Hearing Impairment Follow-up
- Author: Rahul K Shah; Chief Editor: Glenn C Isaacson, MD, FACS, FAAP more...
Further Outpatient Care
Follow-up of the interventions is as important in hearing impairment as in any other disability or medical condition. Physicians too frequently relegate the care of children who are deaf to audiologists and educators. Children who are deaf need ongoing referrals to an audiologist to monitor the progression of their hearing loss and to refit hearing aids to match changing losses and/or growth of their ears.
Pediatricians should monitor the child's linguistic and social development. They should ask about language and school performance. The child's placement in school may not be optimal for his or her abilities. For instance, a child who is not successfully learning lip-reading cannot learn math or science. Pediatricians should ask how the child is doing in school and in the family, how family members interact with the child, and how the parents discipline or instruct the child.
Children who cannot communicate with those around them may be frustrated and, therefore, act out or display withdrawal behaviors. These behaviors may be misinterpreted as being a behavioral or psychological problem rather than being reactions to the child's environment or situation.
Children who are deaf or hard of hearing are at particular risk for abuse. Physical abuse may be inflicted by parents who are frustrated because their child is not acting as they expect. Also, children who perpetrators perceive as being unable to report misconduct are at high risk for sexual abuse. Furthermore, most children with hearing impairment have no physical disabilities and may be attractive to a potential perpetrator. Deaf children may be socially isolated because of the communication barrier, they may be susceptible to individuals who give them special attention. Watch for physical signs of abuse or for behavioral manifestations of child abuse.
Patient Education
Assisting and educating parents
Remember, although most deaf children are otherwise healthy, a parent's response to having a child with hearing impairment is generally the same as that of a parent whose child has a physical disability or chronic medical condition. Some deaf children have both hearing loss and other disabilities or medical conditions. Help parents to identify available resources (such as those listed at the end of this section), or refer them to a social worker or counselor who can provide specialized assistance.
In some states, social workers assigned to families with deaf or hard-of-hearing children are required to have a background in disabilities or hearing loss. Many states assign the next social worker available, in which case the worker has no more ability to determine the best form of communication, the best school environment, or the best resources than the primary care provider.
The information available on the Internet may be selected strictly on geographic location, by the "number of hits" making the site appear at the top of the list, or by alphabet or the manner in which the resource is structured. If possible, the patient’s family should be directed towards people with expertise in the field and towards sources without obvious bias.
Parents must understand that deafness is not an all-or-nothing categorization and that hard-of-hearing is not defined by the hearing loss being in the middle ranges (eg, 30-60 dB). Children with CHL are likely to develop good use of speech with appropriate amplification. Children with SNHL are more variable. In SNHL, the nature of the sound may be distorted, which means that amplification of the deficit may not improve the child's language comprehension in any meaningful way. A helpful analogy is that of a radio signal that is mostly static; it remains unintelligible no matter how loud it is. Therefore, children with moderate SNHL (41-55 dB) may have minimal benefit to improving language comprehension, although they may have good benefit from improved ability to hear environmental cues.
Communication is the most important loss in deafness. Communication is necessary for socialization and integration into the family and into society. All interventions must have the goal of optimizing the child's ability to successfully communicate and interact socially.
Lip-reading (oralism) and sign language
Considerable debate continues regarding the choice of lip-reading (oralism) versus sign language. Lip-reading may be taught alone or supplemented with cued speech. In the United States, sign language can be in the form of American Sign Language (ASL) or Signed English (SE) with Signing Exact English/Seeing Essential English (SEE, which is sometimes distinguished as Signing Exact English [SEE 1] and Seeing Essential English [SEE 2]). PSE (Pidgin Signed English) is not used educationally, but functionally it combines the use of signs using an English-based grammar, but like any Pidgin language uses neither proper English grammar nor ASL grammar.
Parents must be able and willing to accept and then participate in the language that they chose for their child. They should be vigilant and remember that school failures and behavior problems are not inherent to deafness but frequently result from a frustrated child who has no language, no ability to communicate, and no ability to connect with others.
