Two unrelated families with variable expression of Fraser syndrome due to the same pathogenic variant in the FRAS1 gene.

We report on two unrelated families of Polish origin with variable expression of Fraser syndrome (FS; MIM#219000) due to homozygosity for the same pathogenic variant, c.6963_6964dup, of FRAS1. In one family, the disorder presented with perinatal and prenatal lethality. One affected female from family 2 who was followed-up for 32 years, represented a relatively favorable long-term outcome. She displayed the typical craniofacial dysmorphism, including right cryptophthalmos, cutaneous syndactyly, abnormalities of the stomathognatic system, bilateral atresia of the external ear canals resulting in conductive hearing loss, and malformations of the larynx, spleen, kidney, and genitourinary tract. Her intellectual capacities were normal. Our observations illustrate that expression and severity of FS, even when caused by the same pathogenic variant, may be quite different ranging from a lethal disorder to a condition with multiple physical malformations but normal psychomotor development. In addition, we propose that the FRAS1 c.6963_6964dup variant may be a founder mutation in the Polish population. Therefore, it would be reasonable to test specifically for this variant first in any FS1 patient of Polish ancestry.

[Controversies in the treatment of acute otitis media]. / Kontrowersje w zakresie leczenia ostrego zapalenia ucha.

Acute otitis media (AOM) is one of the most frequent diseases in young children and also the most common indication for antibiotic prescribing. It impose substantial burden on pediatricians and general practitioners and leads to negative sequelae and complications in developing countries. Because of growing resistance to antibiotics of respiratory tract pathogens, and the great tendency of AOM to spontaneous resolution there is need for reduction of antibiotic treatment in this disease. The pros and cons of this therapy are presented in this paper. Indications for surgical interventions--adenoidectomy and tympanostomy tubes insertion in cases of recurrent acute otitis media are also presented.

[Clinical and pathological features of cholesteatoma in young children]. / Cechy kliniczne i patologiczne przewleklego zapalenia ucha z perlakiem u malych dzieci.

INTRODUCTION: Children cholesteatoma have been shown to demonstrate aggressive growth with greater extension and higher rates residual and recurrent disease compared with adults, due to anatomic and physiologic differences. AIM: This study aimed to determine the clinical and pathological features of cholesteatoma in young children less than 7 years of age. MATERIAL AND METHODS: Retrospective review of a group of 23 children (24 ears) who had cholesteatoma surgery between 1996 and 2004. Patients ages ranged from 1.8 to 7 years, follow up from 3.5 to 12 years (mean 7.4). Medical history (previous otorrhea, hearing loss) and contralateral ear status was analyzed. The extent of cholesteatoma and ossicular destruction for attic, pars tensa I and pars tensa II type and congenital of cholesteatoma was graded using of Saleh and Mills classification. Residual and recurrent disease depending on type of cholesteatoma, extent of the disease, type of surgery and contalateral ear status was assessed. RESULTS: Otorrhea and hearing loos are the most common clinical symptoms observed in 90% and 59% children. 70% of treated children had otitis media with effusion and/or its sequel in contralateral ear. 60% of acquired cholesteatomas were classified as S3 and S4 extension during surgery with ossicular destruction in 46% of attic cholestatoma. 75% operated ears had a signs of mucosa inflammation during surgery. Residual cholesteatoma was observed in 38.5% ears with attic cholesteatoma and in 25% with pars tensa I cholesteatoma. CONCLUSION: Acquired cholesteatoma in young children are diagnosed in the advanced stages with ossicular chain destruction. High rate of recidivism after surgery, particularly in attic cholesteatomas is observed. Most of operated ears have symptoms of inflammation of middle era mucosa during surgery and otitis media with effusion in contralateral ear and because of that need long-term follow up and constant observation.

[The status of contralateral ear in children with cholesteatoma]. / Stan ucha konralateralnego u dzieci z przewleklym zapaleniem ucha z perlakiem.

