Two case studies are presented about children (now young adults) with multiple disabilities who were fitted with hearing aids at an early age, but their use of the devices was discontinued. These cases suggest a critical need for long-term audiological follow-up for adults who grew up with multiple disabilities including hearing loss to ensure the best possible quality of life.
|Vishakha W. Rawool, PhD, is a professor in the Department of Speech Pathology and Audiology at West Virginia University, Morgantown, WVa.|
As stated in Part 1 of this two-part series (see the January 2010 HR), approximately 25% to 42% of children with hearing loss have at least one additional disability1-4 and about 20% of these children have two additional problems.1 Consideration of these additional disabilities is critical for the overall success of early hearing intervention programs.
Similar to Part 1 in which two case studies and discussion were offered, the purpose of Part 2 is to present case studies of two children (now young adults) who were fitted with hearing aids at an early age, but the use of hearing aids was discontinued and the hearing aids were eventually lost. Both children were seen as part of an interdisciplinary evaluation for feeding and swallowing disorders. These case studies suggest a critical need for long-term audiological follow-up for adults who grew up with multiple disabilities including hearing loss to ensure the best possible quality of life.
The interdisciplinary center does not have any audiological equipment. Hearing screening was performed with a portable audiometer and tympanometer that receive annual calibration and daily biological calibration.
Case Study #3: 28-year-old Woman
Case history. AC is a 28-year-old woman with Möbius syndrome who was seen to address concerns about limited fluid intake due to functional limits of feeding skills. Möbius syndrome is a rare birth defect marked by the absence or underdevelopment of the 6th and 7th cranial nerves; the 3rd, 5th, 8th, 9th, 11th, and 12th cranial nerves can also be affected. Symptoms usually include a unilateral or bilateral congenital facial weakness with impairment of ocular abduction, and frequent occurrence of structural limb defects.5 The specific symptoms in each patient depend on the particular nerves that are involved.
Speech problems have been noted in 70% of the patients.6 Speech difficulties can result from a variety of reasons, including functional problems of the lips, tongue, palate, and occasionally the larynx, cleft palate, micrognathia, microglossia, or hearing loss.5 Johansson et al7 noted hearing loss along with cleft palate and chronic otitis media in 5 out of 19 (25%) patients with Möbius syndrome. Two of these patients had a mild unilateral or bilateral sensorineural hearing loss. With prompt and adequate management, patients with Möbius syndrome are expected to have a normal lifespan.
AC’s family history is marked by hypothyroidism in several family members. Her prenatal history includes Rh factor incompatibility between the mother and the baby. The mother received medications including RhoGAM during pregnancy for anti-Rh antibodies and for stopping bleeding. Natal history was marked by premature delivery after 7½ months and jaundice, which was treated with light exposure. AC was diagnosed with clubfoot and facial nerve paralysis at birth.
Postnatal medical history includes chronic ear infections in childhood, chicken pox at age 3, German measles at age 4-5 years, migraines, and frequent episodes of influenza. She also suffers from seizures; the last seizure was approximately 1 year ago and lasted over a period of 6 hours.
AC is legally blind and has mobility problems. She is currently on about 10 different medications for seizures, asthma, migraines, allergies, and constipation. She was educated in a special setting for children with learning disabilities, and she graduated from high school.
Hearing-related history. As stated previously, AC suffered from chronic ear infections in childhood. She was diagnosed with a bilateral hearing loss at an early age and was fitted with hearing aids. Use of hearing aids was discontinued due to lack of cooperation from the patient and parental uncertainty about benefit. The hearing aids were subsequently lost. She has difficulty hearing individuals speaking at a distance or speaking softly. Her mother reported that AC is constantly concerned about the possibility that other individuals might be talking about her.
Audiological testing. Otoscopic evaluation revealed moderate cerumen in the right ear canal. Tympanometry revealed normal mobility of the tympanic membrane/middle ear system bilaterally suggesting normal middle ear function at the time of testing.
Audiologic testing was conducted under headphones using conventional (hand-raise) test procedures in a quiet room. Results showed the presence of a mild bilateral sensorineural hearing loss.
Trial with amplification. Amplification trial was conducted with a FM device due to lack of availability of trial hearing aids at the test site. AC responded very well to speech amplified through a FM device. She expressed a keen interest in acquiring and wearing hearing aids.
Counseling and recommendations for AC. The results were explained to the patient and her mother. It was further explained that the patient’s feeling that other individuals are talking about her might be related to her hearing loss. Added significance of amplification due to the presence of blindness was also explained. It was further explained that—although AC might be managing without hearing aids—her quality of life, her ability to function independently, her physical orientation and mobility skills, and her ability to realize her full potential could improve with hearing aids. The mother was also reminded that many tools, such as total talk computer terminals, talking calculators, and reading machines that convert books, magazines, letters, and other printed material into spoken English, available for blind individuals rely heavily on the blind person’s ability to hear well. The mother agreed to pursue amplification with an audiologist near the family home.
