Protocol for Managing Babies with



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, 11-14, 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 |27-39 dB HL |40-59 dB HL |60-90 dB HL |100+ dB HL |

|Both unilateral and |152 ears |42 ears |19 ears |21 ears |

|bilateral |(65% of ears with hearing|(18% of ears with hearing |(8% or ears with hearing |(9% of ears with hearing |

| |loss) |loss) |loss) |loss) |

|Bilateral only: |60 ears |16 ears |8 ears |8 ears |

|conductive + |(30 infants) |(8 infants) |(4 infants) |(4 infants) |

|permanent | | | | |

|Unilateral only: | | | | |

|conductive + |92 ears/ infants |26 ears /infants |11 ears /infants |13 ears/ infants) |

|permanent | | | | |

|Bilateral: permanent|18 infants |4 infants |3 infants |4 infants |

| |(1.33% total) |(0.13% total) |(0.1% total) |(0.13% total |

|Unilateral: |57 infants |14 infants |8 infants |11 infants |

|permanent |(1.9% total) |(0.47% total) |(0.27% total) |(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 (26-30). 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 |AUDIOLOGICAL |FAMILY-CENTERED INTERVENTION |

|2 weeks to |Hearing loss indicated via universal newborn hearing screening, high-risk |UNHS personnel inform parents of newborns that do not pass |

|3 months of|indicators, or referral. Confirm presence/degree of loss via OAE/ABR. Refer |universal hearing screening about the results of screening in |

|age |family to local early intervention program (Part C). Share written |a culturally sensitive and language appropriate manner and the|

| |information with parent1. If unilateral loss, consider amplification. If |describe the need for evaluation to rule out the presence of a|

| |hearing aids are recommended, seek medical clearance and begin the necessary |hearing problem. Initial contact with family by early |

| |funding approval process. |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. |

|3-6 months |Appropriate amplification fit to hearing loss by pediatric audiologist2. Fit |Offer to connect the parents with other parents of children |

| |loaner hearing aids as necessary while waiting for 3rd party payer approval. |with unilateral or mild hearing loss. Early interventionist |

| |Special consideration given to instrument specifications such as noise |support to family during the amplification adjustment period. |

| |reduction/suppression, locking battery drawer, FM capability. |Opportunity to assist with establishing the amplification wear|

| |Communicate/consult on specific amplification considerations with early |pattern, provide tips to keep hearing aid(s) on child, |

| |interventionist and parent to assure fit of amplification to natural |increase parent comfort with inserting earmolds, practice of |

| |environment needs. |hearing aid checks using Ling 6-sound test. |

| |Behavioral testing to confirm degree of hearing loss and obtain frequency |Stress the importance of frequent, meaningful parent - child |

|6-9 months |specific information. Compare to early OAE/ABR confirmation for indication of|interactions at close proximity. Emphasize the concept of |

| |possible loss progression. Coordinate with early interventionist to |“Language is caught, not taught” and the importance of early |

| |collaborate with family for completion of Early Listening Function3 (ELF) or |auditory development. Coordinate with audiologist and family |

| |other amplification validation measure. |to complete the ELF or other amplification validation and |

| | |auditory skill development measure such as Little Ears4. |

| |Reevaluate hearing to check stability of hearing loss. Research indicates ¼ |Discuss the possibility of hearing loss progression and need |

|9-15 months|of children with hearing loss may have loss develop in the better ear. Remake|for monitoring of hearing ability. Check communication and |

| |earmolds as needed. Reverify output, gain, frequency response of |cognitive milestones6. Now that child is mobile, discuss |

| |amplification in relation to ear canal growth2. Cross-check appropriateness |impact of listening from a distance and background noise as |

| |of amplification via feedback from parent and early interventionist. Now that|interfering with learning incidental language. Share |

| |child is mobile, consider appropriateness of use of FM in home or child care.|information on child’s development with audiologist. |

| |Reevaluate hearing to check stability of hearing loss. Remake earmolds as |At 24 months obtain more comprehensive assessment of child |

