eMedicine Specialties > Pediatrics: Surgery > Otolaryngology
Hearing Impairment: Follow-up
Updated: Jul 21, 2008
Follow-up
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 behavioral or psychological problems rather than 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. Because 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, a parent's response to having a child with hearing impairment is generally the same as that of a parent whose child has another disability or chronic medical condition. 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.
The diagnosis of deafness may be delayed because infants may respond to many sounds and yet be unable to hear normal conversational speech to understand words that they hear. Speech discrimination cannot be tested in very young children or in children without language.
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, 60 dB).
The nature of the distorted sound in SNHL means that amplification of the deficit may not improve the child's language comprehension in any meaningful way. A radio signal that is mostly static is unintelligible no matter how loud it is.
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. Most children who are deaf are otherwise generally healthy.
Lip-Reading (Oralism) and Sign Language
The debate over lip-reading versus 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. 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]).
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.
The disadvantages are are numerous. First, formal training can begin only at school age. Therefore, language acquisition is delayed beyond the optimal neurobiologic window at approximately 3-5 years of age (though some evidence indicates it may be as young as 18 mo).
Second, almost half of the consonants 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.
Third, lighting, distance, speech impediments, accents, and foreign objects or motion (eg, food, pencils, mustaches, turning the head) can make lip-reading difficult.
Fourth, 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.
Finally, typical lip-readers understand only one third of a 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.
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.
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 uses the signs of ASL but imposes a strict order of the spoken word on the signs. SEE invents prefixes, suffixes, conjugations, and signs that are not necessary in the grammar of ASL. 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. In realistic terms, most people end up omitting or changing some of the signs because SEE is so long. Therefore, children do not benefit from the English-grammar aspect of SEE as much as they might.
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.
A 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
Another debate centers on total communication, the use of signs and the voice simultaneously.
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 understand because the phrase is short and the context is important.)
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 large depend on the choice of language.
Children who are learning to lip-read attend an oral school where lip-reading is taught. A substantial portion of the day is spent in lip-reading instruction, and other subjects cannot truly be taught 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). Many children who are deaf and who have succeeded to this point do well in school with their peers.
If the child learned cued speech in a special program, placement in a regular classroom may be possible if a cued-speech interpreter is provided. Many children who are entering a school where sign language is used have already learned some signs at home or in early intervention. 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. This process is analogous to most kindergarten classes, which tend to be more social than academic. The use of ASL or SEE in schools is debated. Two issues are that most hearing teachers do not use good ASL grammar, and SEE takes too long to perform accurately.
Placing children who are deaf or hard of hearing with hearing children in the least restrictive environment (mainstreaming) may not be as successful for them 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. During when children are engaging in nonacademic subjects or when no interpreter is present, communication between deaf students and their classmates is limited.
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. At a deaf school, where everyone signs, students can participate in many activities, such as debate, football, and cheerleading. Many deaf children live in a household that is linguistically isolated, though a school environment that is completely linguistically accessible to the child promotes self-esteem and social skills. Most deaf adults who attended such residential schools look back on that experience as being the best time of their lives.
Summary: 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. Results of most studies cannot be generalized to apply to the majority of children who are deaf and hard of hearing.
Decisions must almost always be individualized to meet the needs of the particular child, parents, 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, cell phones, and hand-held devices permit children to communicate with email and text messages.
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 consider using 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 - American Academy of Audiology
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 - Gallaudet University
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 Services for the Blind
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)
US Department of Education
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 resources
For excellent patient education resources, visit eMedicine's Ear, Nose, and Throat Center. Also, see eMedicine's patient education article Hearing Loss.
Miscellaneous
Special Concerns
Universal neonatal hearing screening
In 1994, the JCIH recommended that hearing be assessed in all newborns before they are discharged from the neonatal nursery.7 The committee recommended a 2-step testing process with ABR, OAE, or a combination. The slight advantages of the OAE in easy and rapid testing are offset by an increased false-positive rate because cellular debris and amniotic fluid affect it more than the ABR.
In 1999, the AAP supported the committee's statement and endorsed the development of universal hearing screening programs.1 The AAP noted that the goal of testing all newborns is feasible if the referral rate after a 2-step screening process can be maintained more than 4%.
Fluid and debris in the ears frequently cause false-positive results, the fluid might not resolve before discharge. Therefore, retesting may not achieve the stated goal of more than 4%. However, this possibility must be balanced against the loss to follow-up. Approximately 5-20% of parents have a child who may have hearing loss at the time of discharge. If a follow-up test is not performed before discharge, these parents may not return for repeat testing.
To be beneficial, a screening test must identify a problem that is amenable to intervention. Early intervention programs improve the likelihood that children will succeed later in life and were not possible when the mean age at diagnosis was 2.5 years. However, few primary care providers significantly understand deafness. They may not know how to help hearing-impaired children and their parents. Furthermore, specialists in the field of deafness are few. Most advice and support comes from nonmedical sources or otolaryngologists; in general, such information is biased toward one method over another.
Because of the prevalence of hearing loss and the benefit of early intervention, universal screening may still be worthwhile. If the AAP-supported recommendations are implemented, and if deafness is increasingly recognized as a pediatric problem, pediatricians will become more involved than they have been. Pediatricians will be able to gain knowledge in this field and to identify local resources for families with children who are deaf or hard of hearing.
Interpreters
Although the Americans with Disabilities Act (ADA) does not specifically limit the right to a sign-language interpreter to adults alone, children who sign are not usually offered the services of an interpreter.
