Follow-up on the diagnostic level of children covered by the Universal Neonatal Hearing Screening Program in Poland, divided into voivodships.

INTRODUCTION: Thanks to the Polish Universal Neonatal Hearing Screening Program (PUNHSP), all newborns in Poland undergo a free, screening hearing examination. Between 2006 and 2015, the average number of tested children per year was 373,477. According to the analysis of The Central Database (CDB), only 55.8% of the children attended the detailed hearing examinations at the second level of the Program. AIM: The aim of this study is to analyse the dates concerning the attendance of the children at the diagnostic level of PUNHSP in different regions of Poland. MATERIALS AND METHODS: To conduct an analysis of this fact and find out the reasons for low attendance at the second level in 2015, a telephone survey questionnaire was developed for parents who had not registered their babies for further consultation - 3,239 randomly selected parents. RESULTS: The analysis revealed that the number of children examined at the second diagnostic level of the program is in fact much higher than the results of The Central Database show. The actual number is 83.6% as opposed to 55.8%. As a result of the telephone questionnaire some inaccuracies in the input data to the CDB were detected. The main errors in gathering the information for the CDB were incorrect OAE test result and no examination performed. C onclusion: In Poland the worst results (i.e. questionnaire results compared to CDB) for the attendance at the diagnostic level were shown in Pomorskie, Lubelskie, Mazowieckie and Podlaskie regions. In many cases there was a large discrepancy between the reality and the information in the CDB. The improvement of clarity concerning the CDB application is important in order to minimise the possibility of malformation in the CDB.

The report of the Polish Universal Neonatal Hearing Screening Program in 2016.

The Polish Universal Neonatal Hearing Screening Program (PUNHSP) has been carried out in Poland for 14 years. The main aim of this Program is to organize hearing screening tests and to gather the information about risk factors of hearing loss in almost all newborns in Poland. It consists of 496 centers at 3 referral levels. A total of 5 458 114 children had been registered in the Central Database (CDB) of PUNHSP by the 22nd of August 2017. Bilateral sensorineural hearing loss was the most frequently appearing hearing impairment in children. It was diagnosed in 260 cases in 2016. This report presents the most important results and conclusions concerning the running of the PUNHSP in 2016.

Analysis of follow-up at the diagnostic level in the Polish Universal Neonatal Hearing Screening Programme.

Objectives Routine analysis showed that between 1 June and 30 November 2014, only 47.6% of expected follow-up visits at the diagnostic level were registered in the Polish Universal Neonatal Hearing Screening Programme central database. We attempted to detect and analyse the reasons for this low percentage. Methods A telephone survey questionnaire was developed for parents whose children had not registered for consultation at the diagnostic level, or had not received a final diagnosis according to the programme database. Questions aimed to verify the database records and compare these with information received from and given to parents. From the 7888 children not registered at the diagnostic level, 3239 records were randomly selected, i.e. 52.4% of those who had been expected to attend. Results Questions were answered by 1950 parents (60.2% of the selected group). Of these, 52.1% ( n = 734) had attended for diagnostic tests, but this was not recorded in the database. The most common reasons for not attending were the long waiting time for the visit (36.09%), lack of referral to a visit (25.9%) and conscious parent decision (16.35%). Conclusion The telephone survey disclosed omissions in database registration, and that in fact 83.6% of children had attended at the diagnostic level.

Hearing impairment in premature newborns-Analysis based on the national hearing screening database in Poland.

OBJECTIVES: The incidence of sensorineural hearing loss is between 1 and 3 per 1000 in healthy neonates and 2-4 per 100 in high-risk infants. The national universal neonatal hearing screening carried out in Poland since 2002 enables selection of infants with suspicion and/or risk factors of hearing loss. In this study, we assessed the incidence and risk factors of hearing impairment in infants ≤33 weeks' gestational age (wga). METHODS: We analyzed the database of the Polish Universal Newborns Hearing Screening Program from 2010 to 2013. The study group involved 11438 infants born before 33 wga, the control group-1487730 infants. Screening was performed by means of transient evoked otoacoustic emissions. The risk factors of hearing loss were recorded. Infants who failed the screening test and/or had risk factors were referred for further audiological evaluation. RESULTS: Hearing deficit was diagnosed in 11% of infants ≤25 wga, 5% at 26-27 wga, 3.46% at 28 wga and 2-3% at 29-32 wga. In the control group the incidence of hearing deficit was 0.2% (2.87% with risk factors). The most important risk factors were craniofacial malformations, very low birth weight, low Apgar score and mechanical ventilation. Hearing screening was positive in 22.42% newborns ≤28 wga and 10% at 29-32 wga and in the control group. CONCLUSIONS: Hearing impairment is a severe consequence of prematurity. Its prevalence is inversely related to the maturity of the baby. Premature infants have many concomitant risk factors which influence the occurrence of hearing deficit.