Case Study #4: 27-year-old male
AD is a 27-year-old male (AD) with Down syndrome who was seen for reduction in food intake and food selectivity. Down syndrome is a common clinical condition appearing in approximately 1 in 700 newborns. It results due to the presence of extra chromosome material on chromosome 21.8 Among the approximately 40% of children who have multiple disabilities including hearing loss, a significant proportion have a history of NICU stay. The most common condition among these children is Down syndrome.9
Children and young adults with Down syndrome often suffer from middle ear infection and conductive hearing loss.10,11 Up to 78% of children with Down syndrome can have hearing loss in one or both ears, with 16% showing sensorineural hearing loss and 8% showing mixed hearing loss.12 Furthermore, the prevalence and severity of hearing loss increases with age.13,14
Hearing-related history. Parents reported that the patient was fitted with hearing aids in early childhood. The use of the aids was discontinued at an early age due to uncertainty of benefit from aids, lack of cooperation from the patient, and frequent episodes of otitis media. Parents reported that the child’s father occasionally used the hearing aids after the father developed an age-related hearing loss. However, they could not recall where the aids were at the present time. AD appears to have fair receptive language but limited expressive language.
Auditory assessment. Otoscopic evaluation revealed excessive cerumen in both ears. Thus, tympanometry was not conducted. Audiological evaluation was conducted using conditioned play audiometry, and results of a bilateral mild-to-moderate sensorineural hearing loss were considered to have high reliability.
Counseling and recommendations. The results were explained to the parents. Since interdisciplinary evaluation suggested depression as a possible cause for reduced food intake, the connection between depression and untreated hearing loss was explained to the parents. A complete audiological evaluation and hearing aid fitting were recommended.
Conclusions and Implications
These case studies suggest that hearing aid fitting is only the first step in improving the overall outcomes of children with hearing loss who have additional disabilities. Both AC and AD received early intervention, including hearing aid fitting. However, due to the lack of consistent use of hearing aids, the long-term impact of early intervention in these cases is unclear. Without adequate short-term and long-term follow-up, the hearing aids might be completely lost and compromise the quality of life of the patient and the family.
1) Chronic OM
Chronic otitis media has the potential for causing poor extended high frequency auditory thresholds,15 speech and language delays,16 reading problems,17 and attention problems.18 The impact of otitis media can be expected to be worse with poor verbal cognition in the presence of significant medical histories, such as prematurity,19 multiple disabilities, and syndromes including Möbius or Down syndrome. Adequate medical management of otitis media and full access to the auditory environment through appropriate amplification technology can minimize some of these problems.
2) Mild Hearing Loss in Children
The presence of even a mild hearing loss can have a significant impact on a child’s education.20,21 Bess et al22 found that 37% of the children with minimal hearing loss failed at least one grade, which was higher than the district norm failure rate of 3%.23 One possible interpretation of this finding is that many children with minimal hearing loss do not have academic difficulties24 or do not show significant educational problems.25
However, lack of failure in school might not necessarily mean that children with minimal hearing loss are able to reach their full potential without intervention. For example, a child with mild hearing loss who graduates with passing grades or is promoted from one grade to the next due to administrative or legal pressures may not have the same knowledge and capabilities as those of a child without hearing loss. Individuals with hearing loss who graduate from high school can have language and academic achievements below those of normal fourth grade students.26
In addition to any educational impact, the negative impact of mild hearing loss on personal-social quotients27 and psychosocial health-related quality of life28 should also be considered. Children with minimal hearing loss tend to perform worse than normal-hearing children in areas of self-esteem, social support, and stress.23 There is also some evidence of cognitive disadvantage associated with mild hearing loss.29 Although some outcome studies have excluded children with developmental delays or major medical conditions in order to focus on the impact of hearing loss alone,28 the impact of mild hearing loss can be expected to be worse when the loss is accompanied by other disabilities, such as blindness or Down syndrome.
About 25% of the children with mild hearing loss tend not to use their hearing aids even in the presence of parental awareness of benefits from hearing aids, and only about half of the children use their hearing aids all day.30 Lack of hearing aid use by some children with mild hearing loss does not necessarily mean lack of benefit. Children with visual impairments are often reluctant to use eyeglasses. Compliance for wearing eyeglasses can be very low, particularly among older, urban children. The reported reasons for not wearing eyeglasses can vary from broken or lost eyeglasses, headache from eyeglasses, forgetting glasses at home, using eyeglasses at special times or only occasionally, perception that eyeglasses are unnecessary, parental disapproval of eyeglasses, and being concerned or teased about the appearance of eyeglasses.31 However, lack of use of eyeglasses does not mean that the children can function optimally in educational and social environments when teachers, materials on blackboards, and peers are not within their eyesight or are at a distance.
If children with mild hearing loss are fitted with hearing aids at an early age, any delays in speech/language development can be prevented.32 Thus, effective strategies to ensure use of hearing aid technology by children with mild hearing loss can be beneficial. Most of the same reasons that are specified above for lack of use of eyeglasses probably apply to lack of use of hearing aids. Other possible reasons may include failure of parents to keep a fresh supply of batteries available, lack of definitive recommendation or uncertainty about benefits of hearing aids for mild hearing loss by hearing health professionals, and bullying by peers.