INTRODUCTION: Recently published data indicate that over 60% of patients with middle ear cholesteatoma (MEC) have concomitant changes in the contralateral ear (CE). Studies concerning the condition of the CE are few and rarely present the situation in the pediatric population. THE AIM OF THIS STUDY: was to assess the frequency of pathological changes occurring in the CE in children operated on MEC, as well as to determine their clinical evolution. MATERIAL AND METHOD: Retrospective analysis was performed on basis of medical records of 193 patients treated surgically for MEC in 1998-2008 at the Department of Pediatric Otolaryngology in Bialystok. The comparison of otoscopic evaluation before first operation with the latest examination was undertaken. The evolution of abnormalities was analyzed and the effect of age and type of cholesteatoma was assessed. RESULTS: The opposite ears were abnormal in 46.1% of patients. Most frequently observed changes in the CE were retraction pockets (23%). Cholesteatoma was found in 8 of 45 identified cases of retraction pocket. The incidence of abnormalities in the CE was not correlated with patients age or type of cholesteatoma. Cholesteatoma occurred on both sides in 9,3% of children. In these cases, the attic cholesteatoma was presented in 66.67%, tensa cholesteatoma in 16.67%. CONCLUSIONS: In patients with MEC contralateral ear should be systematically examined to detect early any abnormality, or to assess their evolution and take appropriate treatment.

[Propranolol for treatment of subglottic hemangioma]. / Zastosowanie propranololu w leczeniu naczyniaka podglosniowego.

Subglottic hemangiomas are extremely dangerous due to its location and rapid growth during the proliferative phase. Many different treatments are described but these methods are still not satisfactory. Recently propranolol has been used as a new option in hemangioma therapy. We describe a case of 6-week infant with subglottic hemangioma discovered direct laryngoscopy, presented with dyspnoea and inspiratory stridor. After oral propranolol administration all baseline airway symptoms had resolved and endoscopic examination demonstrated significant regression of the hemangioma. We suggest that the propranolol should be used as a first-line treatment in subglottic hemangiomas in children.

[Long term sequelae of otitis media with effusion during childhood]. / Trwale nastepstwa wysiekowego zapalenia ucha w wieku dzieciecym.

INTRODUCTION: Otitis media with effusion is the most common cause of hearing loss in children and myringotomy with tympanostomy tube insertion is recommended procedure to deal with the problem. The objective of the present study was to determine the results of treatment, incidence and prevalence of middle ear sequelae and hearing results among children with chronic otitis media with effusion who received standard treatment with tympanostomy tubes. MATERIAL AND METHODS: The group of 97 patients treated by tympanostomy tubes insertion in the years 1999-2001 was reevaluated after mean period of follow up 7.3 years. At the control examination videootoscopy and audiologic examinations were performed. Audiological assessment consisted of tympanometry and pure-tone thresholds of air and bone conduction. RESULTS: Recurrent otitis media with effusion requiring tube insertion occurred during follow up period in 23.7% of patients. At the control examination 16.5% of children had an ongoing otitis media or ventilation tube in place or tympanic membrane perforation. Most common tympanic membrane abnormality were focal atrophy (67.2% of ears) and myringosclerosis (39.5%) followed by retraction pockets of pars flaccida (29.9%) and tensa (9.6%). Mean pure-tone audiometric threshold were significantly higher in ears with tympanic membrane abnormality by the difference did not excide 5dB HL in ears with focal atrophy and myringosclerosis and 6.5 dB HL in ears with retraction pockets. CONCLUSIONS: As the percentage of recurrences after tympanostomy tubes insertion are not uncommon prolonged period of follow up of those children is recommended. Although ventilation tubes have proven very effective in improving hearing in short term, they have not proven effective in preventing long-term sequelae of tympanic membrane and some degree of hearing loss. The decision about surgical treatment should be taken cautiously taking into account the chance of spontaneous resolution.

[Influence of tympanic membrane changes on immittance and extended frequency audiometric findings]. / Wplyw zmian strukturalnych blony bebenkowej na wyniki audiometrii impedancyjnej i tonalnej w rozszerzonym zakresie czestotliwosci.

