Jackson Roush

Age of Suspicion, Identification, and Intervention for Infants and Young Children with Hearing Loss: A National Study

Objective: This study was designed to seek a nationwide perspective on the status of identification and intervention for infants and young children with hearing loss. Design: Three hundred thirty‐one parents, whose children ranged from infancy to 5 yr of age, returned a mail survey that included respondents from 35 states. Parents were asked to report the approximate age of suspicion, diagnosis, hearing aid fitting, and initiation of early intervention services. Demographic information, risk factors, if known, and reasons for delay were also investigated. Results: Results revealed substantial delays between parental suspicion, audiologic‐medical diagnosis, fitting of acoustic amplification, and initiation of early intervention services; however, the pattern of delay was different for children with known risk factors than it was for those without known risk factors. The median age of identification and intervention was lower than that reported by some previous investigators, although a considerable range was reported for each category. Conclusions: The median age of identification and intervention, although still higher than optimal, may be improving. Further research is needed to identify the many factors that continue to delay the timely management of hearing loss in young children.

Trends in age of identification and intervention in infants with hearing loss.

Objective In 1993, 11 hospitals in the United States were known to screen more than 90% of newborns for hearing loss. By 2000, approximately 1000 hospitals reported screening at least 90% of their babies. This study was designed to identify trends in the age of identification and intervention for infants and young children with hearing loss in light of expanded implementation of newborn hearing screening. Design Parents of children under 6 yr of age with a confirmed hearing loss were surveyed. The survey instrument was designed to investigate three questions: 1) is the age of identification and intervention earlier for babies whose hearing is screened at birth compared with those whose hearing is not screened; 2) when hearing is screened at birth, do ages of dia-gnosis of hearing loss and intervention meet the guide-lines established in 2000, by the Joint Committee on Infant Hearing (Reference Note 1), and 3) what are the barriers to timely identification and intervention? Six hundred fifty-seven parents received the mailing. Results Responses of 151 parents of children with hearing loss, born between 1996 and 2000, were analyzed. Parents from 41 states provided information. Approximately half the children reported on were screened for hearing loss at birth. Age of identification and hearing aid fitting varied substantially based on degree of hearing loss and whether the cause of hearing loss was known or unknown; however, diagnosis and intervention occurred at an earlier age for infants screened at birth. Findings indicate that when hearing is screened at birth, infants with more severe degrees of hearing loss and an unknown cause tend to be identified and receive intervention within the 2000 timelines proposed by the Joint Committee on Infant Hearing. Barriers to timely identification and intervention are discussed. Conclusions Before widespread implementation of newborn hearing screening, age of identification and intervention were consistently reported to exceed 2 yr of age. The results reported here indicate a trend toward earlier identification and hearing aid fitting with the implementation of newborn hearing screening. Although limited to literate and English speaking respondents, the study provides supporting evidence that newborn hearing screening lowers the ages of identification and intervention.

Otitis media, hearing sensitivity, and maternal responsiveness in relation to language during infancy

The relation of otitis media with effusion (OME) and associated hearing loss to language and cognitive skills at 1 year of age was studied to determine whether OME-related hearing loss had a direct association with language and cognitive outcomes at 1 year of age or an indirect association with these outcomes, as mediated by the child-rearing environment. Subjects were 61 black infants attending community-based child care programs. The presence of OME was assessed biweekly from 6 to 12 months of age by otoscopy and tympanometry. Hearing was assessed with visual reinforcement audiometry when children were well and when ill with OME. Language and cognitive skills and the child-rearing environment at home and in child care were examined. The results indicated a modest correlation between hearing loss associated with OME and receptive language. However, the direct association between OME-related hearing loss and all the language and cognitive measures was negligible. Hearing loss had an indirect association with receptive and expressive language, cognitive development, and overall communication as mediated by child-rearing factors. That is, children with more frequent hearing loss tended to have less responsive mothers and home environments, and this association was linked to lower performance on the infant assessments.

