Download Janette Joyce (SLT Crosshouse)

BME Information Day 16th March 2013 (Glasgow)
Information on:
 Cochlear Implants And Auditory Rehabilitation post surgery
by Dr Mary Shanks
 Role of Genetic Counselling for Hearing Impaired in Ethnic
Minority Communities by Dr Juan Mora
 Role of Community Speech And Language Therapy in
Hearing Impaired Children by Fiona Jarvis
The key speakers on the day:
 Dr Juan Mora (Consultant Audio Vestibular Physician)
 Dr Mary Shanks (Consultant ENT Surgeon, Crosshouse),
 Fiona Jarvis (Community Speech and Language Therapist),
 Janette Joyce (Speech and Language Therapist, Advanced
Rehabilitation Advisor, Crosshouse)
 Email response from Lise Henderson (Co-ordinator
Paediatric Cochlear Implant)
Questions asked by parents and some of the responses
given by the speakers in their own words.
1. Because of brain plasticity changes during early
childhood, children who have not acquired a first
language in the early years might never be completely
fluent in any language. If they miss this critical period for
exposure to a natural language, their subsequent
development of the cognitive activities that rely on a solid
first language might be underdeveloped, such as literacy,
memory organization, and number manipulation. Is this
true? My son was diagnosed only when I came into this
country at the age of 9.
Fiona Jarvis (SLT):
“If not diagnosed until age 9, suggests he was capable of
disguising his difficulty. It also suggests that his hearing loss is not
profound in which case, in quieter environments, he may have been
able to access some speech/aspects of speech.
Brain plasticity/neuroplasticity continues throughout an individual’s
lifetime but is most in evidence in the first few years of life
In hearing infants, as a result of repeated sensory stimulation and
experiences of the world, the numbers of synapses or connections in
the brain grow until there is about twice the number found in the adult
brain. Then something called ‘synaptic pruning’ starts to happen and
the weaker synaptic contacts are eliminated while stronger
connections are kept and strengthened. The connections which have
been activated most often by a baby’s repeated experiences, are
preserved while ineffective, unused or weak connections are pruned.
The optimal time for meaningful auditory neural connections to form
is during the first 3 years of life. If the brain is not stimulated by
sound it will reorganise itself to maximise processing through other
senses – primarily vision. After about 3½, the brain has considerably
less flexibility to develop effective skills to process auditory
information which is why HI children identified late have a much
harder time learning to listen and speak proficiently.
There is a lot of documented evidence of the potential impact of
delayed first language acquisition, but few authors offer suggestions
of positive actions to progress the child’s abilities and to prevent
potential gaps in language and cognition. Schick et al (2002) suggest
some ‘positive actions’.
Areas to focus on include :
 - pretend play/role play
 - narrative – experiencing books and stories and progressing to story
telling
 - talking about past events
 - not limiting linguistic experiences – involve child in discussions,
invite opinions, feelings/emotions

relating to life experiences
 Your child is an individual and advice should be tailored to his
individual needs. For general ideas on accelerating language
development, speak to your local specialist speech and language
therapist (specify that you want advice from a specialist in Hearing
Impairment). “
Theory of Mind : Language Cognition in Deaf Children (B.Schick,
J.de Villiers, P.de Villiers, B. Hoffmeister) Dec.03, 2002 in ASHA
2. I have read about long lasting batteries requiring little
electric current will allow for a completely implantable
cochlear device in the future. Are we any closer to getting
such a device for our children? Will children who have
already been implanted be able to have such a device
fitted?
Dr Mary Shanks: “There has been research and development into
TICIs (totally implantable cochlear implants) for many years. There
are still problems to be solved with battery and microphone
technology as well as the need to avoid repeat surgery. At present
upgrading external processors allows children to benefit from the
latest technology without repeat surgery. If a TICI was to become
available in future it is likely that current cochlear implant users could
be upgraded but they would need a further operation to replace their
existing implant.”
