Erratum to: Hearing preservation in children with electric-acoustic stimulation after cochlear implantation : Outcome after electrode insertion with minimal insertion trauma.

The article Hearing preservation in children with electric-acoustic stimulation after cochlear implantation-Outcome after electrode insertion with minimal insertion trauma, written by T. Rader, A. Bohnert, C. Matthias, D. Koutsimpelas, M­A. Kainz, S. Strieth was originally published electronically.

The new Mainz speech test for children 3-7 years old (MATCH) : Design, standardization, and validation.

OBJECTIVE: To develop a new, German, age-appropriate speech audiometry test for children, by using 26 nouns that are most likely part of the lexicon of 2-year-olds. The test is a picture-pointing task with a four-option non-forced choice method. MATERIALS AND METHODS: In total, 179 children aged 2;11 to 6;9 y were included for standardizing and validating the speech test. Of these, 51 had a hearing impairment in both ears ranging up to 90 dB hearing level (HL). The normal-hearing collective was divided into three groups according to age. For each group, the speech reception threshold (SRT) and the slope of the psychometric function of intelligibility were determined. For validation, the test-retest reliability was measured in 85 ears, and the correlation between the pure tone average (PTA) at 0.5, 1, 2, and 4 kHz and the SRT was measured in 86 ears. RESULTS: The sound spectrum of the 26 items was in good accordance with the international long-term speech spectrum, and the relative frequency of phonemes matched the distribution of the 50 more frequent German phonemes. The SRTs ranged from 24.6 ± 0.6 dB sound pressure level (SPL) for the oldest group (> 5.5 y) to 29.3 ± 1.3 dB SPL for the youngest group (< 4.25 y). The slopes of the psychometric function ranged from 4.3 ± 0.5%/dB for the oldest group to 2.6 ± 0.4%/dB for the youngest. The test and retest showed good correlation (r  = 0.89, p < 0.0001) as did the PTA and SRT (r = 0.84, p < 0.0001). CONCLUSION: The newly developed Mainz speech-test effectively measures age-related speech perception from the age of three years.

[The new Mainz speech test for children aged 3-7 years (MATCH) : Design, standardization, and validation. German version]. / Der neue Mainzer Sprachtest für Kinder 3­7 Jahre (MATCH) : Design, Normierung und Validierung.

OBJECTIVE: A new German speech audiometry test was developed using 26 nouns that are most likely part of the lexicon of 2­year-old children. The test was a picture-pointing task using a four-alternative non-forced choice method. MATERIALS AND METHODS: In total, 179 children aged 2 years 11 months to 6 years 9 months were included to standardize and validate the speech test. Of these children, 51 had a hearing impairment in both ears ranging up to 90 dB hearing level. The normal-hearing collective was divided into three groups according to age. For each group, the speech reception threshold (SRT) and the slope of the psychometric function of intelligibility were determined. For validation, the test-retest reliability was measured in 85 ears and the correlation between the pure-tone average (PTA) at 0.5, 1, 2, and 4 kHz and the SRT was determined in 86 ears. RESULTS: The sound spectrum of the 26 items was in good accordance with the international long-term speech spectrum and with the distribution of the 50 most frequent German phonemes. The SRT ranged from 24.6 ± 0.6 dB sound pressure level (SPL) for the oldest group (>5.5 years) to 29.3 ± 1.3 dB SPL for the youngest group (<4.25 years). The slopes of the psychometric function ranged from 4.3 ± 0.5%/dB for the oldest group to 2.6 ± 0.4%/dB for the youngest group. The test and retest showed good correlation (r = 0.89, p < 0.0001), as did the PTA and SRT (r = 0.84, p < 0.0001). CONCLUSION: The newly developed test effectively measures age-related speech perception from the age of 2 years 11 months.

[Hearing aid provision in children]. / Hörgeräteversorgung im Kindesalter.

