Journal of Intellectual Disability Research
45 i
VOLUME 40 PART 5 pp 45I-456 OCTOBER I996
Dutch consensus on diagnosis and treatment of hearing
impairment in children and adults with intellectual disability
H. M. Evenhuis on behalf of the consensus committee*
Hooge Burch Centre for People with Intellectual Disability, Zwammerdam, The Netherlands
Abstract
This is an abridged transiation of a recent Dutch
consensus on diagnosis and treatment of hearing
impairment in children and adults with intellectual
disability. Available diagnostic methods are discussed.
Tbe use of Oto-Acoustic Emissions as a rapid and
low-cost objective screening method is particularly
encouraged. Risk groups, protocols for early
identification and diagnostic screening in older children
and adults, recommendations for treatment and support,
and priorities for research have been formulated.
Keywords hearing loss, mental retardation,
Oto-Acoustic emissions, screening
Correspondence: Dr H.M. Evenhuis, Hooge Burch, Centre for
People with Intellectual Disability, P.O. Box 2027, 2470 AA
Zwammerdam, The Netherlands.
*Members of the consensus committee H.M. Evenhuis (chairman),
physician for intellectually disabled people; R.J.C. Admiraal,
ENT-specialist; C. de Bal, speech and hearing therapist; A.M.
Bierman, speech and hearing therapist; J.P.L. Brokx, audiologist;
Professor J.P.M. van Dijk, behavioural therapist; Professor E.H.
Huizing, ENT-specialist; M. Mul, general practitioner; W.H.M.
Roerdinkholder, speech and hearing therapist; J. Trommelen,
physician for intellectually disabled people; W.I.M.
Veraart-Schretlen, physician; J.A.A.M. Vereiiken, speech and
hearing therapist; R.A.W. Verpoorten, speech and hearing therapist;
J.J. Verschuure, audiologist; M. Vink, physician for intellectually
disabled people; J.J. van der Wai, speech and hearing therapist;
G.A. van Zanten, audiologist; P. ten Have, staff member CBO.
© 1996 Blackwell Science Ltd
Introduction
For a number of reasons, a considerable amount of
undiagnosed hearing impairment is to be expected
in the population with intellectual disability.
Although epidemiological data for this population
are still scarce, the prevalence of early childhood
hearing impairment is higher than in the general
population, since many causes of congenital and
early acquired childhood hearing impairment, such
as intrauterine infections, perinatal asphyxia and
meningitis, may result in cerebral damage as well
(van Rijn 1989). Besides, age-related hearing loss is
increasingly to be expected as a result of the growing
life expectancy in this population (Van
Schrojenstein Lantman-de Valk et al. 1994;
Evenhuis 1995). Moreover, hearing loss as a result
of impacted ear wax is a frequent problem in adults
with intellectual disability (Crandell and Roeser
1993)- People with Down's syndrome are a risk
group both for childhood conductive hearing loss
(Brooks et al. 1972; Roizen et al. 1993) and early
presbyacusis (Buchanan 1990; Evenhuis et al. 1992).
Experience, as well as the studies mentioned
above, shows that hearing loss is seldom
spontaneously mentioned by patients, and
frequently remains unrecognized by parents,
teachers and staff of homes. Therefore, active
diagnostic screening is important in this population.
However, as a result of the tendency towards
community care for people with intellectual
 Journal of Intellectual Disability Research
452
H. M. Evenhuis * Consensus hearing impairment
disability, medical care is usually only provided
when patients or their caregivers ask for it (Wilson &
Haire 1990). Apart from this, specialized skills and
equipment, necessary to overcome diagnostic and
therapeutical problems, especially in those with
moderate and severe intellectual disability, are not
always easily accessible.
Therefore, the Dutch association of physicians
working in the care for people with intellectual
disability (NVAZ) has set up a multidisciplinary
committee to prepare a consensus for diagnosis and
treatment of hearing impairment in children and
adults with intellectual disability. The initiative was
supported by the Dutch association of speech and
hearing therapists working in this field (NGBZ), as
well as the national organization for quality
assurance in hospitals (CBO). The consensus text
produced by this committee has been amended by a
larger group of colleagues, including general
practitioners, ENT specialists, audiologists and
paediatricians. The Journal of Intellectual Disability
Research is glad to publish an abridged translation of
the Dutch report (NVAZ 1995). The committee's
recommendations for practical organization are not
included because of national differences in health
care systems.
Definitions
Hearing loss is defined as 'the mean hearing
threshold loss, measured by pure tone audiometry,
at 1000, 2000 and 4000 Hz' for each ear. This
definition, although different from WHO/ISO
definitions, is currently used in the Netherlands
because of its relevance to speech discrimination. It
also corresponds to the standard proposed by the
British Association of Otolaryngology and British
Society of Audiology (1983).