INTRODUCTION: The aim of the study is to analyze the relationship between otoscopic tympanic membrane abnormalities, results of impedanace and extended-high-frequency audiometry in subjects with history of treatment of secretory otitis media (SOM) and ventilation tube insertion. MATERIAL AND METHODS: 97 subjects treated because of SOM were examined in years 1999-2000. Videootoscopic examinations, extended-high-frequency audiometry and tympanometry with ispilateral acoustic reflex were performed in each patient. The results were analysed in three groups: no otoscopic abnormalities (BZO), retraction pockets (KR) and atrophy and myringosclerosis (AM). The results were compared to otologicaly healthy control group in the same age. RESULTS: The most common tympanic membrane abnormality were focal atrophy (64.7%) of ears and myringosclerosis (37.2%). Mean pure-tone audiometric threshold were significantly higher in groups KR and AM than in control and BZO groups. Low degree of positive correlation was found between the presence of myringosclerosis and atrophy and audiometric thresholds above 1 kHz. No such correlation was observed with the presence of retraction pockets. In the BZO group middle ear admittance was observed significantly higher than in control group. The absent ipsilateral stapedial reflex was observed in 10.8% ears in BZO group, 16.9% in AM and 33.3% in KR. No correlation was found between the parameters of tympanometric evaluation and results of extended-high-frequency audiometry. CONCLUSIONS: In ears treated because of SOM with ventilation tube insertion the middle ear compliance is higher. In the presence of myringosclerosis and atrophy higher audiometric thresholds are observed. Tympanic membrane abnormalities have been more clearly indicated by the absent ipsilateral stapedial reflex than tympanometry.

Selected cytokines in hypertrophic adenoids in children suffering from otitis media with effusion.

OBJECTIVES: The aim of the current study was to assess the levels of MMP-8, MMP-9 and TIMP-1 in the group of children with adenoids who suffered from exudative otitis media. METHODS: The study included 20 patients (10 females and 10 males) with adenoid hypertrophy coexisting with otitis media with effusion. The reference group included 24 patients (10 females and 14 males) with adenoid hypertrophy without otitis media. The levels of MMP-8, MMP-9 and TIMP-1 were determined in supernatants obtained from phytohemagglutinin (PHA)-stimulated cell cultures of the tonsils, using commercial enzyme-linked immunosorbent assay kits (R@D Systems, USA). RESULTS: The median MMP-8, MMP-9 and TIMP-1 concentrations (220.8 ng/mL, 311.1 ng/mL, 53.5 ng/mL, respectively) in the study group were significantly higher (p = 0.000, p = 0.000, p = 0.048, respectively) than those in the reference group (93.5 ng/mL, 112.5 ng/mL, 36.95 ng/mL, respectively). ROC analysis revealed that the area under a curve (AUC) for both metalloproteinases MMP-8 and MMP-9 was 1 with a diagnostic sensitivity of 100% and diagnostic specificity of 95.8%, as compared to 0.690 for TIMP-1. Significant differences were found between the AUC for MMP-8 and TIMP-1 and MMP-9 and TIMP-1 (p < 0.001 for both comparisons). CONCLUSIONS: The changes in the concentrations of MMP-8, MMP-9 and TIMP-1 may indicate an increased remodeling of the extracellular matrix in children with adenoid hypertrophy and otitis media with effusion. The findings can have clinical as well as diagnostic utility. Determination of MMP-8 and MMP-9 may help qualify a child for adenoidectomy and differentiate pediatric patients affected by adenoid hypertrophy with and without otitis media.

[Dendritic cells in hypertrophied adenoid at children with otitis media with effusion]. / Komórki dendrytyczne w tkance przeroslych migdalków gardlowych u dzieci chorych na wysiekowe zapalenie ucha srodkowego.

INTRODUCTION: The adenoids are organized as lymphoepithelial structures that play an important role in protecting both the upper respiratory and alimentary tract regions. This functions requires dendritic cells (DC) which are one of the major populations of immune cells. Due to the presence of specific receptors (DC) are able to respond to both intra- and extracellular antigens. Dendritic cells activating immunological response in tonsil contribute formation immunologic competent cells on necessity of rolling inflammatory process in middle ear. AIM OF STUDY: An investigation was executed in hypertrophied adenoids with or without otitis media with effusion. METHODS: By flow cytometry percentage of CD11c+ myeloid DC and 123+ plasmacytoid DC in hypertrophied adenoid and hypertrophied adenoid and otitis media with effusion was analyzed. RESULTS: The percentage of CD11c+ myeloid DC and 123+ plasmacytoid DC was similar in hypertrophied adenoid and otitis media with effusion compored to the control group. CONCLUSIONS: Our data show that part of dendritic cells has not on course of inflammatory process influence rolling in middle ear.

[Evaluation of percentage of lymphocytes B with expression of co-receptors CD 40, CD22 and CD72 in hypertrophied adenoid at children with otitis media with effusion]. / Ocena odsetka limfocytów B z ekspresja koreceptorów CD40, CD22 i CD72 w przeroslych migdalkach gardlowych dzieci chorych na wysiekowe zapalenie ucha.

