Sensori- Neural Hearing Loss Patient Care Pathways Diagnosis to School Leaver
Combined Child Health Service
Sensori- Neural Hearing Loss
Diagnostic and Patient Care Pathways
Diagnosis to School Leaver
November 2008
Combined Child Health Service
Table of Contents
Pathway 1
Well Baby
Pathway 2
High Risk Baby
Pathway 3
Late Onset Hearing Loss
Pathway 4
Patient Care Pathway
Guidance on the use of Sensori-neural Hearing Loss Patient Care Pathway
Sensori-neural Hearing Loss
Universal Newborn Hearing Screening (UNHS)
Late onset hearing loss
Audiology Service
Speech and Language Service
Information for Parents
Refer to Community Child Health
Universal Child Health Screening
Social Work Department
Education
Joint Assessment of Needs / Integrated Assessment
Individual Educational Plan/ Co-ordinated Support Plan
Key worker
Regular Review
Transfer to Adult Services
Annex 1
Checklist for Reaction to Sounds
Checklist for Making Sounds
Documents used in the development of pathways
References
Working Group Members
November 2008
Combined Child Health Service
Pathway 1
Well Baby
Pathway 2
High Risk Baby
Pathway3
Late Onset Hearing Loss
Pathway 4
Patient Care Pathway
Guidance on the use of Sensori-neural Hearing LossPatient Care Pathway
Sensori-neural Hearing Loss
Sensori-neural hearing loss results from damage to the neural receptors in the inner ear (the hair cell, organ of Corti), the nerve pathway to the brain (notably the auditory nerve), or the area of the brain that receives sound information.
This type of hearing loss is usually permanent.
Level of hearing loss may worsen as the child grows older, depending on the cause of the hearing loss.
Levels of Hearing Loss: If a sound has to be greater than 20dB for a person to hear it, then they are said to have a hearing loss. There are 4 different levels of hearing loss:
Mild: quietest sound heard is between 21-40dB. Person will have difficulty following a conversation if the speaker is more than six feet away or if there is background noise.
Moderate:quietest sound heard is between 41-70dB. Person can hear if speaker spoke loudly about three-five feet away.
Severe: quietest sound heard is between 71-95dB. Person can hear if speaker shouts from about a foot away.
Profound:quietest sound heard louder than 95dB. Person can only hear very loud sounds.
Universal Newborn Hearing Screening (UNHS)
The objective of the UNHS screening programme is to identify, as soon after birth as possible ‘Significant’ (more than 40dB of loss, i.e. infants with moderate, severe or profound losses) Permanent Congenital Hearing Impairment (PCHI), which can then be treated to give the baby the best chance of compensating for their hearing loss. It is well documented that infants whose hearing loss is identified early and dealt with appropriately develop better speech and language skills than those diagnosed after six months of age.
Using the distraction test, (which was carried out when the babies were between 7-8 months old) some children with hearing impairment were not detected until the children were at least 18 months and some not until they were 3-4 years old.
Universal Newborn Hearing Screening is offered to parents of all newborn babies in Scotland within the first four weeks after birth, thus reducing the age in which hearing impairment is confirmed and therefore improving the child educational and social needs.
(NHS Quality Improvement Scotland 2005)
Newborn Hearing Screening allows the identification of two groups of babies:
- Babies who have passed their hearing screening and therefore need only be investigated further if they are at risk of late-onset or progressive hearing loss.
- Babies who have failed their hearing screen and need to have further testing to establish their hearing status.
Prior to hearing screening being carried out parents will receive information leaflets explaining the Newborn Hearing Screening Programme.
- Your Baby’s Hearing Screen
- Your Baby’s Visit to the Audiology Clinic
The information leaflets may be give out at the Antenatal Clinic (at 34 weeks gestation), in maternity hospital prior to testing or sent out to parents with hearing screening appointment.
UNHS is carried out using, Otoacoustic Emission Test (OAE), Automated Auditory Brainstem Response (AABR) testing or a combination of both may be required. Further assessment may also be required using threshold Auditory Brainstem Response (ABR).
