Risk of hearing impairment in pediatric liver transplant recipients: a single center study

As survival rates following liver transplantation have increased, health care providers must assess the impact of transplantation on dimensions other than traditional medical measures. Hearing impairment may adversely impact social, emotional, cognitive, academic, and speech and language development. We hypothesized that children who undergo liver transplantation are at risk for hearing impairment due to exposure to ototoxic drugs. We conducted a review of 74 children who had undergone liver transplantation between December 1996 and September 2000 at Cincinnati Children's Hospital Medical Center. Hearing was assessed at discharge by an audiologist using age and developmentally appropriate techniques. The principal outcome measure was sensorineural hearing impairment. Independent variables were age at transplantation, United Network for Organ Sharing (UNOS) status at transplantation, primary diagnosis, post-transplant length of hospital stay, days of treatment with aminoglycosides, and days of treatment with loop diuretics. Eleven of 74 children (15%) had sensorineural hearing loss, of whom four had severe to profound hearing loss. Multivariate analyses showed that the adjusted relative risk for hearing loss in patients with hepatoblastoma was 66 and that there was a 5% increase risk for hearing loss for each additional day of hospitalization. Age at transplantation, UNOS status, and days of treatment with loop diuretics or aminoglycosides did not achieve significance in the model. Sensorineural hearing impairment occurs in a subset of pediatric patients following liver transplantation. Patients with hepatoblastoma or those who experience prolonged hospitalization after transplantation are at increased risk. Our observations are of particular importance for pediatric liver transplant recipients since the median age at transplantation is 12-18 months, a critical period for language acquisition.     

Sensorineural hearing loss in sporadic congenital hypothyroidism.

Hearing acuity was assessed in 45 children with sporadic congenital hypothyroidism during adequate long-term treatment. Otoscopy was performed in each and additional tympanometry in some of them. Secretory otitis media was found in 6 and was treated medically or by inserting grommets in the eardrum. In these children, hearing acuity was assessed after the otitis had been cured. Hearing acuity was measured either by conventional monoaural pure-tone audiometry (125-8000 Hz) or by binaural free field testing depending on the child''s age (above and below 4 years respectively). Hearing was normal in 36 (80%) children. In the remaining 9, sensorineural hearing loss to some degree was detected affecting the higher frequencies in particular. Perceptive deafness required the use of a hearing aid in 4 children. No relationship could be found between hearing acuity and chronological age or bone age at diagnosis of congenital hypothyroidism or type of hypothyroidism. Sensorineural hearing loss is common in children with congenital hypothyroidism and should be searched for carefully and systematically to avoid difficulties related to speech and language development.     

Childhood hearing impairment

Hearing impairment is one of the most common disabilities in the UK and globally. Worldwide, 60% of cases are thought to be preventable and fewer than 10% globally have access to the hearing support they need. Childhood hearing impairment can impact on development, speech, language, auditory processing, listening skills, behaviour, self-esteem, quality of life and learning. Our responsibility as clinicians is to prevent developmental impairment in these areas and ensure children with a hearing impairment have the same life chances as children who hear well. There are 45,000 children in the UK diagnosed with a hearing impairment. Hearing impairment can be isolated or part of a more complex presentation. Visual, neurodevelopmental, balance and communication difficulties as well as safeguarding issues are more common in children with hearing impairment.     

Contributions of phonological memory, language comprehension and hearing to the expressive language of adolescents and young adults with Down syndrome

BACKGROUND: Expressive language constitutes a major challenge to the development of individuals with Down syndrome. This paper investigates the relationships between expressive language abilities, language comprehension and the deficits in verbal short-term memory and hearing which are also associated with the syndrome. METHODS: Tests of nonverbal ability, expressive language, verbal short-term memory, visuo-spatial memory, language comprehension and hearing were administered. RESULTS: Phonological memory, measured by nonword repetition, was significantly correlated with expressive language abilities measured by MLU and sentence recall. Adjusting for word repetition skills did not reduce this correlation, suggesting that the relationship did not depend on the fact that both tests required spoken output. Hearing did not contribute significantly to expressive language scores of participants who provided an intelligible narrative. However, level of hearing loss as well as other language and memory measures did differentiate these participants from those who were unable to produce an intelligible narrative. CONCLUSION: Phonological memory was closely associated with the expressive language abilities of individuals with Down syndrome. Hearing loss appeared to be less closely related except that individuals with uncorrected mild to moderate hearing loss had difficulty with the narrative task. Further research is necessary to establish the nature of these relationships.     