The importance of polymorphic variants of collagen 1A2 gene (COL1A2) in the development of osteopenia and osteoporosis in postmenopausal women.

OBJECTIVES: Collagen type I plays an important role in the bone matrix and is encoded by COL1A2 (collagen type I alpha 2) gene that may be a potential candidate for osteoporotic fracture. The aim of this study is to determine whether EcoRI, Del38 and PvuII polymorphisms of COL1A2 are associated with the development of osteoporosis and osteopenia in post-menopausal Polish women. Moreover, analysis of relationship between frequency of COL1A2 gene polymorphic variants and clinical parameters of bone turnover and degree of osteoporosis was performed. MATERIAL AND METHODS: The study group comprised of women with osteoporosis (n = 90), osteopenia (n = 56) and healthy individuals (n = 56). The EcoRI, Del38 and PvuII polymorphisms in COL1A2 gene were detected by PCR-RFLP method. RESULTS: In women with osteoporosis the TT genotype of EcoRI polymorphism had the lowest Z-score value compared to other genotypes (p = 0.034). In case of Del28 polymorphism, there was a statistically significant correlation between lower BMI values and the DD genotype in women with osteopenia (p = 0.041). There was no statistically significant correlation between polymorphic variants of Del28 polymorphism and clinical parameters of women with osteoporosis. The analysis of PvuII polymorphism showed that in women with osteopenia the CC genotype had the lowest body weight compared to other genotypes (p = 0.039). PvuII polymorphism and clinical parameters in the group of women with osteoporosis had no statistically significant correlations. CONCLUSIONS: The analyzed COL1A2 polymorphisms seem to be related to osteoporosis development and their particular clinical parameters. Hence, the COL1A2 polymorphism may be a genetic risk factor related to the development of osteoporosis.

Skin flap complications after cochlear implantations.

The objective of the study was to analyse and present the surgical management strategy for major skin flap complications (MSFC) after cochlear implantations. Patients fitted with a titanium-silicone-coated implant of the same kind, operated on between 1994 and 2013 with a standardised procedure (1076 medical charts) were analysed. Analysis aimed to identify and study individuals with skin problems related to the cochlear implant treatment, i.e. requiring surgical treatment in hospital defined as MSFC and focused on incidence, risk factors and treatment of MSFC. MSFC were diagnosed in 1.76 % of patients: 2.06 % of children and 1.35 % of adults, 2.43 % after implantation with a long "C"-shaped incision and 1.28 % after short retroauricular incision. Registered risk factors included head trauma, acute otitis media, poor hygiene in children, and general comorbidities in adults. The primary intervention was dependent on skin complication severity and included revision surgery with wound closure over an implant (52.6 %) and revision surgery with explantation (47.4 %). Revision surgery without explantation was successful in 40 % and the most effective approach was debridement with a two-layer rotational flap. Explantation led to ultimate wound healing in all cases. Major skin flap complications after cochlear implantation are rare, but their treatment is complex and difficult. Revision surgery with resection of infected tissue, formation of a rotational two-layer flap preceded and supplemented by intensive targeted antibiotic therapy can be effective and should be the first treatment option. Spontaneous implant explantation, abscess formation or unsuccessful primary treatment necessitate implant removal as the ultimate solution.

Surgical, functional and audiological evaluation of new Baha(®) Attract system implantations.

Bone-anchored hearing aids are well-established solutions for treatment of hearing-impaired patients. However, classical systems with percutaneous abutments have disadvantages concerning aesthetics, hygiene and adverse soft tissue reactions. The study aimed to evaluate surgical, functional and audiological results of a new Baha(®) Attract system, in which the sound processor is attached by magnetic force. Twenty patients implanted with a Baha(®) Attract system were divided into two groups: A-bilateral mixed and conductive hearing loss, B-single-sided deafness, and evaluated during a 6-month follow-up. Parameters analysed comprised: (1) surgery and wound healing, (2) postoperative functional results (GBI, APHAB and BAHU questionnaires), (3) audiological results (free field speech in noise audiometry in two situations: with signal from implant side and from contralateral side). Obtained results revealed: mean time of surgery-44 min, soft tissue reduction-30 %, bone polishing-20 %, haematoma-10 %. Functional results showed: GBI total score-29.6 points, APHAB global score mean gain-23.5 %, BAHU 'good or very good' score for: aesthetic-85 %, hygiene-100 %, ease of placing the processor-100 %, stability of attraction-75 %. Audiological results-mean gain for the two analysed situations: 32.9 % (group A-36.5 %, group B-27.5 %). To conclude, the data obtained prove the safety and effectiveness of the Baha(®) Attract system in patients with conductive and mixed hearing loss as well as in patients with single-sided deafness. Cosmetic aspects are highly acceptable and the idea of Attract itself is important for patients with limited manual dexterity.