Parents of all newborn babies in Grampian are offered the OAE or AABR test.
Otoacoustic Emissions Test (OAE): is a non invasive test which tests the hearing receptor organ, or cochlea. The OAE tests the auditory pathway up to but not beyond the cochlea. It does not test the connections between the cochlea and the hearing centres of the brain stem.
OAE is an automated system with the hearing screener only having to position the probes in the baby’s ears and help settle baby if required.
A soft tipped probe is placed in baby’s ear canal. This sends clicking sounds down the ear and records the ear’s response using a microphone which is incorporated in the probe. When the ear receives sounds, the cochlea usually produces an echo. Using computer software the hearing screener can see how both ears respond to sound, whether the response is a clear response or no clear response.
Automated Auditory Brainstem Response (AABR):is a non invasive test which tests the pathway up to the brainstem, with results being affected by any problem in the outer or middleear.
This involves three small sensors being placed on the baby’s head. Soft earphones are placed over the baby’s ears and a series of clicking sounds are played while the electrodes record the brain’s electrical activity. The computer software assesses the electrical activity for the presence of an auditory component and gives a decision of clear response or no clear response.
Clear Response
If a clear response is obtained in both ears the baby may be discharged from Audiology care. Parents will receive checklists to refer to if they have concerns about their child hearing, these are, Reaction to Sounds and Making Sound. (See annex1)
A clear response from a baby with any of the following high risk factors may require a repeat hearing test at 9 months old (corrected age if premature).
- Family history of sensori-neural hearing loss
- Admitted to Neonatal Unit
- Repeated courses of aminoglycoside or aminoglycoside levels in toxic range at any time.
- Jaundice where bilirubin level indicated a need for exchange transfusion
- Proven or possible congenital infection
- craniofacial abnormalities including chromosomal or syndromic conditions, (e.g. Down’s Syndrome, cleft palate)
- Neurodegenerative or neurodevelopmental disorder
(NHS Scotland Screening Programmes, 2004)
All professionals involved in the care of children e.g. General Practitioners Health Visitors, Child Development Services, ENT Services, Speech and Language Therapists, Nursery and school staff should remain aware of the possibility of hearing loss at any age despite a clear response on newborn hearing screening. Refer back to Audiology can be made at any time if parents and/or professionals have any concerns.
No Clear Response
If there is no clear response in one or both ears ABR testing will be carried out by the audiologist.
Auditory Brainstem Response (ABR): reflects activity of the cochlea nerve and the auditory brainstem pathways, therefore assessing hearing beyond the cochlea.
Threshold ABR is carried out by an audiologist to establish accurate levels of hearing
Unilateral
Threshold within normal range with ABR testing (In Grampian accepted normal response of 40dB), discharged from Audiology. Checklists given to parents.
No clear response (raised level greater than 40dB), retest at 9 months old (corrected age if premature). If clear response, child discharged from Audiology service.
Bilateral
Mild/Moderate hearing loss, child retested at 9 months old (corrected age if premature). If hearing remains at same level after retesting discuss with parents use of hearing aid. Hearing checked yearly
Severe/Profound hearing loss, use of hearing aids discussed with parents. Ideally hearing aids should be prescribed and fitted before the child is 6 months old.
Late onset hearing loss
Late onset hearing loss may be progressive or acquired and cannot be detected at Newborn hearing screening.
As Health Visitor distraction tests and primary one (school entry) tests are no longer carried out it is important that professionals are vigilant in detecting these types of hearing losses.
Any suspicion of persistent hearing loss of any degree requires prompt referral for hearing assessment.
Age appropriate hearing tests will be carried out by Audiology services e.g.
Visual Reinforced Audiometry (VRA): This is a test which establishes the child’s behavioural response to sound.
Using an audiometer sounds of different volumes and frequencies are played through speakers. When the child hears the sound they turn their head towards the source and are rewarded by a visual display such as a toy lighting up.
