Audiological Management of Children with Single-Sided Deafness

ABSTRACT

The difficulties school-aged children with unilateral hearing loss (UHL) experience have been documented. Children with single-sided deafness (SSD), however, face unique obstacles. These children are more likely to experience difficulties than their peers with lesser degrees of UHL and are not candidates for conventional hearing aids. Newer hearing technology options are emerging for individuals with SSD, but the efficacy of these new devices has not been extensively studied in young children. This article will review the current evidence concerning amplification options for children with SSD, discuss factors the audiologist should take into consideration when contemplating amplification, and review other recommendations the audiologist can make in the best interest of the child.

KEYWORDS

Unilateral hearing loss - single-sided deafness (SSD) - transcranial - bone-anchored hearing aid (Baha) - TransEar - contralateral routing of signal (CROS)

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APPENDIX A: INFANTS AND TODDLERS WITH UHL—A PARENT'S GUIDE[*]

You have been told that your child has a unilateral hearing loss. You probably have lots of questions. This brochure will help answer some of your questions. It will also give you ideas of how to help your child.

Having UHL means hearing is normal one ear and there is hearing loss in the other ear. Sometimes the hearing loss is mild and sometimes it is severe. About 1 child out of 1000 children is born with UHL. Nearly 3% of school-aged children have UHL.

What Causes UHL?

Many times we do not know the cause of hearing loss. Following are some possible causes of UHL in young children:

• Hearing loss that runs in the family

• An outer, middle or inner ear abnormality

• Specific syndromes

• Specific illnesses

Will My Child's Hearing Get Worse?

Although hearing sometimes gets worse, often it does not. The otolaryngologist (ear, nose and throat [ENT] doctor) can order tests that may be able to predict if your child's hearing could get worse. The audiologist will also closely monitor your child's hearing in both ears. If there are any changes, the audiologist will suggest ways to help your child.

Should My Child Be Evaluated by the ENT Now?

Yes, your child should have evaluations with otolaryngology, ophthalmology (vision testing), genetics and speech-language pathology. These evaluations are described below.

• Otolaryngology: Any child with hearing loss should be seen by an ENT doctor. The ENT will check your child's ears. He or she will want to make sure that things such as wax and middle ear fluid are not contributing to your child's hearing loss. The doctor may order some tests, such as a computed tomography scan and a urine test, to see if the cause of the hearing loss can be determined.

• Ophthalmology: An ophthalmologist is a doctor who checks your child's eyes and vision. Since your child has a hearing loss, we want to make sure that his or her vision is normal.

• Genetics: Any child with permanent hearing loss is referred to a geneticist. This doctor may be able to determine a possible cause of the hearing loss and tell you whether it is hereditary. He or she may ask other family members to have their hearing tested. Although it is unlikely, he or she will also check to make sure that your child does not have other medical problems.

• Speech-language pathology: Speech pathologists will evaluate and monitor your child's speech and language development. They may suggest activities to do at home, or may recommend speech therapy.

• Early intervention: Your child should be evaluated by your county's early intervention program. This program is available to your child in your community. They may test your child and recommend that he or she receive services to stimulate speech and language. They may simply want to monitor your child and track his or her development.

What Problems Do Children with UHL Have?

• Your child may have trouble figuring out from which direction sound is coming. This is known as localization. Localization is important when talking and listening in groups of people. It helps direct us to who is talking at any moment. It is also important for safety. If your child is near the street or riding a bike, he or she may have trouble localizing a horn.

• Your child may have difficulty understanding speech in noisy situations. Two normal-hearing ears help us to filter out noise to better hear speech. A child with UHL cannot do this.

• Your child may have a more difficult time hearing you from another room or from outside. When two normal ears hear together, sound seems louder. When a child has a UHL, he or she can often hear speech, but may not always understand what is being said.

• Remember, your child is in the process of developing language. If an adult does not hear a complete sentence, he or she can often figure it out based on his or her experience. Children cannot guess or fill in those gaps based on experience because they do not have the language skills yet.