INTRODUCTION: In hypertrophied adenoid lymphocytes B make up about 60% all lymphocytes. When the lymphocytes B come in interaction with antigens this membranes signal be passed through their receptor (BCR) to interior of cell. This signal affect modulation on gene expression, activation from which depends activation, anergy or apoptosis of lymphocyte B. Accompany BCR co-receptors regulate his functions influence stimulate or inhibitive. To the most important co-receptors stepping out on lymphocyte B belong: CD40, CD22, CD72. AIM OF STUDY: The aim of study was evaluation of lymphocytes B (CD19) with co-expression with CD72 and CD40 receptors in hypertrophied adenoid with at children with otitis media with effusion. MATERIAL: An investigation was executed in hypertrophied adenoids with or without otitis media with effusion. METHODS: By flow cytometry percentage of lymphocytes B with co-receptors CD 40, CD22 and CD72 in was analyzed. RESULTS: The percentages of CD19+CD72+ lymphocytes in the group of children with adenoid hypertrophy and exudative otitis media were lower as compared to the reference group. However, the percentages of CD19+CD22+, CD19+CD40+ in the study group was approximate to the reference group. CONCLUSIONS: The lower percentage of lymphocytes B CD72 + near approximate percentages of lymphocytes B CD40+ and BCD22+ at children with otitis media with effusion can be the cause of incorrect humoral response in hypertrophied adenoid at children. Maybe it is cause reduced spontaneous production IgA and IgG through lymphocyte at children with otitis media with effusion.

[Expression CD27 on T and B lymphocytes in hypertrophied adenoids at children with otitis media with effusion]. / Ekspresja receptora CD27 na limfocytach T i B w tkance przeroslych migdalków gardlowych u dzieci chorych na wysiekowe zapalenie ucha srodkowego.

INTRODUCTION: Adenoid has particular meaning to develop of immunological response to inflammations in upper respiratory inclusive middle ear. The mining of antigen CD27 on lymphocytes T and B in creation of memory cells is still unclear. AIM: CD27 on lymphocytes T and B has a crucial role in development of immune response against inflammatory state. Aim of this study was evaluation functions of lymhocytes with expression CD27 in hypertrophied adenoid in children with otitis media with effusion. MATERIAL AND METHODS: [corrected] We tested 24 children in examine group with hypertrophied adenoid and otitis media with effusion (OME), and 29 children in comparative group only with hypertrophied adenoid (HA). We also divided both groups into two groups, above 5 and over 5 years old. We made the research by flow cytometry method. We used anty-CD19, anty-CD3, anty-CD5 and anty-CD27 monoclonal antibodies to examinations. RESULTS: We showed the lowest percentage lymphocytes B CD5+ with expression of CD27 in subgroup older children with otitis media with effusion (O.M.E. 22.43 +/- 2.66%) in compartative to younger children in the same group (15.96 +/- 5.31%; p < 0.005) and to older comparative subgroup (H.A. 15.50 +/- 8.03; p < 0.001). Percentages of B CD5 cells with expression of CD27 was statistical lower in examinated group (7.25 +/- 3.81) than in comparative group (H.A. 16.26 +/- 5.82; p < 0.04). In group with hypertrophied adenoid showed higher percentages of CD5-CD27+CD19+ cells both in younger and older supgroup than in equivalent supgroups with O.M.E. CONCLUSIONS: Iteraction of CD27-CD70 regulates many functions of T and B lymphocytes. It seems that disorders humoral immunologucal answer in hypertrophied adenoids are supported to develop of otitis media with effusion.

[Expression of chosen chemokine receptors on the Th lymphocytes in children with hypertrophied adenoids with otitis media with effusion]. / Ekspresja wybranych receptorów chemokin na limfocytach Th przeroslych migdalków gardlowych w wysiekowym zapaleniu ucha srodkowego.

