Two pediatric case studies involving children with multiple disabilities are presented, along with some of the gaps in hearing care that appear too often in these cases. The author proposes six areas in improving care for children with hearing impairment and multiple disabilities, including: making sure children have access to all sounds, ensuring parents’ psychological needs are addressed, using effective family educational practices, considering all disabilities in the hearing treatment plan, and recommending genetic workups when appropriate.

Vishakha W. Rawool, PhD, is a professor in the Department of Speech Pathology and Audiology at West Virginia University, Morgantown, WVa.

Recent advances in newborn hearing screenings have led to early identification of hearing loss. However, further improvements are necessary in early diagnosis and treatment of infants with hearing loss.¹

The need for early treatment of hearing loss is even more dire when an infant has other disabilities in addition to the hearing loss. Such infants are at risk for significant delays in speech and language development and associated risks, such as inadequate educational and social development. According to some estimates, approximately 25% to 42% of children with hearing loss have at least one additional disability^2-5 and about 20% of these children have two additional problems.² Given these facts, consideration of additional disabilities is critical for the overall success of early hearing intervention programs.

The purpose of this article is to present case studies of two children who were fitted with hearing aids at an early age but were using the aids minimally. Both children were seen as part of an interdisciplinary evaluation for feeding and swallowing disorders at a community-based center. Evaluation in this context presented clinicians with a unique opportunity to evaluate their auditory outcomes outside the regular audiological context.

These case studies suggest a critical need for audiological follow-up in children whose parents might be overwhelmed by other obviously visible disabilities and thus may pay insufficient attention to hearing loss and hearing aids. Similarly, other professionals, including teachers, involved in the team management may overlook the invisible hearing loss—an oversight that may compromise the child's ability to benefit fully from the various special services and to realize his/her full potential for speech, language, psychosocial, and educational development.

Case Study #1: 6-month-old Girl

Case history. AA was a 6-month-old female at the time of this evaluation and was seen in the interdisciplinary clinic for feeding and swallowing disorders due to concerns about very limited food intake during each mealtime.

Her mother reported two prior miscarriages, and she also had grand mal seizures and was on labium before the pregnancy. Natal history is marked by Cesarean section delivery during the seventh month, a stay in the neonatal intensive care unit (NICU) for 10 days after birth, and bilirubin therapy. AA had hip dysplasia, a webbed neck, and a locked finger at birth. Three surgeries were performed on AA to correct the hip problems. She began having seizures after the surgeries and was on phenobarbital, but currently has no seizures.

Hearing-related history. AA failed a hearing screening at birth. Auditory brainstem response testing was conducted at the age of 2 months, which revealed bilateral severe hearing loss (worse in the right ear). Phonak BTE power hearing aids were fitted 3 weeks prior to the current appointment. AA is enrolled in the "birth to three" program. She was visited by a Ski High program advisor 2 days prior to this appointment who demonstrated the insertion, removal, and maintenance of hearing aids to AA's mother. At the time of the evaluation, AA was not wearing hearing aids. Upon inquiry, the mother reported that her understanding was that the hearing aids were to be worn only during feeding time.

The mother indicated that she was interested in pursuing the total communication mode and was taking sign-language classes. She also reported that she was the only one in the family who was taking these classes.

Hearing aid performance assessment. The interdisciplinary center does not have any hearing aid test equipment. Therefore, hearing aid performance was evaluated through auditory and visual inspection of aids and informal observation of the patient with and without hearing technology.

Auditory inspection suggested good function of the aids. Visual inspection suggested that the right earmold was too loose, which resulted in persistent whistling/feedback. The left hearing aid was coupled to the left ear, and AA did not respond to any speech sounds with the hearing aid, suggesting insufficient gain. She responded very well with a smile when fitted with a trial FM device. AA was remarkably cooperative with placement and acceptance of both the hearing aid and the FM device.

