Audiology features
Living with Usher syndrome
“Usher people often still have sharp, clear central vision. It is the corner of their eye that is missing. This is why the Usher person often does not feel or look blind. In the early days they may not even...
Hearing loss and Alport syndrome
Alport syndrome (AS) includes a group of hereditary diseases caused by mutations in the COL4A3, COL4A4 or COL4A5 genes. These genes are responsible for the biosynthesis of α3, α4 and α5 collagen IV chains, which are located in the glomerular...
The impact of hearing loss in children with Down syndrome
Hearing loss and language development Down syndrome (DS) usually arises due to trisomy 21 and is associated with intellectual disability and risks of developmental delays and difficulties, including hearing loss. Hearing loss is common in children with DS [1]. In...
Branchio Oto Renal syndrome
Branchio Oto Renal Syndrome (BOR) is an autosomal dominant disorder with branchial, otologic and renal manifestations. The presence of clinical signs varies among and within affected families. Three causative genes for BOR syndrome have been reported thus far: EYA1, SIX1...
The changing role of audiology
Audiology has changed dramatically in recent years. Cochlear implants and high power hearing aids have made hearing really available to children with essentially any degree of hearing loss. Those of us who have been in the field for a long...
Evidence based practice in paediatric audiology
Audiology, like most of the health sciences professions, has been working on integrating evidence-based practice principles since the mid-1990s [1]. Professional organisations and regulatory colleges have produced evidence-based clinical practice guidelines, disseminated them to audiologists and collaborated with practitioners to...
A parent’s journey: beyond the diagnosis
Tamsin Coates lives in Wallesey, UK and talks about coming to terms with the difficulties and joys of having two deaf children. Here she explains about the early days and the impact of their diagnosis upon the family. Thinking back...
Screening: evaluating the outcomes of early intervention
Newborn hearing screening is now the accepted standard of care in several countries, and is becoming increasingly more established worldwide. White [1] reported eight countries screening over 90% of newborns, ten screening between 25-89% of births and a further 54...
Current considerations on neural development and hearing loss in young children
The young child’s brain has the ability to change in response to new stimuli, resulting in learning, the foundation of adaptive and intelligent behaviour. For children with hearing loss, a reduction or lack of auditory stimuli can have a ‘lifelong...
Utilisation of cortical auditory evoked potentials in the paediatric population
Introduction Early identification of hearing loss in infants followed by prompt intervention is well established as the key to maximising the development of speech, language and psychosocial skills. Many countries have already adopted early hearing detection and intervention (EDHI) programs,...
Dr Marion Pfaender Down
“Dr Marion P Downs, an innovator in the field of paediatric audiology and a tireless advocate for the early identification of hearing loss, passed away on November 13, 2014. During her exemplary career at the University of Colorado Health Sciences...
Making sense of modern wireless hearing aid technologies
Before diving into the topic of the technology behind wireless hearing aids, it is important to define two key concepts. The first is wireless frequency. This is the frequency at which a wireless signal is transmitted. In the context of...
