Download Mild and Unilateral Sensorineural Hearing Loss

Managing Children with “Little” Hearing Losses
The impact of unilateral and mild hearing loss on the development of infants, preschoolers and school-aged children
has been largely unrecognized or ignored by audiologists, educators, and physicians (1, 3). In the last two decades
research findings have emphasized the subtle and long term effects even “little” hearing losses can have on the
development of academic, social, and behavior skills (2-7). Recognition of the deleterious effects associated with
mild and unilateral degrees of hearing loss in children has resulted in increasing attention toward the need to
consider use of amplification in classrooms and to develop 504 accommodation plans to support the success of these
children (8).
The spread of universal newborn hearing screening becomes across the United States has resulted in an increase in
the early identification of mild and unilateral hearing impairment including the identification of mild hearing loss in
the mid and high frequency ranges that previously would not have been identified prior to school age. With early
identification of these hearing deficits, a variety of management questions arise, such as: What is a reasonable
management plan for a baby with mild or unilateral hearing loss? Is amplification appropriate? If so, when should it
be fit? What services could the parents expect to receive from the early intervention program when their child has
one of these hearing problems?
USHL - Unilateral hearing loss: a sensorineural hearing loss in excess of 40 dB HL in the worse hearing ear;
hearing thresholds not to exceed 15 dB HL in the better hearing ear for the frequencies of 1000, 2000,
and 4000 Hz. Air-bone gap not to exceed 10 dB HL.
BSHL - Mild bilateral sensorineural hearing loss of flat configuration: an average sensorineural hearing loss in
both ears in the range of 20 dB HL to 40 dB HL for the frequencies of 1000, 2000, and 4000 Hz. Air-bone
gap not to exceed 10 dB HL.
HFSHL - Minimal sensorineural hearing loss in the high frequency range: air-conduction thresholds greater than
25 dB HL at two or more frequencies including or above 2000 Hz (3000, 4000, 6000, 8000 Hz) in both
ears with air-bone gaps at 3000 and 4000 Hz no greater than 10 dB.
With the implementation of universal newborn hearing screening, the audiologist and early intervention system may
be encountering the need to appropriately manage “little” hearing losses in infants and toddlers for the first time.
Parents and the medical community may have questions regarding the extent that seemingly minor hearing
problems can be expected to impact child development and how this relates to needed audiological management
and early intervention services.
Prevalence
Prevalence studies have long indicated that 1 baby per 1000 will be born deaf (4). Recent studies have indicated that
a total of 5.4%-5.6% of the childhood population will have some degree of hearing impairment (9,10). Even mild and
unilateral sensorineural hearing loss will cause children to be at risk for the following problems:
1. difficulty understanding speech under adverse listening conditions (background noise, reverberation,
distance greater than one meter, etc.), especially if the hearing loss is not identical in both ears (5, 1114, 37)
2. increased family tension, family disruption, breakdowns in family communication, greater fatigue of
listener’s with hearing loss, and social isolation (16-17 )
3. diminished self-esteem, social-emotional health at risk, increased incidence of behavior problems (2, 7,
18-20)
4. delayed development of verbal skills and reduced academic achievement (21-25)
As with other disability conditions there are fewer children with severe and profound degrees of impairment and a
much higher number of children with lesser degrees of hearing loss. Of 2995 the 8 – 12 months old infants that were
tested, 168 had identifiable hearing loss (5.6% of infants tested). Of this number, 18 infants had permanent bilateral
mild hearing loss (0.6% of total infants tested) and 51 infants (1.7% of total) had unilateral permanent hearing loss
from mild to profound degree9. Of 1218 children in grades 3, 6, and 92, the prevalence of bilateral hearing loss in
children was 2.2% and the prevalence of unilateral hearing loss in children was 3.4%.