Counseling and recommendations. The mother was informed about the need for repeated ear impressions and gain readjustments due to the growing ear-canal during infancy and early childhood. Additional counseling was provided about the importance of consistent use of hearing aids to promote incidental listening and language learning during the critical period of language development. The importance of involvement of all family members in any activities related to learning sign language was also explained.

The types of responses that can be expected from the child to auditory stimuli were demonstrated to the mother. AA's mother appeared very responsive to all suggestions. Although the child walked in with the hearing aids in the box, she walked out with the left hearing aid coupled to her ear. The following recommendations were made:

• Continued monitoring of auditory sensitivity and hearing aid performance.
• Intensive speech and language stimulation to promote speech and language development during the critical period of language development.
• Genetic referral to rule out spondyloepiphyseal dysplasia (SED) congenita due to the presence of hip dysplasia, a webbed neck, and a locked finger at birth.

Several gaps were apparent relative to the optimal auditory care for AA. These included:

1. Short- and long-term follow-ups are important components of an effective early hearing detection and intervention (EHDI) program.1 Continuous adjustments to hearing aid gain and earmold impressions for new earmolds were necessary for AA. Appointments for new earmolds at regular intervals should be made at the time of the initial fitting in early infancy. Without such appointments, the 6-month "trial period" for hearing aids before considering cochlear implants is, in effect, much shorter than the time period based on the date of the initial fitting.
2. A gap between the mother's understanding of when and how often to use hearing aids was apparent with the mother being under the impression that she was supposed to place the hearing aids on AA only during feeding times.
3. If it is assumed that the family has made a well-informed choice about adopting total communication, the mother was unaware of the need for all family members to be involved in sign-language training.
4. There was not an initial referral for genetic evaluation to rule out SED congenita. According to the mother, no genetic referrals were previously made. SED is an inherited disorder of bone growth that results in dwarfism or short stature and skeletal abnormalities. Approximately 25% of individuals with SED have hearing loss.⁶ Inner and middle ear abnormalities are possible, including fixation of the footplate of the stapes.^7,8 Since the hearing loss can be progressive,⁷ early diagnosis and intervention is critical.

In SED, the bones of the spine (spondylo-) and the ends (epiphyses) of long bones in the arms and legs are affected. Therefore, patients with SED can suffer from arthritis and decreased joint mobility early in life, which has the potential of restricting hand movements for sign-language communication. Furthermore, severe nearsightedness and other eye problems, such as retinal detachment or cataracts,6 can impair vision, which can have an additional impact on sign-language communication.

Case Study #2: 6-year-old Girl

Case history. AB was a 6-year-old female at the time of this evaluation and was seen in the interdisciplinary clinic for feeding and swallowing disorders to evaluate oral skills and to provide recommendations for advancing current oral skills. The child had a gastrostomy (G) tube at the time of this evaluation.

Family history is marked by a left outer ear malformation and possible hearing loss in one of the paternal cousins. Medical history includes the discovery of a large cyst in one of the lungs at the age of 10 months, which was surgically treated with lobectomy. AB suffers from acid reflux, hypothryroid, and seizures, and she appears to have cerebral palsy. She does not walk, but displays fair hand movements. AB's vision is very poor; she has prescription eye-glasses but does not wear them.

Hearing-related history. AB suffers from chronic ear infections, which have been treated with antibiotics and placement of pressure equalization tubes. She was diagnosed with a moderate-to-severe hearing loss at around age 3. She has two digital BTE Widex Senso hearing aids, which were fitted at the age of 3 years. However, the hearing aids have not been worn on a consistent basis because the family members are unsure if AB derives any benefit from the use of hearing aids. The parents reported that, after the initial hearing aid fittings, the hearing aids have never been readjusted.

Communication and education. AB does not speak, but can vocalize. Previous communication intervention attempts included use of augmentative communication devices and sign-language. AB attends a special education classroom and receives occupational therapy, speech therapy, and physical therapy on a weekly basis.