Hearing impairment is defined as a mean
threshold loss of 25 dB and over. Definitions of the
Tabie I Degree of hearing impairment
Degree Loss (dB)
Mild 25-30 • further diagnostic assessment. Nowadays, the test is
Moderate 30-60
Severe 60-90
Profound 90-120
(Sub) total deafness >I2O
© 1996 Blackwell Science Ltd, Journal of Intellectual Disability Research 40, 451-456
VOLUME 4 0 PART 5 OCTOBER I996
degrees of hearing impairment are presented in
Table i. The consensus committee is aware that
these definitions are usually related to 500, 1000 and
2000 Hz, but decided to redefine them in relation to
1000, 2000 and 4000 Hz.
Diagnostic methods
Hetero-anamnestic interview and otoscopy
A direct anamnesis is not normally relevant in
individuals with intellectual disability. The interview
of caregivers is aimed at the cause of intellectual
disability and possible hearing loss. Impacted ear
wax and middle ear pathology are detected by
otoscopy.
Tympanometry
This method of oto-admittance measurement is
easily applicable in most people with intellectual
disability for a quick detection of certain forms of
middle ear pathology, such as Eustachian tube
dysfunction, middle ear infection and some types of
eardrum pathology (Brooks et al. 1972).
Screening of hearing function
People with mild intellectual disability can be
screened by general practitioners using a screening
audiometer. Screening with whispered speech at 3 m
distance for each ear apart is easily applicable in
people with developmental ages of 3 years and over
(Evenhuis 1995). All individuals who do not
respond adequately should be referred for further
diagnosis.
Measurement of Oto-Acoustic Emissions (OAE) is a
recently developed objective method to establish
hearing losses of about 25 dB and over (Kemp &
Ryan 1990; Probst et al. 1991). This rapid and
low-cost method is not intended to define the exact
auditory threshold but allows to select persons for
mainly used for screening of newborn babies (NIH
Consensus Statement 1993), but it is also promising
for use in people with intellectual disability (Gorga
etal. 1995).
 Journal of Intellectual Disability Research
453
H. M. Evenhuis * Consensus hearing impairment
Subjective response audiometry
Regular pure-tone and speech audiometry can
usually be applied to assess hearing function in
people with mild intellectual disability. However,
many people with moderate and severe intellectual
disability or with behavioural problems have to be
assessed with adapted methods, using conditioning
techniques (Fulton & Lloyd 1975). This requires
specialized skills and equipment. In some cases only
global, binaural hearing thresholds can be obtained.
Adapted pure tone audiometry (play audiometry)
People are trained to respond by means of playing
(e.g. drawing, puzzling, putting bricks into a box or
building towers) as a reaction to pure tones. This
test is applicable at developmental ages of 2.5-3
years and over.
Visual reinforcement audiometry
Visual reinforcement audiometry is based on the use
of visual rewards, following an adequate reaction to
pure tones or broad-band noise (Thompson et al.
1989; Moore 1995). It is applicable at developmental
ages of 1.5-2 years and over.
Behavioural response audiometry
Behavioural response audiometry is based on
observation of behavioural responses as a reaction to
certain noises (Gans & Flexer 1983; McCormick
1995)- It is applicable at developmental ages under
1V2 years.
TOUCH-procedure
The TOUCH-procedure is a promising method,
using tactile stimuli for conditioning of hearing, that
was originally developed for autistic children but is
also applicable in persons with severe intellectual
disability (Verpoorten & Emmen 1995). Evaluation
of its reliability and sensitivity is in preparation.
Adapted methods of speech audiometry
Methods of speech audiometry developed for young
children are also used in people with intellectual
disability.
© 1996 Blackwell Science L.td, Journal of Intellectual Disability Research 40, 451-456
VOLUME 4 0 PART 5 OCTOBER I996
Auditory Brainstem Response Audiometry (ABR)
Auditory brainstem response audiometry (ABR)
Gacobson 1985; Drift et al. 1987), an objective
method of audiometry not requiring any active
cooperation, has been successfully applied in
difiScult-to-test children and adults with intellectual
disability (Stein et al. 1987; Evenhuis et al. 19925
Maurizi et al. 1995). It quantifies high-frequency
hearing loss for each ear apart with an estimate of
conductive losses. It can also be applied in people
with severe neurological damage; in case of
abnormalities, OAE may be additionally necessary
to distinguish cochlear from neurological pathology.
Early identification
Early diagnosis and intervention of childhood
hearing impairment is of crucial importance for the
development of the audition system, the future
development of speech and language and therefore,
the social and emotional development of the child.
Risk groups (Table 2) should be screened before the
age of 6 months by means of OAE, followed by
behavioural response audiometry or ABR in case of
abnormalities (NIH Consensus Statement 1993). At
the age of 9 months, hearing function of all Dutch
children is screened by means of a behavioural test.
