A Case Report of Melanoma as Acute Mastoiditis in a 10-Month-Old Female Child.

We present a rare case of transplacental-transmitted maternal melanoma to the placenta and foetus during the pregnancy of a 34-year-old woman. She was diagnosed with a melanoma at the age of 25, for which she was treated with chemotherapy. During her pregnancy, she presented with a recurrence of the disease and died 3 months after delivery. The 10-month-old female child presented with a recurrent retroauricular oedema on the left side. A trephination of mastoid apophysis followed. Multiple fragments of dark-coloured tissue were sent for histological examination, and the immunophenotype showed a melanocytic tumour in the mastoid. A full radiological assessment showed no sign of metastasis. The child remained without treatment. Complete remission of bone metastatic lesion has been confirmed by follow-up; now, the child is 4 years old, alive, and without evidence of disease.

Use of chloral hydrate as a sedative for auditory brainstem response testing in a pediatric population.

OBJECTIVE: Chloral hydrate (CH) is an oral sedative widely used to sedate infants and young children during auditory brainstem response (ABR) testing. The aim of this study was to record effectiveness, complications and safety of CH as a sedative for ABR. METHODS: From January of 2003 until December of 2007, 1903 children were tested for ABR, 568 of them being under the age of 6 months. CH (8%) was used for sedation at a dose of 40 mg/kg with a repeat dose, if necessary, for an adequate sedation, in 20-30 min. We recorded the effectiveness of CH as a sedative for ABR examination, as well as all complications related to the use of CH such as vomiting, rash, hyperactivity, respiratory distress and apnea. The statistical method used was the absolute and percentage frequency distribution of the occurrences. RESULTS: Sedation with CH was necessary to perform testing in 1591 (83.6%) of the examined children. However, in the population of the examined infants, only 341 (60%) were sedated with CH, because the remaining 227 (40%) fell asleep by themselves. Complications included hyperactivity in 152 children (8%), minor respiratory distress in 10 children (0.4%), vomiting in 217 children (11.4%), apnea in 4 children (0.2%) and rash in 10 children (0.4%). The complications of hyperactivity, vomiting and rash resolved without any medical treatment. The apnea cases were managed effectively by supplying ventilation to the children via a mask in the presence of an anesthesiologist. CONCLUSIONS: The use of CH at a dose of 40 mg/kg up to 80 mg/kg is safe and effective when administered in a setting with adequate equipment and the presence of well trained personnel.

The impact of the heptavalent pneumococcal conjugate vaccine on the epidemiology of acute otitis media complicated by otorrhea.

BACKGROUND: The heptavalent pneumococcal conjugate vaccine (PCV7) has a considerable effect on the epidemiology of pneumococcal disease. The aim of this observational hospital-based study was to examine the effect of the PCV7 (introduced in our settings in 2004) on the epidemiology of spontaneously draining acute otitis media. METHODS: Results of all middle ear fluid cultures (n = 3446) obtained from children with acute otitis media complicated with otorrhea before the introduction of immunization (between 2000 and 2003) were compared with those (n = 2134) obtained during a similar post-PCV7 period (between 2005 and 2008). Results of cultures obtained between 2006 and 2008 were examined prospectively, whereas those obtained in previous years were retrospectively reviewed. RESULTS: Following PCV7 immunization, the rates of otorrhea visits per 10,000 emergency department visits decreased by 38% from 133 to 83 (95% confidence interval of the difference, 42-53; P < 0.001), mainly as a result of the decrease in the incidence of pneumococcal disease (48% decrease-25 vs. 13 per 10,000 emergency department visits; P < 0.001). Otorrhea due to Haemophilus influenzae decreased by 20% (20-16 per 10,000 visits; P < 0.001). Serotype 19A accounted for 1 of 47 (2%) pneumococcal strains in 2006, for 5 of 34 (15%) in 2007, and for 13 of 53 (25%) in 2008 (P for trend: 0.001). In the postvaccine years, penicillin-resistant pneumococcal strains (minimum inhibitory concentration ≥ 2 µg/mL) increased from 4% to 13% (P < 0.001). However, the proportion of pneumococci resistant to macrolides decreased (44% vs. 35%; P = 0.01). CONCLUSIONS: After the introduction of immunization, otorrhea incidents decreased considerably, mainly because of the decrease in pneumococcal disease. H. influenzae is now the predominant organism. Serotype 19A has increased significantly and is the most common nonvaccine pneumococcal serotype. Penicillin resistance has increased in recent years.

Pseudohypacusis: the most frequent etiology of sudden hearing loss in children.