Audiological screening. The interdisciplinary center does not have any audiological equipment. Therefore, hearing screening was performed with a portable audiometer and tympanometer. Tympanometry revealed reduced mobility of the tympanic membrane/middle-ear system, suggesting middle-ear dysfunction bilaterally at the time of testing. Audiological screening was conducted using behavioral observations by presenting warbled tones and speech stimuli under headphones in a quiet setting. Responses suggested a severe-to-profound bilateral hearing loss.

Hearing aid performance assessment. The interdisciplinary center does not have any hearing aid test equipment. Thus, hearing aid performance was evaluated through auditory and visual inspection of aids and informal observation of the patients with and without hearing technology.

Visual inspection of the digital hearing aids revealed slightly loose earmolds. Auditory inspection suggested that the hearing aids were providing minimal amplification. No behavioral responses to conversational speech were apparent with binaural hearing aids. However, AB did respond to speech sounds when an FM device was directly coupled to her left ear. The mother reported that the hearing aids have never been readjusted since the initial fitting.

Conclusions. Overall these findings suggest a severe-to-profound hearing loss in both ears. Behavioral responses to aided conversational speech suggest that the current gain settings may be insufficient, probably due to lack of adjustment of hearing aid parameters after the initial hearing aid fitting and a probably transient conductive component.

Counseling and recommendations. Parents were informed about the results. AB's ability to respond to sounds was demonstrated to them with the help of the FM device. The following recommendations were made:

• An otolaryngological evaluation to assess the middle ear;
• A complete audiological reevaluation to determine the current degree and type of hearing loss;
• New earmolds and reevaluation of the current gain parameters in the hearing aids to ensure adequate amplification, sufficient to elicit behavioral responses to conversational speech;
• Acquisition of an FM system for classroom use. AB's hearing aids are equipped with a t-coil. Therefore, the assistive listening device can be coupled to the hearing aid through an induction neck-loop.
• Frequent hearing and hearing aid re-evaluations and follow-ups due to the history of chronic ear infections. The monitoring should ensure adequate and consistent use of hearing aids to capitalize on the critical period of language development.
• Reevaluation and ongoing monitoring of vision to ensure consistent and adequate use of eyeglasses. Without proper vision, the child is expected to draw minimum benefit from instruction in the use of assistive communication devices or sign-language.
• Genetic workup due to the family history of outer ear deformity.

The gaps apparent in optimal auditory care for AB include:

1. Lack of follow-up hearing evaluations and continuous adjustments to hearing aid gain and earmold impressions for new earmolds.
2. A gap in the caregiver's ability to recognize the child's ability to respond to sounds and thus ensuring consistent use of hearing aids.
3. Failure to implement the use of adequate assistive listening technology for classroom use.
4. Attempts to teach sign language and use of assistive communication devices without ensuring adequate visual access.
5. Lack of referral for genetic workup in the presence of family history of outer ear deformity. Since AB is showing symptoms of cerebral palsy and developmental delays in the absence of any prenatal, natal, or postnatal cause for brain injury, it is also essential to evaluate her for any underlying genetic syndrome or a progressive neurological or metabolic condition. A genetic contribution to cerebral palsy risk is possible, and variants of several genes have been associated with cerebral palsy in girls.⁹

Clinical Implications

These case studies demonstrate that hearing aid fitting is only the first step in improving the overall outcomes of children with hearing loss who have additional disabilities. Without adequate short- and long-term follow-up, the hearing aids will not be used on a consistent basis, depriving the child of incidental learning opportunities. Additionally, if hearing aids and appropriate classroom technology (eg, FM devices) are not used, any benefit the child can get in formal learning environments—including therapy sessions in noisy or reverberant environments such as classrooms—may be minimal or nonexistent depending on the degree of hearing loss.

