Congenital cytomegalovirus (CMV) infection is the most common vertically transmitted disease with the rate of the infection ranging from 0.2 to 2.4% in newborn infants. Congenital CMV infection ...causes multiorgan affection, but the most severe and permanent sequelae are those affecting central nervous system such as mental retardation, cerebral palsy, sensorineural hearing loss, chorioretinitis and seizures as a result of direct interference of the virus with neurogenesis. The time of acquiring infection is strongly connected to the level of child's disability. Infection in early pregnancy results in severe neurological sequelae, while later infection has less prominent signs. Radiological findings show connection between onset of infection and brain imaging, from lissencephaly, pachygyria, polymicrogyria, schizencephaly, calcification, cerebellar hypoplasia and/or hypoplasia/agenesis of corpus callosum as a result of an early infection, to white matter abnormalities including disturbed myelination as a result of a late infection. We present nine patients with proven congenital CMV infection and malformations of cortical development and their computed tomography/magnetic resonance (CT/MRI) findings along with clinical assessments. According to CT/MRI results we assume that two of our children with lissencephaly had an early onset of infection. The other seven with less severe cortical dysplasia in form of pachy/polymicrogyria were probably infected later Cerebellar hypoplasia and/or calcifications in our patients also confirm an early onset of infection. Developmental outcome in all of our children was poor: moderate to severe psychomotor retardation has been diagnosed in all children; five of them have developed cerebral palsy (four have bilateral spastic and one dyskinetic) and one is estimated to have minor motor dysfunction. Seven out of nine developed epilepsy, chorioretinitis was found in three of them and sensorineural deafness in two of them. All of our children, except one, were presented by symptomatic infection, yet only four of them were recognized at birth. Therefore, congenital CMV infection should be considered as one of the reasons for childhood disability more often.
Lenticulostriate vasculopathy (LSV) is an ultrasound (US) visible lesion of the brain, which appears as echogenic streaks or spots in the arteries of thalamus and basal ganglia. LSV has varied ...etiology. Transfontanelar Color Doppler (TFCD) can easily display lenticulostriatal blood flow and assess: stage I LSV with present flow within echogenic changes and stage II LSV in which the flow disappears, despite a presence of streaks and spots, which at this stage most probably correspond to calcification. The objectives of this study are to determine: (1) Whether there are differences in distribution (unilateral or bilateral) and presence (during first year of age) of TFCD flow between congenital CMV infection positive and negative group of children with LSV (2) Could US and TFCD findings of LSV be an indication for further investigation of possible congenital CMV infection, because of their variable and often adverse neurodevelopmental outcome? We examined and followed-up 98 infants with LSV One group (37/98) with congenital CMV infection and second (61/98) negative. All infants had clinical signs of neuromotor delay and ultrasound and TFCD markers of LSV Our study shows that most of the patients from both groups had TFCD visible flow at the age of 0-4 months. In majority of them in both groups, at the age of 5-8 months, there was no more visible flow. TFCD showed no statistically significant difference among congenital CMV infection positive group and negative group, nor in youngest age period (0-4 months), nor in later course of flow in LSV unilaterally or bilaterally. Although the LSV presents nonspecific marker for intracranial infection (ICI), all infants presenting with LSV should be evaluated for possible ICI. Thus, the Doppler findings of LSV in infants require a detailed examination, monitoring and follow-up of neuromotor outcome.
Perception and discrimination of auditory and speech stimuli in children aged 7-9 years with either receptive (n=6) or expressive (n=5) type of special language impairment and 7 healthy age-matched ...controls was investigated using evoked potential technique. The measurements were performed with a 32-channel Neuroscan electroencephalographic system. Two types of stimuli were applied, pure tones (1 kHz and 2 kHz) and double syllabi consisting of one consonant and one vocal characteristic of Croatian language. The stimuli were presented in an oddball paradigm, requiring a conscious reaction for the subjects. Latencies and amplitudes of P1, N1, P2, N2, P3, N4, and SW waves were analized, as well as the reaction time and number of responses. There were found no statistically significant difference between children with special language impairment and the control group in average response time and number of responses to tone burst or double syllable. Analysis of variance of all used variables showed a statistically significant difference in P3 and Sw wave latencies after double syllable stimulation, P3 and N4 waves latencies after target stimulation, P2 and Sw wave amplitude; and in N1 wave amplitude after pure tone stimulation. Our study showed that children with speech and language disorder take longer time to perceive and discriminate between either tonal or speech auditory stimuli than children with typical speech and language development.
