BOR and BO syndromes are allelic defects of EYA1.

Branchio-oto-renal (BOR) syndrome is an autosomal dominant disease characterized by varying combinations of branchial, otic and renal anomalies. By positional cloning, a candidate gene, EYA1, homologous to the drosophila eyes absent gene, has recently been identified at 8q13.3 and shown to underlie this syndrome. The name branchio-oto (BO) syndrome has been used to describe a similar combination of branchial and otic anomalies, without the association of renal anomalies. Whether BOR and BO syndromes involve the same gene was unknown. To address this question, we analyzed two large independent families for which each of the 8 affected members present exclusively with BO syndrome. In both families, linkage analysis mapped the causative gene to the same chromosomal region as EYA1. A search for mutations in 9 of the EYA1 coding exons identified a 2-bp insertion segregating in one family and an 8-bp deletion segregating in the other. These results demonstrate that EYA1 also underlies BO syndrome, and that BOR and BO syndromes are allelic defects of this gene.

Endemicity, molecular diversity and colonisation routes of Pseudomonas aeruginosa in intensive care units.

OBJECTIVE: We carried out a prospective study to evaluate the endemicity of Pseudomonas aeruginosa in intensive care units (ICUs). Pulsed-field gel electrophoresis (PFGE) was used to determine the genotypes of P. aeruginosa isolates. This allowed us to determine the importance of cross-colonisation and the colonisation routes of P. aeruginosa. DESIGN: We screened epidemiological specimens (rectal swab, nose swab and tracheal aspiration) and routine clinical cultures from patients admitted to ICUs during a 2-year period, from 1st January, 1998, to 31st December, 1999. SETTING: The study was carried out in four separate adult ICUs located in the Franche-Comté region of France. These four units admitted a total of 1,500 patients per year. RESULTS: A total of 1686 specimens were collected from 473 patients; 122 of these patients were positive on admission, 351 became positive during hospitalisation. The overall incidence of P. aeruginosa was 15.7 cases per 100 patients and 15.1 cases per 1000 days of hospitalisation. Of 184 patients with at least one ICU-acquired positive clinical culture, 104 had been previously identified as carriers by a similar genotype. Typing of 208 non-replicate isolates revealed 101 major DNA patterns. Approximately 50% of P. aeruginosa carriage or colonisation/infection was acquired via cross-transmission; the other cases probably originated from endogenous sources. CONCLUSION: Cross-colonisation seems to play an important role in the general spread of P. aeruginosa in ICUs.

Myocardial protection through cold cardioplegia using diltiazem, a calcium channel blocker.

During two hours of aortic clamping, two groups of 10 dogs each were given an intermittent infusion of a cold solution in the aortic root. In one group, the solution contained 20 mEq per liter of potassium chloride (KCl) and in the other, a calcium channel blocker (diltiazem). Left ventricular (LV) performance was measured by calculation of LV pressure, left ventricular end-diastolic pressure (LVEDP), cardiac index (CI), and stroke-work index (SWI). Regional myocardial function was assessed through ultrasonic crystals implanted in the subendocardial areas of both the left anterior descending (LAD) and circumflex coronary arteries. Dogs receiving KCl displayed deterioration of LV performance as evidenced by a return of maximal LV pressure, maximal rate of rise of LV pressure (dP/dtmax), CI, and SWI to 74 +/- 4%, 87 +/- 5%, 74 +/- 6%, and 59 +/- 6%, respectively, of the initial (before clamping) values. Animals that received diltiazem, on the other hand, had for the same variables a return to 85 +/- 4%, 99 +/- 7%, 129 +/- 8%, and 111 +/- 10% of the initial values. The rate of relaxation (peak negative dP/dt) decreased in both groups but less in dogs receiving diltiazem. Regional function in the area of the LAD and circumflex arteries showed little change in either group. We conclude that cold cardioplegia with a solution containing KCl or diltiazem protects the myocardium during prolonged ischemic cardiac arrest. Return of LV function on the whole is superior when diltiazem is used.

Early detection of hearing loss in children with cleft palates by brain-stem auditory response.

The hearing of 23 children with cleft palates was studied at an early age (before 12 months) and before pharyngoplasty using the electrophysiologic method of auditory brain-stem response. Nineteen children showed important degrees of conduction deafness of 50 or 60 dB. Results were compared with those obtained for a group of normal children tested under the same method before age 6 months. No hearing loss was observed in this group. It is concluded that hearing defects appear at an early age in children with cleft palates and are in direct relationship to the malformation. Hypotheses concerning the etiopathogeny of these hearing losses are proposed.