Will My Child Learn to Talk Like Other Children?

Your child's UHL will not keep him or her from learning to talk. However, some children with UHL have delays in their speech and language development. We do not yet know why some children have difficulties and others do not.

How Can I Help My Child?

There are lots of ways you can help your child now. Following are some ideas:

• When you are holding or feeding your baby, try to make sure his or her good ear is facing you.

• Always be aware of where your child's “normal” hearing ear is facing. It should always be facing you or those talking to your child. Think about this when your child is at dinner, in the car, in the stroller.

• Try to avoid letting your child's “normal”-hearing ear face noise (i.e., dishwasher, room air conditioner, radio).

• Try to make eye contact and use facial expressions.

• Get your child's attention before talking to him or her.

• Talk about what you are doing within your daily routine (“I'm making your sandwich, here is your milk”).

• Start conversations; take turns talking.

• Encourage listening (“Did you hear the telephone ring?”)

• Help your child localize sound (“Look over there at that dog barking.”)

• Use repetition.

• Look for cues that your child understands what you are saying.

• Expand your child's vocabulary. If he or she says “Good,” expand by saying “Great, wonderful, terrific.”

• Play listening games. (“What do you hear? I hear a car and a bird”)

• Raise your voice slightly and face your child when you are at a greater distance (walking your baby in the stroller).

How Will I Know If My Child's Speech Is Developing Normally?

Speech development varies from child to child. If you have concerns about your child's speech, you should tell his or her physician. As mentioned earlier, your child should also be evaluated by a speech-language pathologist. Following are milestones for speech and language development published by The American Speech-Language and Hearing Association. Milestones give us examples of normal or typical development.

BIRTH TO 3 MONTHS

• Makes happy sounds (cooing)

• Cries differently for different needs

• Smiles at you when he or she sees you

4 TO 6 MONTHS

• Babbles using different sounds such as p, b, and m

• Expresses excitement and unhappiness

• Makes gurgling sounds

7 MONTHS TO 1 YEAR

• Babbles with long and short groups of sounds (tata, bibibi)

• Uses speech or noncrying sounds to get attention

• Initiates different speech sounds

• Has one or two words (bye-bye, dada), although may not be clear

1 TO 2 YEARS

• Says more words every month

• Uses some two- to three-word questions (Where's Daddy?)

• Puts two words together (more juice)

• Uses many different consonant (p, b, m, w) sounds in the beginning of words

2 TO 3 YEARS

• Has a word for almost everything

• Uses two- to three-word sentences to talk about most things

• Speech is understood by familiar listeners most of the time

• Often asks for objects by naming them

3 TO 4 YEARS

• Talks about activities at school or at friends' homes

• People outside family understand child's speech

• Uses sentences that have four or more words

• Usually talks easily without repeating words or syllables

4 TO 5 YEARS

• Uses sentences that give lots of details

• Communicates easily with other children and adults

• Tells stories that stick to a topic

• Uses the same grammar as the rest of the family

When My Child Is Older, Will He or She Have Trouble in School because of His or Her Hearing Loss?

Studies have shown that children with UHL are at risk for difficulties in school. Some children will have difficulties and some will not. Although school may be years away for your child, being aware may help you and/or your child's daycare/nursery school teacher to catch any problems early. Help is always available for your child.

How Will I Know If My Child Is Having Difficulty?

• Your child might get easily frustrated.

• Your child might seem overly tired at the end of the day.

• Your child might seem like he or she is not paying attention.

• Your child may respond incorrectly to a question or request.

• Your child might “act out” due to frustration.

How Can I Help My Child in Daycare/Preschool?

• Share with your child's teacher what you have learned about UHL.

• Give the teacher written information about UHL.

• When choosing a day care center or preschool for your child, look closely at their “setup.” Try to avoid situations where many classes are within one large area separated by partitions. This will be noisy.

• Ask if the center has scheduled reviews of your child's progress.

