Audiology & Down Syndrome Abstracts

Int J Pediatr Otorhinolaryngol 61 (3): 199-205 (2001 Dec 1)

Hearing loss in children with Down syndrome

Shott SR, Joseph A, Heithaus D
Department of Pediatric Otolaryngology, Children's Hospital Medical Center, Cincinnati, OH

Objective: Previous studies report a 38-78% incidence of hearing loss in children with Down syndrome (DS). The purpose of this study was to establish more up to date information about hearing loss in children with DS. Methods: A 5-year longitudinal study following the otolaryngologic problems seen in children with DS was initiated in February, 1999 at the Children's Hospital Medical Center in Cincinnati, OH. Aggressive, 'state of the art' treatment, both medical and surgical, was provided to a group of children, (n=48), all of whom were entered into the study at an age under 2 years. Specific interventions and treatments were reviewed in regards to following and treating the children's chronic ear disease. Hearing level results at the end of the first year of the study were evaluated in this publication. This includes both pre-treatment and post-treatment audiologic results. Results: After treatment of easily reversible hearing loss from chronic otitis media, either with medical or surgical treatment with PETs (Pressure Equalization Tubes), 98% of the children had normal hearing levels. Only two children had residual mild hearing losses after treatment interventions. Conclusion: Aggressive, meticulous and compulsive diagnosis and treatment of chronic ear disease in children with DS, started soon after birth, provides significantly improved hearing levels than reported previously.
Int J Pediatr Otorhinolaryngol 49 (2): 143-9 (1999 Aug 5)

Efficacy of tympanostomy tube insertion for otitis media with effusion in children with Down syndrome

Iino Y, Imamura Y, Harigai S, Tanaka Y
Department of Otolaryngology, Teikyo University School of Medicine, Tokyo

OBJECTIVE: Although the insertion of tympanostomy tubes is regarded as an effective treatment for otitis media with effusion in the general population, it remains to be determined whether tympanostomy tube insertion is also effective for otitis media with effusion in children with Down syndrome. The present study was carried out to determine the efficacy of tympanostomy tube insertion in children with Down syndrome. PATIENTS AND METHODS: We studied 28 children (18 males and ten females) with Down syndrome and 28 age-matched control children who underwent tympanostomy tube insertion and were followed up for more than 2 years, up to 7 years of age or older. The children were followed up every month for 6 months after the operation and every 2 months thereafter. The tympanostomy tubes were not removed unless granulation tissue appeared around the ventilation tubes. RESULTS: The cure rate for otitis media with effusion was lower in the children with Down syndrome than in the age-matched control children. Sequelae of otitis media with effusion (atelectatic eardrum, permanent perforation of the eardrum and middle ear cholesteatoma) were significantly often encountered in the former group. The children with Down syndrome had more frequent episodes of otorrhea from the tympanostomy tubes than the control children and antibiotic-resistant-bacteria were frequently isolated. Moreover, improvement in hearing acuity after the placement of tympanostomy tubes was not always achieved in children with Down syndrome. CONCLUSION: The efficacy of the tympanostomy tube insertion for children with Down syndrome was much lower than in control children. We propose that in children with Down syndrome conservative management should be the treatment of first choice and that the insertion of tympanostomy tubes should be indicated only when hearing loss due to middle ear effusion is in a severe degree and when pathological changes of the eardrum, such as adhesion and deep retraction pocket formation, are going to occur.
J Intellect Disabil Res 38 (6): 549-60 (1994 Dec)

Abnormal pinna type and hearing loss correlations in Down's syndrome

Mazzoni DS, Ackley RS, Nash DJ
Colorado State University, Fort Collins 80523

Significant hearing loss and external pinna malformations are two of the most common defects evident in Down's syndrome. The external and middle ears are linked embryologically, both arising from the first and second branchial arches. Evidence indicates that the majority of hearing loss in Down's syndrome is conductive in nature, originating from malformations of the middle ear ossicles and/or the eustachian tube. Recent studies also have indicated that hearing loss is a contributing factor to the IQ and learning deficits that afflict most individuals with Down's syndrome. Therefore, an early, external diagnostic feature for predicting conductive hearing loss would be desirable. In the current study, people with Down's syndrome, people with non-Down's mental retardation and control subjects were examined in a clinical environment for the presence of hearing loss and pinna defects. It was found that 90% of the Down's syndrome population had significant hearing loss, compared to slightly more than 50% in the non-Down's group and no hearing loss in the controls. Also, the majority of hearing loss among individuals with Down's syndrome was conductive, while all hearing loss in the non-Down's group was sensorineural. The Down's syndrome population exhibited nearly 3.5 pinna defects per ear, with malformations of the helix being very evident. The non-Down's population exhibited 2.5 pinna defects per ear, with concha defects being the most common.
Laryngoscope 104 (9): 1065-70 (1994 Sep)

Otological and Habilitative Management of Children with Down Syndrome

Pappas DG; Flexer C; Shackelford L
Pappas Ear Clinic, Birmingham, AL 35233 (205) 251-7169, Fax: (205) 254-3013

It has been well-established that children with Down syndrome experience a high incidence of persistent conductive hearing loss because of their predisposition to stenosis of the external auditory canal (EAC), cerumen impaction, serious otitis media (SOM), and cholesteatoma. Because this hearing problem may be a primary cause of the spoken communication skills in these children being far below their cognitive ability, assertive management is required. The authors present an aggressive multi-discipline treatment model that is instituted during the first year of life in an infant with Down syndrome. Reconstruction of the EAC, amplification technology, and speech/language intervention that emphasizes auditory-verbal (A/V) therapy are discussed. In a pilot study, we compared the language development of six infants with Down syndrome who received aggressive treatment during their first year of life (group A) to six infants who did not (group B). The results showed age-appropriate oral language development for the infants in group A.
Clin Pediatr (Phila) 33 (7): 439-43 (1994 Jul)