Can the mode of death be predicted in patients with angiographically documented coronary artery disease?

To determine whether sudden versus non-sudden cardiac death could be predicted in high risk patients, 1157 medical patients were followed for an average of 46 months after a diagnostic coronary angiogram and 18 clinical, hemodynamic, and angiographic variables known to be associated with a high risk of mortality were analyzed. The total group of 141 deaths was classified into 3 subgroups: (1) 82 sudden deaths (less than 1 hour after onset of symptoms); (2) 46 deaths due to acute myocardial infarction with or without heart failure, and (3) 13 deaths unrelated to cardiac symptoms. In a subset of 64 patients, the duration of electrical systole (QTc) was calculated before angiography and before death. A comparison was made of QTc measurements at entry with QTc values of subjects with normal coronary arteries and normal left ventricular function. Deaths from cardiac causes could often be predicted from older age, male sex, history of myocardial infarction, unstable angina, congestive heart failure, abnormal cardiothoracic ratio, multivessel disease, abnormal left ventricular contraction, and abnormal ejection fraction. However, these variables did not discriminate between sudden and nonsudden cardiac deaths and both modes of death were characterized by depressed left ventricular function and multivessel coronary disease. During follow-up the incidence of acute myocardial infarction was not different in patients with cardiac and noncardiac deaths and in long-term survivors. However, patients dying from cardiac causes had a higher incidence of heart failure. Patients dying suddenly did not present new infarctions during follow-up whereas patients dying from acute myocardial infarction had a 13% incidence of prior infarction and a higher incidence of heart failure. In addition, QTc at entry was longer in nonsurvivors than in normal subjects (p less than 0.0001) and patients experiencing sudden death exhibited the highest incidence of QTc prolongation (greater than or equal to 440 ms) during follow-up (p less than 0.05). We conclude that: (1) although the severity of coronary disease and left ventricular dysfunction are closely related to cardiac mortality, they do not discriminate between sudden and nonsudden cardiac deaths; (2) patients experiencing sudden death are characterized by a low incidence of new myocardial infarction or congestive heart failure and prolongation of the QTc interval during follow-up.

Assessment of coronary artery disease by thallium scan in type-1 diabetic uremic patients awaiting combined pancreas and renal transplantation.

Twenty-five type-1 diabetic uremic patients (14 men, 11 women, mean age forty +/- eleven years, range, nineteen to sixty) were prospectively analyzed for coronary artery disease (CAD) by thallium scan (TLS) before synchronus pancreas and kidney transplantation. Duration of diabetes ranged from ten to thirty-two years (mean twenty-two +/- five). Fifteen patients (60%) were in dialysis from two to sixty months (mean twenty +/- eighteen). Advanced diabetic degenerative complications were present in all patients. Twenty exercise and five pharmacologic thallium scans were performed. Forty-four percent of patients (6 men, 5 women, mean age forty-two +/- eleven years, range, twenty-six to sixty) had a positive (reversible or permanent defects) TLS. In 2/11 cases, severe CAD required further cardiac investigations for therapeutic management decision. A statistical correlation with resting ECG nonspecific ST-segment and T-wave abnormalities was observed (p less than 0.05) despite the absence of angor in 8 of the 11 patients. On the contrary, no statistical correlations were found regarding high blood pressure, smoking, hypercholesterolemia, duration of diabetes, and duration of dialysis. In this particular diabetic population (young age, male/female ratio = 1.2) with high incidence of silent ischemia, resting ECG repolarization abnormalities, though predictive, were not specific or prognostic of CAD severity; in these cases, exercise and pharmacologic TLS, a noninvasive and sensitive cardiovascular test, may be of great interest in diagnosis of CAD, allowing adequate cardiac management before, during, and after pancreas transplantation.

[Myocardial contusions. Contribution of scintigraphy using thallium-201. A prospective series]. / Contusions myocardiques. Apport de la scintigraphie au thallium 201. Série prospective.