It is necessary to find the reasons for lack of use of hearing aids by each child and to address their concerns. For example, if cosmetic issues are a concern, completely-in-the-canal (CIC) hearing aids can be considered around the age of 10 years when the ear canal growth is expected to be complete.33
3) Addressing Parental Needs
Parents who are caring for adult children with multiple disabilities may experience a learned hopelessness or helplessness over time. These parents can benefit from a break once or twice a month from their children. Community organizations can develop a pool of trained volunteers who visit the home and take care of individuals with multiple disabilities. Alternatively, the children can be dropped off at a community center and looked after by a group of volunteers. These breaks allow parents to get recharged and take care of not just the basic food and shelter needs of their children but to also address special needs such as hearing aids. Additionally, in these situations, adult children who are unemployed and spend most of their time at home have an opportunity to mix with other individuals who have special needs.
4) Refitting with Integrated FM
Refitting of hearing aids with integrated FM technology and training in the consistent use and care of hearing aids should be attempted for adults such as AC and AD. Adults can use hearing aids even with moderate or severe intellectual disability after a period of individual habituation training over several months.14 Neural plasticity is possible in adulthood34 and should be capitalized on before the hearing loss becomes worse due to the effects of aging.13,14 Individuals such as AC and AD can be expected to benefit from hearing aids due to the mild and moderate degree of hearing loss. Emotional, social, and cognitive status of adults with untreated hearing loss can be negatively affected,35 which includes feelings of inadequacy and social isolation as apparent in AC and depression, as apparent in AD. Furthermore, caregivers need to be informed about the fact that use of hearing aids can improve Health Related Quality of Life (HRQoL) by reducing psychological, social, and emotional effects of hearing loss.36
5) “Revealing” Hearing Loss as an Important Disability to Address
Because hearing loss is an invisible disability, on the surface it may seem insignificant to parents in the presence of other obvious conditions, such as blindness or Down syndrome. All professionals and caregivers need to be highly vigilant in addressing the needs related to hearing loss and ensuring the use of hearing aids in the presence of other disabilities. With adequate vigilance, adults with multiple disabilities such as AC and AD can be expected to improve their quality of life by improving social interactions, overall cognition, and employment status.
6) Improve Funding of Appropriate Audiological Care
In approving grant proposals designed for service provision to individuals with multiple disabilities, funding agencies and grant reviewers need to carefully review grants and, more specifically, budgets to ensure that audiologists are included and adequately funded. The current practice of either not including audiologists, or allowing minimally qualified individuals to provide audiological services (or allocating minimal funding for these services), can further minimize the significance of care of an already invisible disability and guarantee poor auditory care.
- Fortune H, Davis A. Epidemiology of permanent childhood impairment in Trent region, 1985-1993. Br J Audiol. 1997;31(6):409-446.
- 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: GRI, Gallaudet University; 2005.
- Holden-Pitt L, Albertario J. Thirty years of the Annual Survey of Deaf and Hard-of-Hearing Children and Youth: a glance over the decades. Am Annals Deaf. 1998;143(2):72-76.
- 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.
- Briegel W. Neuropsychiatric findings of Möbius sequence—a review. Clin Genet. 2006;70:91-97.
- Sjögreen L, Andersson-Norinder J, Jacobsson C. Development of speech, feeding, eating, and facial expression in Möbius sequence. Int J Pediatr Otorhinolaryngol. 2001;60(3):197-204.
- Johansson M, Wentz E, Fernell E, Strömland K, Miller MT. Autistic spectrum disorders in Möbius sequence: a comprehensive study of 25 individuals. Dev Med Child Neurol. 2001;43:338–345.
- Mégarbané A, Ravel A, Mircher C, et al. The 50th anniversary of the discovery of trisomy 21: the past, present, and future of research and treatment of Down syndrome. Genet Med. 2009;11(9):611-6.
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- Evenhuis HM, Van Zanten GA, Brocaar MP, Roerdinkholder WHM. Hearing loss in middle-age persons with Down syndrome. Am J Mental Retard. 1992;97:47-56.
- Gravel JS, Roberts JE, Roush J, et al. Early otitis media with effusion, hearing loss, and auditory processes at school age. Ear Hear. 2006;27:353-368.
- Klein JO, Teele DW, Pelton SI. New concepts in otitis media: results of investigations of the Greater Boston Otitis Media Study Group. Advances in Pediatrics. 1992;39:127-56.
- Updike C, Thornburg JD. Reading skills and auditory processing ability in children with chronic otitis media in early childhood. Ann Otol Rhinol Laryngol. 1992;101(6):530-537.
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Correspondence can be addressed to HR or Vishakha Rawool, PhD, at .
Citation for this article:
Rawool VW. Invisible hearing loss among obvious multiple disabilities, Part 2: The case of the missing hearing aids. Hearing Review. 2010;17(2):32-37.