[Analysis of causes and treatment of hearing loss in children from Department of Infant Diseases the Children's Memorial Health Institute, Warsaw]. / Analiza przyczyn niedosluchu oraz zastosowanego leczenia u dzieci w materiale Kliniki Niemowlecej Instytutu "Pomnik - Centrum Zdrowia Dziecka" w Warszawie.

UNLABELLED: The aim was to identify the frequency of different causes of congenital hearing loss and to investigate the age of treatment intervention. MATERIAL: 197 children with hearing loss, hospitalized in the Department of Infant Diseases between 2007-2009. METHODS: Three-level audiological examinations, clinical investigations, specific tests for selected congenital infections and GJB2 mutations, neuroimaging. RESULTS: In 14 children with negative screening test hearing loss was confirmed; in 14 with positive was excluded; in 5 newborns screening test was not performed. In 179/197 the confirmation of hearing impairment was obtained up to 6 months (90%). Sensorineural (176/197), bilateral (157/197) hearing loss dominated; conductive and mixed was in 21/197. In 97/176 children with sensorineural hearing loss, congenital CMV infection was confirmed; in 47/176 - GJB2 mutations; in 21 simultaneous CMV infection and GJB2 mutation; in 26 the reason was not identified. The hearing aids were applied in 128, in 76 up to 6 months; the cochlear implants received 36, mainly in the 1st. and 2nd. year of life. The improvement of hearing was obtained in 33. CONCLUSIONS: 1. Early identification of infants with hearing loss allows for an earlier introducing of comprehensive treatment and improvement of hearing. 2. The significant proportion of children with hearing loss in the course of congenital cytomegalovirus infection indicates the need to carry out tests to identify infection in newborns with abnormal hearing screening test. 3. Cochlear implants are now in Poland the standard method of treatment in partial and complete deafness in children, also the youngest.

Evaluation of intellectual development of children following congenital, mildly symptomatic cytomegalovirus (CMV) infection. A prospective study.

AIM: Assessment of intellectual development of 6-year-old children following asymptomatic or mildly symptomatic congenital cytomegalovirus infection in infancy. MATERIAL AND METHODS: A longitudinal, prospective cohort study concerning 38 (2%) children with congenital cytomegalovirus infection confirmed by specific serological and molecular tests, selected from the group of 1895 neonates preliminarily enrolled into the study. The first specialistic clinical assessment was performed during the neonatal and early infancy period, the second at the age of 12-18 months, and the final comprehensive clinical evaluation was carried out at the age of 6-6.5 years. RESULTS: Psychological evaluation showed normal mental development (Intelligence Quotient ranged from 88 to 114), but 12 (32%) children showed abnormalities in speech development and in 3 (8%) poor visual-motor integration was observed. Emotional and social functioning indicate a normal level of maturity, but 14 (37%) children exhibited increased emotional sensitivity. Psychological assessment indicate that 6 (16%) children may have problems with school maturity. CONCLUSIONS: Long-term follow-up of children with congenital cytomegalovirus infection is necessary, including those with a mild clinical course, in view of the possible late sequelae, especially concerning intellectual development and hearing impairment.

Polish universal neonatal hearing screening program-4-year experience (2003-2006).

OBJECTIVE: The aim of this paper is to share our experience and observations in running the Universal Neonatal Hearing Screening Program on a national level, present results and indicate some problems that have arisen during these 4 years. METHODS: Polish Universal Neonatal Hearing Screening Program started back in 2002 in all neonatal units in Poland. Implemented testing methods consisted of test of transient evoked otoacoustic emission (TEOAE) performed in all new born children in their first 2-3 days of life and auditory brainstem response testing (ABR) conducted on children, who did not meet the TEOAE pass criteria. Additional questionnaire registered information on ototoxic drugs and family history of hearing impairment in every newborn. Diagnosed children were further referred for treatment and rehabilitation. RESULTS: After 4 years of running the program (between 2003 and 2006) a total number of 1,392,427 children were screened for hearing impairment, what stands for 96.3% of all delivered babies, registered in Poland. The screening program enabled to identify and refer for further treatment 2485 children with various types of hearing loss, 312 with profound (0.02% of population) and 145 with severe sensorineural hearing loss (0.11% of population). CONCLUSIONS: Our results indicate the accuracy of newborn hearing screening which remain an issue. Although improvement is needed in both intervention systems and diagnostic follow-up of hospitals, the Polish Universal Neonatal Hearing Program fully has achieved the main goal, the identification and treatment of hearing impaired children.