Table 1. Of 2995 8-12 month-old infants tested, 168 had identifiable hearing impairment (5.6% of infants tested) in
234 ears (4% ears tested had hearing impairment)9
Hearing Loss
Both
unilateral and
bilateral
Bilateral only:
conductive +
permanent
Unilateral
only:
conductive +
permanent
Bilateral:
permanent
Unilateral:
permanent
27-39 dB HL
152 ears
(65% of ears with
hearing loss)
60 ears
(30 infants)
40-59 dB HL
42 ears
(18% of ears with
hearing loss)
16 ears
(8 infants)
60-90 dB HL
19 ears
(8% or ears with
hearing loss)
8 ears
(4 infants)
100+ dB HL
21 ears
(9% of ears with
hearing loss)
8 ears
(4 infants)
92 ears/ infants
26 ears /infants
11 ears /infants
13 ears/ infants)
18 infants
(1.33% total)
57 infants
(1.9% total)
4 infants
(0.13% total)
14 infants
(0.47% total)
3 infants
(0.1% total)
8 infants
(0.27% total)
4 infants
(0.13% total
11 infants
(0.37% total)
Amplification for children with hearing loss in only one ear
A large proportion of children who are being identified by universal newborn hearing screening have unilateral
hearing impairment. There are several compelling reasons to provide direct audiological management to children
with this type of hearing impairment. First, it is recognized that there is a critical time period from birth to the first
months and years of life during which neural development and important synaptic connections are being formed (2630). Children with unilateral hearing loss in excess of 40 dB HL are at risk for sensory deprivation due to a lack of
stimulation of the impaired ear’s cochlea and the auditory pathways. In the case of children with unilateral hearing
loss who may later lose the hearing ability in their better hearing ear, the prevention of sensory deprivation in the
poorer hearing ear can be highly important to their future auditory function. This is the strongest argument for early
amplification of hearing loss in one ear. Second, when two ears work together the ability to hear in noise is better
than for either ear alone. A loss of this binaural summation advantage results in significant negative effects on
speech recognition for persons with unilateral hearing loss (31-34). For example, one study found that listening with
two ears together can result in a word recognition score that is approximately 18-30% better than the score that is
obtained when only one ear is used to listen (35). Third, two ears also work together to allow us to listen more
effectively in noisy situations and to localize the direction of sound sources (36). That is, without auditory input into
two ears, it is very difficult for a listener to attend to one conversation in the midst of competing conversations or
noise (36). Taken together, research findings clearly support the need for early identification, amplification, and
intervention for infants and toddlers with unilateral hearing loss.
Comment: The use of amplification does not “inoculate” a child with hearing loss from developing delays in
language, cognitive, or social skills. The use of amplification in early childhood can be assumed to decrease a child’s
risk for developing these delays. Development of competence of subtle social cues, fast-paced conversation, and
understanding of humorous or idiomatic expressions requires consistent hearing of incidental language surrounding
a child in infancy, toddlerhood and as a preschooler. The consistent use of amplification can allow a child to master
these skills prior to school age. Once a child reaches school age, cosmetic and social concerns may arise that can
cause the parent/child to choose to discontinue consistent hearing aid wear as the child enters school or shortly
thereafter. In the classroom, hearing aid benefit for mild or unilateral hearing losses can be limited due to
background noise in the classroom that also will be amplified and will interfere with listening, unless technology
(e.g., FM system) is also used by the child in the classroom. Unless classroom hearing technology is used consistently
in conjunction with personal amplification, the level of interference in communication due to amplified noise may be
more disruptive to a child’s attention and learning than not wearing hearing aids. Although discontinuing use of
personal amplification is not a preferred choice, early use of hearing aids can provide gains in language, listening,
learning, and social skills that will provide continuing longterm benefit to the child.
Suggestions for Evaluation and Management of Infants Identified by Newborn Screening:
Mild and Unilateral Sensorineural Hearing Loss
AGE
2 weeks
to 3
months
of age
3-6
months
6-9
months
AUDIOLOGICAL
FAMILY-CENTERED INTERVENTION
Hearing loss indicated via universal newborn hearing screening,
high-risk indicators, or referral. Confirm presence/degree of loss via
OAE/ABR. Refer family to local early intervention program (Part C).
Share written information with parent1. If unilateral loss, consider
amplification. If hearing aids are recommended, seek medical
clearance and begin the necessary funding approval process.