There are several recommendations that can be made for children with multiple disabilities, and many fall into the six areas covered below:

1) Ensure Access To All Sounds

Perform real-ear measures. Real-ear measures need to be performed in fitting hearing aids to children at the initial fitting and on an ongoing basis. In the cases described here, parents could not recall placement of probe tubes in the ear canal or performance of real-ear measures by the dispensing audiologists. A survey in 1996 suggested that only about 15% to 40% of the audiologists always used probe microphone measures.¹⁰ This can lead to rejection of hearing aids by children when the gain is excessive or lack of benefit from hearing aids when the gain is insufficient.

Provide assistive listening technology. The speech recognition scores of children with even mild hearing loss are poorer than those of normal-hearing children in noise.¹¹ In addition, these children need to exert more listening effort,¹² which can leave them with less mental energy to perform other activities or to accommodate for other disabilities such as visual impairment. Thus, hearing aids with integrated FM technology should always be considered for children with hearing loss, especially for those with multiple disabilities, to allow access to sounds in academic and other noisy and reverberant conditions.

Provide effective guidance to parents for hearing aid and FM use. Parents should be guided properly to ensure continued use of hearing aids. They should be informed about the appropriate expectations from hearing aids during the initial period so that, when the child does not respond, they do not discontinue the use of aids.

Additionally, they need to understand that a child with multiple disabilities may not respond to sounds in the same manner that a normal child responds, and they need to look for other types of clear responses that are "time locked" to the presence of stimuli, such as stopping, smiling, increase in alertness, vocalization, etc. If strong motivation, knowledge about the need for amplification (especially during the critical period of language development), potential impact of lack of amplification on communication, education, and social development, and the confidence in ensuring proper hearing aid use is not provided, hearing aids will not be used. Also the emotional barriers, including any embarrassment related to the child's hearing aids, should be addressed on an individual family basis.

2) Address Psychological Needs of Parents

Support networks for children with multiple disabilities can be helpful for parents of children who have hearing loss and additional disabilities. Such parents may feel isolated in a support group of parents whose children have only hearing loss.

Without psychological support, parents can get overwhelmed by chronic illnesses, numerous surgeries, and many appointments with health professionals.¹³ They may feel inadequate and might seek a rescuer professional. The rescuing professional, without realizing it, can reinforce the feelings of inadequacy and learned helplessness in parents.¹⁴ Some professionals are so focused on addressing the disability related to their specialty that they may overlook the need for ongoing emotional support; even just providing undivided attention to parents' concerns and active listening can be of great help to the family.

Parents of AA and AB were extremely articulate in expressing all of their hurdles and needs during the brief informational counseling session provided to them. Both seemed relieved after being able to recognize their children's responses to sounds. However, in both cases, the need for continuing emotional support was obvious. Professionals need to remember that parents can effectively process information only when their emotional needs are met.

3) Use Effective Family Education Practices

An important component of a comprehensive EHDI program is effective family education. Development and use of effective strategies is necessary to ensure that information is understood and retained by family members.

Provide videotapes of informational sessions. A study suggests that mothers of babies in neonatal intensive care units who receive taped conversations with neonatologists are 75% more likely to recall all of the information about treatment compared to mothers who do not receive such taped conversations.¹⁵ Providing the parents with a videotape of any demonstrations and conversations with the audiologist may allow the parents to review and digest the information later when they have had a chance to overcome the initial shock and are psychologically better ready to absorb the information in a more relaxed home environment. Taping may also motivate audiologists to carefully and interactively cover essential information.

Minimize information overload. Initial information load should be minimized in such a way that parents can understand and apply the information for their specific child. If too many recommendations are made at the beginning, the parents may not recall them accurately or they may marginalize the recommendations as being impractical.

Provide individualized information. It is important to address the specific concerns of each family. For example, if a child cries excessively when the hearing aids are placed, the parents may feel unsure about whether or not the hearing aids are causing discomfort to the child due to either extreme loudness or uncomfortable earmolds. Under such circumstances, real-ear measures can be explained to parents in nontechnical language. In addition, aided responses to both loud and soft stimuli can be demonstrated in a sound-treated booth.