Sclerosis of the mastoid air cells as an indicator of undiagnosed otitis media in children with Down's syndrome

Roizen NJ, Martich V, Ben-Ami T, Shalowitz MU, Yousefzadeh DK
University of Chicago Pritzker School of Medicine, La Rabida Children's Hospital and Research Center, IL 60649

We hypothesized that many children with Down's syndrome have undiagnosed otitis media. In a 1989 study of lateral neck radiographs of 22 children with Down's syndrome, we found that 64% had sclerosis of the mastoid air cells, indicating untreated or inadequately treated otitis media. We reviewed the lateral neck radiographs of 53 children with Down's syndrome and interviewed their parents regarding the diagnosis of otitis media. Mastoid air cells were found to be sclerotic in 22 (42%) of study subjects; 32% of these had no known history of otitis media. For the 68% of children diagnosed as having had otitis media, the most common symptoms were fever (61%) and cough or coryza (58%). Sclerosis of the mastoid processes was not associated with a high frequency of otitis media (> 20 episodes) or a higher frequency of hearing loss, but was associated with subsequent myringotomy and insertion of a ventilatory tube (P = .038). Our finding of sclerotic mastoids in 42% of children with Down's syndrome raises the possibility that children with Down's syndrome have unidentified or inadequately treated episodes of otitis media.
J Pediatr 123 (1): S9-12 (1993 Jul)

Hearing loss in children with Down syndrome

Roizen NJ, Wolters C, Nicol T, Blondis TA
Department of Pediatrics, University of Chicago Pritzker School of Medicine, Illinois

Conventional behavioral testing of hearing shows that children with Down syndrome have a high prevalence of hearing deficits. However, most young (< 3.5 years of age) children with Down syndrome are unable to cooperate in this type of testing. We evaluated auditory brain-stem responses of 47 unselected patients 2 months to 3½ years of age. Thirty-four percent had normal hearing, 28% had unilateral loss, and 38% had bilateral loss. Type of loss was conductive in 19 ears, mixed in 14, and sensorineural in 16. Degree of loss was mild in 33 ears, moderate in 13, and severe to profound in 3. Language quotients and degree of hearing loss were compared and an association was not found. Because of the high prevalence of hearing loss in children with Down syndrome, we recommend evaluation of auditory brain-stem responses in the first 6 months of life
Dev Med Child Neurol 35 (6): 511-5 (1993 Jun)

Short-term efficacy of tympanostomy tubes for secretory otitis media in children with Down syndrome

Selikowitz M
Tumbatin Developmental Clinic, Prince of Wales Children's Hospital, Randwick, Australia

Twenty-four children with Down syndrome, aged six to 14 years, were tested with audiometry six to nine weeks after insertion of tympanostomy tubes for bilateral secretory otitis media (SOM). There was no improvement in hearing in 40 per cent of ears, compared with only 9 per cent of ears in 21 age-matched controls with bilateral SOM. Tympanostomy tubes for SOM in children with Down syndrome have a high short-term failure rate, which should be explained to the parents before insertion, and the children should have audiometry tests shortly after the operation. Persistent hearing-loss may require the fitting of hearing aids. Management should involve ensuring that the children are in a situation in which they can hear as well as possible, and making allowances for the hearing impairment.
Pediatr Radiol 19 (5): 311-2 (1989)

Mastoid abnormalities in Down syndrome

Glass RB, Yousefzadeh DK, Roizen NJ
Department of Radiology, Loyola University Medical Center, Maywood, Illinois

Hearing loss and otitis media are commonly associated with Down syndrome. Hypoplasia of the mastoids is seen in many affected children and sclerosis of mastoid bones is not uncommon in Down syndrome. Awareness and early recognition of mastoid abnormality may lead to appropriate and timely therapy, thereby preserving the child's hearing or compensating for hearing loss; factors which are important for learning and maximum development.
Am J Ment Defic 85 (5): 467-72 (1981 Mar)

Hearing loss of Down syndrome adults

Keiser H, Montague J, Wold D, Maune S, Pattison D

Audiological hearing-test data were obtained for 51 Down syndrome adults. Depending upon hearing-loss criteria, from 51 to 74 percent of the subjects had some degree of hearing impairment. Correlation coefficients between these hearing-acuity measurements and receptive hearing vocabulary scores suggest a moderate relationship. These data indicate that Down syndrome adults are prone to a wide variety of hearing difficulties that may reflect a variety of ear pathologies. These data and other studies have shown that both Down syndrome adults and children need frequent hearing screening and, when indicated, otological and/or audiological management. When otological treatment will be of long duration, aural rehabilitation, including prescription amplification, may be indicated as an adjunctive therapeutic procedure.
Clin Pediatr (Phila) 18 (2): 116-8 (1979 Feb)

Hearing loss in Down's syndrome. A treatable handicap more common than generally recognized

Balkany TJ, Downs MP, Jafek BW, Krajicek MJ

Significant hearing loss was found in about two-thirds of a series of 107 individuals with Down's syndrome. Hearing loss of that degree causes retardation of speech, language and intellectual development in normal children. Down's children are presumably affected even more by this sensory deprivation and without correction are prevented from reaching their full potential.