• Ask preschools if their TV or music areas are separate from teaching areas.

• If your child is having difficulty, request that a hearing support teacher from your county attend any meetings about your child.

• During instructional time (circle time) make sure that your child is seated near the teacher with his or her better ear facing the teacher.

• If your child wears a hearing aid or uses an FM system, make sure the teacher knows how they work. (These devices are described later.)

• Keep in close communication with your child's teacher to monitor his or her progress. If you or the teacher has concerns, request further testing from your county's early intervention program.

• Let your child's teacher know that he or she may have trouble localizing sound (i.e., being called on the playground).

How Can I Help My Child at Home?

• Make your home “listening friendly.” There are things you can do to reduce noise. Use carpeting and cloth curtains. Use corkboards instead of magnetic boards. Replace buzzing fluorescent lights. Operate noisy appliances (dishwasher, washing machine) when your child is not home or is sleeping.

• Teach your child's siblings things that you have learned about helping him or her.

• Do not have the TV or radio on while eating dinner or at other times when you are talking with your child.

• Be aware of where you place your child at the dinner table. Make sure his or her good ear is facing those talking to him or her.

• Create a quiet listening environment while your child is watching TV.

• Do not give your child instructions from another room. He or she will likely hear your voice, but not understand what you are saying.

• Remember the tips listed earlier on helping speech and language development.

• If your child wears a hearing aid, make sure it is functioning properly at all times. A hearing aid that does not work is much worse than no hearing aid at all.

Should My Child Wear a Hearing Aid?

A hearing aid might be an option for your child. Some children with UHL benefit from using a hearing aid. We will need to consider your child's age and the amount of hearing loss he or she has. Your audiologist will help you to decide what will best meet your child's needs. These amplification systems are described below.

• Conventional hearing aid: A hearing aid may be appropriate for some children with UHL. It is recommended if there is some usable hearing in the impaired ear. The goal is for your child to be able to hear and understand speech in the aided ear. This may allow your child to have more “balanced” hearing. It is not recommended if your child has a severe or profound UHL. Most young children are fit with a BTE type of hearing aid. Any child under the age of 18 years requires medical clearance by a physician prior to the fitting of a hearing aid.

• FM system: At some point, your audiologist may recommend an FM system for your child. An FM system provides the most benefit to children with UHL. With this device, the speaker (teacher or parent) wears a microphone and the child has a receiver. This receiver may be a headset, an attachment to the hearing aid, an ear bud-style earphone, a desktop speaker, or room speakers. An FM system allows the speaker's voice to be heard at a volume that is louder than the existing background noise. An FM system can be used in different situations, such as the stroller, nursery school, church, and the car. It is important to note that in group situations, young children should be able to hear all who are contributing to a conversation. In other words, a young child learns not only from a parent or teacher, but also from other children.

• Baha: You may read or hear about this device. This hearing device is placed surgically in the bone behind the ear. It was initially intended for individuals with primarily conductive hearing loss (outer or middle ear). It has recently been marketed for individuals with “single-sided deafness.” The sound travels from the impaired ear through bone conduction to the normal-hearing ear. The Baha is approved for children over 5 years of age. There is a nonsurgical option with the Baha using a headband. Little is known about the effectiveness of the Baha in young children with UHL at this time.

• CROS aid: This hearing aid is for individuals with a severe or profound UHL. It picks up sound on the impaired side and delivers it to the good ear. This system may be useful in quiet listening situations. It is not recommended in situations where noise could enter on the impaired side and be sent to the “normal” side. This could actually make it more difficult for your child to understand what is being said. A CROS aid is not a good choice unless your child can determine when it helps and when it does not. For this reason, it is not recommended for young children.

• Cochlear implant: A cochlear implant is not an option for children with UHL. This device, which is placed surgically in the inner ear, is only for children with severe or profound bilateral hearing loss.

How Often Should My Child's Hearing Be Tested?