Sudden hearing loss is a rare pathology in children. Several factors may be responsible for it although the exact etiology remains frequently undiagnosed. Among them, pseudohypacusis has been reported. However, the extent to which this pathology contributes to sudden hearing loss in children is unknown. This study evaluates the incidence of pseudohypacusis in children presented with sudden hearing loss. The medical records of 48 children presented to our department because of sudden hearing loss from 2002 to 2007 were reviewed. Diagnostic process included both subjective and objective audiological tests while organic hearing losses were further subjected to proper evaluation and treatment. 26 cases (54%) of pseudohypacusis and 22 cases (46%) of organic sudden hearing loss were diagnosed. In the pseudohypacustic group, girls outnumbered boys (16:10) and their mean age was 10.5 years. Pseudohypacusis represents the most frequent etiology of sudden hearing loss in children. Its detection is relatively simple using conventional audiological tests though in some cases even experienced clinicians may come to incorrect diagnosis.

Maturation of the auditory system: 1. Transient otoacoustic emissions as an index of inner ear maturation.

The transiently evoked otoacoustic emission amplitude of 42 preterm babies (84 ears; post-conceptional age [PCA] 30-36 weeks) was compared with the TEOAE amplitude of 39 full-term babies (78 ears; PCA 37-45 weeks) in order to trace the inner ear maturation characteristics. An ILO-92 otoacoustic emission recording system was used with linear clicks of 70 dB peak equivalent SPL. The results obtained indicated: (1) There was no statistically significant difference between preterm and full-term ears; (2) There was no significant difference between males and females; (3) There was a significant difference between left and right ear TEOAE amplitude; (4) The interaction of ear with age in relation to TEOAE amplitude was statistically significant; (5) Noise and stimulus parameters did not reveal any significant differences between right and left ears; (6) A positive correlation existed between birthweight and TEOAE amplitude; and (7) A negative correlation existed between aminoglycoside treatment and TEOAE amplitude. The results indicate subtle changes in TEOAE amplitude over time, showing a natural development of the inner ear function during the process of maturation.

Transient otoacoustic emissions in the detection of olivocochlear bundle maturation.

OBJECTIVE: The medial olivocochlear bundle, forms a neural pathway connecting the superior olivary complex to the outer hair cells of mainly the contralateral cochlea. Although the biological significance of the bundle remains ambiguous, it is clear that activation of medial olivocochlear fibers alters cochlear output and this can be experimentally evident through a series of changes on otoacoustic emissions, called otoacoustic emission suppression. The aim of this study is to observe the maturation of the medial olivocochlear bundle by measuring the suppression effects on transient otoacoustic emissions. METHODS: The study population consisted of 27 premature babies (53 ears) from the P&A Kyriakou Neonatal Intensive Care Unit (conceptional age: 31-36 weeks) and 43 fullterm babies (61 ears) (conceptional age: 37-42 weeks) from the same unit. The ILO-92 system is used to deliver both the linear clicks to the ear examined (producing the emissions) and the white noise to the contralateral ear. The mode used is based on the alternating on and off presence of the white noise in the contralateral ear. RESULTS: 12/53 (22%) ears of preterm babies presented suppression > or =1 dB. 32/61 (52.4%) ears of fullterm babies presented suppression > or =1 dB. The mean suppression for the ears of premature infants was 0.52 dB, (+/-0.1 S.E.M.). The mean suppression for the ears of fullterm infants was 0.90 dB, (+/-0.09 S.E.M.). At the second half of the time window, suppression of fullterms rises above 1 dB (a pattern similar to that of adults), whereas it remains below 1 dB for preterms. CONCLUSIONS: Suppression of otoacoustic emissions helps us to study the function of the medial olivocochlear bundle in different populations. It appears in premature babies but becomes stronger as the postconceptional age advances.

Prevalence of GJB2 mutations in prelingual deafness in the Greek population.

OBJECTIVE: Mutations in the gene encoding the gap junction protein connexin 26 (GJB2) have been shown as a major contributor to prelingual, sensorineural, nonsyndromic, recessive deafness. One specific mutation, 35delG, has accounted for the majority of the mutations detected in the GJB2 gene in Caucasian populations. The aim of our study was to determine the prevalence and spectrum of GJB2 mutations in prelingual deafness in the Greek population. METHODS: In a collaboration with the major referral centers for childhood deafness in Greece, patients were examined by an extensive questionnaire to exclude syndromic forms and environmental causes of deafness and by allele-specific polymerase chain reaction (PCR) for the detection of the 35delG mutation. Patients heterozygous for the 35delG mutation were further analyzed by direct genomic sequencing of the coding region of the GJB2 gene. RESULTS: The 35delG mutation was found in 42.2% of the chromosomes in 45 familial cases of prelingual, nonsyndromic deafness (18 homozygotes and 2 heterozygotes) and in 30.6% of the chromosomes in 165 sporadic cases (45 homozygotes and 11 heterozygotes). Direct genomic sequencing in heterozygous patients revealed the L90P (2 alleles), W24X (2 alleles), R184P (2 alleles), and 291insA (1 allele) mutations. CONCLUSION: Mutations in the GJB2 gene are responsible for about one third of prelingual, sensorineural, nonsyndromic deafness in the Greek population, and allele-specific PCR is an easy screening method for the common 35delG mutation.