3. I was told my child was born without auditory nerves...is
this rare. Will my child ever be able to use any listening
device to hear?
Dr Juan Mora: “The absence of auditory nerves is very rare,
particularly when it is in both ears. There are devices like brainstem
implants or other simpler devices which use vibration but they will
help with hearing to a limited extent.”
Janette Joyce (SLT Crosshouse): “I have located the info I was
seeking last summer for one of the Scottish SLT's who had a little boy
on her caseload with no auditory nerves. He was referred to our CI
programme initially but as we can only offer cochlear implants at this
time I believe he was referred on to the Manchester CI programme.
This appears to be the only CI centre in the UK offering Auditory
Brainstem Implants (ABI) to children. Both Manchester and
Cambridge have experience with adults who have received ABI. The
experiences I am hearing about at the moment is similar to the early
days of cochlear implant when there were no guarantees about the
benefits. Unlike now when we see the great results on a regular daily
basis. ABI surgery is much more daunting at this time and will only
usually be undertaken when there really is no other option. This is an
extremely difficult decision for any parent to make so the collection of
relevant information and the opportunity to talk to others who have
been through the experience will be invaluable. Dr Colletti at the
University of Verona in Italy is the main specialist in this field and I
understand that at least one child from the UK has travelled to Italy
for surgery.
I am copying the e-mail I received from Lise Henderson, the
Specialist SLT and Auditory Verbal Trained therapist at Manchester
CI programme. If the family who asked the question would like to
speak to her I am sure she would be happy to offer some advice
based on her experiences. The outcomes are not terribly positive at
this stage with only one out of six children achieving the outcome of
being able to understand spoken communication without lip reading.
But this was last summer and there may be more news by this time.”
Email response from Lise Henderson (Co-ordinator Paediatric
Cochlear Implant)
“In Manchester we have done 60 adult and 6 paediatric ABIs
(Auditory Brainstem Implants) the outcome is much poorer for ABI
patients than CI and there is some data to suggest that only 5%
obtain significant benefit. There are a small number of patients who
are able to understand speech without lip reading but many adults
find it an aid to lip reading. However adults who get an ABI will have
had NF2 so their brainstem has been damaged by a tumour so you
may anticipate a poorer outcome.”
“With the children we have only 1 who demonstrates an ability to
understand speech without lip reading the others generally are aware
of speech and like to wear their implants but have no open set
recognition. Due to the difficulty in placing the electrode there is often
non auditory stimulation so 2 children do not obtain any auditory
sensation from their implant they only have non auditory sensations
such as eye twitches /coughing/pain in their ear etc. “
“ABI in children is not recommended under the age of 18 months and
currently Manchester is the only UK centre doing paediatric ABI and
Manchester and Cambridge the only place doing adult ABIs. I believe
London is looking into setting up a paediatric Programme as well”
“Hope that helps a little feel free to get in touch if you need any
further info.”
Kind Regards
Lise
4. CI’s given to children with complex and multiple
disabilities, such as those with visual, motor, or
learning disabilities in addition to deafness it has
been argued that there will be benefits in relation to
perception of environmental sounds. Is this true?
Dr Juan Mora “Visual, motor and learning disabilities are certainly
NOT a contraindication to undergo assessment for cochlear implants.
However significant developmental delays will influence whether the
child will benefit from cochlear implants and therefore will have to be
considered very carefully to decide is the child can be helped by
cochlear implants. “
Fiona Jarvis (SLT): “The research suggests that benefits may be
limited to perception and some recognition of environmental sounds.
However, we need to remember that every child is an individual
and consider a number of factors including:
- child’s age
- cognitive level
- the support available from the family
Parents considering cochlear implantation for their child, need to be
provided with comprehensive and honest information in order to
make an informed decision.
Case example - implanted July 2011, aged 3,
This child was referred for assessment for cochlear implantation at
age 2. She is blind and deaf – the most profoundly dual sensory
impaired child I have encountered. Research suggests that for
deafblind children, the best outcome is awareness/recognition of
environmental sounds and maybe recognition of a few words after a
lot of input. This child is not, however, typical of deafblind children.