Advantages and disadvantages of lip-reading
The greatest advantage of lip-reading is that parents and society are required to make only minimal adjustments for the deaf individual. Therefore, deaf children must learn to speak for themselves and understand the speech of others.
The disadvantages are numerous. First, formal training can begin only at school age. Therefore, language acquisition is delayed beyond the optimal neurobiologic window for language acquisition, between approximately age 3-5 years (although some evidence indicates it may be as young as 9-18 mo).
Second, almost half of the consonants in English appear similar when spoken (eg, d-t, f-v, g-k, b-p-m). That is, they look identical on the lips but are distinguishable to hearing people when spoken. Other sounds appear the same such as /ch/, /j/, and /sh/, making chew, Jew, and shoe indistinguishable from each other (this includes the "soft g" as in "George" and all variations of /sh/ as in fi sh, Charlotte, and na tion. Many vowel sounds appear similar, especially compared with their written equivalent. For example, the sound /oo/ may be written as “t o,” “ too,” “th rew,” “thr ough,” “d ue,” or “sh oe.”
Lip-reading is hard to master and tiring to perform. To appreciate the difficulty, imagine reading this page with no spaces between the words and with only periods as punctuation. Then, imagine the text passing in front of you as if on an electronic billboard, and that every /p/ might be a /b/ or /m/, every /d/ could be a /t/, every /ch/ could be /j/ or /sh/, and so on.
Fourth, lighting, distance, speech impediments, accents, and foreign objects or motion can make lip-reading more difficult (eg, faces silhouetted by light, food, pencils, fingers, mustaches, turning the head).
As a result, typical lip-readers understand only one third of a one-to-one conversation. The best lip-readers understand about two thirds. In general, children with more hearing and better speech discrimination than others are most successful with lip-reading.
Finally, the lip-reader cannot localize a speaker. During a one-to-one conversation between the lip-reader and one other person, there is only the one set of lips to watch. Even a third person makes the conversation difficult because when the speaker’s lips stop moving, it could be a pause, or the other speaker has started speaking. The lip-reader must guess if he or she should continue to look at the speaker’s lips, risking missing the opening words of the second party (those which best set up context, for example agreement or disagreement with the first speaker). To look at the other person’s lips risks missing the continued comments of the speaker.
In a group setting, the lip-reader may miss several entire comments by the time his or her eyes identify the speaker. Having more hearing is helpful in group situations. Although hearing aids help to localize sound, they also amplify each voice equally, which may decrease the ability to accurately use the sounds that are heard.
Deaf and hard-of-hearing people who use either lip-reading or cued speech must learn to speak for themselves. They have varying success, but many can make themselves understood in most situations. The challenge is simple. They must learn to create sounds they cannot hear. A simple analogy would be teaching a blind person to paint in watercolors. It would be possible for blind people to make the design by embossing the paper with an image they can feel, and then fill in the areas (like painting by numbers) with colors they cannot truly imagine. Deaf people use surrogates for sound to produce noises they cannot imagine. Traditional “sound surrogates” would be making a feather blow with plosive sounds, or feeling the throat vibrate to understand when the larynx is engaged.
More modern technologies include matching speech patterns on oscilloscopes or similar equipment, or “green light” when the deaf person’s spoken word matches the computer’s. Many hearing people cannot even imitate another regional or foreign accent, and few people speaking a new language ever speak without a residual accent from their native language, despite the fact that they can hear the new language and compare their voice with it. Just as some hearing people go to a specialist to learn to speak with an accent or to ablate their native accent, some deaf people who already have learned to speak will return to speech therapists every few years to maintain their speech quality.
Puberty and growth, changes in their vocal cords due to maturation and use (or misuse), and changes in their mouth and shape of their oropharynx occur throughout their lives, altering their ability to produce speech sounds. Without being able to hear themselves and self-correct, they seek professional help to do so.