Cilj istraživanja: ispitati koliko se često slušna neuropatija javlja u Hrvatskoj te bi li, s obzirom na njenu učestalost, u naša rodilišta osim ispitivanja otoakustičke emisije (OAE) valjalo uvesri ...i automatsko ispitivanje evociranih potencijala moždanog debla (A-ABR).
Ispitanici i metode: u razdoblju od 1. 10. 2002. do 1. 10. 2007. u Hrvatskoj je rođeno oko 200 000 djece. U oko 90% djece je kod otpusta provjeren sluh metodom OAE. Svoj djeci u koje je OAE bila odsutna, kao i u neurorizične djece ili one koja su poslije nesigurno reagirala na zvučne podražaje, učinjeni su dijagnostički evocirani potencijali moždanog debla (ABR). Kriteriji za dijagnozu slušne neuropatije bili su uredan OAE i odsutan ili značajno promijenjen ABR.
Rezultati: u petogodišnjem razdoblju jednostrano ili obostrano trajno slušno oštećenje nađeno je zasad u 240-ero djece u prosječnoj životnoj dobi od 6 mjeseci. Obostrana slušna neuropatija nađena je u 4-ero djece (1,7% slušno oštećene djece, odnosno, 0,02‰ opće populacije novorođenčadi). Sva su ta djeca bila neurorizična. U jednog je djeteta nađena Arnold-Chiarijeva malformacija, a u jednog agenezija corpus calosuma i kraniosinostoza.
Zaključak: s obzirom na rezultate ovog istraživanja, prirođena slušna neuropatija je u Hrvatskoj rijetka, pa zasad ne postoji opravdana potreba za uvođenjem sustavnog A-ABR-a u naša rodilišta. Ipak, naglašava se potreba praćenja razvoja slušanja i govora i prema potrebi dijagnostički ABR, pogotovu u neurorizične djece, bez obzira na uredan OAE u rodilištu.
Neonatal hearing screening program (NHSP) started in Croatia at the beginning of year 2002 (University
hospital „Sv Duh“, Zagreb), and by the end of year 2002 it was implemented in almost all ...maternity units
in Croatia. From the year 2006 the NHSP is mandatory and there is a specific health legislation.
In General hospital Pula, the NHSP is performed before the time of discharge home, using A-OAE method.
Those who are positive on hearing impairment (HI), are checked again using the same method 3-4 weeks after
the discharge. Those who are positive again are checked using A-ABR method and if they are positive twice, they
are sent to the University hospital for a specific audiologic evaluation.
The aim of this study is describe the outcome of NHSP in General hospital Pula (Pula, Croatia) in a five-year
period (2011.-2015.). In the above mentioned period, there were 6799 live births and NHSP was performed in
6535 neonates (96,12%). Thirty-two children were positive on HI (Two A-OAE checkings plus two A-ABR checkings)
and were sent to the University hospital for audiologic evaluation. HI was diagnosed in 12 children; four of
them had unilateral HI and eight of them had billateral HI. The incidence of HI in our population is 0,18%.
Promjene koje su posljedica prezbiakuzije opisane su na svim razinama slušnog puta. samo ne postoji slaganje gdje su promjene najčešće, mijenja li se lokalizacija oštećenja s porastom životne dobi i ...zašto je razumijevanje govora sve slabije. Metodom slučajnog uzorka ispitano je ukupno 106 osoba između 60 i 99 godina (72% žena), podijeljenih u četiri dobne skupine. Nalazi slušnih potencijala moždanog debla uspoređeni su s nalazima tonskog audiograma, razlikovnih pragova visine i trajanja tonova različitih frekvencija, razumljivosti riječi u standardnoj, usporenoj i otežanoj govornoj audiometriji. razumljivosti rečenica standardne brzine i ubrzanih rečenica kao i rečenica davanih naizmjenično na desno i lijevo uho. (Usporedba rezultata pretraga i statistička obrada pokazala je da se s povećanjem životne dobi pravilno pogoršava periferna razina sluha i to zbog oštećenja receptora, da su promjene spiralnoga ganglija i subkortikalnih struktura rijetke i nisu povezane s dobi, a da su kortikalne promjene najizraženije i prevladavaju nad receptornim. Standardna govorna audiometrija uglavnom odgovara oštećenju receptora i dosta je neosjetljiva na centralne smetnje slušanja. a otežana govorna audiometrija. ispitivanje razumljivosti ubrzanih rečenica i rečenica davanih naizmjenično na desno i lijevo uho najbolje pokazuju prisustvo centralnih smetnji.