Vesicular Contact Reaction May Progress into Erythema Multiforme.

Dear Editor, Erythema multiforme is considered an acute skin condition, characterized by a self-limiting and sometimes recurrent course. It is regarded as a type IV hypersensitivity reaction associated with certain infections, medications, and other various triggers. Allergic contact dermatitis is in turn a delayed type of induced allergy as a result of cutaneous contact with a specific allergen to which the patient develops specific sensitivity. This type of cutaneous reaction is associated with inflammation manifesting with erythema, edema, and vesicles. A 27-year old female patient presented with a 3-day history of erythematous and vesicular lesions which developed 24 hours after cesarean section. Initially the lesions were localized in the area of surgery (mainly the abdomen and upper thighs) and on the next day progressed to the buttocks and lumbar area. The patient was referred to the Outpatient Clinic and was treated with antihistamines, but her dermatological state deteriorated rapidly. At the day of admission to the Department of Dermatology, numerous erythematous and vesicular lesions were present on the skin of the abdomen, thighs, and back (Figure 1, a), but the skin of the neck, chest, and extremities was also covered with erythematous and edematous patches. On the second day of hospitalization, we observed the evolution of lesions localized within the chest and extremities into an erythema multiforme-like targetoid eruption (Figure 1, b). Initially the patient was treated with intravenous injections of dexamethasone and ceftriaxone and orally with second-generation antihistamines (in four-fold doses), followed by intravenous metyloprednisolone pulse-therapy (total dose of 3 g). As the new vesicobullous lesions started to appear on the face and arms, we introduced cyclosporine A orally 400 mg daily. We could then observe gradual remission, but on the seventh day of hospitalization the patient developed a massive labial herpes simplex infection and had to be treated with acyclovir intravenously. Eight days after admission, we switched from intravenous metyloprednisolone to its oral formula. Diagnostic methods included: laboratory analyses (leukocytosis, neutrophilia, lymphopenia could be observed, and also serum CRP elevation). Pemphigoid gestationis was excluded on the basis of a direct immunofluorescence from perilesional skin and on the basis of indirect immunofluorescence and also serum analysis using ELISA for serum IgG antibodies to BP180-NC16A (courtesy of Prof. Marian Dmochowski). Histopathological examination revealed: massive edema of dermal papillae, leading to the formation of sub-epidermal vesicles; individual cell necrosis was observed in the upper epidermis. Within the dermis, a dense, perivascular inflammatory infiltrate was detected: the clinical picture suggested erythema multiforme. Another histopathological examination was performed at the University Clinic of Dermatology and Venereology in Magdeburg, courtesy of Prof. Dr. Harald Gollnick and Dr. Med. I. Franke; it also suggested the bullous form of erythema multiforme (dermal type). Three months after remission, the patient was hospitalized again to perform allergological diagnostics. Patch tests were performed with the European Baseline Series (Chemotechnique Diagnostics) supplemented with disinfectants and textiles used during surgical procedure. For patch testing, Finn Chambers on Scanpor were used. Results were recorded at 48 and 72 hour time points. According to the ICDRG (International Contact Dermatitis Research Group), reactions evaluated as ++ and +++ pluses were considered as positive and reaction evaluated as + plus was considered as doubtful. Patch testing revealed polyvalent contact allergy (Table I), (Figure 2a). The patient also reacted to Kodan Tinctur forte used as a skin disinfectant (contains brown dye LF 1889 - mixture of quinoline yellow, sunset yellow, brilliant black) (Figure 2b). It has to be emphasized, that patch test reading procedure was difficult due to patient's skin reactivity toward a plaster mounting Finn chambers. Literature data suggests that erythema multiforme may occasionally occur in conjunction with allergic contact dermatitis to various non-related substances including chemicals (epoxy-based compound, fragrances, epichlorydrine, bromofluorene), medications (antibiotics, acetaminophen, triamcinolone, bufexamac), plant-derived allergens (poison ivy, tea tree oil, red cedar essential oil), but also rubber ingredients and nickel. The severity of the reaction varies from mild erythema to generalized erythema multiforme or even toxic epidermal necrolysis (1,2,3,4). Lesions characteristic for erythema multiforme may appear during the episode of acute contact dermatitis or may follow a nearly resolving vesicular eczematous eruption. The pathomechanism Patomechanism of an erythema multiforme-like eruption developing in association with allergic contact dermatitis still remains unclear. Immune complex-mediated and T-cell-mediated reactions have been proposed as the cause. However, T-cell-mediated cellular mechanisms seems to be more likely, since generalized erythema multiforme often follows contact dermatitis, which is a type IV allergic reaction mediated by T cells (5,6). According to Bushkell et al. (7), an allergen penetration through the skin may result in a type III hypersensitivity reaction, with involvement of circulating immune complexes, and to confirm that, IgM, IgA, C3, and fibrin deposits are detected in some cases of targetoid lesions in erythema multiforme. On the other hand, Wiedemeyer et al. (8) suggest that contact allergens (i.e. paraphenylenodiamine) may be transported in peripheral blood mononuclear cells from the area of initial skin contact even to distant sites. According to Shiohara et al. (9) and Gonzalez-Delgado et al. (10), epidermal expression of adhesion molecule - 1 (ICAM-1) and the number of CD4+ T cells is increased within the iris lesions of erythema multiforme. Thus, it is possible that adhesion molecules may facilitate epidermal invasion of lymphocytes in these lesions, which is also the place of the expression of maintained allergen molecules. In conclusion, in the described case the causative factor also remained uncertain. The patient was found to have contact allergy to six haptens included in the European Baseline Series and also to a disinfectant used during cesarean section. Among these, both colophonium and formaldehyde are used in adhesives and glues or surface coatings. However, formaldehyde is mainly associated with this type of the reaction - in fact, hapten description supplied by Chemotechnique Diagnostics includes the information that "formaldehyde may produce erythema multiforme-like eruptions".