Traumatic myocardial contusion is observed in 15% of autopsy studies; however, it is much more difficult to detect in survivors. Thirty-two consecutive, unselected patients admitted to the intensive care unit for serious thoracic injuries, underwent Th 201 myocardial scintigraphy in 2 to 4 different projections, 2 to 13 days after admission. The results were interpreted double blind and considered positive when a zone of hypofixation creating a defect greater than 25% was observed. The results were correlated with daily ECG recordings, serum CPK MB levels and echocardiography. Thirteen patients (41%) had abnormal scintigraphy. Border line appearances in 1 case excluded any severe myocardial lesions. The other investigations (56%) were normal. These results did not correlate with the ECG or echocardiographic appearances but a significant relationship was found with serum CPK MB levels (p less than 0.05). Therefore, the first investigations did detect cardiac damage but were not specific for myocardial lesions. Thallium 201 myocardial scintigraphy is superior to other non-invasive investigations. It provides information as to the size of the lesion and, by repeated studies, can differentiate simple myocardial contusion from a true traumatic myocardial infarction.

[Complications of myocardial infarction between the 2d and 4th week]. / Complications de l'infarctus du myocarde de la 2e à la 4e semaine.

The complications of myocardial infarction after transfer from the Coronary Care Unit on the 6th day were analysed bu a retrospective study of 3,460 computerised case reports (1973-1980). The mortality rate was 6% (1/3 of hospital deaths) in the period from the 7th day to discharge from hospital (14th to 30th day). Cardiac arrest as not uncommon (20% of all cardiac arrests) but the prognosis was better thn during the initial phase (p less than 0.05) as the mechanism was more commonly ventricular fibrillation or tachycardia (p less than 0.05). This series was compared with a similar population from 1970-1973; an improvement was observed in global hospital mortality (27% previously compared to 17%, in the study series, p less than 0.001). As the population were comparable, this phenomenon seems to be related to better treatment of shock and cardiac failure and the advances in cardiac surgery during the initial phase of infarction. Thd commonest mechanical complication was ventricular aneurysm; its occurrence does not influence the vital prognosis during this period. The incidence of cardiac arrest and death due to cardiac failure is not negligible after the first week of myocardial infarction. Therefore, we do not believe that the hospital period should be reduced after myocardial infarction. Special training of the nursing staff is essential for the successful treatment of these complications. The global prognosis could be improved by the rehabilitation of digitalis therapy and the introduction of new sympathomimetic amines in the acute phase of myocardial infarction.

[Gamma-angiography in the diagnosis of akinesia and dyskinesia after myocardial infarction]. / Apport de la gamma-angiographie au diagnostic des akinésies et des dyskinésies après infarctus du myocarde.

A one year prospective double blind trial included all patients with myocardial infarction and clinical, electrocardiographic or radiographic signs of left ventricular aneurysm. All 36 patients underwent ventriculography and radionuclide angiocardiography in the same projections: right anterior oblique, antero-posterior, left anterior oblique and left lateral. The angiographic diagnosis of left ventricular aneurysm was based on the finding of a deformation of the ventricular contour persisting in diastole; 22 patients were classified as having a left ventricular aneurysm and the 14 others had akinesia alone. The radionuclide diagnosis of left ventricular dyskinesia was based on the finding of the following 3 criteria in at least one projection: crossing of the systolic and diastolic isocontours; over 4 p. 100 of LV pixels having a negative ejection fraction; the dephased infarcted region having a movement separate from that of the remaining healthy myocardium. None of the clinical criteria of inclusion allowed diagnosis of LV aneurysm when compared with the results of ventriculography. The results of radionuclide and conventional ventriculography correlated 100 p. 100 in the diagnosis of severe contractile abnormalities. When compared with ventriculography, radionuclide angiocardiography had a specificity of 95 p. 100 and a sensitivity of 86 p. 100 for the diagnosis of aneurysm. The only false negative was a non-surgical septal aneurysm. The multiplication of the incidences of examination increases the sensitivity of the results of radionuclide angiography.

[Comparison of magnetic resonance imaging, echocardiography and catheterization in the diagnosis of congenital heart diseases]. / Evaluation comparée de l'IRM, de l'échocardiographie et du cathétérisme pour le diagnostic des cardiopathies congénitales.

