KidsAudiologist

Archive for the ‘newborn hearing screening’ Category

Yesterday I attended the launch of the new National Commissioning Framework for Hearing Loss Services.

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This is a document was developed by members of the Hearing And Deafness Alliance (a group of representatives from professional organisations, charity sector and patient groups) with NHS England and follows the launch of the Government’s cross-sector and cross-departmental Action Plan on Hearing Loss last year.

The new framework is aimed at supporting NHS commissioners in ensuring they understand the importance of services for people with hearing loss and the potential impact of un-managed hearing and communication difficulties. The document clearly indicates that it covers the whole age range from birth onwards but understandably given the much larger numbers involved, does have some emphasis on age-related hearing loss. But section 3.1 does make it clear that CCGs should be familiar with their commissioning responsibilities in relation to hearing and wider audiology services and appendix 3 helpfully clarifies the responsibilities of CCGs, NHS England and PHE in the complex environment of commissioning the various parts of a child’s audiology journey. Finally, section 8 stresses the need to move towards more outcome based commissioning and the crucial role of service specifications in setting out the key requirements for delivery of the service.

I was therefore very pleased be invited onto the Children’s Services Content Group and to lead on developing a model service specification for commissioners on paediatric audiology services along with a series of suggested outcome measures for children plus service performance outcomes, that services and commissioners can use to measure quality of the service. A link to this document is contained within the Framework or can be downloaded here. This is the first time we have had children’s outcomes used as commissioning measures of quality and we look forward to feedback and developing these further.

I’m now looking forward to working with NDCS colleagues to share this suite of documents widely with our networks, including service professionals and commissioners.

 

 

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Yesterday I published news that specialist paediatric audiology services are to be nationally commissioned from next April. Discussion with colleagues and on Twitter has confirmed confusion about the plans. And in response to the link posted on the BAA Facebook page I received some varied responses:

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In summary:

The biggest question appears to be how much of a typical paediatric audiology service will fall under “Specialist Audiology Services. Specialised audiology services consist of those audiological services which are part of a paediatric hearing aid service”? We don’t know at this stage – it could mean just the advanced audiological tests such as diagnostic ABRs (used mostly for diagnosis following newborn screening) or it could also include hearing aid fittings since we know that fitting aids to babies is very complex. My working assumption is that they are using the same kind of definition that the Department of Health used in their Transforming services for children with hearing difficulties and their families; A good practice guide (2008) that used the term to mean fitting of hearing aids on the under 3’s and associated testing.

Whichever parts of the service are included the issues remain the same, with the most important of all – how to ensure good integrated care across the child’s care pathway when several different services and commissioning bodies are involved? Lots to tease out and lots of clarity still needed about what this announcement will mean.

UPDATE 18th September 2012: The NDCS response to this news can now be read here

As part of the new Health and Social Care Act 2012 the commissioning (or purchasing) of NHS services will be the responsibility of local Clinical Commissioning Groups (CCGs) except where the service is prescribed in legislation. Prescribed services will be commissioned by the NHS Commissioning Board (NHSCB). Yesterday the DH published the recommendations of the Clinical Advisory Group for the services that should be nationally commissioned from April 2013. (The document is dated June 2012 but it was only publically available yesterday!)

The recommended services that are included and of interest to the audiology and ENT profession, as well as parents of deaf children are:

  • Specialist Ear, Nose and Throat Services for Children and Young People including surgical management of rare conditions, surgical management of more common conditions when the child or the procedure is high risk (including the need for PICU or Specialist Anaesthetic management), provision of specified procedures and Specialist Audiology Services. Specialised audiology services consist of those audiological services which are part of a paediatric hearing aid service. In some cases specialised audiology services may not be co-located with specialised ENT surgery services. (p21 & 130)
  • Cochlear Implantation Services (p26 & 200)
  • Bone Anchored Hearing Aid Services (p26 & 201)
  • Auditory Brainstem Implantation Service (p26 & 203)
  • Middle Ear Implantable Hearing Aid Services (p26 & 205)
  • Other Specialised Ear Surgery (the plastic surgery element is included within the proposed regulation on Specialist Plastic Surgery for Children and Young People.) (p26 & 207)
  • Specialist Mental Health Services for Deaf Adults & Deaf Children & Adolescents (p17 & 103)