She is bright, inquisitive, creative and determined. The parents were
invited to discuss their concerns and given clear information about
the procedures and the potential outcomes. For their child, if she
made no gains post-implant, she remained, as before, deafblind. No
exaggerations were made re the outcomes but staff expressed the
positive potential in such an able child. The parents decided to go
forward with implantation and currently their daughter is showing
understanding of familiar phrases in both her first language and in
English. She is interested in words and will copy some but also uses
a growing number of words independently and appropriately.”
5. Implantation is taking place at increasingly earlier
ages, with evidence that normally hearing infants
make use of audition before birth, both for recognition
of mother’s voice and also in relation to identification
of the syllable structure of the mother’s language. Is
there scope for such antenatal testing and techniques
for antenatal implantation in the future?
Dr Mary Shanks: “Antenatal surgery is very hazardous to the
unborn child and unlikely to be considered for non- life-threatening
conditions such as deafness, even if antenatal hearing testing was
available. Children implanted around the time of their first birthday
make excellent progress and their spoken language skills quickly
catch up with normal hearing children so it is not necessary to expose
younger children to the additional risks associated with general
anaesthesia in very young babies. Many deaf children have residual
low frequency hearing which allows them to hear environmental and
some speech sound in the womb”.
6. Neurotrophic treatments-I have read about these:
Neurotrophic factors which support survival and
stimulate axonal growth of spiral ganglion neurons
within the cochlea may provide enhanced outcomes
for cochlear implants or as a means of regenerating a
fully functional auditory system (Miller et al., 2007).
Research in this area is likely to provide alternatives
to current surgical interventions. Please could you
explain this better?
Dr Mary Shanks: “Neurotrophic or ‘nerve re-growth’ treatment is
being developed but it will be many years until this type of treatment
is available. Electrode design is being constantly improved to
minimise damage to the spiral ganglion cells. In the near future it will
also be possible to deliver drugs directly to the cochlea by coating
implant electrodes or having drug delivery channels within the
electrode. Steroids delivered in this way would reduce inflammation
and scar tissue in the cochlea and help preserve spiral ganglion cells
following surgery. In the future neurotropic factors could be delivered
in this way.”
7. Approximately 5% of recessive deafness is in south
Asians than other populations. Does this mean that if
people don’t have consanguineous marriages they
can prevent recessive deafness?
Dr Juan Mora “Recessive hearing loss can appear in the children of
both
consanguineous and non-consanguineous marriages.
However in general Recessive medical conditions are less common
in non-consanguineous marriages. “
8. False positives in universal neonatal screening for
permanent childhood hearing impairment-What is
this?
Dr Juan Mora “Some newborns do not pass the hearing screening
test although they may have normal hearing. However then they will
undergo a thorough assessment to find out about their hearing which
is very reliable.”
9. Please could you explain auditory neuropathy in
layman’s terms? Would you consider a child with
auditory neuropathy for an implant?
Dr Mary Shanks: “The term auditory neuropathy includes a number
of different conditions. It means that there is a problem with the
nerve of hearing. Some of these conditions may be improved by
cochlear implantation but some are not. If there is a problem with the
hair cells in the cochlea transmitting sounds to the auditory nerve
(hair cell dysynchrony) this can sometimes improved by cochlear
implantation as the electrode stimulates the nerve directly. Other
types of auditory neuropathy might also benefit but every child needs
to be assessed individually”.
Janette Joyce (SLT Crosshouse): “Cochlear implantation should
certainly be considered for someone with auditory neuropathy and
that normal speech can be an expected outcome. As with all CI
surgery the level of ability is not guaranteed and is dependent on
many other factors. In layman's terms an auditory neuropathy means
that there is a fault somewhere along the auditory pathway but it can
be difficult to tell exactly where. This is why the outcomes for a child
with this diagnosis can be very variable”
10. In ear hearing aids-How effective are they?
Dr Juan Mora “They are effective and have advantages and
disadvantages compared with behind the ear hearing aids. In general
they are not recommended for young children. They are not adequate
for all degrees of hearing loss, they are lost more easily than behind
the ear aids and are more difficult to replace if they have a technical
problem, which may lead to o lack of consistency wearing the aids.”