The authors compared the diagnostic value of magnetic resonance imaging (MRI), echocardiography and cardiac catheterisation with angiography in 66 patients with congenital heart disease, to determine a diagnostic strategy in the use of these methods of cardiac imaging. The patients were 8 days to 44 years old. The congenital cardiac malformations were classified in three groups: 29 isolated vascular malformations (Group 1), 17 isolated intracardiac malformations (Group 2) and 20 complex malformations (Group 3). MRI was performed in all patients using a high field (1.5 tesla) magnet and spin-echo sequences in multiple incidences. The results were compared with those of echocardiography in 60 patients and/or cardiac catheterisation in 39 cases. Technical evaluation of MRI showed images of diagnostic quality in 62/66 cases (93.9%). MRI provided a diagnostic contribution in 56 cases (85%) which was less important in intracardiac malformations than in the other groups (p less than 0.05). In comparison with other imaging techniques, globally, the diagnostic value of MRI was lower than that of cardiac catheterisation (p less than 0.005) but there was no significant difference between MRI and echocardiography. When the type of malformation was taken into account, MRI was not as useful as catheterisation and echocardiography for the diagnosis of isolated intracardiac malformations (p less than 0.01) but gave comparable results in other malformations. On the other hand, MRI associated with echocardiography was more useful (p less than 0.05) than catheterisation in the diagnosis of complex congenital lesions.(ABSTRACT TRUNCATED AT 250 WORDS)

[Recurrent angina after coronary surgery. Results of reoperations for myocardial revascularization]. / L'angor récidivant après chirurgie coronarienne. Résultats des réinterventions de revascularisation myocardique.

40 patients presented with invalidating angina after initial revascularisation surgery (44 operations). The relapse of angina was attributed to thrombosis or stenosis of the graft (33 p. 100), graft failure plus the presence of non-bypassed coronary lesions (progression of coronary artery disease or incomplete initial revascularisation) (52 p. 100) or to the presence alone of coronary lesions which had not been bypassed (15 p. 100). At reoperation 37 stenosed or thrombosed grafts were replaced or repaired and 36 new aortocoronary bypasses were implanted. The perioperative risk was comparable to that of the initial operation (4,5 p. 100 mortality, 13,6 p. 100 myocardial infarction). The long term clinical improvement (34 months) was however less satisfactory (30 p. 100 unimproved). Several factors may account for the relatively high failure rate: the incidence of incomplete revascularisation; perioperative myocardial infarction and, above all, rapid progression of the coronary artery disease. Reoperation for myocardial revascularisation should therefore be restricted to patients with severe angina despite medical therapy, in whom the angiographic appearances suggest that optimal revascularisation might be possible.

[Prognostic value after 1 year of post-infarction exercise test with measurement of oxygen consumption]. / Valeur pronostique à un an du test d'effort postinfarctus avec mesure de la consommation d'oxygène.

A symptoms-limited exercise stress test with measurement of myocardial oxygen consumption (VO2) was carried out in 56 patients on the 44th +/- 16 days after infarction and in 48 patients on the 119th +/- 31 days. Analysis of the expired gases was performed by mass spectrography, cycle by cycle. The second test was coupled with an exercise gamma-angiography in 40 cases. The parameters on exercise were analysed in two groups at one year: group 1 asymptomatic and group 2 symptomatic (subgroup 2a with angina but no dyspnoea, subgroup 2b with dyspnoea). At the second test, the peak VO2 was lower in group 2 (19.46 +/- 5.78 ml/mn/kg) than in group 1 (24.2 +/- 6.5 ml/mn/kg) (p less than 0.008) irrespective of the symptom (angina and/or dyspnoea). The oxygen pulse was lower in group 2a with angina at one year (42.4% +/- 14.3%) compared with asymptomatic group 1 patient (58.5 +/- 12.4%) (p = 0.03). The 3 parameters: VO2, blood pressure and ejection fraction on exercise were independent. The lack of physical fitness may partially explain the absence of variation of the peak VO2 at the first test. These preliminary results should be confirmed by a multivariate analysis in a larger patient group.

[Thallium 201 myocardial scintigraphy and left branch block. Comparative value of exercise test and dipyridamole test]. / Scintigraphie myocardique au thallium 201 et bloc de branche gauche. Intérêt comparé de l'épreuve d'effort et de l'épreuve au dipyridamole.