|16-24 |needed. Reverify amplification performance. Cross-check appropriateness of |development6. Delays in expressive language may begin to be |

|months |amplification via feedback from parent and early interventionist (i.e., via |apparent by 18 months. If concerns, address the need for |

| |ELF). Communicate any changes in hearing status with early interventionist. |communication intervention services. Emphasize the importance |

| |Discuss lifelong need to avoid overexposure to noise and for regular hearing |of reading aloud to the child on a daily basis. |

| |tests. | |

| |Reevaluate hearing, including WIPI half-list at 40 dB HL. Remake earmolds as |Monitor communication development at 30 months. Discuss |

|25-36 |needed. Reverify amplification performance. Discuss the need to begin to |transition to community or public school preschool setting at |

|months |train child to put on own hearing aids. Provide modeling on how to answer |age 3, especially if there are deficit areas present. Discuss |

| |peer questions about wear of hearing aid(s). As child approaches entry into |need for child to develop independent skills with hearing |

| |preschool, in conjunction with the early interventionist, pursue the purchase|aid(s)7 Final communication inventory and evaluation prior to |

| |of a personal, classroom, or desk-top sound field amplification system. |age 3. Provide information to parent on the availability of |

| |Consider FM need based on support from parent report (CHILD7 report form) or |establishing a 504 plan at school-age and potential need for |

| |if WIPI test results for perception of low level speech in +5 S/N are < 88% |FM technology in school classrooms. Exit from early |

| |correct. |intervention. |

1. Example: So Your Child Has A Hearing Loss,

2. ASHA Pediatric Working Group on Amplification Fitting

3. Early Listening Function (ELF), or

4. Little Ears Auditory Questionnaire

5. Suggested instruments: Communication and Symbolic Behavior Scales- Developmental Profile (CSBS-DP) The Mullen Scales of Early Learning; Ages and Stages Questionnaires (ASQ),

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) or

Sequence of Development for Infants and Toddlers:

Auditory, Language, and Speech

|Approx. Age |Auditory Development |Language Development |Speech Development |

| |Startle response; attends to music and| | |

|0-28 |voice, soothed by parent’s voice; some| | |

|days |will synchronize body movements to | | |

| |speech patterns; enjoys time “enface” | | |

| |position; hears caregiver before being| | |

| |picked up | | |

| |Looks for sound source; associates |Startles to loud sounds; smiles when |Makes pleasure sounds (cooing, gooing); |

| |sound with movement; enjoys parent’s |spoken to; seems to recognize parent |cries differently for different needs. |

|1-3 months |voice; attends to noise makers; |voice and quiets if crying; increases |Smiles when sees known caregiver |

| |imitates vowel sounds |or decreases sucking behavior in | |

| | |response to sound. | |

| |Uses toys/objects to make sounds; |Recognizes some words; responds to |Babbling sounds more speech-like with many |

| |plays with noise makers; pays |verbal commands (bye-bye); learning to|different sounds, including p, b, and m. |

|4-7 months |attention to music; enjoys rhythm |recognize name; |Vocalizes excitement and displeasure; makes |

| |games; responds to changes in tone of| |gurgling sounds when left alone and when |

| |caregiver voice; notices toys that | |playing with caregiver. |

| |make sound; moves eyes in direction of| | |

| |sounds | | |

| |Attends to TV; localizes to |Recognizes words for common items like|Babbling has both long and short groups of |

| |sounds/voices; enjoys rhymes and |“cup,” “shoe,” “juice.” Begins to |sounds such as “tata upup bibibibi.” Uses |

|8-12 months |songs; enjoys hiding game; responds to|respond to requests. Understands NO. |speech or non-crying sounds to get and keep |

| |vocal games (e.g., So | |attention. Imitates different speech sounds.|

| |Big!!,Peek-a-boo) | |Has 1 or 2 words (no, dada, mama) although |

| | | |they may not be clear. |

| |Dances to music; sees parent answer |Points to pictures in a book when |Says more words every month. Uses some 1-2 |

