Mild impairment of neuro-otological function in early treated congenital hypothyroidism

Pure tone audiometry, tympanometry, acoustic stapedial reflex thresholds (ASRTs), and auditory evoked brain stem responses (AEBRs) were carried out in 38 children with early treated congenital hypothyroidism aged 10-12 years, together with tests of vestibular function (electronystagraphy, rotational, and caloric tests). Sensorineural hearing loss with thresholds of greater than 15 dB was detected in 18 children (10 at 8 kHz only); only two children had more than 40 dB hearing loss, each in one ear. Raised ASRTs were found in eight children and two children had abnormal AEBRs. Of the 29 children tested, 12 had an abnormality of vestibular function. Although not significant at the 5% level, there was a tendency for the abnormalities to be more prevalent and severe in the children with more severe hypothyroidism, as judged by pretreatment plasma thyroxine. It is concluded that (i) mild abnormality of hearing is still common in children with congenital hypothyroidism despite early treatment but this is much less severe than that found before neonatal screening and (ii) mild abnormalities of vestibular function may be common in early treated congenital hypothyroidism.     

Mild impairment of neuro-otological function in early treated congenital hypothyroidism.

Pure tone audiometry, tympanometry, acoustic stapedial reflex thresholds (ASRTs), and auditory evoked brain stem responses (AEBRs) were carried out in 38 children with early treated congenital hypothyroidism aged 10-12 years, together with tests of vestibular function (electronystagraphy, rotational, and caloric tests). Sensorineural hearing loss with thresholds of greater than 15 dB was detected in 18 children (10 at 8 kHz only); only two children had more than 40 dB hearing loss, each in one ear. Raised ASRTs were found in eight children and two children had abnormal AEBRs. Of the 29 children tested, 12 had an abnormality of vestibular function. Although not significant at the 5% level, there was a tendency for the abnormalities to be more prevalent and severe in the children with more severe hypothyroidism, as judged by pretreatment plasma thyroxine. It is concluded that (i) mild abnormality of hearing is still common in children with congenital hypothyroidism despite early treatment but this is much less severe than that found before neonatal screening and (ii) mild abnormalities of vestibular function may be common in early treated congenital hypothyroidism.     

上海地区2378名新生儿听力筛查分析

为探索如何开展新生儿听力筛查工作，研究正常新生儿和新生儿监护室（ＮＩＣＵ）重症监护儿的听力障碍发病情况及听力障碍高危因素。对上海地区２３７８名新生儿进行听力筛查。正常新生儿组和ＮＩＣＵ重症监护儿组分别为１９７９人和３９９人。分别做听觉行为测定、信访和电话访问、脑干听觉诱发电位（ＡＢＲ）检查。结果：正常新生儿组和ＮＩＣＵ组的听力障碍发生率分别为０．３０％和２２．６０％，其中重度以上听力障碍分别为０．０５％和１．００％。提示高胆红素血症、庆大霉素注射史、卡那霉素注射史、低出生体重和化脓性脑膜炎可能是听力障碍的高危因素。     

Persistent fetal circulation. Neurodevelopmental outcome

Forty children who had persistent fetal circulation (PFC) were followed up for one to four years. At the most recent examination, 16 (40%) were normal, eight (20%) had neurosensory hearing loss, three (7.5%) were profoundly impaired, six (15.0%) had suspect or abnormal results of neurologic examinations exclusive of hearing loss, three (7.5%) had speech impairment and normal hearing, and one had a delay in motor development. The remaining three had clinically suspect speech. Neurosensory hearing loss correlated only with having a mother with insulin-dependent diabetes, hand-to-mouth facility on the Brazelton scale, and eight-month neurologic status. Earlier diagnosis of hearing loss may be facilitated by testing of neonatal auditory behavioral responses, neurologic status at eight months, and by attention to parental concern about abnormal speech development. Neonatal and infancy auditory screening are recommended in children who have PFC in the neonatal period.     

Hearing,speech, and language in survivors of severe perinatal asphyxia.

Hearing, speech, and language were studied in 26 children who survived severe perinatal asphyxia. The results of hearing tests showed that most children had a favourable outcome. Only 1 child had sensorineural deafness. Hearing loss in 6 others was due to middle-ear disease which resolved after treatment, and on retesting was found to be normal. The study also showed that neither gentamicin treatment nor incubator noise seemed to affect hearing. The results of speech and language assessment were less encouraging and about one-third of the children without serious mental or physical handicap had deficits in speech and language. It is suggested that the quality of life in such children could be improved if these deficits were detected early and adequately treated.     