INTRODUCTION: Chemokine receptors play a crucial role in the recruitment of leucocytes into inflamed tissue from secondary lymphoid organs. AIM OF THIS STUDY: was evaluation of the percentage Th (CD4+) lymphocytes with expression of chemokine receptors: CCR3, CCR4, and CCR5 in hypertrophied adenois tissue in children with otitis media with effusion. MATERIAL AND METHODS: 36 children with otitis media with effusion and 25 children with hypertrophied adenoids were tested. Expression of chemokine receptors CCR3, CCR4 and CCR5 on T CD4 lymphocytes of hypertrophied adenoid's tissue was estimated by flow cytometry method. RESULTS: Average percentage of T CD4+ lymphocytes with expression CCR4 in hypertrophied adenoid in children group with otitis media with effusion (OME = 64.11%) was significantly higher than in comparative group with hypertrophied adenoid (HA = 75.05%, p < 0.04). We showed statistically lower percentage of CD3+CD4+ cells with expression CCR3 in examinated group (OME = 47.19%) than HA group (62.66%; p < 0.003). CONCLUSION: Results suggests that intensify of humoral immunological answer in hypertrophied adenoid depends on Th2 lymphocytes with expression CCR3 and CCR4 chemokine receptors in course of inflammatory state in middle ear.

Expression of collagens type I and V in healing rat's tympanic membrane.

OBJECTIVES: Our preliminary study performed on perforated rat's tympanic membrane (TM) using Rat Wound Healing RT2 Profiler PCR Array showed significantly increased levels of mRNA for collagens type I and V. Enhanced expression of those genes does not assure that their protein products are indeed present, and in what quantity. Therefore, this study was undertaken to analyze the collagen type I and V content in the healing TM. METHODS: Sixty rats were used, of which 10 served as controls and the others had their TM perforated. The experimental animals were divided into five subgroups on the basis of time points (03, 06, 09, 14, 20 day after injury). Videootoscopy and histology were employed to assess the morphology of the healing process. The expression of collagen type I and V was evaluated using Western blot analysis. Tissue localization of collagens was determined by the immunofluorescence method. RESULTS: The collagen type I expression was three times higher on the third day after injury and remained on that level for whole period of observation, up to day 20. The increase of the collagen type V expression was gradual, reaching the highest level on day 14 following injury. In comparison to the control TM statistically significant increase in the level of expression was observed starting from day 09 to the end of observation period. In healing TM immunofluorescent labeling of collagen type I and V was seen on the surface of remnants of previous lamina propria and in the loose proliferating fibrous tissue. On day 20 immunofluorescence was present mainly on the surface of thin connective tissue layers forming the scar in the place of previous perforation. CONCLUSION: Although the collagens type I and V are present only in subepithelial layer in the normal rat's TM they play significant role in TM healing process.

Adenoid as a source of lymphocytes in the surface secretions of nasopharynx.

OBJECTIVE: The significance and protective efficacy of surface secretions on mucosal membranes in the upper airways are well recognized. The aim of our study was to reveal the role of the adenoids as a source of cellular components in the mucosal secretion. METHODS: The adenoid removed because of its hypertrophy and the samples of surface secretions taken by "imprint method" described by Ebenfelt et al. from the group of 38 children were examined by flow cytometry. The lymphocyte subsets with following antigens: CD3, CD4, CD8, CD19, CD23, CD16+56, CD45RA, CD45RO, HLA-DR were analyzed. Two groups were studied: (1) AH, children with adenoid hyperplasia without otitis media; (2) OME, children with adenoid hyperplasia and persistent otitis media with effusion. Age-related changes in percentages of lymphocyte subpopulations were analyzed. RESULTS: The percentage of lymphocyte subsets in adenoid and secretion were similar, however, in the surface secretion there was higher proportion of lymphocytes B and NK and lower of T. There was no correlation between percentages of lymphocyte in adenoid and surface secretion except for lymphocytes Th with memory phenotype and lymphocytes B with marker CD23(+). In adenoid the percentage of T and Th lymphocytes increased with age and the percentage of B lymphocytes decreased. In the secretion age-related changes were less pronounced and only the percentage of lymphocytes B CD23(+) showed positive correlation with age. In cases of otitis media with effusion there was higher proportion of lymphocytes B (CD19(+) and CD19(+)CD23(+)) and lower of lymphocytes Ts and Th with naive phenotype in the secretion. In adenoid however, the cases of OME revealed lower percentage of lymphocytes B and higher of lymphocytes Ts. CONCLUSIONS: Similar proportions of lymphocyte subpopulations in surface secretion and in adenoid imply that adenoid is the source of those cells. However, lack of correlation between the percentages of lymphocytes in secretion and in adenoid suggest that the process of lymphocyte migration has active and selective character.