| |telephone/doorbell; answers to name |named; points to a few body parts when|word questions (“Where kitty?”). Puts 2 |

|1-2 |call; listens to simple stories, |asked; follows simple commands and |words together (“More cookie”). Uses many |

|years |songs, and rhymes |understands simple questions (“Roll |different consonant sounds at the beginning |

| | |the ball” “Where’s your shoe?”) |of words. |

| |Listens on telephone; dances to music;|Understands differences in meaning |Has a word for almost everything. Uses 2-3 |

| |listens to story in a group; goes with|(“go/stop,” “up/down”). Follows two |word “sentences” to talk about and ask for |

|2-3 |parent to answer door; awakens to |requests (“Get the book and put it on |things. Speech is understood by familiar |

|years |smoke detector. |the table”). Attends to travel |listeners most of the time. Often asks for |

| | |activities and communication. |or directs attention to objects by naming |

| | | |them. |

Adapted from: Ear Infections and Language Development, pubs/edpubs.html and Developmental Index of Auditory and Listening (DIAL),

References

1. Bess, F. H. & Tharpe, A. M. (1986). An introduction to unilateral sensorineural hearing loss in children. Ear and Hearing, 7(1), 3-13.

2. Culbertson, J. L. & Gilbert, L. E. (1986). Children with unilateral sensorineural hearing loss: cognitive, academic, and social development. Ear and Hearing, 7(1), 38-42.

3. Bess, F. H. (1985). The minimally hearing-impaired child. Ear and Hearing, 6, 43-47.

4. Bess, F. H., Dodd-Murphy, J., & Parker, R. A. (1998). Children with minimal sensorineural hearing loss: Prevalence, educational performance, and functional status. Ear and Hearing, 19(5), 339-354.

5. Crandell, C. (1993). Speech recognition in noise by children with minimal degrees of sensorineural hearing loss. Ear and Hearing, 14, 210-214.

6. Davis, J. M., Elfenbein, J., Schum, D., & Bentler, R. A. (1986). Effects of mild and moderate hearing impairment on language, educational., and psychosocial behavior of children. Journal of Speech and Hearing Disorders, 51, 53-62.

7. Giolas, T. G. & Wark, D. J. (1967). Communication problems associated with unilateral hearing loss. Journal of Speech and Hearing Disorders, 41, 336-343.

8. Flexer, C. L. (1996). Amplification for children with minimal hearing loss. In F. H. Bess, J. S. Gravel, & A. M. Tharpe (Eds.). Amplification for Children with Auditory Deficits (pp. 321-337). Nashville, TN: Bill Wilkerson Center Press.

9. Widen, J., Folsom, R., Cone-Wesson, B., Carty, L., Dunnell, J., Koebsell, K., Levi A., Mancl, L., Ohlrich, B., Trouba, S., Gorga, M., Sininger, Y., Vohr, B., & Norton, S. (2000). Identification of neonatal hearing impairment: Hearing status at 8 to 12 months corrected age using a visual reinforcement audiometry protocol. Ear and Hearing, 21(5), 471-487.

10. Joint Committee on Infant Hearing Position Statement, Principles, and Guidelines (2000). American Journal of Audiology, 9, 9-29.

11. Blair, J. C. (1977). Effects of amplification, speech reading and classroom environments on reception of speech. Volta Review, 79, 443-449.

12. Boney, S., & Bess, J. H. (1984). Noise and reverberation effects on speech recognition in children with minimal hearing loss. Paper presented at the American Speech-Language-Hearing Association, November, San Francisco.

13. Goetzinger, C. (1962). Effect of small perceptive losses on language and on speech discrimination. Volta Review, 64, 408-414.

14. Ross, M., & Giolas, T. G. (1971). Effect of three classroom listening conditions on speech intelligibility. American Annals of the Deaf, 116, 580-584.

15. Davis, J. M. (1988). Management of the school age child: A psychosocial perspective. In F. H. Bess (Ed.). Hearing Impairment in Children. Parkton, MD: York Press.