Stress Thallium 201 myocardial scintigraphy in patients with left bundle branch block often shows reversible septal perfusion defects even in the absence of coronary artery disease. This phenomenon seems more common when the patients have tachycardia. With the working hypothesis that dipyridamole stress testing, which does not greatly increase the heart rate, would be more appropriate than exercise stress testing to unmask coronary artery disease in this condition, the authors compared the results of two Thallium 201 scintigraphies performed after exercise and then after dipyridamole under the same conditions three weeks later, in 67 patients with complete left bundle branch block. Scintigraphy showed one or more reversible perfusion defects in 64/67 patients after exercise but only 32/67 patients after dipyridamole (p < 0.001). There was poor uptake in the septal region in 59 patients (88%) after exercise and in 25 patients (37%) after dipyridamole (p < 0.001). The specificity was evaluated in 23 patients estimated to have no coronary artery disease. If only unequi vocal perfusion defects were considered, the specificity after dipyridamole was higher than that after exercise, increasing from: 35% to 83% for septal defects (p < 0.01); 65% to 96% for anterior wall defects (p < 0.05); 61% to 87% for inferior wall defects (p < 0.05); 57% to 91% for apical defects (p < 0.01); 17% to 83% overall (p < 0.001). Lower values but with a comparable difference were observed when all forms of hypofixation (even minimal) were taken into account.(ABSTRACT TRUNCATED AT 250 WORDS)

[Detection of residual myocardial ischemia by Thallium-201 myocardial scintigraphy after myocardial infarction. Apropos of 53 patients treated with thrombolytic agents during the acute phase and with coronary transluminal angioplasty]. / Appréciation de l'ischémie résiduelle par scintigraphie myocardique au thallium-201, après infarctus du myocarde. A propos de 53 patients traités par thrombolytiques à la phase aiguë et par angioplastie transluminale coronaire.

The prognosis of silent ischemia after myocardial infarction is similar to that of post-infarction angina. In order to detect this condition two stress myocardial scintigraphies were performed: three weeks after hospital admission for myocardial infarction treated by thrombolytic therapy without any complications or recurrence of chest pain; one month later, after percutaneous transluminal coronary angioplasty on the infarct-related artery in 24 patients or after medical therapy alone when this procedure was not possible (29 patients). Silent ischemia, initially present in two thirds of patients, was less frequently observed in the patients undergoing angioplasty (p < 0.05). In the remaining one third of patients with no silent ischemia, myocardial scintigraphy was unchanged at the follow-up procedure whether or not angioplasty had been performed. These results show that silent ischemia is commonly observed during stress myocardial scintigraphy after acute myocardial infarction treated by thrombolysis, but that this condition can be significantly reduced by coronary angioplasty. When no silent ischemia is observed, coronary angiography and angioplasty do not seem to be indicated.

[Meningitis and interferon-alpha in the cereprospinal fluid. What diagnostic and therapeutic approach? A case report]. / Méningite et présence d'interféron alpha dans le liquide céphalorachidien. Quelle attitude diagnostique et thérapeutique? A propos d'un cas.

BACKGROUND: In meningitis without germs, the existence of an inflammatory syndrome leads toward a bacterial etiology while the detection of interferon-alpha (IFN-alpha) in the cerebrospinal fluid (CSF) argues for a viral meningitis. The coexistence of the inflammatory syndrome and the presence of IFN-alpha in the CSF makes this differentiation difficult. The reported case yields the picture and begs the question on the diagnostic approach and the required therapeutic attitude. CASE REPORT: A six-week-old infant, exclusively breast-fed, was hospitalized for fever. The examination showed an important inflammatory syndrome and meningeal attempt with a cellularity at 94/mm3 with 53% polymorphonuclear neutrophils, contrasting with normal proteinorrhachia and glycorrhachia. The IFN-alpha in the CSF was present at 4 UI/mL while the bacteriological culture and the viral search by PCR were negative. The clinical and biological worsening within the first 36 hours, in spite of the parenteral dispensation of a triple antibiotic therapy (amoxicillin, ceftriaxone, netilmicin), then a favorable clinical and biological response after adjunction of vancomycin, led toward a pneumococcal meningitis with reduced sensitivity to beta-lactams. The maternal antibiotic therapy by amoxicillin and its presence in the maternal milk favored the hypothesis of a decapitated bacterial meningitis. CONCLUSION: In the presence of a meningitis without germs, the coexistence of a sizable inflammatory syndrome and the detection of IFN-alpha in the CSF must be considered as an unusual phenomenon and motivate the pursuit of antibiotic therapy until viral identification.

[Neurodevelopmental outcome of premature infants born at less than 33 weeks of gestational age and not cerebral palsy at the age of 5 years]. / Devenir neurodéveloppemental à cinq ans des prématurés nés avant 33 semaines d'aménorrhée et indemnes d'infirmité motrice d'origine cérébrale.