Cued speech
Cued speech aids lip-reading because hand shapes are placed near the mouth. These shapes help in discriminating sounds that are difficult to distinguish by observing the lips alone.
Parents must learn how to cue. The technique is similar to shorthand in that as sounds, not letters, are cued. For example, the /sh/ sound in fi sh, ch ard, and na tio n are all cued and in the same way "L" or "gun" hand shape placed by the chin. Whereas the /p/, /b/, and /m/, which are identical on the lips, are cued by the chin with one finger, 4 fingers, or all 5 fingers with the fingers flat and close together, respectively.
Because cued speech is the language of neither the parents nor society, cued-speech interpreters may be required in situations such as interviews or public events. These interpreters are harder to locate than sign-language or oral interpreters.
Similar to instruction in lip-reading, instruction in cued speech cannot begin at an early age; therefore, language acquisition is delayed.
Systems of manual and visual sign language
In the United States, manual and visual signing systems include SEE and ASL.
SEE is visually encoded English, using or adapting the signs of ASL and imposing exact order of the spoken word on the signs. SEE invents suffixes (dog vs dog s), conjugations (see vs sees, -ing, -ed), and signs (the) that are not necessary in the grammar of ASL. These make the signs and sign order identical to English. SEE is long and tiresome. However, children who use SEE grow up signing what they learn to read and write, just as hearing children speak and hear what they later read and write. Because it takes too long, most people end up omitting or changing some of the signs, and they use PSE. Therefore, children do not benefit from the English-grammar aspect of SEE as much as they might; they learn neither proper English grammar nor proper ASL grammar.
ASL has a unique grammar. It requires fewer signs than SEE does to complete most thoughts because it incorporates space and time into the motion of signs in a way that spoken language cannot. It is efficient and beautiful to watch. However, the child must grow up being bilingual. The grammar used for ASL must be translated into English in order to write.
An advantage of ASL or SEE is that instruction may begin immediately when hearing loss is diagnosed. In fact, children of parents who are deaf learn to sign as their first language, and they begin to sign babble as early as 6-9 months, when hearing children begin to babble normally. In addition, signs are clearly visible at distances, and signing is the preferred language of the Deaf community. Research suggests that a strong linguistic background is as important to reading and language development as the actual language itself. Therefore, learning ASL may aid the development of English language skills rather than confuse them. Remember, it was not too long ago that educators told immigrant parents not to confuse their children with their foreign languages and to focus on English. Now it is accepted that children can easily learn several languages simultaneously, and it may enhance their ability to acquire languages later in life.
The biggest disadvantage is that sign language is not the language of the hearing world; therefore, interpreters are necessary. It is usually not the language of the family, and many families are intimidated by having to learn a new language to communicate with their child. In fact, 20% of children who are deaf who sign have no family members who sign, and 40% have only 1 family member who signs. Parents must be reminded that as long as they are 1 sign ahead of their child in early childhood, they know all they need to maintain communication and linguistic development. With only a few signs, love and discipline can be clearly expressed.
Total communication with sign language and voice
Total communication allows children with residual hearing to benefit from supplemental auditory information. It may also help students in lip-reading because signs with meaning can be associated with movements of the mouth. (For example, mouth the words "I'm going to bed" to your spouse at bedtime; he or she probably understands because the phrase is short and the context is clear; if you said it at a baseball game, your spouse initially would assume he or she had misunderstood.)
The main disadvantage is that speaking English while signing ASL at the same time is almost impossible. As a result, neither of the grammars is effectively or consistently applied.
School placement and schools for children with hearing impairment
Educational placements largely depend on the choice of language.
Children who are learning to lip-read attend an oral school where lip-reading is taught. A portion of the day is spent in lip-reading instruction, and other subjects cannot truly be learned until adequate language is established. As children age, they can be placed in a regular hearing classroom ("mainstreamed") if their lip-reading and vocal skills are sufficient. Their experience there depends on the teacher's ability to accommodate the needs of the student (eg, by not spending much time facing the blackboard and away from the student). Oral interpreters may also be provided to the student in a mainstream classroom. Many children who are deaf and who have succeeded to this point do well in school with their peers.