16. Maliszewski, S. J. (1999). Reflections of family support: One parent’s perspective. In F. H. Bess (Ed.). Children with Hearing Impairment: Contemporary Trends, Nashville, TN: Vanderbilt Bill Wilkerson Center Press.

17. Blake, P. E. & Bess, F. H. (1992). The use of functional health assessments with hearing impaired adolescents. Poster presented at the annual convention of the Florida Language Speech and Hearing Association. Tarpon Springs, FL.

18. Pudlas, K. A. (1996). Self-esteem and self-concept: Special education as experienced by deaf, hard of hearing, and hearing students. British Columbia Journal of Special Education, 20(1), 23-39.

19. Stein, D. (1983). Psychosocial characteristics of school-age children with unilateral hearing loss. Journal of the Academy of Rehabilitative Audiology, 16, 12-22.

20. Quigley, S. P. & Thomure, F. E. (1968). Some Effects of Hearing Impairment upon School Performance. Springfield, IL, Illinois Office of Education.

21. Bess, F. H., & McConnell, F. E. (1981). Audiology, Education and the Hearing Impaired Child. St Louis: The C. V. Mosby Company.

22. Kodman, F. (1963). Educational status of hard of hearing children in the classroom. Journal of Speech and Hearing Research, 28, 297-299.

23. Sarf, L. S. (1981). An innovative use of free-field amplification in regular classrooms. In R. J. Roeser & M. P. Downs (Eds.). Auditory Disorders in School Children (pp. 263-272). New York: Thieme Medical Publishers, Inc.

24. Young, C. & McConnell, F. E. (1957). Retardation of vocabulary development in hard-of-hearing children. Exceptional children, 23, 368-370.

25. Webster, D. B., & Webster, M. (1977). Neonatal sound deprivation affects brain stem auditory nuclei. Archives of Otolarynogology, 103, 392-396.

26. Webster, D. B., & Webster, M. (1979). Effects of neonatal conductive hearing loss on brainstem auditory nuclei. Annals of Otology, Rhinology, Laryngology, 88, 684-688.

27. Trune, D. R. (1982). Influence of neonatal cochlear removal on the development of mouse cochlear nucleus, I. number, size, and density of its neurons. Journal of Comprehensive Neurlology, 209, 425-434.

28. Tess, R. C. (1967). The effects of early auditory restriction in the rat on adult duration discrimination. Journal of Auditory Research, 7, 195-207.

29. Tess, R. C. (1967). Effects of early auditory restriction in the rat on adult pattern discrimination. Journal of Comprehensive Physiological Psychology, 63, 389-393.

30. Keys, J. W. (1947). Binaural versus monaural hearing. Journal of the Acoustical Society of America, 19, 629-631.

31. Shaw, W. A., Newman, E. B., & Hirsh, I. J. (1947). The difference between monaural and binaural thresholds. Journal of Exceptional Psychology, 37, 229-242.

32. Lochner, J. P., & Burger, J. F. (1961). The binaural summation of speech signals. Acustica, 9, 313-317.

33. Bergman, M. (1957). Binaural hearing. Archives of Otolaryngology, 66, 572-578.

34. Konkle, D., & Schwartz, D. M. (1981). Binaural amplification: A paradox. In F. H. Bess, B. A. Freeman, & S. Sinclair (Eds.). Amplification in Education. Washington, DC: Alexanger Graham Bell Association for the Deaf.

35. Licklider, J. (1948). The influence of interaural phase relations upon the masking of speech by white noise. Journal of the Acoustical Society of America, 20, 213-232.

36. Jensen, J. H., Johansen, P. A. & Borre, S. (1989). Unilateral sensorineural hearing loss in children and auditory performance with respect to right/left ear differences. British Journal of Audiology, 23, 207-13.

37. Sung, R. J. & Sung, G. S. (1982). Low frequency amplification and speech intelligibility in noise. Hearing Instruments, 33(1), 20-24.

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.

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