If parents are worried about excessive crying due to the presence of other health conditions, such as congenital heart problems, they should be guided to increase the use of hearing aids slowly over time. Other parents may simply try to prioritize "family peace" over any benefits the child may receive from hearing aids. Obviously, effective informational counseling is critical so that such parents can make well-informed choices after clearly understanding the long-term impact of such choices.

If the family perceives the child's cognition to be too poor to benefit from hearing aids, the connection between sensory deprivation and cognition should be explained, and consideration of developmental age rather than chronological age in infancy and early childhood should be emphasized.

Provide information in a clearly written format, and offer recommendations at the beginning of the report. All of the information should be given in writing in a clear and easy to understand format so that parents can review it later. Recommendations usually appear at the end of audiology reports. Although from a professional viewpoint this is logical, many parents who are not professionals may give up reading the report due to the technical nature of the first part before they ever reach the recommendations part. The situation is worse when parents of children with multiple disabilities are bombarded with several reports and recommendations from several different disciplines. Under such circumstances, the recommendations related to hearing can be easily lost or forgotten.

For these reasons, it is better to provide a limited set of clearly written recommendations that are easy to follow at the beginning of the report. More relevant recommendations can be provided later, at the time of each evaluation based on the child's progress at that time.

4) Consider All Disabilities in Planning Intervention

Visual communication systems have been suggested in the presence of significant language delay or additional learning needs.¹⁶ However, in order for families to make a well-informed choice, comprehensive information should be provided about visual communication systems and any required modifications that might be necessary when additional disabilities such as restricted hand movements or progressive visual impairments are present. Children who have a history of a combination of very low birth weight, extreme prematurity, and assisted ventilation for 10 or more days should be considered at risk for visual problems. Confirmed sepsis and seizures with intraventricular hemorrhage are addition risk factors for visual impairments.¹⁷

5) Refer for Genetic Workup

Referral for genetic workup and counseling should be made as needed. Parents expect to have a diagnosis of their child's condition¹⁸ and they have a need to understand the cause of their child's hearing loss.¹⁹ A diagnostic label related to the multiple disabilities can assist parents in accepting the several dimensions of the disability and in explaining the disability to others.¹⁸

The results of genetic workup can allow for prediction of hearing loss or vision problems and detection of other abnormalities such as restricted hand movements. These in turn can have an impact on selection of communication mode. Although genetic workup might not always reveal the cause, it can be psychologically helpful for parents to know that everything possible was done to find the cause.18 Additionally, for future offspring, prenatal diagnosis for some conditions (eg, SED) is possible and can lead to early diagnosis and intervention.²⁰

6) Prevent the "Invisible" Hearing Loss from Being Marginalized

It is important to ensure that the invisible hearing disability is not lost or minimized among other obvious physical disabilities. Because hearing loss is invisible, it can be easily ignored in the presence of other obvious conditions, such as skeletal abnormalities or physical handicaps.

All professionals need to be vigilant in addressing the needs related to hearing loss in the presence of other disabilities. Treatments provided for many other disabilities—such as physical therapy or occupational therapy—often include verbal instructions and reinforcements to the child. Ensuring adequate auditory access can enhance the child's progress in other areas. Audiologists need to remind professionals from all specialties that, without adequate vigilance about hearing loss, individuals with multiple disabilities may not be able to reach their full social, educational, and employment potential.