[Acoustic rhinometry evaluation of adenoid hypertrophy and adenoidectomy efficacy]. / Zastosowanie rynometrii akustycznej w ocenie przerostu migdalka gardlowego oraz skutecznosci zabiegu adenoidectomii.

INTRODUCTION: Adenoid hypertrophy is a problem of more than 1/3 of children and is the predominant reason of otolaryngology consultations in this population. Diagnosis and surgical qualification are based on physical examination and other methods introduced more than 100 years ago. Development of endoscopic techniques has been found this method to be a gold standard in adenoid assessment up to date. However, the method is difficult to performed in some young patient because of their invasiveness. Acoustic assessment of nosopharyngs seems to be a new promising method. THE AIM OF THE STUDY: Were an assessment of nasal obstruction due to adenoid hypertrophy in group of patients referring to adenoidectomy and an objective evaluation of changes in the volume of the nasopharynx after adenoidectomy. MATERIAL AND METHODS: The examination was carried on the group of 30 patients aged 5-10 y.o. with adenoid hypertrophy admitted for adenoidectomy. The control group consisted of 10 children free of otolaryngological problems. All studied individuals had acoustic rhinometry performed and additionally, endoscopic method such as rhynofiberoscopy and endoscopy of nasopharyngs were introduced in the patient's group. RESULTS: The study showed that children with adenoid hypertrophy have statistically significant reduction of nasopharyngeal volume (NPV) vs control group. Adenoidectomy increases the NPV parameter and makes it equal to control group. CONCLUSIONS: The acoustic rhynometry seems to be a promising method in assessment of nasopharyngeal volume. This and further studies may help to reduce the number of "unnecessary" adenoidectomies, by making standards for NPV in different group of age.

[Myringoplasty in children--success factors]. / Myringoplastyka u dzieci--czynniki wplywajace na wynik leczenia chirurgicznego.

OBJECTIVE: Myringoplasty is successfully performed in children since many years. However still considerable controversy surrounds the subject of proper timing of surgery and other factors influencing the success rate. The aim of this study was to assess the results of myringoplasty in children and to determine which factors influence the postoperative results. MATERIAL AND METHODS: Retrospective study of the anatomic and functional results of 82 consecutive myringoplasties performed in children between 1996-2004. Seventy one children (mean age 13 +/- 2,9 years, range 6,4-18 years) who had undergone myringoplasty because of central perforation or retraction pocket in the pars tensa were evaluated. All of the children underwent myringoplasy using endaural or post-auricular approach and underlay technique. Temporalis fascia, perichondrim or cartilage were used as a graft material. The results were assessed on the basis of otoscopic and audiometric examination pre and postoperatively. Following factors have been analyzed to assess their influence on the success rate: age, size and site of perforation, status of the operated ear (dry/discharging), status of the contralateral ear. RESULTS: Closure of the perforation was achieved in 91,5% of children. A good anatomic outcome was observed in 87,8%. The Air Bone Gap between 0-20 dB was observed in 95,6% of patients. Age below 10 years was found to significantly affect the results of surgery. The rate of success was lower in cases with pathological process in the contralateral ear but the difference was not statistically significant. CONCLUSIONS: Myringoplasty with underlay grafting of the fascia temporalis or perichondrium gives good anatomic and functional results in children. The results are significantly worst in children below 10 years of age. Pathological changes in the contralateral ear influence the risk of an abnormal postoperative tympanic membrane.

[Acute otitis media--rational antibiotic treatment]. / Ostre zapalenie ucha--racjonalna antybiotykoterapia.

Acute otitis media is the most common reason for which antibiotics are prescribed to children. Because use of antibiotics has been implicated in the selection and progression of resistance among upper respiratory tract pathogens a concerted effort has been established to promote their judicious use. To reduce use of antibiotics recently published guidelines recommend observation option (watchful waiting) to children >2 years of age and non severe illness. Amoxicillin at conventional or high-doses remains an appropriate choice for first-line therapy. Appropriate options for second-line therapy include high-dose amoxicillin/clavulanate and ceftriaxon. Tympanocentesis is useful for identifying causative pathogen and may be beneficial for those who have failed second-line therapy. Natural history of untreated otitis media, most common pathogens and their resistance pattern are presented in the paper.

[Video endoscopic analysis of eustachian tube function in children with middle ear pathology]. / Wideoendoskopowa ocena funkcji gardlowego ujscia trabki sluchowej u dzieci z chorobami ucha srodkowego.