Otitis Media, the Caregiving Environment, and Language and Cognitive Outcomes at 2 Years

Objective. To examine the relationship between otitis media with effusion (OME) and associated hearing loss between 6 and 24 months of age and childrens language and cognitive development at 2 years of age. Study Design. A prospective cohort design in which 86 African-American infants who attended group child-care centers were recruited between 6 and 12 months of age. Between 6 and 24 months, assessments included serial ear examinations using otoscopy and tympanometry, serial hearing tests, two ratings of the childrearing environment at home and in child care, and language and cognitive outcomes at 2 years. Results. Children experienced either unilateral or bilateral OME an average of 63% and reduced hearing sensitivity an average of 44% of the time between 6 and 24 months of age. Although proportion of time with OME or with hearing loss was modestly correlated with measures of language and cognitive skills, these relationships were no longer significant when the ratings of the home and child-care environments were also considered. Children with more OME or hearing loss tended to live in less responsive caregiving environments, and these environments were linked to lower performance in expressive language and vocabulary acquisition at 2 years. Conclusions. Both OME and hearing loss were more strongly related to the quality of home and child-care environments than to childrens language and cognitive development. Study results might be explained either by suggesting that children in less responsive caregiving environments experience conditions that make them more likely to experience OME and/or by suggesting that it may be more difficult for caregivers to be responsive and stimulating with children with more OME.

Early Otitis Media with Effusion, Hearing Loss, and Auditory Processes at School Age

Objectives: To examine the effect of conductive hearing loss (HL) secondary to otitis media with effusion (OME) in the first 3 years of life on physiologic, peripheral, and higher-order behavioral auditory measures examined at school age. Methods: Peripheral hearing sensitivity for conventional and extended high-frequency audiometric ranges, physiologic (distortion product otoacoustic emissions, contralateral and ipsilateral acoustic middle ear muscle reflexes), auditory brain stem response (ABR), and higher-order auditory processing measures (masking level difference; Virtual Auditory Localization, Speech Intelligibility Gain; adaptive Pediatric Speech Intelligibility task) were examined at the end of the second grade of elementary school in two cohorts (North Carolina, N = 73, and New York, N = 59). All participants (mean age, 8 years) were followed prospectively in infancy and early childhood (7 to 39 months) for middle ear status and hearing loss (using pneumatic otoscopy/tympanometry and repeated conditioned behavioral audiometric response procedures). Multivariate analyses were conducted to address whether early OME and early conductive HL were related to physiologic, peripheral, and higher-order auditory processes. Results: Early hearing loss and OME were significantly associated with peripheral hearing at school age; extended high-frequency thresholds accounted for the result. Similarly, hearing loss in early life and OME were significantly associated with the acoustic middle ear muscle reflex: The contralateral stimulation condition accounted for the association. Significant associations with both early OME and early HL were also found for the auditory brain stem response measure and were explained by the correlations between early hearing loss and the ABR Wave V latency but not other ABR indices. There were no reliable associations between either early OME or early HL on any other auditory processes evaluated at the end of second grade. Conclusions: Extended high-frequency hearing and brain stem auditory pathway measures in childhood were significantly associated with children’s experiences with OME and hearing loss from 7 to 39 months of age. However, no significant associations were found for psychoacoustic measures of binaural processing or a behavioral adaptive speech-in-noise test at school age.

Otitis media in early childhood in relation to preschool language and school readiness skills among black children