U neke djece uredna sluha s funkcionalnom dislalijom fonematski sluh je slabije razvijen, što upućuje na središnje smetnje slušanja, iako one do sada neurofiziološkim tehnikama nisu dokazane. Nije ...jasno niti jesu li smetnje posljedica subkortikalne ili kortikalne disfunkcije, iako literatura uglavnom upućuje na kortikalnu lokalizaciju smetnji. Autor je vrlo preciznom tehnikom (slušni evociram potencijali moždanog debla - ABR) ispitao vrijeme provođenja jednostavnog slušnog podražaja (clicka) kroz strukture moždanog debla u tri različite skupine djece:
• djece s funkcionalnom dislalijom i slabije razvijenim fonematskim sluhom
• djece s funkcionalnom dislalijom, ali urednim fonematskim sluhom
• kontrolne skupine djece bez smetnji slušanja i govora
Djeca su bila u dobi između 6 i 7 godina, uredna periferna sluha, uredna neurološkog nalaza i uredne inteligencije. Prema razultatima istraživanja proizlazi da ispitana djeca sa slabije razvijenim fonematskim sluhom imaju duže centralno vrijeme provođenja kroz moždano deblo nego ostala djeca, i to slično kao u djece mlađe od godinu dana u koje maturacija tog područja slušnog puta još nije dovršena. Autor zaključuje da slabiji fonematski sluh u djece nije nužno posljedica korit i ka l ne disfunkcije, te da je možda u osnovi smetnji usporeno provođenje i obrada slušnog podražaja na razini moždanog debla. U djece s funkcionalnom dislalijom, slabije razvijenim fonematskim sluhom i "nezrelim " nalazom ABR-a predlaže naziv "razvojna dislalija ".
Perception and discrimination of auditory and speech stimuli in children aged 7–9 years with either receptive (n=6) or expressive (n=5) type of special language impairment and 7 healthy age-matched ...controls was investigated using evoked potential technique. The measurements were performed with a 32-channel Neuroscan electroencephalographic system. Two types of stimuli were applied, pure tones (1 kHz and 2 kHz) and double syllabi consisting of one consonant and one vocal characteristic of Croatian language. The stimuli were presented in an oddball paradigm, requiring a conscious reaction for the subjects. Latencies and amplitudes of P1, N1, P2, N2, P3, N4, and SW waves were analized, as well as the reaction time and number of responses. There were found no statistically significant difference between children with special language impairment and the control group in average response time and number of responses to tone burst or double syllable. Analysis of variance of all used variables showed a statistically significant difference in P3 and Sw wave latencies after double syllable stimulation, P3 and N4 waves latencies after target stimulation, P2 and Sw wave amplitude; and in N1 wave amplitude after pure tone stimulation. Our study showed that children with speech and language disorder take longer time to perceive and discriminate between either tonal or speech auditory stimuli than children with typical speech and language development.
Lenticulostriate vasculopathy (LSV) is an ultrasound (US) visible lesion of the brain, which appears as echogenic streaks or spots in the arteries of thalamus and basal ganglia. LSV has varied ...etiology. Transfontanelar Color Doppler (TFCD) can easily display lenticulostriatal blood flow and assess: stage I LSV with present flow within echogenic changes and stage II LSV in which the flow disappears, despite a presence of streaks and spots, which at this stage most probably correspond to calcification. The objectives of this study are to determine: (1) Whether there are differences in distribution (unilateral or bilateral) and presence (during first year of age) of TFCD flow between congenital CMV infection positive and negative group of children with LSV; (2) Could US and TFCD findings of LSV be an indication for further investigation of possible congenital CMV infection, because of their variable and often adverse neurodevelopmental outcome? We examined and followed-up 98 infants with LSV. One group (37/98) with congenital CMV infection and second (61/98) negative. All infants had clinical signs of neuromotor delay and ultrasound and TFCD markers of LSV. Our study shows that most of the patients from both groups had TFCD visible flow at the age of 0–4 months. In majority of them in both groups, at the age of 5–8 months, there was no more visible flow. TFCD showed no statistically significant difference among congenital CMV infection positive group and negative group, nor in youngest age period (0–4 months), nor in later course of flow in LSV, unilaterally or bilaterally. Although the LSV presents nonspecific marker for intracranial infection (ICI), all infants presenting with LSV should be evaluated for possible ICI. Thus, the Doppler findings of LSV in infants require a detailed examination, monitoring and follow-up of neuromotor outcome.