Eustachian tube (ET) dysfunction play an important role in the development, persistence and recurrence of otitis media with effusion (OME) and chronic otitis. Evaluation of the type of obstruction in the cartilaginous portion of ET is important for decision concerning methods of treatment and prognosis of surgical outcome. The aim of this study was to identify characteristics of dynamic function of the pharyngeal orifice of ET in children with OME and chronic otitis by video endoscopy. Transnasal endoscopic examination of the nasopharyngeal opening of ET during swallowing was performed on 21 children - 13 with OME, 8 with chronic otitis. Video recording were made for dynamic slow-motion analysis of ET dilation and closing processes. Most cases of ET dysfunction in children were obstrucive (81%), associated with mucosal oedema and hyperplasia. Dynamic type of dysfunction is caused by the reduced movement of the tensor veli palatini. Dynamic video analysis is useful in the identify type of ET dysfunction in children. Obstructive dysfuntion need diagnostic procedures for chronic infection, nasopharyngeal reflux and alergic diseases.

[Analysis of usefulness of central auditory processes tests in children]. / Analiza przydatnosci badan oceniajacych centralne procesy sluchowe u dzieci.

INTRODUCTION: Diagnostic of central auditory processing should be done in some group of children, especially with learning disabilities. MATERIAL AND METHODS: The audiological examination was performed in the group (n=24) of pupils from first class of secondary school (13-14 years) without any otological and neurological problems. The battery of audiological tests was used: pure tone, impedance and speech audiometry, two tests with low redundant speech: filtered speech (FS) and compressed sentences, sequencing tasks (FPT, DPT) and ABR. Normal answer: in FS - 80% correctly repeated words, FTP and DPT 75% correctly repeated sequences. RESULTS: Results of this examination were referred to the scores of test after primary school (max 40 points). Children were divided into 3 groups: group III (33-40 points), group II (21-32), group I (0-20). Because seven children havenít had stapedial reflex we put them into special group - NR group. Tympanometry and pure tone audiometry were normal in all children. Speech audiometry was performed in intensity 30dB and correct answers were repeated in 80-100%. In compressed sentences test answers of 22 children were normal. The average dyscrimination score were: FS g. III 90%, g. II 84,3%, g. I 77,5%; FPT 89,2%, 72,9%, 62,5%; DPT 82,5%, 71,4%, 62,5%. The percentage of normal answer were: FS g. III 100%, g. II 100%, g.I 75%, FPT 100%, 42,9%, 25%, DPT 66,7%, 42,9%, 25%. The comparison between group I-III and NR was performed. The average dyscrimination score FS g. I-III 84,7%, g. NR 71,4%; FPT g. I-III 76,2%, g. NR 72,1%; DPT g. I-III 73,5%, g. NR 50,7%. The percentage of normal answer were: FS g. I-III 94,1%, g. NR 42,9%; FPT g. I-III 58,8%, g. NR 28,6%, DPT g. I-III 47,1%, g. NR 28,6%. CONCLUSIONS: In central auditory tests (FS, FPT, DPT) worse results were in children without stapedial reflex. Good pupils (group III) had better results than poor ones (group I). The better results in audiological tests, the better scores in school test.

[The high position of the jugular bulb in children]. / Wysoko polozona opuszka zyly szyjnej u dzieci.

INTRODUCTION: Variations of the vascular structures related to the temporal bone may cause important problems in diagnosis, treatment planing and surgery. A high jugular bulb is not an uncommon anomaly. It has been reported to occur in up to 6% of temporal bones. A high jugular bulb is often discovered as an asymptomatic, incidental finding. Case reports indicate that abnormal location of the jugular bulb may be the cause of some audiological problems. Conductive hearing loss in association with this anomaly may occur, but has been reported infrequently in the literature. MATERIAL AND METHODS: Two children with high jugular bulb are presented. RESULTS: One boy presented as a profuse bleeding from the right middle ear space after raising a tympanomeatal flap during surgery due to bilateral retraction pockets. Another one had unilateral conductive hearing loss with abberant, bluish mass seen behind tympanic membrane. CT revealed high position of jugular bulb in those patients. Results of otoscopic, audiological and radiological examinations are presented. CONCLUSION: ENT specialists and otologic surgeons should be aware of this entity to perform differential diagnosis of conductive hearing loss and avoid penetration of the jugular bulb during surgery.