If practitioners verbally ask children aged 6 years why they are sick, they should consider hiring an interpreter for a similar child who is deaf. Many children who are deaf grow up and know nothing about their families' health histories. For instance, they might be inadvertently excluded from discussions about a family member's illness that their hearing siblings overhear. They often know as little about their own health history. As adults, they may remember being in the hospital when they were aged 15 years, but they never knew why.
If a child requires a procedure, an operation, and/or anesthesia, an interpreter should certainly be hired to help communicate. Parents who know sign language are generally not proficient enough to explain the procedure to their child, even the simplified version of the situation that children are told. Furthermore, practitioners should permit the parents to focus on their roles (eg, as worried parents) and not have to be the child's interpreter. For some routine health-maintenance visits and for minor illnesses, interpreters are highly recommended but not as essential as they are when a child is being hospitalized or undergoing a surgical procedure.
Cochlear implants are implantable devices inserted through the skull and into the cochlea by using a drill.8 The device is connected, with a wire, to a subcutaneous magnet left behind the ear. The patient wears an exterior magnet connected by wire to a computer processor. Cochlear implants are not invisible. The processor alone is at least the size of a cigarette box or deck of cards, and it is worn on a large harness. However, new technologies are reducing the size of the devices.
Cochlear implants create sound in the brain by directly stimulating the auditory nerve. The processor divides sound in the hearing frequencies into 22 channels (current technology). Sound received by the processor in a given frequency is converted into an electronic impulse across the magnets to the cochlear implant itself, which then stimulates the nerve at 22 corresponding locations along the cochlea. Cochlear implants may be considered for implantation in children with profound hearing loss.
The training required for use is substantial and very important. Unless the child and parents participate in the training, much of the potential benefit of the cochlear implant is lost.
Cochlear implants are frequently mischaracterized as a cure for deafness. Many people believe that they may have potential adverse health effects, in addition to the risks of surgery and infection. Conversely, as the technology of the cochlear implant improves, it could potentially signal the demise of the deaf community. A cure for blindness or spinal cord injury would not destroy the communities of individuals with those disabilities in a similar fashion. Cochlear implants do provide many children with substantial hearing and auditory language benefit. However, predicting who will do well or how well they will do is impossible. Therefore, balanced and realistic counseling is important.
Deaf culture and the deaf community
Deafness creates a language barrier and thus isolates persons who are deaf from their families and communities. As a result, deaf people have formed a community linked by sign language. The core of the deaf community uses ASL more than they use SEE. ASL, folklore, jokes, and puns that are unique to the language highlight the shared experiences of the community.
Some parents fear losing their child to the deaf (signing) community. Sometimes, this fear is part of the reason parents opt to have their child read lips. However, children thrive when they are surrounded by people who can understand them.
Instead of denying the child access to the deaf culture, parents should be encouraged to learn about the deaf community and to participate in it. A person who is deaf participates in the hearing world by default. Parents should be encouraged to meet both oral and signing people who are deaf and to talk to them about their experiences. They should be helped to identify resources in the deaf community. The goal is for families to understand that existence in a world predicated on a signed language is liberating and not isolating.
More on Hearing Impairment |
| Overview: Hearing Impairment |
| Differential Diagnoses & Workup: Hearing Impairment |
| Treatment & Medication: Hearing Impairment |
 Follow-up: Hearing Impairment |
| References |
| « Previous Page |
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Further Reading
Keywords
hearing loss, deaf, deafness, hard of hearing, hard-of-hearing, conductive hearing loss, CHL, sensorineural hearing loss, SNHL, mixed hearing loss, American Sign Language, ASL, English Sign Language, Signed English, SE, Signing Exact English/Seeing Essential English, SEE, Signing Exact English, SEE 1, Seeing Essential English, SEE 2, lip-reading, lipreading, lip reading, total communication, voice and sign language, brainstem audio-evoked response, BAER, automated auditory brainstem response, ABR, AABR, otoacoustic emissions, OAEs, audiometry, otosclerosis
cholesteatoma, glomus tympanicum, glomus jugulare, schwannomas of the facial nerve, hemangiomas, encephalocele, Waardenburg syndrome, Gernet syndrome, Winter syndrome, Rosenberg syndrome, Turner syndrome, Klinefelter syndrome, DiGeorge syndrome, Townes-Brocks syndrome, Miller syndrome, Bixler syndrome, coloboma, heart disease, atresia choanae, retarded growth, ear anomalies, CHARGE syndrome, Jervell Lange-Nielson syndrome, limb-oto-cardiac syndrome, Alport syndrome, branchio-oto-renal syndrome, Kearns-Sayre syndrome
Epstein syndrome, Barakat syndrome, Killian/Teschler-Nicola syndrome, Noonan syndrome, Cockayne syndrome, Gustavson syndrome, LEOPARD syndrome, Senter syndrome, BADS syndrome, Davenport syndrome, Pendred syndrome, Johanson-Blizzard syndrome, Refetoff syndrome, Wolfram syndrome, Kallmann syndrome, Goldenhar syndrome, frontometaphyseal dysplasia, Escher-Hirt syndrome, Levy-Hollister syndrome, Usher syndrome, Marshall syndrome, Harboyan syndrome, Fraser syndrome, Jensen syndrome, craniometaphyseal dysplasia, OSMED syndrome, cytomegalovirus, CMV, herpes, rubella, syphilis, toxoplasmosis, varicella, meningitis, mumps