Achieving high-quality hearing aid fitting in children is an interdisciplinary task and a challenge for all involved specialist disciplines. According to quality agreements (standards), comparative hearing aid fitting is mandatory prior to prescription. Furthermore, RECD measurements and, in older children, in-situ measurements should be applied on a regular basis for finetuning and adjustment of the hearing aid fitting. ENT physicians, phoniatricians and pediatric audiologists, pediatric acousticians, teachers of the hearing impaired, and special educational needs teachers should work together in an interdisciplinary approach.

Hearing preservation in children with electric-acoustic stimulation after cochlear implantation : Outcome after electrode insertion with minimal insertion trauma.

BACKGROUND: Cochlear implantation in patients with functional residual low-frequency hearing is performed according to an established hearing-preserving surgical technique in order to cause minimal trauma of inner ear structures. Due to the increasing number of cochlear implants in children, the preservation of residual hearing is becoming increasingly important in this patient collective. OBJECTIVES: Short- and mid-term hearing preservation outcome in pediatric patients is investigated. MATERIALS AND METHODS: A group of 9 children (12 ears) between 5 and 12 years of age were examined after hearing-assisted cochlear implantation with respect to the pure tone audiometric thresholds. Retrospectively, short-term hearing preservation (up to 3 months after surgery) was examined. In a subgroup of 5 children, mid-term hearing preservation (7.5 to 16 months after surgery) was also analyzed. The mean values of hearing preserved (HL%) and hearing loss (HL) due to electrode insertion were calculated as measured values. RESULTS: In the whole group, the mean values of the preoperative PTAlow were 29.8 dB and the short-term postoperative PTAlow 42.6 dB. The mean value of the HL% was 73.6%, corresponding to an HL of 9.4 dB. In the subgroup, the mean PTAlow postoperatively was 46.0 dB in the mid-term and the HL% at 80.7% with a HL of 6.6 dB. CONCLUSIONS: The results in children are consistent with the results in adults. Electric-acoustic stimulation (EAS) should be used in the treatment of children with existing low-frequency residual hearing, as good residual hearing preservation can also be achieved in children after implantation.

[Hearing preservation in children with electric-acoustic stimulation after cochlear implantation : Outcome after electrode insertion with minimal insertion trauma (German version)]. / Hörerhalt bei Kindern mit elektrisch-akustischer Stimulation nach Cochleaimplantation : Ergebnisse nach Elektrodeninsertion mit minimalem Insertionstrauma.

BACKGROUND: Cochlear implantation in patients with functional residual low-frequency hearing is performed according to an established hearing-preserving surgical technique in order to cause minimal trauma of inner ear structures. Due to the increasing number of cochlear implants in children, the preservation of residual hearing is becoming increasingly important in this patient collective. OBJECTIVES: Short- and mid-term hearing preservation outcome in pediatric patients is investigated. MATERIALS AND METHODS: A group of 9 children (12 ears) between 5 and 12 years of age were examined after hearing-assisted cochlear implantation with respect to the pure tone audiometric thresholds. Retrospectively, short-term hearing preservation (up to 3 months after surgery) was examined. In a subgroup of 5 children, mid-term hearing preservation (7.5 to 16 months after surgery) was also analyzed. The mean values of hearing preserved (HL%) and hearing loss (HL) due to electrode insertion were calculated as measured values. RESULTS: In the whole group, the mean values of the preoperative PTAlow were 29.8 dB and the short-term postoperative PTAlow 42.6 dB. The mean value of the HL% was 73.6%, corresponding to an HL of 9.4 dB. In the subgroup, the mean PTAlow postoperatively was 46.0 dB in the mid-term and the HL% at 80.7% with a HL of 6.6 dB. CONCLUSIONS: The results in children are consistent with the results in adults. Electric-acoustic stimulation (EAS) should be used in the treatment of children with existing low-frequency residual hearing, as good residual hearing preservation can also be achieved in children after implantation.

Links between the organization of the family home environment and child obesity: a systematic review.