Sensorineural hearing loss

Hearing losses in children are disabling and frequently unrecognized. Children with persistent uncorrected hearing impairment are at high risk for speech and language delays, behavioural problems, and socialization and learning difficulties. Although most hearing impairment in childhood is conductive in type. about 1 in 1000 Australian children have significant sensorineural hearing losses (SNHL) of mild to profound degree.     

Impact of recurrent otitis media on middle ear function, hearing, and language

Whether recurrent otitis media in infants and young children is followed by delayed language development was addressed by following 210 normal subjects longitudinally through the first 2 years of life with pneumatic otoscopy and tympanometry performed at every physician encounter. Otitis accounted for 26% of the medical visits. One hundred fifty-six of these children had speech and hearing evaluation at 2 years of age. Thirty percent of the children with recurrent otitis media had a mild or moderate hearing loss. However, after multiple speech and language tests, we could not identify a delay in language acquisition in the otitis-prone children. At 3 to 4 years old, 36 children, including nine with a hearing loss at 2 years of age, were retested; all nine had normal hearing. Recurrent otitis media induced a temporary decrease in hearing sensitivity demonstrable at 2 years of age, which appeared to resolve as the children matured and which was not associated with delay in language acquisition.     

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

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.     

Genetic causes of hearing loss in children

Hearing loss affects various related aspects of a childs overall development. It is the cause of impaired development of speech and language, as well as poor performance in school and loss of cognitive potential. Hearing loss may also hinder and impair the childs social and emotional relationships. Genetic causes of congenital deafness in children are rare, nevertheless they are important, since early detection and effective treatment of hearing loss is one of the most urgent duties of any physician who cares for small children. Diagnosis is equally important as well, in terms of genetic counseling.     

Theory-of-mind development in oral deaf children with cochlear implants or conventional hearing aids

BACKGROUND: In the context of the established finding that theory-of-mind (ToM) growth is seriously delayed in late-signing deaf children, and some evidence of equivalent delays in those learning speech with conventional hearing aids, this study's novel contribution was to explore ToM development in deaf children with cochlear implants. Implants can substantially boost auditory acuity and rates of language growth. Despite the implant, there are often problems socialising with hearing peers and some language difficulties, lending special theoretical interest to the present comparative design. METHODS: A total of 52 children aged 4 to 12 years took a battery of false belief tests of ToM. There were 26 oral deaf children, half with implants and half with hearing aids, evenly divided between oral-only versus sign-plus-oral schools. Comparison groups of age-matched high-functioning children with autism and younger hearing children were also included. RESULTS: No significant ToM differences emerged between deaf children with implants and those with hearing aids, nor between those in oral-only versus sign-plus-oral schools. Nor did the deaf children perform any better on the ToM tasks than their age peers with autism. Hearing preschoolers scored significantly higher than all other groups. For the deaf and the autistic children, as well as the preschoolers, rate of language development and verbal maturity significantly predicted variability in ToM, over and above chronological age. CONCLUSIONS: The finding that deaf children with cochlear implants are as delayed in ToM development as children with autism and their deaf peers with hearing aids or late sign language highlights the likely significance of peer interaction and early fluent communication with peers and family, whether in sign or in speech, in order to optimally facilitate the growth of social cognition and language.     

Otoacoustic emissions as a screening test for hearing impairment in children.

Transient evoked otoacoustic emissions (TEOAEs) are low amplitude sound waves produced by the healthy cochlea. They can be recorded with a microphone in the external ear. TEOAEs are abolished by hearing losses of 30 dB or more. The feasibility of using TEOAEs as a screening test for hearing loss in children was studied. TEOAE recordings were attempted in 56 children attending an audiology clinic. Recordings were possible from both ears in 52 children; of these 104 ears, 32 had hearing deficits of 30 dB or more. Hearing status was compared with the results of six TEOAE screening criteria. All criteria had a sensitivity of 1.00. Four standard TEOAE criteria yielded specificities of 0.46-0.58. Two new criteria derived from analysis of limited frequencies from the TEOAE waveform gave specificities of 0.76 and 0.82. It can be concluded that, when appropriate pass/fail criteria are employed, TEOAEs are a feasible screening test in children.     