Exposure to volatile anaesthetics is not followed by a massive induction of single-strand DNA breaks in operation theatre personnel.

Volatile anaesthetics such as halothane, isoflurane and others were expected to produce a health challenge for operation room personnel because of prolonged occupational exposure to anaesthetic gases. To estimate a molecular background of adverse health effects, a cohort of 100 exposed individuals was studied by the single-cell gene electrophoresis (comet assay) test. DNA lesions in lymphocytes of the exposed group did not differ significantly compared with non-exposed blood donors. Then, the exposed group was further divided according to job position. A highest level of DNA lesions was established in nurses but without significant difference compared with other groups. When a time period of exposure was taken into account, a tendency to cumulate DNA lesions was found only in the group of anaesthesiologists. A very weak genotoxic effect established in this study is discussed in relation to DNA repair, adaptative response and potential self-elimination of sensitive individuals.

[The long-term results of treatment of Ménière's disease with intratympanic injections of gentamicin]. / Odlegle wyniki leczenia choroby Ménière'a za pomoca dobebenkowych wstrzykniec gentamycyny.

OBJECTIVE: To estimate the results of treatment of Ménière's disease with intratympanic injections of gentamicin. MATERIAL: 37 patients with defined, pharmacological treatment resistant Ménière's disease treated in Department of Otolaryngology and Laryngological Oncology of University of Medical Sciences in Poznan with intratympanic injections of gentamicin from 2001 to 2010 year. METHODS: Patients were injected intratympanic with 0.3 ml (12 mg) of gentamicin once or few times with 7 days or longer breaks and a number of injections depended on the reaction of the inner ear. We estimated the patients' subjective feelings and results of equilibrium and hearing organ examination in early (3 months) and late (2 years) period after treatment. RESULTS: Complete control of vertigo (class A) was achieved in 84.6%, and complete and essential control (class A and B) in 96.1%. Hearing deterioration usually mild or moderate was observed directly after treatment in 16.2% and after 2 years in 23% patients. The results of pure tone audiometry showed deterioration of hearing in 16.2% (early) and 26.9% (late). In 1 patient hearing deterioration was essential. CONCLUSIONS: Intratympanic injections of gentamicin are effective and not troublesome method of treatment of pharmacological treatment resistant Ménière's disease. In most of patients hearing can be preserved, but they should be always informed about possible risk of hearing deterioration. The number of injections and breaks between them depends on the effect of therapy and of expectations of patients.

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.