Achieving early functional auditory access in paediatric cochlear implantation.

Cochlear implantation (CI) is a viable option for providing access to auditory stimulation in severe-to-profound hearing loss/impairment of cochlear origin. It has been demonstrated that CI is safe and effective for deaf children. Younger age at activation after CI is linked with better outcomes. It is important to study variables and issues that can interfere with an early fitting and access to sound after CI. They range from patient characteristics, family compliance and support, to technical, medical or organisational problems. A SWOT analysis and a subsequent TOWS matrix was conducted to discuss issues and propose recommendations to be considered when operating an early switch on of the CI.

Life-threatening unilateral hearing impairments. Review of the literature on the association between inner ear malformations and meningitis.

BACKGROUND: Bacterial meningitis is a life threatening disease that can be triggered by a CSF leak through an inner ear malformation. Early identification of the specific type of cochleovestibular dysplasia and the associated risk of meningitis is of vital importance. OBJECTIVES: The objective of this review is to collect and discuss available data on the association between inner ear malformations and meningitis in children. METHODS: Electronic databases were crosschecked for obtaining relevant papers published in the last 20 years, and further cases were identified by hand searching through the references. Demographic data were extracted from full texts, together with information on the severity of hearing impairment, the type of inner ear anomaly, the site of cerebrospinal fluid leak, the number of recurrent meningitis episodes. RESULTS: Sixty-seven cases of meningitis related to inner ear malformation have been identified among 45 papers. Mean age at presentation is 3.60±3.00 (range 0.1-14) years. Average diagnostic delay from the first episode of meningitis is 3.44±3.41 (range 0.00-10.00) years. The number of meningitis episodes that occurred before the correct diagnosis and definitive surgical treatment is 3.27±1.81 (range 1.00-10.00). Unilateral hearing impairment affects 70% of patients. Six patients had normal hearing at presentation. Two children are dead from inner-ear-malformation-related meningitis among reviewed reports. CONCLUSION: A high number of paediatric patients carrying inner ear malformations, especially when associated with unilateral hearing impairment, could be at risk to develop recurrent bacterial meningitis. Universal newborn hearing screening programs should prompt a diagnostic work-up even in the case of unilateral hearing impairment, in order to prevent inner ear malformation-related meningitis.

Novel connexin 30 and connexin 26 mutational spectrum in patients with progressive sensorineural hearing loss.

OBJECTIVE: Mutations in the gap junction protein beta-2 gene ('GJB2') are known to be responsible for mild to profound congenital and late-onset hearing loss. This study aimed to investigate the molecular basis of progressive hearing loss compared with non-progressive hearing loss. METHODS: Following clinical otorhinolaryngological evaluation, a genetic analysis was performed in a cohort of 72 patients with progressive sensorineural hearing loss. RESULTS: Pathological genotypes were established in 16 patients (22.2 per cent). Six different gap junction protein beta-2 gene mutations were detected in 15 patients, with the c.35delG mutation responsible for 56 per cent of the mutated alleles. A novel gap junction protein beta-6 gene ('GJB6') mutation (p.Met203Val) was observed in one patient with mild progressive hearing loss. CONCLUSION: Analyses of gap junction protein beta-2 and -6 genes revealed that similar pathological genotypes, occurring with similar frequencies, were responsible for progressive hearing loss, compared with reported genotypes for non-progressive hearing loss patients. Thus, genotype cannot be used to differentiate non-progressive from progressive hearing loss cases; in this study, patients both with and without an established pathological genotype had a similar clinical course.

Analysis of the causes and consequences of decreased antibiotic consumption over the last 5 years in Slovenia.

OBJECTIVES: Compared with European countries, the use of antibiotics in Slovenia is moderate. In the period 1999-2002 an 18.67% decrease in outpatient antibiotic consumption was noted. The aim of the present study was to analyse this decrease and its consequences. METHODS: The data on outpatient antibiotic consumption were obtained from the Institute of Public Health and Health Insurance Institute of Slovenia and expressed in defined daily doses (DDD)/1000 inhabitant-days. The number of media publications on 'antibiotic drugs' and 'bacterial resistance' during the study period was obtained. In 2000, the prescription of co-amoxiclav and fluoroquinolones was restricted because of a constant increase in the consumption of these drugs. The data on incidence of acute mastoiditis and penicillin resistance among invasive pneumococci were obtained. RESULTS: The total outpatient consumption of antibacterials increased from 15.21 DDD/1000 inhabitant-days in 1996 to 20.08 in 1999, and decreased to 16.97 in 2003. The consumption of restricted antibiotics decreased from 7.29 in 1999 to 5.25 DDD/1000 inhabitant-days in 2003. There was a positive correlation between antibiotic consumption and the number of newspaper articles (r=0.92), and a negative correlation between the number of diagnostic tests and antibiotic consumption (r=-0.73 for the C-reactive protein test and -0.68 for the streptococcal antigen detection test). Reduced antibiotic consumption was paralleled by a decrease in penicillin resistance among invasive pneumococci. No increase in mastoiditis cases was observed in spite of reduced antibiotic consumption. CONCLUSION: Restriction of antibiotic prescription proved to be effective in reducing outpatient antibiotic consumption. The effect was prolonged and affected restricted antibiotics as well as non-restricted drugs.