Due to increasingly high rates of child overweight and obesity, it is important to identify risk and protective factors that may inform more effective prevention and intervention. The degree of organization in the family home environment is a studied, but not well-specified, factor that may impact child weight. Prior research on household organization has examined an array of constructs, including family routines, limit setting, household chaos, crowding and the broad home environment. This study systematically reviews literature on organization within the family home environment and weight among children ages 2-12. Six hundred thirty-seven studies were reviewed by four coders for eligibility, and 32 studies were included in the final synthesis. Overall, 84% of studies provided evidence for relations between at least one indicator of organization within the family home environment and child weight. Studies provided compelling evidence across several constructs, suggesting that the relevance of household organization to child weight extends beyond a single indicator. Directions for future work include (i) examining the mediating role of health behaviours, (ii) examining the moderating role of socioeconomic factors, (iii) broadening this evidence base across cultures and nationalities and (iv) integrating constructs to develop a comprehensive model of organization within the home environment.

Becoming American, becoming obese? A systematic review of acculturation and weight among Latino youth.

Latino youth have the highest rate of overweight and obesity across ethnic and racial groups, placing these individuals at increased risk for a variety of negative immediate and long-term health outcomes. Many studies have shown that acculturative factors play a role in this process for adults, while less is known about the impact of these factors for children and adolescents. This study systematically reviews the current literature on acculturative factors and obesity among Latino children. Three hundred and seventy-nine studies were independently reviewed by two coders for eligibility. Twenty-nine studies met eligibility criteria and were included in the final review. Results indicated that relations between acculturation and obesity among Latino children are equivocal. Across studies reviewed, the significance and directionality of this relation differed. Heterogeneity across studies reviewed, including age, specific population and measures used for assessing acculturation, likely contributed to the mixed results. To provide greater clarity on the role of acculturative factors on obesity, future studies should (i) utilize a longitudinal design; (ii) control for potential confounding factors such as socioeconomic status; and (iii) examine potential moderating and mediating influences.

Reductions in physical pain predict lower risk of relapse following alcohol treatment.

OBJECTIVE: Physical pain is considered a potential predictor of relapse in alcohol-dependent individuals after treatment. The aim of this study was to evaluate whether reductions in pain level during the follow-up period after treatment were associated with lower relapse risk. METHOD: A sample of 366 participants was recruited from alcohol treatment centers in Warsaw, Poland. At baseline, information was obtained about pain level, demographics, childhood abuse, impulsivity, depressive symptoms, severity of alcohol and sleep problems. After finishing the alcohol treatment program, patients were followed for 12 months and alcohol drinking (relapse) as well as pain severity were evaluated. RESULTS: In the followed-up group, 29.5% of patients confirmed that they drank any alcohol during past 4 weeks. Comparing follow-up to baseline pain, 48.6% of subjects reported an increased severity of pain, 28.8% reported the same level of pain, 22.6% reported decreased level of pain. There was a significant association between the decrease in level of pain and the lower risk of relapse. Other factors associated with relapse during 4 weeks prior to the follow-up were baseline severity of depressive symptoms, low baseline social support and number of drinking days during 4 weeks prior to entering treatment. In multivariate analysis, a decrease in pain level was associated with a lower likelihood of relapse (OR=0.159; 95%CI:0.04-0.62; p=0.008) even when controlled for other factors associated with relapse. CONCLUSIONS: Decreases in pain level following treatment for alcohol dependence are associated with, and may contribute to, a lower risk of alcohol relapse.

Understanding subtypes of inner-city drug users with a latent class approach.