An audiologist will want to monitor your child's hearing closely. Children with UHL should be evaluated every 3 to 6 months. The number of appointments depends on your child's age, degree of hearing loss, and tests already completed. Close follow-up will allow the audiologist to determine if your child's hearing is changing. If your child wears a hearing aid, the audiologist will want to monitor use and progress closely.

Important Considerations

• • If your child seems to be having more difficulty hearing, make an appointment with your audiologist as soon as possible. Signs of difficulty may include:

•  ˆ ignoring sounds to which he or she used to respond

•  ˆ saying “huh?” more often

•  ˆ needing the TV turned up

•  ˆ acting more frustrated

• • Remember that your child will have difficulty localizing sounds. This is important for safety. He or she may not be able to tell from where someone is calling him or her or from which direction a horn is honking.

• • If your child starts to show signs of an ear infection, take him or her to your doctor as soon as possible. Such signs may include: tugging on the ear/s, increased irritability, difficulty sleeping, and/or fever. If your child has an ear infection or fluid in the ear, it could cause temporary hearing loss. Middle ear fluid may cause hearing loss in your child's “good” ear and/or increased hearing loss in their “impaired” ear. Although middle ear problems are common in young children, it can be more harmful to your child than to a child with normal hearing in both ears.

• • Make sure your child's ears do not get plugged by earwax. Although earwax is normal, if it blocks your child's ear canal, it can cause temporary hearing loss. If you notice that your child has excessive earwax, take him or her to the doctor.

• • Has your doctor told you that your child has enlarged vestibular aqueduct syndrome? This is a particular formation of the inner ear that can be seen on a computed tomography scan. If so, you will want to try to avoid situations where your child could get bumped in the head. Sometimes a blow to the head can cause the hearing to worsen. If your child has enlarged vestibular aqueduct syndrome and gets bumped in the head, you should schedule an appointment with your audiologist.

• • Start to think about “hearing conservation.” This refers to preserving the hearing that you have. Have your child use earplugs when he or she going to be at loud events (fireworks, concerts). Extremely loud sounds can cause additional hearing loss. If your child is using headphones, make sure to keep the volume at a reasonable volume. As your child grows, teach him or her about hearing conservation. It should become a habit. As your child gets older, a fun and informative website for you and your children to visit is http://www.dangerousdecibels.org or http://www.noisyplanet.nidcd.nih.gov. Share these sites with your child's teachers.

• • You now know the risks and the difficulties associated with UHL. Help your child now. Teach your child how to help him- or herself. What you do now will make a difference!

Additional Resources

• American Academy of Audiology: http://www.audiology.org

• American Academy of Otolaryngology-Head and Neck Surgery: "http://www.entnet.org

• American Academy of Pediatrics: http://www.aap.org/healthtopics/visionhearing.cfm

• American Speech-Language-Hearing Association: http://www.asha.org

• Boys Town National Research Center: http://www.babyhearing.org

• The Center for Disease Control, Early Hearing Detection and Intervention Program http://www.cdc.gov/ncbddd/ehdi

• The Center for Hearing Loss in Children, Boys Town National Research Hospital: http://www.boystown.org/chlc/

• Colorado Home Inventory Program: http://www.csdb.org

• Educational Audiology Association: http://www.edaud.org/

• National Center for Hearing Assessment and Management: http://www.infanthearing.org

• Hands and Voices: http://www.handsandvoices.org

APPENDIX B: CHILDREN WITH UHL—A TEACHER'S GUIDE[*]

UHL refers to hearing loss in one ear and normal hearing in the other ear. Approximately 1 in 1000 children are born with UHL and approximately 3% of school-aged children have UHL.

Research has shown children with UHL to be at risk for:

• Speech and language delays

• Cognitive delays

• Academic difficulties, specifically in the areas of reading and math

• Grade failure (10 times more likely to fail a grade in school than their normal-hearing peers)

• Need for resource room assistance (almost 50% of children with UHL need resource room assistance)

• Social/emotional difficulties

Always remember, with UHL speech is often audible, but not always understandable.

