National study of newborn hearing screening: programme sensitivity and characteristics of undetected children.

UNLABELLED: The success of universal newborn hearing screening (UNHS) programmes is usually evaluated by determining the effect of the early detection of hearing loss on developmental outcome. However, in practice, these programmes do not detect all children with permanent childhood hearing impairment. In this study we determine the sensitivity of the current UNHS programme and analyse the characteristics of the children not detected by UNHS. We performed a nationwide, population-based, retrospective follow-up study in The Netherlands. All children born in 2003-05 and screened in a hearing screening programme (well babies and neonatal intensive care (NICU) graduates) were included for study. The main outcome measure was the sensitivity of the UNHS programme (based on the proportion of children known to have a permanent childhood hearing impairment in 2008 who were identified by UNHS). We also evaluated age at diagnosis, severity, and aetiology of hearing impairment in the children not detected by UNHS. We found that the sensitivity of the current UNHS programme was 0.83 (0.79 for well babies and 0.96 for NICU graduates). Permanent childhood hearing impairment was confirmed before 36 months of age in 96% of the study cohort. Of the children unidentified by the UNHS, > 50% had moderate hearing loss. No predominant cause of hearing impairment was found in these children. CONCLUSION: Our current UNHS programme identified the majority of children with a permanent hearing impairment of congenital cause.

Auditory neuropathy in a low-risk population: a review of the literature.

OBJECTIVE: Collect all available published evidence on the prevalence of auditory neuropathy in the well baby population and calculate the contribution of this to the false negative rate of oto-acoustic emission based newborn hearing screening programs. METHOD: PubMed and EMBASE were searched for relevant articles published between 1996 and 2010. Medical Subject Headings terms included 'Auditory disease', 'Prevalence' and 'Child' and their relevant synonyms. Included were original studies, which focused on well babies and reported the prevalence of auditory neuropathy. RESULTS: Of 519 citations 4 articles met the inclusion criteria. The population based prevalence of auditory neuropathy in children in population hearing screening was found to vary between 0.006% (SD 0.006) and 0.03% (SD 0.02). The false negative rate, caused by missed children with auditory neuropathy, is between 4 and 17%. CONCLUSION: The available information on the prevalence of auditory neuropathy in the well baby population is poor. However, if oto-acoustic emission screening is used in the first stage of a neonatal hearing screening program, children with auditory neuropathy are missed. The cost-effectiveness of population-based screening using auditory brainstem response should be studied.

Hearing aids: expectations and satisfaction of people with an intellectual disability, a descriptive pilot study.

BACKGROUND: In spite of an increased risk of hearing impairment in persons with an intellectual disability (ID), rehabilitation with hearing aids often fails. We performed a descriptive pilot study with the following study questions: 1 Do comparable elements as in the general population contribute to expectations of and satisfaction with hearing aids in adults with mild or moderate IDs? 2 To what extent do adults with an ID depend on carers in use and maintenance of hearing aids? STUDY POPULATION: 16 adults with a mild or moderate ID and a recent diagnosis of hearing impairment. Method: information by means of specially designed booklets; semi-structured interviews prior to hearing aid fitting and 6 months afterwards. ANALYSIS: descriptive. RESULTS: In total, 14/16 participants were able to give reliable answers. Most were aware of their hearing loss and familiar with reasons for hearing aids. A minority expressed positive expectations. Some expressed explicit wishes on the looks of hearing aids. All satisfaction domains as described for the general population could be recognized. Most participants were partially or totally dependent on carers in use and maintenance of hearing aids. CONCLUSIONS: Adults with ID may have explicit ideas and wishes about hearing aids and, if specifically asked, are capable of expressing these. Given information should be checked and repeated. In satisfaction with hearing aids, comparable elements may play a role as in the general population: benefit, cosmetics, sound quality/acoustics, comfort/ease of use, and service delivery. These findings, however, are from a small-scale study. Additional research is necessary to find out whether they are applicable more generally.