AIM: To appreciate the impact of prematurity, fetal hypotrophy and familial environment on the neurodevelopmental performances of very premature infants without cerebral palsy at the age of five years. POPULATION AND METHODS: We followed a regional cohort of 171 very premature infants (< or = 32 weeks of gestation) until they were five years of age. Cognitive functions were tested with the WPPSI test and the development quotient was assessed by the ability to draw a "bonhomme". Twenty-two premature infants suffered from cerebral palsy diagnosed before the age of two years. Another infant had a moderate diplegia at the five-year examination. We had no information for 16 prematures (9.3% of survivors). Twenty-eight premature infants were considered as having no severe disability on phone or mailed contact, and another child had a severe isolated mental retardation. We examined 104/148 infants, and 96/148 survivors without cerebral palsy passed the tests. The cognitive functions of these premature infants are compared to the performances of a control group made up of 108 children born at term > or = 37 weeks, matched for birthplace and single or twin characteristics of the pregnancy. RESULTS: The values of the different quotients are significantly decreased in the preterm group. The global IQ and the performance IQ are 0.8 SD, verbal IQ is 0.5 SD and the development quotient is 0.4 SD below the values observed in the control group. A performance IQ less than -2 SD for the mean of the control group is observed three times more than in the controls (13.5% vs 3.7%, P < 0.01). Multiple linear regression shows that prematurity explains, independent of hypotrophy and socioeconomic environment, 8% of the variation of the performance IQ (P < 0.01), 2% of the variation of the verbal IQ and 2% of the development quotient (P < 0.05). CONCLUSION: The five-year neurologic outcome of the children born prematurely in this regional study is similar to the results observed in regional studies conducted in Europe: 13.4% of the survivors have cerebral palsy, and the cognitive functions of the children with no cerebral palsy are significantly lower than the term control group. Other risk factors such as hypotrophy, which modulates the developmental quotient, and the socioeconomic status, which modulates the verbal IQ, are underlined.

[Effect of propofol on left ventricular function in the coronary patient. Combined isotopic and hemodynamic studies]. / Impact du propofol sur la fonction ventriculaire gauche du coronarien. Etudes isotopique et hémodynamique couplées.

The present study investigated the effects of propofol (P) as sole anaesthetic agent of left ventricular (LV) function using gated radionuclide ventriculography (RNV) in unpremedicated patients with chronic coronary arterial disease (CAD). After informed consent, seven ASA 111 patients (51-74 yr) undergoing major urologic surgery took part in this study. All patients suffered from documented angina pectoris secondary to CAD. None gave a history of congestive heart failure or valvular heart disease. No patients were premedicated, but all received their chronic medications (nifedipine and isosorbide) up to and including the morning of surgery. The study was performed in the Nuclear Medicine Laboratory just before surgery. Heart rate (HR) was obtained from standard limb lead II of the ECG. A 7.5 F thermodilution Swan-Ganz catheter and a radial artery cannula were inserted under local anaesthesia. All patients were studied by RNV using red blood cells (RBC) labelled in vivo with 99m-technetium (Tc). A first RBC-Tc preparation containing 2-3 mCi f Tc was counted 10 cm from a gamma-camera. A first-pass study was carried out in the left anterior oblique position following the bolus i.v. injection of the source, allowing the evaluation of the isotopic dilution cardiac output (COiso) and the attenuation factor (F). Then, a second RBC-Tc preparation containing 20-25 mCi of Tc was injected intravenously. At equilibrium, 16 ECG-synchronized frames were acquired by computer for processing. Mean end-diastolic (ED) and end-systolic (ES) counts, left ventricular-end diastolic and systolic areas and ejection fraction (EF) were calculated every 1.5 min.(ABSTRACT TRUNCATED AT 250 WORDS)

[Analysis of word perception in deaf children. A longitudinal study]. / Analyse de la perception de la parole de l'enfant sourd. Etude longitudinale.

The assessment of speech perception and speech reading has been studied with the phonetic test of J.C. Lafon (lists of 17 french words with 3 phonemes each). 168 children from 7 to 17 have been collected. Deafness was moderate, severe or profound. It was always congenitaly sensoneural hearing loss. In order to precise data, results are giving regard to educational program: mainstream or special classes. Comparative data for each degree of deafness are given for speech perception, lipreading and both auditory and visual sensorial ways.