The experience for students using Cued Speech is not dissimilar. Once their language skills are established, they may stay in a program that uses Cued Speech, or they may enter a mainstream classroom with a Cued Speech interpreter.
Many children who are entering a school where sign language is used have already learned some signs at home or in early intervention. The classroom may consist of deaf children of deaf adults whose first language is ASL and children with minimal sign language skills. Because sign language is visual, young children who are immersed in a signing setting rapidly acquire the signs for objects, people, and, ultimately, grammar. Shortly thereafter, formal educational programming can begin. The process is analogous to most kindergarten classes, which tend to be more social than academic, and language acquisition occurs in a similar fashion in many bilingual school programs for foreign languages. The use of ASL or SEE in schools is debated by educators, but it is of relatively little importance early in the child’s education, especially when the goal is to foster the development of language, communication, and social skills.
Another debate is the location of deaf/signing programs, residential or mainstream programs. Placing children who are deaf or hard of hearing with hearing children in the least restrictive environment (mainstreaming) may not be as successful for deaf children as it is for children with other disabilities. Many so-called mainstream classrooms are isolated from those for hearing children, and the children who are deaf are instead grouped with children who have learning disabilities or mental retardation. Even in an integrated classroom, instruction happens through the interpreter.
When children are engaging in nonacademic subjects or when no interpreter is present, communication between deaf students and their classmates is limited. Children who have acquired only minimal sign skills will not understand the interpreter, and they have minimal opportunity to practice their signing with their classmates who do not sign. Children who have more advanced sign language may do much better in a hearing classroom from the academic perspective, but their ability to socialize with other students or participate in extracurricular activities is limited.
In some cities, there are deaf programs at hearing schools. Some of these programs put deaf children of varying ages together with a signing teacher. The educational model is more like independent study, with each child working on their assignments. However, the language of instruction is sign, and they interact with signing classmates. Other programs are able to assemble sufficient numbers of deaf students to have entire classes of deaf students with a signing teacher, but on the campus of a hearing school. Depending on the programs resources, students usually are able to participate in extracurricular activities.
Residential deaf schools are on the decline because of the recent desire to keep deaf children at home with their families. Relatively few independent day schools for the deaf exist, no more than 1 or 2 in each state. At a deaf school, where everyone signs, students can participate in many extracurricular and academic activities, such as debate team, football, and cheerleading. Because most deaf children live in a hearing household that is linguistically isolated to them, a school environment that is completely linguistically accessible to the child promotes self-esteem and social skills. The children are able to use sign language all day and all night. They engage and interact with peers and deaf children of different ages. They can develop leadership skills in a way that they may not be able to living at home. Most deaf adults who attended such residential schools look back on that experience as being the best time of their lives.
Choice of language and school placement
The debates rage on, and all parties can be vehement in their views. No one opinion is right, and little literature of adequate quality strongly supports any particular standpoint. Few well-performed studies exist, and the results of most studies cannot be generalized to apply to the majority of children who are deaf and hard of hearing. Therefore, the best approach is to make decisions based on the individual, to meet the needs of a particular child, including the parents’ beliefs and resources, and programs available to them. The goal of pediatricians should be continual monitoring of the child's progress. If the child is not succeeding in one environment or with one choice, suggest a trial in a different one.
Devices to aid children with hearing impairment
Young children need only hearing aids. As they grow, the family should be encouraged to obtain devices such as strobe lights connected to doorbells, timers, alarm clocks, and fire alarms. Telecommunication Devices for the Deaf (TDDs) and teletypewriters (TTYs) are machines than enable deaf people to use the phone. Computers with modems or video/webcam, cell phones with text messaging or instant messaging, and other hand-held devices all permit children to communicate using modern technologies.