11. Is there any way of diagnosing deafness during
pregnancy?
Dr Juan Mora “Not really unless you are referring to genetic studies
during pregnancy which could identify mutations which may cause to
hearing loss.”
12. Will a child with auditory neuropathy spectrum
develop normal speech?
Fiona Jarvis (SLT):
“As with any group, the outcome varies from individual to individual.
Australian study in International Journal of Audiology 2009; 48(6):
313-20. Rand, G., Barker EJ.
Aim – to assess the receptive language and speech production of
school aged children with Auditory Neuropathy/dys-synchrony type
hearing loss. .
Three groups were included in the research:
Group 1
10 ch with AN and Cochlear Implant (CI) in one/both ears
Group 2
10 implanted children from the general population of CI
recipients
Group 3
10 with AN who were long-term hearing aid users
Outcome – generally the same for each of the groups, with similar
levels of receptive language and speech production, except for 1/10
from Group 1 who gained little perceptual benefit from CI and showed
severely delayed spoken language. If considering cochlear
implantation, this may suggest that it would be better to give a single
implant and see if the child benefits rather than automatically
proceeding with bilateral implants.
Research into auditory neuropathy spectrum disorder is continuing.
With our current level of knowledge, outcomes can be unpredictable.
A child with AN may or may not benefit from hearing aids/cochlear
implant(s). Additional evidence suggests increased difficulty listening
against background noise. Speech and language outcomes will be
affected by the child’s level of hearing loss and on their ability to
make sense of sound with some developing good speech and
language and others struggling to process what they hear. Some may
benefit from the use of sign language.”
13. Is there a link between certain syndromes and
ethnicity? My child has Alport syndrome.
Dr Juan Mora “Some mutations are more common in certain
populations but I think Alport syndrome would not be a good example
of those.”
14. If my child gets an implant am I changing his/her
chance of future developments.
Dr Mary Shanks: “Improvements in external processors which
improve understanding of speech, and more recently music, are
already available to current implant users. It will also possible to
replace the internal receiver and electrode during a child’s lifetime,
perhaps two or three times. Future developments which involve
regrowing hair cells will not be possible if an ear is implanted.
However unless this treatment was carried out during the critical
period of language development, from birth to four years old, it would
not to be effective. It is likely to be more than ten years until there is
any alternative to cochlear implantation for the treatment of profound
hearing loss”.
15. My child wears an implant and has tinnitus. Any
tips on tinnitus?
Janette Joyce (SLT, Cross House):
“Child with tinnitus:1. Check programming of CI and let scientists know that tinnitus is a
problem.
2. If only one CI consider a hearing aid in contra lateral ear if that is
where the tinnitus is a problem.
3. If tinnitus is only a problem when CI is not worn then considers
wearing the equipment during quiet periods and at bedtime.
4. As suggested by another parent - try changing position of bed at
night time.
5. I would also add that it may be necessary to discuss sounds with
the child. It could be that the problem may be down to lack of
knowledge of the natural sounds around us.”
Dr Mary Shanks “Tinnitus is often improved by cochlear implantation
and when the processor is switched on the tinnitus is less prominent.
If tinnitus is severe at night it may be advisable to wear the processor
in bed”.
Some Research Articles read by parents:
http://news.bbc.co.uk/1/hi/health/7582640.stm
http://www.bbc.co.uk/news/health-21320859
http://news.bbc.co.uk/1/hi/health/5102486.stm
http://news.bbc.co.uk/1/hi/health/6755897.stm
http://www.bbc.co.uk/news/health-20960418
http://www.bbc.co.uk/news/health-19570024