References

1. American Speech-Language-Hearing Associ-ation. Lost to Follow-up in Early Hearing Detection and Intervention [Technical Report]: 2008. Available at: www.asha.org/policy.
2. Fortune H, Davis A. Epidemiology of permanent childhood impairment in Trent region, 1985-1993. Br J Audiol. 1997;31(6):409-446.
3. Gallaudet Research Institute. Regional and National Summary Report of Data from the 2004-2005 Annual Survey of Deaf and Hard of Hearing Children and Youth. Washington, DC: Gallaudet University; 2005.
4. Holden-Pitt L, Albertorio J. Thirty years of the Annual Survey of Deaf and Hard-of-Hearing Children and Youth: a glance over the decades. Am Ann Deaf. 1998;143(2):72-76.
5. Van Naarden K, Decoufle P, Caldwell K. Prevalence and characteristics of children with serious hearing impairment in metropolitan Atlanta, 1991-1993. Pediatrics. 1999;103:570-575.
6. Wynne-Davies R, Hall C. Two clinical variants of spondylo-epiphysial dysplasia congenita. J Bone Joint Surg Br. 1982;64:435-441.
7. Cole WG, Hall RK, Rogers JG. The clinical features of spondyloepiphyseal dysplasia congenita resulting from the substitution of glycine 997 by serine in the alpha 1(II) chain of type II collagen. J Med Genet. 1993;30:27-35.
8. Dahiya R, Cleveland S, Megerian CA. Spondyloepiphyseal dysplasia congenita associated with conductive hearing loss. Ear Nose Throat J. 2000;79(3):178-82.
9. Gibson CS, Maclennan AH, Dekker GA, Goldwater PN, Sullivan TR, Munroe DJ, Tsang S, Stewart C, Nelson KB. Candidate genes and cerebral palsy: a population-based study. Pediatrics. 2008;122(5):1079-85.
10. Hedley-Williams A, Tharpe AM, Bess FH. The status of amplification and earmolds worn by children in the United States. In: Bess FH, Gravel JS, Tharpe AM, eds. Amplification for Children with Auditory Deficits. Nashville, Tenn: Bill Wilkersen Press; 1996.
11. Crandell CC. Speech recognition in noise by children with minimal degree of sensorineural hearing loss. Ear Hear. 1993;14:210-216.
12. Bourland-Hicks C, Tharpe AM. Listening effort and fatigue in school-age children with and without hearing loss. J Speech Hear Lang Res. 2002;45:573-584.
13. Edwards BM, Kileny PR, VanRiper LA. CHARGE syndrome: a window of opportunity for audiologic intervention. Pediatrics. 2002;110(1):119-126.
14. Luterman D. Counseling of families of children with hearing loss and special needs. Volta Review. 2004;104(4)[monograph]:215-220.
15. Koh TH, Butow PN, Coory M, Budge D, Collie LA, Whitehall J, Tattersall MH. Provision of taped conversations with neonatologists to mothers of babies in intensive care: randomised controlled trial. Br Med J. 2007;334(7583):28-31.
16. Anderson K, Matkin N. Relationship of Degree of Long-Term Hearing Loss to Psychosocial Impact and Educational Needs (Appendix A). Manitoba Education, Citizenship and Youth School Programs Division Winnipeg, Manitoba, Canada. 2009. Available at: www.edu.gov.mb.ca/k12/docs/support/dhh_resource/appendices.pdf.
17. Cone-Wesson B, Vohr BR, Sininger YS, et al. Identification of neonatal hearing impairment: infants with hearing loss. Ear Hear. 2000;21(5):488-507.
18. Cass H, Price K, Reilly S, Wisbeach A, McConachie H. A model for the assessment and management of children with multiple disabilities. Child Care Health Dev. 1999;25(3):191-211.
19. Harrison M, Roush J. Information for families with young deaf and hard of hearing children: reports from parents and pediatric audiologists. In: Seewald RC, Gravel JS, eds. A Sound Foundation Through Early Amplification. Warrenville, Ill: Phonak; 2002:233-249.
20. Cui YX, Xia XY, Bu Y, et al. Rapid molecular prenatal diagnosis of spondyloepiphyseal dysplasia congenita by PCR-SSP assay. Genet Test. 2008;12(4):533-6.

Correspondence can be addressed to HR or Vishakha Rawool, PhD, at .