These and other aids help children with hearing impairment to develop a sense of independence and accomplishment, just as hearing children do when they complete tasks such as waking up for school using their own alarm clock or baking a cake for the first time.
Schools should also use FM amplification systems to transmit the teacher's voice to a small headphone speaker the child wear just behind the hearing aid. This system amplifies the teacher's voice over extraneous noise.
All new televisions are equipped with closed captioning, which decodes the captioning of dialogue and action provided with most television shows, videotapes, and DVDs. This not only makes television accessible but also promotes reading skills in deaf and hearing children alike.
Resources
Alexander Graham Bell Association for the Deaf and Hard of Hearing
3417 Volta Place, NW
Washington, DC 20007
Voice: (202) 337-5220
TTY: (202) 337-5221
Fax: 202-337-8314
E-mail: info@agbell.org
11730 Plaza America Drive, Suite 300,
McLean, VA 22102
Voice: (800) AAA-2336, (703) 790-8466
Fax: (703) 790-8631
E-mail: info@audiology.org
American Deafness and Rehabilitation Association (ADARA)
ADARA National Office
PO Box 480
Myersville, MD 21773
E-mail: ADARAorgn@aol.com
American Hearing Research Foundation
8 South Michigan Avenue, Suite 814
Chicago, IL 60603-4539
Voice: (312) 726-9670
Fax: (312) 726-9695
E-mail: ahrf@american-hearing.org
Laurent Clerc National Deaf Education Center
and
National Center for Law and the Deaf
800 Florida Avenue, NE
Washington, DC 20002
Hearing Loss Association of America (HLAA, formerly Self Help for Hard of Hearing People [SHHH])
7910 Woodmont Ave, Suite 1200
Bethesda, MD 20814
Phone: (301) 657-2248
Helen Keller National Center for Deaf-Blind Youths and Adults (HKNC)
141 Middle Neck Road
Sands Point, NY 11050
Voice, TTY: (516) 944-8900
E-mail: hkncinfo@hknc.org
House Ear Institute (HEI)
2100 West Third Street
Los Angeles, CA 90057
Voice: (213) 483-4431
TTD: (213) 483-2642
Fax: (213) 483-8789
E-mail: info@hei.org
National Association of the Deaf (NAD)
8630 Fenton Street, Suite 820
Silver Spring, MD 20910-3819
Voice: (301) 587-1788
TTY: (301) 587-1789
Fax: (301) 587-1791
National Cued Speech Association (NCSA)
5619 McLean Drive
Bethesda, MD 20814-1021
Voice, TTY: (800) 459-3529, (301) 915-8009
National Fraternal Society of the Deaf
1188 South Sixth Street
Springfield, IL 62703
Voice: (217) 789-7429
TTY: (217) 789-7438
Office of Special Education and Rehabilitative Services (OSERS)
400 Maryland Avenue, SW
Washington, DC 20202-7100
Voice: (202) 245-7468
Parmly Hearing Institute
Loyola University
6525 North Sheridan Road
Chicago, IL 60626
Voice: (773) 508-2710
Fax: (773) 508-2719
E-mail: rfay@luc.edu (Richard R. Fay, director)
Rainbow Alliance of the Deaf (RAD)
Steven Schumacher, RAD Secretary
9804 Walker House Road, Suite 4
Montgomery Village, MD 20886-0506
Registry of Interpreters for the Deaf, Inc
333 Commerce Street
Alexandria, VA 22314
Phone: (703) 838-0030
Telecommunications for the Deaf, Inc (TDI)
8630 Fenton Street, Suite 604
Silver Spring, MD 20910
Voice: (301) 589-3786
TTY: (301) 589-3006
Fax: (301) 589-3797
E-mail: info@tdi-online.org
Triological Society (The American Laryngological, Rhinological, and Otological Society, Inc.)