AIMS: We empirically identified subtypes of inner-city users of heroin and cocaine based on type of drug used and route of administration. METHOD: The sample was recruited from the communities in Baltimore, MD (SHIELD study) and consisted of 1061 participants who used heroin and or cocaine in the past 6 months on a weekly basis or more. Latent class analysis (LCA) was used to identify subtypes of drug users based on type of drug and route of administration. Logistic regression was used to compare the subtypes on depressive symptoms, injection risk and drug network compositions. FINDINGS: Inner-city drug users were classified into five subtypes: three subtypes of injection drug users (IDUs) [heroin injecting (n=134; 13%), polydrug and polyroute (n=88, 8%), and heroin and cocaine injecting (n=404, 38%)], and two subtypes with low proportions of IDUs (LIDUs) [heroin snorting (n=275, 26%) and crack smoking (n=160; 14%)]. The polydrug and polyroute subtype had the highest depressive symptoms risk among all subtypes. Injection risk was lowest in the heroin injecting subtype and significantly differed from heroin and cocaine injecting subtype. The IDU subtypes also varied in the drug network compositions. The LIDU subtypes had similar depressive symptoms risk but vastly differed in the drug network compositions. CONCLUSIONS: Subgroups of inner-city cocaine and heroin users based on type and route of administration differed in their depressive symptoms, injection risk and drug network compositions. Future studies should longitudinally examine factors associated with transitioning across these subtypes to better inform prevention and treatment efforts.

Association of polymorphisms in the mannose-binding lectin gene and pulmonary morbidity in preterm infants.

Deficiency in the collectin mannose-binding lectin (MBL) increases the risk for pulmonary and systemic infections and its complications in children and adults. The aim of this prospective cohort study was to determine the genetic association of sequence variations within the MBL gene with systemic infections and pulmonary short- and long-term complications in preterm infants below 32 weeks gestational age (GA). Three single-nucleotide polymorphisms (SNPs) in the coding region and one SNP in the promotor region of MBL2 were genotyped by direct sequencing and with sequence-specific probes in 284 newborn infants <32 weeks GA. Clinical variables were comprehensively monitored. An association was found between two SNPs and the development of bronchopulmonary dysplasia (BPD), defined as persistent oxygen requirement at 36 weeks postmenstrual age, adjusting for covariates GA, grade of respiratory distress syndrome and days on mechanical ventilation (rs1800450 (exon 1 at codon 54, B variant): odds ratio dominant model (OR)=3.59, 95% confidence interval (CI)=1.62-7.98; rs7096206 (-221, X variant): OR=2.40, 95% CI=1.16-4.96). Haplotype analyses confirmed the association to BPD, and a single haplotype (frequency 56%) including all SNPs in their wild-type form showed a negative association with the development of BPD. We detected no association between the MBL gene variations and the development of early-onset infections or further pulmonary complications. Frequent variants of the MBL gene, leading to low MBL concentrations, are associated with the diagnosis of BPD in preterm infants. This provides a basis for potential therapeutic options and further genetic and proteomic analysis of the function of MBL in the resistance against pulmonary long-term complications in preterm infants.

Dendritic cell deficiency in the blood of kidney transplant patients on long-term immunosuppression: results of a prospective matched-cohort study.

Evidence from in vitro studies suggests that immunosuppressive drugs interfere with key functions of dendritic cells (DCs), but the in vivo relevance of these findings is elusive. We prospectively analyzed the major DC precursor subsets in the blood of kidney transplant recipients on long-term immunosuppression (> or =1 year). A total of 87 patients were compared to 87 age- and sex-matched controls. Total DC numbers and the precursor subsets, myeloid type 1 DCs, myeloid type 2 DCs (mDC1, mDC2) and plasmacytoid DCs (pDCs) were identified by four color flow cytometry. Long-term immunosuppression was associated with significant reduction of all major DC subsets in comparison to healthy controls (mDC1 p < 0.001; mDC2 p < 0.0001; two-tailed Mann-Whitney U-test) with the strongest negative impact on pDCs (p < 0.00001). In contrast, total leukocyte numbers were not significantly affected. Analysis of the relative impact of different agents revealed a significant impact of prednisolone on pDCs (p = 0.009) and mDCs2 (p = 0.006). The functional relevance of pDC deficiency was confirmed independently by Interferon-alpha analysis after Toll-like receptor 7 (p < or = 0.001) and 9 (p < 0.05) stimulation. These results indicate for the first time a profound negative impact of long-term immunosuppression on major DC subsets in kidney transplant recipients. DC deficiency may have important implications with respect to viral infections and tumor development.