However, since this test is not reliable in children
with developmental delay, hearing function of this
Tabie 2 Risk factors for hearing impairment in the
population with intellectual disability
Corjgenital
1. Hereditary sensorineural hearing impairment
2. Cranlofacial syndromes
2. Inborn errors of metabolism (e.g. mucopolysaccharidoses,
mucolipidoses and Refsum's disease)
Pregnancy and birth
4. Intra-uterine infection: rubella, cytomegalovirus, syphilis
and toxoplasmosis
5. Ototoxic drugs
6. iHyperbilirubinaemia
7. Asphyxia (pre- and dysmaturity)
Lote-onset
8. Meningitis (especially pneumococcal)
9. Ototoxic drug use
10. Conditions related to Down's syndrome
11. Old age
 Joumal of Intellectual Disability Research
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H. M. Evenhuis * Consensus hearing impairment
group is usually not assessed at all. Therefore, the
consensus committee recommended that all children
with developmental delay at this age should be
screened by means of OAE. We are aware that OAE
is ruled out by middle ear infections, which are
increasingly frequent during the first year of age.
Recent insights are, that screening before the age of
6 weeks would be the most appropriate.
Diagnostic screening in older children and
adults
Annual otoscopy is recommended in all children
and adults with intellectual disability for detection of
impacted ear wax and unrecognized middle ear
pathology. In children and adults with Down's
syndrome, otoscopy might even be performed twice
a year. Hearing function should be screened at 5,10
and 15 years of age and every 5 years from 50 years
of age. In case of regular noise exposure (>8o dB),
annual screening is recommended. However,
screening of hearing function is recommended every
3 years during life in children and adults with
Down's syndrome.
Treatment
The frequent exposure to background noise in
homes and daycare centres on one hand, and
problems with hearing aid habituation and control
on the other, may hamper treatment of hearing
impairment in people with intellectual disability.
Background noise
The design of homes and daycare centres should
generally be tuned to hearing impaired people and
acoustical circumstances should be be optimalized
(e.g. partition of large rooms, soft floor-coverings
and slightly elastical wall-coverings). Education of
staff to reduce background noises from sources such
as radio and TV sets is important.
Hearing aids
Adjustment of hearing aids is seldom problematic in
people with mild intellectual disability. Most people
with more severe intellectual disability can also be
© 1996 Blackwell Science Ltd, Joumal of Intellectual Disability Research 40, 451-456
VOLUME 4 0 PART 5 OCTOBER I996
trained to use hearing aids (McCracken & Bamford
1995). Even in cases of severe hearing impairment,
which hampers verbal communication, a hearing aid
can be useful to identify environmental noises. In
people with severe or profound intellectual
disabilities, the aim of a hearing aid is not verbal
communication in the first place. In our experience,
these people can gradually be trained to accept a
hearing aid; as a result, they may become more
involved with their environment and more
explorative.
If adjustment of the hearing aid is not optimal,
people with moderate and severe intellectual
disabilities may not complain, but refuse their
hearing equipment. This may especially be
anticipated if amplification has to be based on
limited audiometric data. Therefore, technical
adjustment should be advised by audiologists with
sufiRcient experience with this population.
Individuals with moderate or severe intellectual
disability often need a habituation period of several
months.
Moreover, especially for those living in homes,
varying caregivers may be responsible for the use
and control of the apparatus. In our experience, the
success of hearing aid adjustment in a person with
intellectual disability is dependent on their
permanent support and understanding. Therefore,
amplification should be followed by a longitudinal
process of training, support and control of the
hearing impaired person and his caregivers by a
specialized speech and hearing therapist.
Alternative or supportive communication
If adjustment of a hearing aid is not feasible or does
not enable verbal communication, the specialized
speech and hearing therapist may train the hearing
impaired person in the use of an alternative
communication method. Many methods of
communication, like body language, sign systems,
the use of pictures, pictograms or object symbols,
are available and applicable for people with different
levels of intellectual disability.
Practical organisation
Organization of early identification and optimal
diagnosis and treatment of hearing impaired
 Joumal of Intellectual Disability Research
455
H. M. Evenhuis * Consensus hearing impairment
children and adults with intellectual disability will
vary with the health care system in each country.
Nevertheless, structured cooperation of an
audiological physician, ENT surgeon, audiological
scientist, specialized speech and hearing therapist
and hearing aid technician (e.g. by cooperation of
district audiological centres and centres for
intellectual disability) is recommended. Referral to
such structures by general practitioners,
paediatricians and ENT specialists should be
encouraged.
Priorities for research
Finally, the consensus committee formulated the
following priorities for further scientific research in
the population with intellectual disability:
• Epidemiological studies of thefi-equencyof
hearing impairment in the population with
intellectual disability as well as further
identification of risk groups.
• Reliability and sensitivity of adapted and objective
diagnostic methods.
• Longitudinal studies on the effect of hearing loss
and hearing aid adjustment on communication
and social development.
• Effects of early intervention of congenital and
childhood hearing impairment.
• Evaluation of structures of professional
cooperation.
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© 1996 Blackwell Science Ltd, Journal of Intellectual Disability Research 40, 451-456