Performance Testing: In this test a child is conditioned to wait for an auditory stimulus and then respond in a play activity such as placing the man in a boat.
The test is started with a demonstration by the tester. The tester holds the man ready to respond then promptly places the man in the boat when the sound stimulus is heard. After a few demonstrations the child should be offered the toy and the response guided by the tester. Once the child is prepared they should hold the item ready to respond for up to 10 seconds. The tester should vary the time between sound stimuli from 1-10 seconds to reduce the possibility of anticipatory responses.
Nottingham Toy Test (McCormick): The test is administered by setting out a display of toys containing only paired items known to the child. The child is conditioned to point to the toys on request. Children with normal hearing can identify the items at a minimal listening level of 40dB the aim of the test is to determine the quietest level at which the child can identify the items with 80% success.
Pure Tone Audiometry: an audiometer generates sounds at accurate volumes and frequencies. Sounds are played through headphones and the child is asked to respond when they hear them e.g. moving a toy, placing a peg into a board, pressing a button or saying yes.
Tympanometry: Shows how mobile the eardrum is. An eardrum needs to be flexible to allow sound to pass through it. A small earpiece is placed gently into the ear canal allowing gentle air pressure changes to be applied. The eardrum should move freely in and out with the change in pressure. The earpiece measures this by checking the sounds reflected by the eardrum. If the eardrum is too ridged (there is fluid (glue) behind it) the sounds bounce back off the eardrum instead of going through it. The amount of sound bounced back determines if the child has glue ear.
Audiology Service
The Audiology service provides age appropriate hearing tests to evaluate the degree, extent and nature of any hearing loss.
When hearing loss is identified in a child appropriate Audiology support is provided, this may involve monitoring the situation or the fitting of hearing aids.
Audiology support will continue even although a child/young person declines the option of having a hearing aid fitted.
If significant permanent hearing loss is identified, attendance at Audiology clinic will be required for hearing aid fitting/ trial.
Review will take place approximately 4-6 weeks after the initial fitting then as required to test hearing, verify and evaluate hearing aid function. Once satisfactory fit of hearing aid established the child/ young person will have an annul review. The child/young persons hearing levels, use and effectiveness of the hearing aid and ear-mould impressions are usually taken at this time.
A written report of the child’s Audiology results and findings will be sent to, Hearing SupportService, Speech and Language Therapy and Community Child Health Department. A copy of the report may also be sent to Social Work Department if they are involved with the child/ young person and parental/patient consent is obtained.
Hearing aids and ear moulds are maintained on a regular basis and repaired/replaced as required by the Audiology Department at the Royal Aberdeen Children’s Hospital.
Pre-School followed up monthly - yearly at Audiology clinic.
School aged children followed up yearly at Audiology clinic.
If the child has any underlying ENT problem they will be reviewed at the combined ENT/Audiology clinic.
If evidence of little or no benefit from use of conventional hearing aids the child may be referred through the ENT Services for cochlear implant assessment.
The cochlear implant team based at Crosshouse Hospital Kilmarnock will guide parents and professionals to the child’s suitability for a cochlear implant. (suitability of anatomical structure of cochlea, medical cause of hearing loss, age of child in relation to hearing loss) Many factors have to be taken into account which will be discussed with parents at time of assessment, to decide whether proceeding to surgical implant is in the child’s best interest.
Speech and Language Service
Communication assessment and progress of language skills is carried out by Speech and Language therapy.
TheSpeech and Language Service is available throughout Grampian and can be accessed by referral to:-
North AberdeenshireAberdeenCity
c/o Manager / Lead Therapistc/o Manager / Lead Therapist
SLT DepartmentSLT Department
ChalmersHospitalAiryhall Clinic
Clunie StreetSpringfield Road
BanffAberdeen
AB45 1JAAB15 7RF
Tel: 01261 879149Tel: 01224 310582
Central AberdeenshireMoray
c/o Manager / Lead Therapistc/o Manager / Lead Therapist
9 Watson AvenueSLT Department
HuntlySpynieHospital
AB54 8BFElgin
Tel: 01466 765013IV30 5PW
Tel: 01343 567162
South Aberdeenshire
c/o Manager / Lead Therapist
Arduthie Lodge
CommunityHospital
Kirkton road
Stonehaven
AB39 2NJ
Tel: 01569 792067
Information for Parents
Parents of children diagnosed with Sensori-neural hearing loss will require clear verbal and written information about the condition and of any associated health issues.