A promoter polymorphism in the CD14 gene is associated with elevated levels of soluble CD14 but not with IgE or atopic diseases.

BACKGROUND: A polymorphism in the promoter region of the CD14 gene, C-159T, has been shown to be associated with increased levels of soluble CD14 (sCD14) and decreased serum immunoglobulin E (IgE) and the expression of a more severe atopic phenotype in previous studies. METHODS: To test if these associations are consistently found in different populations and different age groups, we genotyped 2048 children of different age groups as well as 888 adults from different regions of Germany for the CD14 C-159T polymorphism. RESULTS: While an association between this promoter polymorphism and levels of sCD14 could be confirmed in our study population (CC: 1017 ng/ml vs TT: 1370 ng/ml, P = 0.03), no association between CD14 C-159T genotypes and IgE levels or the prevalence of atopic diseases was seen. CONCLUSIONS: The lack of association between CD14 genotypes and IgE as well as atopic outcomes in this large German study population seems to indicate that CD14 genotypes may not directly be involved in the development of allergies during childhood.

Th2-mediated atopic disease protection in Th1-mediated rheumatoid arthritis.

OBJECTIVE: The balance between CD4+ T-helper (h) cell subsets (Th1 and Th2) plays an important role in the pathogenesis of rheumatoid arthritis (RA) and atopy. While RA is believed to be a Th1 mediated disease, Th2 cells predominate in atopic disorders. The purpose of this study was to investigate differences in the occurrence of allergy, hay fever, house dust mite sensitivity and asthma, as well as total serum IgE levels in RA patients and controls. METHODS: The case history of atopic disorders was assessed in 134 RA patients and compared to those found in 305 healthy blood donors. RA patients also answered clinical questions concerning disease activity and severity. Total serum IgE levels were measured in both groups, taking into consideration disease modifying therapy. RESULTS: A significantly lower occurrence of medical history of hay fever (2.3%) and house dust mite sensitivity (3.1%) was found among RA patients compared to controls (24.2% and 12.2%, respectively; p < 0.0001 and p < 0.003 respectively). Moreover, RA patients had significantly lower total serum IgE levels than control subjects (p < 0.0001). RA was less severe in patients with atopy compared to non-atopic RA patients. CONCLUSION: These results support the concept that RA and atopy antagonize each other and that a change in the cytokine patterns of Th1 and Th2 cells could provide an indication for curative effects on RA.

Novel genetic variation of human interleukin-21 receptor is associated with elevated IgE levels in females.

The interleukin-21 receptor (IL21R) was recently discovered as a novel member of the class-I-cytokine-receptor family and is selectively expressed in lymphoid tissues. IL21R shows strong sequence homologies to the interleukin-4 receptor alpha chain gene (IL4RA). In addition, both genes are adjacent and share structural similarity. We analyzed all the exons of the human IL21R gene and its 5' flanking region for sequence variation. We identified four novel single nucleotide polymorphisms (SNPs) and genotyped 300 healthy blood donors. Total serum IgE levels were measured in all subjects and associated with IL21R SNPs. Results revealed a significant association of one IL21R polymorphism (T-83C) with elevated IgE levels (>100 kU/I) in females (OR=3.000, CI=[1.163;8.385], P=0.015, n=138). This was confirmed in a second prospectively collected group of female blood donors (OR=2.535, CI=[0.927;6.733], P=0.046, n=123). In contrast, no effects were observed in male subjects in either population. These findings identify IL21R as a possible novel target locus influencing IgE synthesis in female individuals.

No linkage of the interleukin-4 receptor locus on chromosome 16p11.2-12.1 with sarcoidosis in German multiplex families.

