, especially cloudiness and distinct hypomobility of the TM, seemed to be good in both groups.

The middle ear fluid (MEF) bacteriology of 107 attacks of acute otitis media (AOM) in 101 infants less than 3 months old was analyzed. A total of 108 bacteria were isolated from 85 attacks. Major AOM-pathogens, S. pneumoniae (19%), H. influenzae (9%) or B. catarrhalis (7%) were cultured in approximately one-third of all the attacks. S. aureus (17%) and coagulase-negative staphylococci (22%) without the above pathogens were commonly found, whereas gram-negative enteric bacteria were culturable from only 5 attacks. Only 8% of the MEFs were polymicrobial. More than half of all the bacterial strains produced beta-lactamase. The bacteriology of those younger than one month was not different from that of the others. The same was true with attacks of out-patients and in-patients, except for a larger proportion of beta-lactamase producing strains in in-patients. Nasal-nasopharyngeal and MEF samples showed the same bacteriology in only 20% of cases. Two-thirds of AOM attacks were present in infants with perinatal or other concomitant morbidity, but their bacteriology was not different from those without other morbidity. In addition to the examination of ears in infants presenting with any illness before the age of 3 months, the study stresses the importance of bacteriological analysis of MEF in all cases of AOM at this age.