Also of interest to us because of the populations involved:

  • Specialist Services to support patients with complex physical disabilities which including Specialist Augmentative and Alternative Communication Aids (p11 & 44)
  • Cleft Lip and Palate Services (p15 & 77 & 152 & 158)
  • Specialist Genetic Services (p20 & 95)
  • Specialist Plastic Surgery Services for Children and Young People which includes congenital malformations of the ear including: absence of ear, absence/atresia/stricture of auditory canal, macrotia, microtia, misshapen ear, misplaced ear. And congenital malformation syndromes predominately affecting facial appearance incl Goldenhar syndrome, Robin syndrome and Treacher-Collins syndrome. (p23 & 158)

There are also some exisitng services that are already regionally or nationally commissioned that are recommended for transfer to new regulations:

  • Alström services (p28)
  • Complex neurofibromatosis type 1 services  (p29)
  • Mental health services for Deaf children and adolescents will include services provided by Highly Specialist Deaf Child and Adolescent Mental Health centres including outreach when delivered as part of a provider network. (p29)
  • Craniofacial services (p29 & 152)
  • Neurofibromatosis type 2 services (p31)
  • Primary ciliary dyskinesia management services (p32 & 166)
  • Rare mitochondrial disorders services (p32)
  • Stickler syndrome diagnostic services (p34)

What do you think?

National commissioning has the potential to be a very positive move for those low volume, highly complex, high cost interventions such as cochlear implants, and this could mean much greater equity of access across the country to these types of procedures. The big surprise to me is the paediatric hearing aid services included in the recommendations as it neither fits the low incidence or high cost descriptions used to identify national specialist services. More so than many of the other services this recommendation has the potential to have a very major impact on how current services are provided in terms of location, staff, and integration with the rest of the paediatric audiology service. Specialist services are likely to have more experienced staff who see larger numbers of children and build up more expertise. But how is this organised? It isn’t fair to expect families with young deaf children to travel great distances on a regular basis to attend specialist services so how will care closer to home be managed? With a split between local and specialist centres? With a network arrangement where staff travel to see children in outreach centres and/or work with local teams? Or another way?

What are your views? What would you like to see happen?

Update 14th September 2012: Some of your views can be found here.

Update 18th September 2012: The NDCS response to this news can now be read here.

Last updated 24th November 2016

Useful resources for families on the ear, hearing and hearing loss in languages other than written English. If you know of others that are publically available to share please comment below.

Cochlear implants

Communicating with deaf children

Ear infection & glue ear

Hearing aids

Hearing tests

Helping your deaf child to learn

Newborn hearing screening

Parenting & families

  • Positive Parenting DVD with options for spoken English, Polish, Urdu, Sylheti, Punjabi and Somali with English subtitles and British Sign Language
  • Parenting a deaf child parenting tips available in Welsh
  • Who am I? DVD with options for spoken English, Polish, Punjabi and Urdu, with English subtitles and British Sign Language
  • All Together DVD with options for spoken English, Urdu, Sylheti and Punjabi, with English subtitles and British Sign Language
Speech and language development

Vision care

In addition to above the New South Wales Multicultural Health Communication Service in Australia hosts a selection of leaflets in other languages, and the National Institute on Deafness and other Communication Disorders has a large selection of leaflets on hearing, deafness, tinnitus, speech & language development etc in Spanish.

At a recent NDCS Family Weekend for families of newly diagnosed children I was reminded of a question I’m asked alot – is the newborn hearing screen accurate? And the parents most likely to ask are those whose child passed newborn hearing screening but was later diagnosed with a hearing loss. So is it? Or have these children been misdiagnosed?

I believe that there is an issue here but that it might not be what is immediately thought!