We typed 241 members of 62 sarcoidosis families with 136 affected siblings for three single nucleotide polymorphisms of the interleukin-4 receptor alpha-chain gene (IL4R). Allele frequencies in patients were compared to those of healthy unrelated control individuals. The segregation of the three-point IL4R haplotypes completed by two flanking highly polymorphic microsatellite polymorphisms revealed no evidence for linkage of the IL4R gene locus with sarcoidosis.

A novel polymorphism in the 5' promoter region of the human interleukin-4 receptor alpha-chain gene is associated with decreased soluble interleukin-4 receptor protein levels.

Interleukin (IL)-4 exerts its biological effects through binding to the IL-4 receptor (IL4R) complex, plays a central role in stimulating B-cell differentiation, and is crucial for the development of T helper 2 cells. Recently, a soluble form of the human IL4R alpha chain (sIL4R alpha), which is produced by alternate mRNA splicing of exon 8, was discovered. sIL4R is thought to play an important role in either enhancing or inhibiting IL-4 signalling. We analyzed the 5' promoter region of the human IL4R alpha-chain gene (IL4RA) of healthy volunteers by DNA sequencing and found three novel single-nucleotide polymorphisms (SNPs; T-890C, T-1914C, C-3223T) and one novel short tandem repeat [(CAAAA)(5-7)-3600]. The two common promoter region SNPs T-1914C and C-3223T as well as six known coding SNPs in the IL4RA gene were genotyped in healthy blood donors by PCR with sequence-specific primers; total sIL4R levels were measured by ELISA. Results revealed a highly significant association of the -3223T variant with lowered sIL4R levels (two-tailed t-test, P=0.0002). Results remained highly significant after Bonferroni adjustment for multiple comparisons (P=0.0017). Moreover, the C-3223T variant was found to be in strong linkage disequilibrium with the extracellular 150V variant (P<0.001), which was recently described to be associated with atopic asthma in a Japanese population. Since this novel IL4RA promoter region SNP is common (allele frequency 29.8%), we conclude that it may be of importance for the genetic regulation of the IL-4 signalling pathway.

Termination of stem cell maintenance in Arabidopsis floral meristems by interactions between WUSCHEL and AGAMOUS.

Floral meristems and shoot apical meristems (SAMs) are homologous, self-maintaining stem cell systems. Unlike SAMs, floral meristems are determinate, and stem cell maintenance is abolished once all floral organs are initiated. To investigate the underlying regulatory mechanisms, we analyzed the interactions between WUSCHEL (WUS), which specifies stem cell identity, and AGAMOUS (AG), which is required for floral determinacy. Our results show that repression of WUS by AG is essential for terminating the floral meristem and that WUS can induce AG expression in developing flowers. Together, this suggests that floral determinacy depends on a negative autoregulatory mechanism involving WUS and AG, which terminates stem cell maintenance.

Analysis of interleukin-4 receptor alpha chain variants in multiple sclerosis.

A recent candidate gene study employing microsatellite markers suggested a possible linkage of multiple sclerosis (MS) with the interleukin-4 receptor (IL4R) gene. Consequently, we investigated the association of different IL4R variants with MS in 341 German MS patients and 305 healthy controls. Analysis of the first 100 MS patients for six IL4R variants showed an increased frequency of the R551 variant in MS patients versus healthy controls and carriage of the same IL4R variant was weakly associated with myelin oligodendrocyte glycoprotein (MOG) autoantibody production. However, further analysis of all 341 MS patients did not confirm the finding that this IL4R variant represents a general genetic risk factor for MS but revealed an increased frequency of the R551 variant in MS patients with primary progressive MS (PPMS, n=48) as compared to patients with relapsing remitting MS or secondary progressive MS (RR/SPMS n=284; P=0.005 for genotype differences) and to 305 healthy controls (P=0.001 for genotype differences). This association was statistically independent of the presence of the well-known MS susceptibility allele HLA-DRB1*15. After correction for multiple comparisons only the genotype differences between PPMS patients and healthy controls remained statistically significant. These results indicate, that the IL4R variant R551 may influence the genetic predisposition for PPMS but does not represent a general genetic risk factor for MS.