[Analysis of specificity and sensitivity of Polish "Universal Newborn Hearing Screening Program"]. / Analiza czulosci i swoistosci ogólnopolskiego "Programu przesiewowych badan sluchu u noworodków".

INTRODUCTION: Polish National Universal Hearing Screening Program (PNPUHS) has got three-level structure. Audiology Outpatient Clinic in The Childrens' Memorial Health Institute in Warsaw is a center of the second and the third level. Newborns are referred to us from neonatal units, the first level in the program. We found that referred children, both with unilateral as well as bilateral referral rating in TEOAE, show a high proportion of false positive results. AIM: To analyze a diagnostic process of healthy neonates referred to our Audiology Outpatient Clinic with referral rating in one and both ears measured by TEOAE in 2005 and 2006. Assessed parameters include: attendance of patients to further diagnostics tests, with respect to their results, specificity and sensitivity of methods: TEOAE, DPOAE, analysis of final diagnoses of hearing loss. PATIENTS: 1764 infants without risk factors of hearing loss, aged from one to six months referred with referral rating in one or both ears in TEOAE. METHODS: Diagnostics methods include: otoscopy, DPOAE, AI, BOA. Incorrect results are indication for ABR examination, if not conclusive ASSR. Collected data were statistically analyzed by chi-squared test and Czupurow and V-Cramer coefficient. RESULTS: 998 (56,5%) of 1764 examined children passed DPOAE. Only 512 (66,8%) of remaining 766 neonates carried on further investigations. Diagnosis of hearing loss was made in 449 patients (25%), including 320 (71%) with bilateral and 129 (29%) with unilateral involvement. Sensorineural hearing loss was detected in 274 children (61%), conductive in 161 (35,8%), and mixed in 14 patients (3,1%). Global PNUHSP specificity are 95,45% and sensitivity results are 95,08% respectively. Although in our Audiology Outpatient Clinic, sensitivity rate of TEOAE and DPOAE is 94,5%, and 94,4% respectively, the specificity rate of TEOAE appeared to be 36,9% and of DPOAE 79,7%. CONCLUSIONS: Universal newborn hearing screening program permit early diagnosis and intervention in neonates and infants before 6th month of age. High rate of specificity and sensitivity characterizes the Polish Program. Low rate of specificity detected in our outpatient clinic indicates the necessity to effect and cost analysis in neonatal units. Comparison of two groups with bilateral and unilateral referral rating in screening shows that higher number of patients follow diagnostic process from the group with bilateral incorrect results, more diagnoses of hearing loss was established in this group as well.

[The 50-years' history of recommendations for organization of universal hearing screening]. / Rekomendacje dotyczace organizowania badan przesiewowych sluchu noworodków na przestrzeni ostatnich 50 lat.

Diagnoses of hypoacousis in children based on behavioral examination in 60-ies brought about the idea of creation recommendation for hearing screening program in children. Joint Committee of Infant Hearing, organized in US in early 70-ies by the speech and hearing therapists, has edited several recommendations concerning hearing screening in children. The point of view on this problem has been changing with the development of new techniques and increase of knowledge. Initially, hearing screening was introduced only in children of high risk population. Nationals Institutes of Health, another American organization created in 90-ies, has edited details concerned implementation of the universal hearing screening program. The idea to introduce this program has expand from the USA to Europe. The document European Consensus established in 1998 regulates the status of nationals programs of universal hearing screening which are widely implemented to the clinical practice.