The goal of newborn hearing screening is to enable the identification of as many newborns as possible who do have a hearing loss (high sensitivity) but also exclude as many newborns as possible who do not have a hearing loss (high specificity). Depending on the type of screen carried out, around 15% of babies who are tested using otoacoustic emissions (OAE), and 3% of babies tested using automated auditory brainstem response (AABR) will be referred for further testing. Between one to two babies in every 1000 are born with some level of hearing loss in one or both ears, so we know that it is much more likely that a baby is referred from screening and found to have normal hearing than the other way round. But there is a real issue with information sharing between professionals, as well as between professionals and parents…

Only half the children with permanent childhood hearing loss are born deaf. The other half develop deafness during the first few months/years. There are therefore young children who passed newborn screening and diagnosed as deaf early in life that can look like an inaccurate or late diagnosis but may have actually developed the deafness post-screening. There are some common causes of childhood deafness that cause this, such as CMV infection and genetics, that are commonly believed to be congenital (from birth) but are actually progressive early in life. One of the reasons we test for unilateral (one-sided) hearing loss in newborns in this country is because we know that a proportion go on to develop bilateral hearing loss soon after so it’s important to monitor these babies and young children. Another issue is that the screen is designed to pick up moderate or greater deafness but this isn’t explicitly laid out to parents and therefore again parents feel misled if later their child is diagnosed with mild deafness (or maybe was born with mild deafness that progressed early). Some of this we have only begun to understand better in the last few years and since universal screening was implemented – in the past children were always diagnosed later and therefore assumed to have been deaf from birth.

Although the information leaflet that is provided with newborn screening clearly says that deafness can happen at any age, most people’s perception is that a passed screen = normal hearing. Parents continue to express concerns that they have problems accessing an audiology assessment for their child. A GP may fail to refer concerned parents because everything must be OK as ‘they passed their newborn test’ and Health Visitors who used to test babies at 8 months may now assume that there’s no need to be concerned because of the early screen.

Having said all this, there is of course always the chance of error or mis-diagnosis within the system, or a false report of passing the screen. In which case any concerns of the parents should be treated seriously and investigated fully. For other families it can be helpful to discuss the ‘aetiology’ or cause of their child’s deafness and that may help to understand how and why the hearing loss happened/progressed. NDCS publishes a great booklet that is free to download called Understanding your child’s hearing tests that includes a section ‘Medical tests used to help diagnose the cause of
permanent deafness’ with more information.

I wanted to share a couple of excellent articles by Dr Carol Flexer, Ph.D. on the importance of early hearing aid fitting and use, & associated development of the auditory brain…

“Hart and Risley (1999) studied children from professional families and determined that they have heard 46 million live-spoken words by age 4. This is the magnitude of practice that is critical. This speaks volumes to the fact that less than every waking hour of technology use will not cut it for children with hearing loss. Dehaene (2009) talked about the listening basis for reading, and children with hearing loss require three times the exposure to learn new words and concepts because of reduced acoustic bandwidth compared to typical hearing peers.”

The Auditory Brain: Conversations for Pediatric Audiologists (2011)

“Robbins et al. (2004) found that skills mastered as a course of normal development result in developmental synchrony. Therefore, it appears we are pre-programmed to develop specific skills during certain periods of development. If those skills can be triggered at the intended time, we will be operating under a developmental and not a remedial paradigm. That is, we will be working harmoniously within the design of the human structure.”

Neuroplasticity is greatest during the first 3 ½ years of life; the younger the infant, the greater the neuroplasticity. Rapid infant brain growth requires prompt intervention, typically including amplification and a program to promote auditory skill development. In the absence of sound, the brain re-organizes itself to receive input from other senses, primarily vision; this process is called “crossmodal re-organization” and it reduces auditory neural capacity. Early amplification or implantation stimulates a brain that has not yet been reorganized, allowing the brain to be more receptive to auditory input resulting in greater auditory capacity.”

Auditory Brain Development: The key to developing listening, language and literacy (2012)

More than 9 out of every 10 chldren born with a hearing loss have hearing parents. It’s therefore entirely natural that early on they are going to ask “What can my child actually hear, what does it sound like for them?” They share a need to try and get to grips with this as part of the process of understanding what they need to do to help their child. It is a very difficult question to answer. Some of the things we know at this stage include the degree and type of deafness: we know how much volume is lacking for a mild (20-40dB), moderate (41-70dB), severe (71-95dB) or profound (95+dB) hearing loss, and we know that loss of hair cells in sensorineural hearing loss means that some natural processing that happens within the cochlea is lost – including the cochlea amplifier, pitch discrimination and the ability to recognise different sound levels (reduced dynamic range). We also know a good amount about the benefits and limitations of various types of hearing technologies that are used. Now more than any time in history, we know that almost all deaf children can perceive the full range of speech sounds with their hearing aids or cochlear implants. But we also know that they can’t do this as easily as hearing children and that no hearing technology can replace normal levels or quality of hearing.

