[Life expectancy of extremely preterm infants]. / Extrém alacsony gesztációs korú koraszülöttek életkilátásai.

Extremely preterm infants [gestational age (GA) between 24-28 weeks] should be delivered optimally in an institute where neonatal intensive care unit (NICU) is available and their short- and long-term care is ensured. At the Department of Obstetrics and Gynecology, Medical School, University of Pécs, 7499 infants were born between 1st of January, 2000 and 31st of December, 2004. During this period the rate of preterm deliveries was 20% (1499/7499). Among preterm infants the incidence of extremely preterm babies (GA 28 weeks or less) was 18% (272/1499), the rate of profoundly preterm infants (GA less than 25 weeks) was 3.2% (48/1499). Advancing with gestational age the survival rate is increasing. At the department, the rate of handicapped infants among extremely premature babies was 15.3%. The majority of the handicapped infants were profoundly preterm, meanwhile, more than 50% of infants born at the 26 gestational weeks were free of symptoms influencing social activities. It is important to stress the prognostic value of the screening for hearing loss (otoacoustic emission), visual problems, and intracranial bleeding for the early detection and cure of the possible complications of prematurity.

Maternally inherited deafness and unusual phenotypic manifestations associated with A3243G mitochondrial DNA mutation.

The mitochondrial DNA A3243G transition is a fairly common mutation which often associates with a MELAS (mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes) phenotype, however, a broad variety in the associated clinical picture has also been described. The patient reported here developed a generalized seizure at age 12, which was followed by bilateral hearing loss and occasional fatigue. The maternal inheritance pattern of hearing loss pointed to a possible mitochondrial origin, which was confirmed by molecular analysis of the mitochondrial DNA, revealing a heteroplasmic A3243G transition. Interestingly, muscle biopsy showed ragged-red fibers in the proband, which is unusual in the deafness-associated forms of this mitochondrial disorder. In addition to hearing impairment in four generations of the family, fatal cerebral embolization in the mother and fatal heart attack in the maternal grandmother (both at age 33) also occurred. On the contrary, diabetes, which usually accompanies the hearing loss variant, was specifically absent in all generations. The unusual manifestations associated with this mutation somewhat differentiate this family from the already known variants.

Retrospective analysis of tympanoplasty in children with cleft palate: a 24-year experience. II. Cholesteatomatous cases.

BACKGROUND: Contradictory experience has been published on the outcomes of ear surgery in patients with cleft palate. OBJECTIVES: The authors of this study investigated whether there were differences in the short- and long-term outcomes of tympanoplasty performed due to cholesteatoma in children with or without cleft palate. SETTING: Tertiary care medical centre. METHODS: The authors retrospectively analyzed the first author's 24-year experience of paediatric tympanoplasty using the software programme developed by the fourth author. The outcomes of 268 tympanoplasties on 172 ears with cholesteatoma in 151 'NoCleft' patients were compared to the outcomes of 35 tympanoplasties on 20 ears of 19 'Cleft' patients. The average age of the patients was 10.7±3.6 years and 9.5±2.7 years respectively. The average follow-up time was 4 and 4.1 years. RESULTS: Preoperative PTA-ABGs (31.22/34.88 dB; p=0.058), best postoperative PTA-ABGs (17.04/16.4 dB; p=0.499), last postoperative PTA-ABGs (19.93/20.98 dB; p=0.298), the final hearing improvement (11.29/13.9 dB; p=0.193) and postoperative PTA-ABG deterioration with time (2.89/4.58 dB; p=0.117) were statistically compared between the 'NoCleft' and 'Cleft' groups. The same parameters were analyzed separately in the case of tympanoplasty performed with intact ossicular chain and the different type of columella ossiculoplasty. No significant differences were found between the two groups in any of these parameters. However, significant difference was found in the necessity for grommet insertion (8-fold difference, p≈0), and conversion to open techniques (p≈0). CONCLUSIONS: The authors conclude that the achievable audiological outcomes of tympanoplasty in children with cleft palate and cholesteatoma do not differ significantly from those of the general child population. However, this more frequently requires ventilation tube insertion and more frequent follow-up visits. The latter is ensured by patient care within the frameworks of the 'Cleft Palate Team'. We have to accept that in some cases Eustachian tube dysfunction caused by the underlying disease (cleft palate) 'takes over' and we have to resort to open techniques.

Retrospective analysis of tympanoplasty in children with cleft palate: a 22-year experience: I. Mesotympanic (non-cholesteatomatous) cases.

BACKGROUND: Contradictory data have been published on the outcomes of ear surgeries in cleft patients. OBJECTIVES: To investigate whether there are differences in the short and long term outcomes of tympanoplasty performed due to childhood chronic mesotympanic otitis media in patients without and with cleft palate. SETTING: Tertiary care pediatric medical centre. METHODS: The authors retrospectively analysed the first author's data on pediatric tympanoplasties of the past 22 years with the help of a computer programme developed by the third author. The outcomes of 159 'NoCleft' tympanoplasties (119 patients, 144 ears) were compared to the outcomes of 31 'Cleft' tympanoplasties (21 patients, 27 ears) with the average age of the patients being 10.8 and 10.7 years accordingly. RESULTS: The preoperative ABG (28.76/28.94dB, p = 0.468), the best postoperative ABG (12.78/10.04dB, p = 0.096), the last postoperative ABG (15.59/13.19dB, p = 0.192), the final hearing gain (13.17/15.75dB, p = 0.253) and the postoperative ABG deterioration associated with time (2.81/3.15dB, p = 0.376) were statistically compared in the 'NoCleft'/'Cleft' groups. No significant difference was found between the outcomes of the two groups. The same parameters were examined separately after tympanoplasties performed with intact ossicular chain and after those requiring columella ossiculoplasty. The 'Cleft' group did not have worse outcomes in this respect, either. The average follow-up period of the patients was more than 3 years in both groups. The graft take rate was 100%, reperforation occurred in 3.5% of the cases in both groups. In their study, grommet insertion was more likely to be necessary in the 'Cleft' group. CONCLUSION: The authors concluded that there was no significant difference between the expectable outcomes of pediatric tympanoplasties in patients with and without cleft palate regarding mesotympanic cases; therefore, the indications for tympanoplasty are the same in the two groups. They outline the importance of the therapeutic management of cleft patients in the frameworks of a "Cleft Palate Team" and the necessity for their lifelong otorhinolaryngological care due to their vulnerable Eustachian tube function. They are planning to publish the same comparative analysis of their patients with cholesteatoma in a following study.