Otoacoustic emissions as a screening test for hearing impairment in children

Transient evoked otoacoustic emissions (TEOAEs) are low amplitude sound waves produced by the healthy cochlea. They can be recorded with a microphone in the external ear. TEOAEs are abolished by hearing losses of 30 dB or more. The feasibility of using TEOAEs as a screening test for hearing loss in children was studied. TEOAE recordings were attempted in 56 children attending an audiology clinic. Recordings were possible from both ears in 52 children; of these 104 ears, 32 had hearing deficits of 30 dB or more. Hearing status was compared with the results of six TEOAE screening criteria. All criteria had a sensitivity of 1.00. Four standard TEOAE criteria yielded specificities of 0.46-0.58. Two new criteria derived from analysis of limited frequencies from the TEOAE waveform gave specificities of 0.76 and 0.82. It can be concluded that, when appropriate pass/fail criteria are employed, TEOAEs are a feasible screening test in children.     

Hearing impairment in children: early diagnosis is essential]

Diagnosis of hearing impairment is possible during the first days of life. Hearing tests are noninvasive and should not be delayed when hearing loss is suspected. Among children's hearing impairments, conductive hearing loss is the most frequent; it is generally acquired and reversible. At the opposite pole, sensorineural hearing loss has more severe consequences because it is irreversible and often present from birth. Early diagnosis and treatment are necessary in all cases to prevent speech delay. In cases with sensorineural hearing impairment, hearing aid fitting, or even cochlear implantation, and intensive speech therapy will help deaf children learn speech, with the view of optimal social and professional integration.     

Hearing and verbal-cognitive abilities in high-risk preterm infants prone to otitis media with effusion

Preterm infants with a history of perinatal complications are at risk for language learning difficulties, and are more likely than full-term infants to show recurrent otitis media. The present study looks at the association between these risk outcomes in the preschool period. Twenty-three otitis-prone preterm children (referred to as cases) were compared with 20 non-otitis-prone children with similar perinatal and demographic characteristics (controls). Hearing thresholds were depressed for the cases in conjunction with abnormal tympanograms, and hearing was significantly poorer than for controls. Some language and verbal cognitive abilities were significantly poorer for the cases. The findings suggest the importance of medical intervention, audiometric assessment, and speech and language follow-up for high-risk premature infants prone to otitis media with effusion.     

Hearing assessment in infants and children: recommendations beyond neonatal screening

Congenital or acquired hearing loss in infants and children has been linked with lifelong deficits in speech and language acquisition, poor academic performance, personal-social maladjustments, and emotional difficulties. Identification of hearing loss through neonatal hearing screening as well as objective hearing screening of all infants and children can prevent or reduce many of these adverse consequences. This report outlines the risk indicators for hearing loss, provides guidance for when and how to assess hearing loss, and addresses hearing referral resources for children of all ages.     

Intellectual, behavioral, and linguistic characteristics of three children with 18p- syndrome

Developmental characteristics of three girls with 18p- syndrome are described, with detailed history and results of psychological and speech/language evaluations completed at various ages. Results suggest that some children with 18p- syndrome have average abilities in selected areas, despite previous reports that these children have mild to severe mental retardation. Two of the three girls had more severe deficits in linguistic, as opposed to nonlinguistic, abilities. All had similar difficulties with speech articulation. It is suggested that children with speech/language delays who have physical features associated with this syndrome have chromosome analysis performed. It is also recommended that children identified as having 18p- syndrome be referred for comprehensive developmental assessments and remedial special education programming before a specific prognosis is determined.     

Phonological processing, language, and literacy: a comparison of children with mild-to-moderate sensorineural hearing loss and those with specific language impairment

Phonological skills, language ability, and literacy scores were compared for four groups: 19 children with mild-to-moderate sensorineural hearing loss (SNH), 20 children with specific language impairment (SLI), 20 controls matched on chronological age to the SNH group (CA), and 15 controls matched on receptive vocabulary level to a subset of the SLI group (CB). In common with the SLI group, mean scores of children with mild-to-moderate hearing loss were significantly poorer on tests of phonological short-term memory, phonological discrimination, and phonological awareness than CA controls. No differences between group means were observed in SNH and CA control groups on vocabulary, digit and sentence recall, sentence comprehension, and literacy scores. However, there was considerable individual variation within the SNH group. Nearly 50% of the SNH group showed phonological impairment associated with poorer expressive and receptive vocabulary and higher hearing thresholds than remaining children without phonological impairment. Nonword repetition deficits were observed in SNH subgroups with and without phonological impairment and were of a similar magnitude to those observed in children with SLI. Indeed, poorer repetition in children with SLI could only be differentiated from children with SNH on phonologically complex nonwords. Overall, findings suggested major problems in nonword repetition and phonological impairment occurred without clinically significant deficits in wider language and literacy abilities in children with mild-to-moderate sensorineural hearing loss. Implications for theories of SLI are discussed.     