NHS Scotland, Newborn Hearing Screening Programme has produced a booklet “Your Baby has Hearing Loss”, available from the Audiology clinic.
Providing information on local support groups and other national and local resources will empower parents in the care of their child.
Further sources of information include:
Grampian Deaf Children’s Society
Website:
Contact chairperson or vice chairperson for details
ChairpersonVice Chairperson
Jim NeishCarrie Heddle
60 Highfield Walk63 Alder Drive
TurriffPortlethen
AberdeenshireAberdeen
AB53 4LGAB12 4WA
Tel: 01888 562089Tel: 07866 856356
Email: mail:
National Deaf Children’s Society Scotland
187-189 Central Chambers
93 Hope Street
Glasgow
G2 6LD
Tel: 0141 248 4457
Website:
NDCS Family Support Workers
Alan MacQueenDebbie Taylor
Tel: 01382 500580National Deaf Children’s Society
Email;16 Kenneth Street
Inverness
IV3 5NR
Tel: 01463 226509
Mobile: 07800658286
Email:
Aberdeen & North East Deaf Society
13 Smithfield Road
Aberdeen
AB24 4NR
Tel: 01224 494566
Email:
Website:
Refer to Community Child Health
Community Child Health is available at local clinics throughout Grampian and can be accessed by referral to:-
Community Child Health Department
Royal Aberdeen Children’s Hospital
Westburn Road
Aberdeen
AB25 2ZG
Raeden Centre orDr A Liebenberg
Midstocket RoadWard 2
AberdeenDr Gray’s Hospital
AB15 5PDPluscarden Road
Elgin
IV30 1SN
The Audiology department liaises regularly with Community Child Health. Further to receiving this information a medical appointment for Community Child Heath may be offered to manage health and development needs. This may include aetiological investigation and management of any medical condition if parents wish. Referral to Genetic services is offered to assess risk to any further planned children.
Community Child Health will liaise with parents and relevant professionals involved with the child’s care to help plan and provide appropriate support for the child and their family as required.
Referral to Ear, Nose and Throat (ENT) Services may be required for wax and middle ear effusion (glue ear) as this may compromise efficiency of hearing aids.
Liaison with ENT Services, Audiology, Health Visitor, Allied Health Professionals, Social Work, Hearing Support Service, Educational Psychology and Nursery School/School may be required to ensure the child’s care and social needs are being met.
Universal Child Health Screening
The early identification of a child requiring an assessment for health problems and developmental disorders is the aim of the child health screening and surveillance programmes.
Health for all Children (Hall 4) has led to a significant change in screening and surveillance in Scotland.
All children receive a health check when starting primary school, with little formal child health surveillance after that. In school aged children detection of problems should be part of mainstream school life.
Hall 4 states:-
Formal screening should be confined to the evidence based programmes agreed by the National Screening Committee.
Health professionals must respond promptly to parental concerns.
Formal universal screening for speech and language delay, global developmental delay and autism is not recommended.
Children starting school should receive the agreed screening programmes.
Health care of school-age children should include support for children with problems and special needs.
Scottish Executive 2005
Early identification, early therapeutic and educational intervention, and seamless delivery of services may help a child to maximise their potential.
Social Work Department
An assessment of need may be carried out to determine what support and services are required by the child and their family. This may include respite care, play schemes for child or siblings and details of available financial assistance.
The Aberdeen and North East Deaf Society (ANEDS) provide a service for children/young people and their family in AberdeenCity, Aberdeenshire and Moray.
This service can be accessed by contacting:-
Aberdeen & North East Deaf Society