UNHS personnel inform parents of newborns that do
not pass universal hearing screening about the results
of screening in a culturally sensitive and language
appropriate manner and the describe the need for
evaluation to rule out the presence of a hearing
problem. Initial contact with family by early
intervention program (EIP). EIP to provide written
information describing the schedule of communication
and auditory development1. Provide parents with a list
of pertinent websites, written materials.
Offer to connect the parents with other parents of
children with unilateral or mild hearing loss. Early
interventionist support to family during the
amplification adjustment period. Opportunity to assist
with establishing the amplification wear pattern,
provide tips to keep hearing aid(s) on child, increase
parent comfort with inserting earmolds, practice of
hearing aid checks using Ling 6-sound test.
Appropriate amplification fit to hearing loss by pediatric
audiologist2. Fit loaner hearing aids as necessary while waiting for
3rd party payer approval. Special consideration given to instrument
specifications such as noise reduction/suppression, locking battery
drawer, FM capability. Communicate/consult on specific
amplification considerations with early interventionist and parent to
assure fit of amplification to natural environment needs.
Behavioral testing to confirm degree of hearing loss and obtain
frequency specific information. Compare to early OAE/ABR
confirmation for indication of possible loss progression. Coordinate
with early interventionist to collaborate with family for completion
of Early Listening Function3 (ELF) or other amplification validation
measure.
Stress the importance of frequent, meaningful parent child interactions at close proximity. Emphasize the
concept of “Language is caught, not taught” and the
importance of early auditory development. Coordinate
with audiologist and family to complete the ELF or
other amplification validation and auditory skill
development measure such as Little Ears4.
Discuss the possibility of hearing loss progression and
need for monitoring of hearing ability. Check
communication and cognitive milestones6. Now that
child is mobile, discuss impact of listening from a
distance and background noise as interfering with
learning incidental language. Share information on
child’s development with audiologist.
At 24 months obtain more comprehensive assessment
of child development6. Delays in expressive language
may begin to be apparent by 18 months. If concerns,
address the need for communication intervention
services. Emphasize the importance of reading aloud
to the child on a daily basis.
Monitor communication development at 30 months.
Discuss transition to community or public school
preschool setting at age 3, especially if there are
deficit areas present. Discuss need for child to develop
independent skills with hearing aid(s)7 Final
communication inventory and evaluation prior to age
3. Provide information to parent on the availability of
establishing a 504 plan at school-age and potential
need for FM technology in school classrooms. Exit
from early intervention.
Reevaluate hearing to check stability of hearing loss. Research
indicates ¼ of children with hearing loss may have loss develop in
the better ear. Remake earmolds as needed. Reverify output, gain,
frequency response of amplification in relation to ear canal growth2.
Cross-check appropriateness of amplification via feedback from
parent and early interventionist. Now that child is mobile, consider
appropriateness of use of FM in home or child care.
Reevaluate hearing to check stability of hearing loss. Remake
16-24
earmolds as needed. Reverify amplification performance. Crossmonths check appropriateness of amplification via feedback from parent
and early interventionist (i.e., via ELF). Communicate any changes in
hearing status with early interventionist. Discuss lifelong need to
avoid overexposure to noise and for regular hearing tests.
Reevaluate hearing, including WIPI half-list at 40 dB HL. Remake
25-36
earmolds as needed. Reverify amplification performance. Discuss
months the need to begin to train child to put on own hearing aids. Provide
modeling on how to answer peer questions about wear of hearing
aid(s). As child approaches entry into preschool, in conjunction with
the early interventionist, pursue the purchase of a personal,
classroom, or desk-top sound field amplification system. Consider
FM need based on support from parent report (CHILD7 report form)
or if WIPI test results for perception of low level speech in +5 S/N
are < 88% correct.