Evaluation and Management of Hearing Loss in Survivors of Childhood and Adolescent Cancers: A Report From the Children's Oncology Group

Hearing loss (HL) is common in childhood cancer survivors exposed to platinum chemotherapy and/or cranial radiation and can severely impact quality of life. Early detection and appropriate management can mitigate academic, speech, language, social, and psychological morbidity resulting from hearing deficits. This review is targeted as a resource for providers involved in aftercare of childhood cancers. The goal is to promote early identification of survivors at‐risk for HL, appropriate evaluation and interpretation of diagnostic tests, timely referral to an audiologist when indicated, and to increase knowledge of current therapeutic options.     

Developmental outcomes of children with hearing loss born in Colorado hospitals with and without universal newborn hearing screening programs

The developmental outcomes of children born in hospitals with universal newborn hearing screening programs were compared with children born in hospitals without universal newborn hearing screening programs. Eight-four percent of children born in screening hospitals were early-identified with hearing loss prior to 6 months of age as compared to 8% of the children in the non-screen group. The participants in the screen group had an average language quotient of 82 while the participants in the non-screen group had an average language quotient of 62. Children in the screen group had better receptive and expressive language quotients, more different consonants in the spontaneous phonetic repertoire, better speech intelligibility, and larger expressive vocabulary inventories. Odds risk ratio estimates indicate that 80% of the children with cognitive quotients 80 or greater or four out of five children had language quotients within the normal range, 80 or greater, when they were in the screen group.     

Audit from preschool developmental surveillance of vision,hearing, and language referrals.

Referrals from preschool medical examinations were followed up for two years to assess attendance rate, waiting time for appointment, appropriateness of the referral, the diagnosis and management of the condition. Altogether 184 children were referrals for ophthalmology, 285 for audiology, and 195 for speech therapy. The median waiting time for an appointment was 46 days in ophthalmology, 175 days in audiology, and 83 days in speech therapy. The poorest attendance rate was identified in speech therapy (75%). Approximately 60% of examined children had a justified referral to ophthalmology and 20% had a clear defect. Over half the children in audiology (55%) had an altered impedance or hearing impairment. Of those with a hearing problem kept under review only half improved spontaneously. In speech therapy 80% of those assessed had a language problem. Many health problems were detected for which parents were unaware or did not use the service. Parental awareness alone will not uncover the sizable level of lingual and sensorial problems in inner city areas. This audit identified specific deficiencies in the provision of services and a number of organisational changes are suggested to improve their effectiveness.     

Auditory interhemispheric transfer deficits, hearing difficulties, and brain magnetic resonance imaging abnormalities in children with congenital aniridia due to PAX6 mutations

OBJECTIVE: To assess auditory processing, hearing difficulties, and brain magnetic resonance (MR) imaging abnormalities in children with panocular developmental aniridia due to PAX6 mutations. DESIGN: Case-control study. SETTING: Great Ormond Street Hospital and Institute of Child Health. PARTICIPANTS: Eleven case subjects with PAX6 mutations and 11 age-matched and sex-matched healthy control subjects. INTERVENTIONS: All subjects completed a structured hearing questionnaire, baseline audiometry, and central auditory tests (dichotic speech tests, frequency and duration pattern tests, and gaps-in-noise test). Case subjects underwent brain MR imaging with volumetry, and the results were compared with those of age-matched and sex-matched healthy control subjects randomly selected from the Radiology and Physics Unit database. MAIN OUTCOME MEASURES: Brain MR imaging, central auditory test results, and questionnaire scores. RESULTS: The corpus callosum area was significantly smaller on brain volumetry in the cases compared with the controls. The anterior commissure was small in 7 cases and was normal in 3 cases on visual inspection of brain MR images (conducted in 10 of 11 cases). Audiograms showed no abnormalities in any of the children. Central auditory test results were normal in all the controls and were abnormal in all the cases except for 1 case with a pattern of abnormalities consistent with reduced auditory interhemispheric transfer. The cases had greater difficulty localizing sound and understanding speech in noise than the controls. CONCLUSIONS: Despite normal audiograms, children with PAX6 mutations may experience auditory interhemispheric transfer deficits and have difficulty localizing sound and understanding speech in noise. In view of their additional visual difficulties, thorough audiological evaluation of these children is indicated to initiate appropriate management.