Difficulties Encountered by a Child with UHL

Children with UHL can often hear speech, but cannot understand what is being said. This is due to the following:

• Trouble localizing sounds

• Difficulty understanding speech in noisy situations

• Difficulty hearing sounds from a distance

Signs/Symptoms Experienced by Children with UHL

• Fatigue—due to increased effort and strained listening.

• Social problems—due to missing or misunderstanding parts of conversations. They may also miss subtle social cues.

• Apparent inattentiveness—If your student does not understand what is being said, it is difficult for him or her to continue to attend.

• Frustration.

• Issues with self esteem—may lack confidence.

• Behavioral problems—If a child experiences the difficulties listed, he or she may uncharacteristically “act out.”

Audiologist's Recommendations/Options for a Child with UHL

• Hearing aid: A hearing aid may be appropriate for some children with UHL if there is some usable hearing in the impaired ear. Most children are fit with a BTE type of hearing aid. An ITE hearing aid may be an option for older children. You can help your student by checking to make sure the hearing aid is properly functioning.

• CROS aid: This hearing aid is for individuals with a severe or profound UHL. It picks up sound on the impaired side and delivers it to the good ear. This system may be useful in quiet listening situations. It is not recommended in situations where noise could enter on the impaired side and be sent to the “normal” side. This could actually make it more difficult for your child to understand what is being said. A CROS aid is not a good choice unless your child can determine when it helps and when it does not. For this reason, it is not recommended for young children.

• Baha: You may read or hear about this device. This hearing device is placed surgically in the bone behind the ear. It was initially intended for individuals with primarily conductive hearing loss (outer or middle ear). It has recently been marketed for individuals with SSD. The sound travels from the impaired ear through bone conduction to the normal-hearing ear. The Baha is approved for children over 5 years of age. There is a nonsurgical option with the Baha using a headband. Little is known about the effectiveness of the Baha in young children with UHL at this time.

• FM system: An FM system is often recommended for any child with hearing loss. With this device, the teacher wears a small microphone and the child has a receiver. This receiver may be in the form of a headset, a component of their hearing aid, an ear bud, a desktop speaker, or classroom speakers. An FM system allows the speaker's voice to be heard at a loudness level greater than the existing background noise. This is very beneficial to a hearing impaired child. In the case of classroom speakers, all children are found to benefit. If your student changes classes, he or she will likely need to take the transmitter with him or her to the next teacher. Help your student with this routine while still encouraging responsibility.

• Preferential seating: Teachers should always be cognizant of the placement of a child with UHL in all listening situations. The child's normal hearing ear should always be facing the speaker (i.e., teacher) and never be facing a noise source. In addition, a child with UHL should always be seated close to a speaker (within 6 feet).

• Vision testing: An audiologist refers children with hearing loss to various medical professionals. Because the child already has one sense that is compromised, the audiologist wants to ensure that vision is normal. If you are concerned about your student's vision, refer him or her for vision testing.

Practical Strategies to Help Your Child with UHL in the Classroom

• Teach your class speaking and listening etiquette (one person talks at a time, everyone listens).

• Always make sure that your student's “normal”-hearing ear is facing you and is in close proximity to you (within 6 feet).

• Always make sure that your student's “normal”-hearing ear is not facing a noise source such as the hallway, HVAC vents, and computers.

• Be aware of your student's placement relative to the other students. Other student's responses and contributions add to the educational experience.

• Try to get your students' attention by calling their names before giving oral instructions.

• Try to give written instructions in conjunction with oral instructions.

• Ensure optimal lighting.

• Try not to speak with your back to the student.

• Have a “preunderstood” nonverbal cue to use with your student (e.g., a tap on the shoulder to regain attention).

• Give your student a “note buddy.”

• Provide visual aids; however, do not place your student next to a slide or LCD projector.

• Allow your older student to record lectures.

• Teach your student to advocate for him- or herself (e.g., Can you repeat that?).