So it’s relatively easy to mock up some simulations of what a hearing loss might sound like using some software to reduce the overall level of sound and filter out some of the frequencies that make up speech sounds. There are some good ones available online and my favourite is the Better Hearing Institute which has mild and moderate hearing loss in different situations. The NDCS also have a couple of examples that are specific to children in classrooms.

But there is a health warning about these simulations and that is that we don’t know, especially in the early days following diagnosis in young babies, what is happening beyond the ear and how well they can make sense of the sound they hear. Hearing, listening and understanding are sophisticated processes that are determined by a complex interaction between the physical properties of the ear as well as attention, memory and auditory processing (what happens when your brain recognises and interprets sound so that it becomes meaningful). The brain has the incredible power of plasticity in the early years and is continually moulding and learning from new experiences and sounds. As adults the brain is much less plastic and it takes much longer to adapt. This means that the experience of hearing and listening is hugely variable between individuals and is influenced by whether someone is born deaf or what age they became deaf, how long it was between developing a hearing loss and being fitted with hearing aids, their listening experience, and their cognitive and auditory processing abilities. Children born deaf and those who become deaf early in life experience deafness as the norm. Older children, teenagers and adults who become deaf have a very different experience of deafness to which they need to psychologically and physically adapt to. Much of what we understand hearing loss to sound like comes from adults who have previously experienced normal hearing levels. This is a particular issue when parents are considering a cochlear implant for their child for example. It is commonly reported that cochlear implants sound electronic, that they aren’t natural, and that voices sound like ‘a Dalek underwater’. This can be a devastating idea for parents considering this option but in fact when you speak to these same implanted adults later on they describe their implants as sounding very natural as their brain has acclimatised to the new sound. I recently chatted with a group of teenagers who all used cochlear implants and they agreed – “people think they sound like robots, but they don’t”. Just from listening to the voice quality of the thousands of implanted children, we can be certain that they do not experience this kind of ‘electronic’ sound. But to listen to this type of electronic simulation the best one I’ve found is Scientific American Frontiers.

Auditory Neuropathy Spectrum Disorder (ANSD) affects approximately one in 10 deaf children and causes distortion of sound and difficulty discriminating speech over and above what we would expect from the hearing loss alone. The level of distortion is highly variable from very slight to very severe and it is not measurable. So in young children we do not know early on how it will affect their speech and language development. Simulations of ANSD from mild to profound neuropathies can be heard here and make scary listening but again we know that with the right intervention children with ANSD are successfully using their hearing and using spoken language.

There are 10 million people in the UK have some degree of hearing loss and the majority of these people experience age-related deterioration of hearing. At the other end of the age spectrum there are just 45,000 under 18’s with a permanent hearing loss. Inbetween there are those adults who grew up with a hearing loss as well as those who have lost their hearing as an adult. My experience is that individuals (and their parents or family) use a wide variety of terms to describe their hearing level – deaf, hearing loss, partially hearing, hearing impaired, hard of hearing – and they rarely relate to their audiogram level. There are those people who associate themselves culturally with the Deaf community who we may traditionally think of as those who are profoundly deaf sign-language users. But there are also people who have developed a mild hearing loss for the first time who may tell their audiologist they are ‘stone deaf’ or ‘can’t hear a thing’ and this is their perception compared with their normal experience.

Every child’s hearing levels and hearing experience is unique and it is impossible for these types of simulations to represent how all deaf people process and experience sounds heard. But they are useful in giving the listener an understanding of the difficulties encountered and I particularly like those that can highlight the big differences experienced between listening in quiet and noisy environments and which are an important reminder of how much easier we can make life for children by improving their listening environments in school and elsewhere.


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