555 North 30th Street
Omaha, NE 68131
Voice: (402) 346-5500
Fax: (402) 346-5300
E-mail: info@triological.org
USA Deaf Sports Federation (USADSF, formerly American Athletic Association of the Deaf [AAAD])
102 North Krohn Place
Sioux Falls, SD 57103-1800
Voice: (605) 367-5760
TTY: (605) 367-5761
E-mail: HomeOffice@usdeafsports.org
World Recreation Association of the Deaf, Inc (WRAD)
PO Box 3211
Quartz Hill, CA 93586
Videophone: (661) 943-8879
Additional patient education resources
For patient education resources, see Ear, Nose, and Throat Center, as well as patient education article Hearing Loss.
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| Organ or System | Syndrome | Inheritance Pattern | Hearing Loss | Obvious Physical Abnormalities |
| External ear | DiGeorge sequelae | Sporadic | CHL | Yes |
| Branchio-oto-facial syndrome | AD | CHL | Yes | |
| Townes-Brocks syndrome | AD | SNHL | Yes | |
| Miller syndrome | AR | CHL | Yes | |
| Bixler syndrome | AR | CHL | Yes | |
| Cardiac | Coloboma, heart disease, atresia choanae, retarded growth, and ear anomalies (CHARGE) syndrome | AD, AR, X linked, sporadic | SNHL, mixed | Yes |
| Jervell Lange-Nielson syndrome | AR | SNHL | No | |
| Limb-oto-cardiac syndrome | AR | CHL | Yes | |
| Renal | Alport syndrome | AD, AR, X linked | SNHL | Yes or no |
| Branchio-oto-renal syndrome | AD | SNHL, CHL | Yes | |
| Kearns-Sayre syndrome | Sporadic | SNHL | Yes | |
| Epstein syndrome | AD | SNHL | No | |
| Barakat syndrome | AR | SNHL | No | |
| Mental (retardation) | Noonan syndrome | Sporadic | SNHL | Yes |
| Killian/Teschler-Nicola syndrome | Sporadic | SNHL | Yes | |
| Cockayne syndrome, type I | AR | SNHL | Yes | |
| Gustavson syndrome | X linked | SNHL | Yes | |
| Dermatologic | Waardenburg syndrome | AD | SNHL | Yes |
| Lentigines, ECG, ocular, pulmonary, abnormal, retardation, and deafness (LEOPARD) syndrome | AD | SNHL | Yes | |
| Senter syndrome | AR | SNHL | Yes | |
| Black locks with albinism and deafness (BADS) syndrome | AR | SNHL | Yes | |
| Davenport syndrome | AR | SNHL | Yes | |
| Endocrine and/or metabolic | Pendred syndrome | AR | SNHL | Yes or no |
| Johanson-Blizzard syndrome | AR | SNHL | Yes | |
| Refetoff syndrome | AR | SNHL | Yes | |
| Wolfram syndrome | AR | SNHL | Yes or no | |
| Kallmann syndrome | AD, AR, X linked | SNHL, mixed | Yes or no | |
| Facial | Goldenhar syndrome | AD, AR | CHL, SNHL | Yes |
| Frontometaphyseal dysplasia | X linked | Mixed | Yes | |
| Escher-Hirt syndrome | AD | CHL | Yes | |
| Levy-Hollister syndrome | AD | SNHL | Yes | |
| Ophthalmologic | Usher syndrome | AR | SNHL | Yes or no |
| Marshall syndrome | AD | SNHL | Yes | |
| Alström syndrome | AR | SNHL | Yes | |
| Harboyan syndrome | AR | SNHL | Yes or no | |
| Fraser syndrome | AR | CHL | Yes | |
| Jensen syndrome | X linked | SNHL | No | |
| Orthopedic | Klippel-Feil sequelae | Sporadic | CHL, SNHL | Yes |
| Stickler syndrome | AD | CHL, SNHL, mixed | Yes | |
| Craniometaphyseal dysplasia | AD, AR | CD | Yes | |
| Oto-spondylo-megaepiphyseal dysplasia (OSMED) syndrome | AR | SNHL | Yes |
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