Objective. To examine whether otitis media with effusion (OME) and associated hearing loss (HL) during the first 5 years of life were related to childrens language skills during the preschool years and to school readiness skills at entry to kindergarten. Methods. In a prospective study, the ears of 85 black children primarily from low-income families and recruited from community-based childcare programs were repeatedly examined from 6 months to 5 years of age for the presence of OME and from 6 months to 4 years of age for HL when well and ill with OME. Assessments were made annually of the childrens child-rearing environments at home and in childcare, and childrens language skills between 3 and 5 years of age and readiness skills in literacy and math were evaluated at entry into kindergarten. Results. Children had either bilateral or unilateral OME ∼30.4% and HL 19.6% of the observation time. OME and associated HL were significantly positively correlated with some measures of expressive language at 3 and 4 years of age; however, these direct relationships were no longer significant when the childs gender, socioeconomic status, maternal educational level, and the responsiveness and support of the home and childcare environments were also considered. Further, both OME and HL were moderately correlated with school readiness skills at entry to school, with children having more OME scoring lower in verbal math problems and with children with more HL scoring lower in math and recognizing incomplete words. These associations continued to remain significant even after partialing out the child and family background factors. Conclusions. There was not a significant relationship between childrens early OME history or HL and language skills during the preschool years. However, children with more frequent OME had lower scores on school readiness measures. These associations were moderate in degree, however, and the home environment was more strongly related to academic outcomes than was OME or HL. These results should be interpreted cautiously when generalizing to other populations.

Family-Centered Early Intervention: The Perceptions of Professionals

This paper, based on a nationwide survey, examines the attitudes and opinions of professionals regarding parent-professional involvement in the planning and implementation of goals and services for hearing-impaired infants and toddlers and their families. The implications of the findings are discussed as they pertain to PL 99-457, the new federal mandate for services to young children with special needs and their families.

Methods for assessing neurodevelopment in lysosomal storage diseases and related disorders: a multidisciplinary perspective

Lysosomal storage diseases and related disorders (LSRDs) are a heterogeneous group of rare diseases caused by genetic mutations that result in deficiencies of specific lysosomal enzymes. Some of these enzymes are necessary for normal development of the central and peripheral nervous systems. Because of the heterogeneity in clinical presentation and complexity of these disorders, evaluation of disease progression poses unique challenges. In recent years, recombinant enzyme replacement therapy and haematopoietic stem cell transplantation have been developed to treat some of these diseases. With the development of specific therapies and screening programmes, there is a need to systematically follow the natural course and effects of treatment in these disorders with standardized and validated tools. This review describes the limitations of currently available neurobehavioural tools in longitudinally tracking disease outcomes in patients with neurodegenerative LSRDs. A multidisciplinary team reviewed over 750 evaluations in 274 patients. These patients were found to have neurological, sensory and somatic problems that considerably influence the results of neurobehavioural testing.

Prospective surveillance for otitis media with effusion among black infants in group child care

OBJECTIVE To document the prevalence of otitis media with effusion (OME) in 102 black children observed prospectively between 6 and 24 months of age. METHODS Study children attended nine different center-based child care facilities. Middle ear status was assessed by pneumatic otoscopy and tympanometry every 2 weeks. RESULTS All children, except one, had OME during the period of observation. The proportion of child-examinations revealing bilateral OME ranged from 76% between 6 and 12 months of age to 30% between 21 and 24 months of age. Effusions were considered purulent in only 13% of examinations revealing middle ear fluid. The mean incidence of purulent OME was 2.13 episodes per child per year. Sixty-six children had at least 4 months of continuous bilateral OME during the period of observation; 57 were followed without placement of tympanostomy tubes. Bilateral OME had resolved before the second birthday in 95% of these children, and within 3 months of achieving the 4-month criterion in 50% of subjects. CONCLUSIONS Persistent bilateral OME occurs commonly between 6 and 18 months of age in infants who enter group child care during the first year of life. In this study, spontaneous resolution of bilateral effusion by 2 years of age was typical.

Application of an implantable bone conduction hearing device to patients with unilateral sensorineural hearing loss

This investigation, comprised of five studies, was undertaken to determine if individuals with newly acquired profound unilateral hearing losses would benefit from an implantable bone‐conduction hearing device. The bone conductor was implanted on the side of the deaf ear at the time of translabyrinthine acoustic tumor resection. Two areas greatly affected by unilateral hearing loss, speech recognition in noise and sound localization, were examined. No improvement in aided performance could be documented in either area.