1. Example: So Your Child Has A Hearing Loss, http://www.agbell.org
2. ASHA Pediatric Working Group on Amplification Fitting
3. Early Listening Function (ELF), http://www.phonak.pl/com_elf_questionnaire_gb.pdf or www.sifteranderson.com
4. Little Ears Auditory Questionnaire http://www.medel.com/US/img/download/BRIDGE_Catalog.pdf
5. Suggested instruments: Communication and Symbolic Behavior Scales- Developmental Profile (CSBS-DP) The Mullen Scales of
Early Learning; Ages and Stages Questionnaires (ASQ), http://firstwords.fsu.edu
6. Examples: Minnesota Inventory of Early Child Development (24-40 months), ELM; Denver Developmental Screening Test
7. Children’s Home Inventory for Listening Difficulties (CHILD) www.sifteranderson.com or
http://www.oticonusa.com/eprise/main/SiteGen/Uploads/Public/Downloads_Oticon/Pediatrics/CHILD_Questionnaire.pdf
9-15
months
Sequence of Development for Infants and Toddlers:
Auditory, Language, and Speech
Approx.
Age
0-28
days
1-3
months
4-7
months
8-12
months
1-2
years
2-3
years
Auditory Development
Startle response; attends to
music and voice, soothed by
parent’s voice; some will
synchronize body
movements to speech
patterns; enjoys time
“enface” position; hears
caregiver before being
picked up
Looks for sound source;
associates sound with
movement; enjoys parent’s
voice; attends to noise
makers; imitates vowel
sounds
Uses toys/objects to make
sounds; plays with noise
makers; pays attention to
music; enjoys rhythm games;
responds to changes in tone
of caregiver voice; notices
toys that make sound; moves
eyes in direction of sounds
Attends to TV; localizes to
sounds/voices; enjoys
rhymes and songs; enjoys
hiding game; responds to
vocal games (e.g., So
Big!!,Peek-a-boo)
Dances to music; sees parent
answer telephone/doorbell;
answers to name call; listens
to simple stories, songs, and
rhymes
Listens on telephone; dances
to music; listens to story in a
group; goes with parent to
answer door; awakens to
smoke detector.
Language Development
Speech Development
Startles to loud sounds;
smiles when spoken to;
seems to recognize parent
voice and quiets if crying;
increases or decreases
sucking behavior in response
to sound.
Recognizes some words;
responds to verbal
commands (bye-bye);
learning to recognize name;
Makes pleasure sounds (cooing,
gooing); cries differently for
different needs. Smiles when sees
known caregiver
Recognizes words for
common items like “cup,”
“shoe,” “juice.” Begins to
respond to requests.
Understands NO.
Babbling has both long and short
groups of sounds such as “tata
upup bibibibi.” Uses speech or
non-crying sounds to get and keep
attention. Imitates different
speech sounds. Has 1 or 2 words
(no, dada, mama) although they
may not be clear.
Says more words every month.
Uses some 1-2 word questions
(“Where kitty?”). Puts 2 words
together (“More cookie”). Uses
many different consonant sounds
at the beginning of words.
Points to pictures in a book
when named; points to a few
body parts when asked;
follows simple commands
and understands simple
questions (“Roll the ball”
“Where’s your shoe?”)
Understands differences in
meaning (“go/stop,”
“up/down”). Follows two
requests (“Get the book and
put it on the table”). Attends
to travel activities and
communication.
Babbling sounds more speech-like
with many different sounds,
including p, b, and m. Vocalizes
excitement and displeasure;
makes gurgling sounds when left
alone and when playing with
caregiver.
Has a word for almost everything.
Uses 2-3 word “sentences” to talk
about and ask for things. Speech is
understood by familiar listeners
most of the time. Often asks for or
directs attention to objects by
naming them.
Adapted from: Ear Infections and Language Development, www.ed.gov/pubs/edpubs.html and Developmental Index of Auditory
and Listening (DIAL), www.edaud.org
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.
23.
24.
25.
26.
27.
28.
29.
30.
31.
32.
33.
34.
35.
36.
37.
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DEVELOPED BY KAREN L. ANDERSON, ED.S., AUDIOLOGY CONSULTANT, EARLY HEARING LOSS DETECTION AND
INTERVENTION PROGRAM, FLORIDA DEPT OF HEALTH, CHILDREN’S MEDICAL SERVICES 2001.