• Make sure one person talks at a time.

• During group time, separate groups and place “noisy” groups off on their own.

• Train volunteers to be good communication models. This is particularly important with student teachers and parent volunteers. Parents should not be talking to children or other parents while the teacher is giving instructions.

• Take interest in your student's hearing aid or FM system. If your student is in preschool or a lower grade, he or she might want to share his or her hearing aid for “show and tell.” There are children's books available that explain hearing loss and hearing aids. If your student is older, he or she might want to coteach the class a lesson about the anatomy of the ear or how hearing aids work. Of course, ask your student and his or her parent's permission first.

Ways to Help Your Classroom Be More “Listening Friendly”

• Avoid “open class” teaching with many classes within a large space.

• Carpeting—if there is not wall-to-wall, place some area rugs.

• Request acoustically treated, hanging ceiling tiles.

• Avoid situations where the class is split and half of the students are listening to teacher instruction and the other half is watching TV or listening to tape recorder.

• Place window treatments (thick material).

• Replace buzzing lights.

• Hang long pieces of felt on the wall in which children can pin artwork.

• Use “creative” artwork, hang egg crates and strips of carpet from the ceiling.

• Use corkboards whenever possible.

• Place flat surfaces (movable boards) at an angle.

• Place tennis balls or rubber tips on chair feet.

• Have soft chairs (small beanbag chairs) in leisure/reading areas.

• Do not have noisy equipment (i.e., computers, projectors) on if you are not using them.

• Try to keep doors and windows closed.

• Give your class an age-appropriate lesson about sound. Show them how it is difficult to hear when many children are talking at once.

Other Recommendations

• Because your student is at risk for educational delays, monitor progress closely. Keep in close contact with the parents, and if delays are suspected, refer for appropriate educational testing.

• Report any suspected changes in hearing to the student's parent. UHL can be progressive, and it can also develop into bilateral hearing loss.

• The Individuals with Disabilities Education Improvement Act (IDEA, 2004) mandates “proper functioning of amplification devices used in school.” Establish a daily routine with your student to ensure that his or her hearing aid and/or FM system is functioning properly. A hearing support professional will be able to help you establish this routine.

• Use published outcome measures as a tool to monitor your student's function or progress. Sometimes results of these tools help to validate the need for services or assistive devices. One such tool to use in school is the Screening Instrument for Targeting Educational Risk. This tool is available in a preschool format as well.

• Ask to have a hearing support teacher involved in any meetings involving the child.

• Teach your students about hearing conservation (protecting their ears from loud sounds). Although this is particularly important for individuals with hearing loss, the information also pertains to the general population. A fun and informative website for you and your students to visit is http://www.dangerousdecibels.org. Go to “Virtual Exhibit” or to “Teacher Guide” for the most useful information.

• Last, this child has the same potential as your “normal-hearing” students. Help the child now to reach his or her full potential!

Additional Resources

• American Academy of Audiology: http://www.audiology.org

• American Speech-Language-Hearing Association: http://www.asha.org

• Boys Town National Research Center: http://www.babyhearing.org

• The Center for Disease Control, Early Hearing Detection and Intervention Program http://www.cdc.gov/ncbddd/ehdi

• The Center for Hearing Loss in Children, Boys Town National Research Hospital: http://www.boystown.org/chlc

• Educational Audiology Association: http://www.edaud.org

• Hands and Voices: http://www.handsandvoices.org

• National Center for Hearing Assessment and Management: http://www.infanthearing.org

1 Developed by Sarah McKay, Au.D., as part of doctoral project, Central Michigan University (2006), Committee Chair: Anne Marie Tharpe, Ph.D. Subsequently published by The American Speech-Language-Hearing Association (ASHA) and available at http://www.asha.org/public/hearing/disorders/UHLchildren.htm.

2 Developed by Sarah McKay, Au.D., as part of doctoral project, Central Michigan University (2006); Committee Chair: Anne Marie Tharpe, Ph.D.