Treatment of symptomatic congenital cytomegalovirus infection with intravenous ganciclovir followed by long-term oral valganciclovir

Congenital cytomegalovirus infection is the most common cause of nonhereditary sensorineural hearing loss and an important cause of psychomotor retardation. Earlier studies showed that 6-weeks’ treatment with ganciclovir, starting in the neonatal period, prevented hearing deterioration at 6 months, but in one-fifth of the infants, the effect was not sustained at age 12 months. The aim of this preliminary retrospective study was to investigate the effectiveness and safety of long-term treatment with ganciclovir/valganciclovir for congenital cytomegalovirus infection. Twenty-three infants with culture-proven symptomatic congenital cytomegalovirus infection were treated with ganciclovir for 6 weeks followed by oral valganciclovir to age 12 months. Audiometry was performed at least three times in the first year, in addition to physical examination including neurological and developmental assessment. At age ≥1 year, hearing was normal in 76% of affected ears compared to baseline (54%). In 25 normal ears at birth no deterioration was found at ≥1 year. These results were significantly better than reported in a historical control group of similar infants treated for 6 weeks only (P= 0.001). Viral load monitoring demonstrated sustained virological response. Four of the children (18%) had mental retardation. The main side effect of treatment was transient neutropenia. In conclusion, prolonged therapy of symptomatic congenital CMV infection with intravenous ganciclovir followed by oral valganciclovir is safe, and it appears to lead to a better auditory outcome than short-term treatment.     

Late Otological Sequelae of Tuberculous Meningitis

An otological follow-up study in 1956 and 1959—62 of 103 children treated for and cured of tuberculous meningitis in 1949—54 is presented. Impaired hearing was observed in 63 per cent, severe loss of hearing in 15 per cent and total deafness in 7 per cent. In 36 per cent there was a hearing loss of more than 10 dB in the speech range (500—2,000 cps). The initial and the follow-up results of otological investigations were in close accordance with each other — only in 3 cases was there some improvement and in 11 some deterioration of hearing. The cases with hearing loss were evenly distributed between the different age groups excepting the youngest one — 0—12 months — which displayed a considerably lower frequency of impaired hearing as a whole but a much higher frequency of severe hearing loss. There was no correlation between hearing sequelae and the duration of meningeal symptoms prior to hospitalization or the severity of the disease on admission. There was, however, a correlation between the frequency of hearing sequelae and the daily and total doses of streptomycin given: the frequency of hearing loss was 49 per cent (severe hearing loss 22 per cent) when 50 mg/kg daily was used compared with an incidence of 15 per cent (severe hearing loss in 4 per cent) when 10—20 mg/kg of streptomycin was used intramuscularly and 1—3 mg/kg intrathecally daily.
No correlation was found between loss of hearing and neurological, mental, eye or EEG disturbances.
Disturbances of vestibular function were found in 54 per cent altogether and they were severe in 31 per cent.
Five of the 15 children with severe hearing loss used hearing aids with considerable success, 3 got along without them in ordinary schools and 10 children attended schools for the deaf.     

Hearing and neurodevelopmental outcome in survivors of persistent pulmonary hypertension of the newborn.

Thirty-four infants who had a diagnosis of severe persistent pulmonary hypertension of the newborn at birth (alveolar-arterial oxygen difference greater than 600) were treated without paralysis or hyperventilation to induce alkalosis. All survived. Twenty-seven of these 34 eligible infants (79%) underwent neurologic, intelligence, and audiologic testing between 10 months and 6 years of age. Children who were younger than 1 year of age at the initial hearing test were retested after they reached 2 years of age. The average IQ was within the normal range (mean = 96.23). None had sensorineural hearing loss. Severe neurologic abnormalities were seen in 4 children, 3 of whom had been severely asphyxiated at birth (determined by biochemical criteria). Mild neurologic abnormalities were observed in 5 children. Two infants had bronchopulmonary dysplasia because they required supplemental oxygen for 29 and 66 days, respectively, and had abnormal chest roentgenograms; 1 patient takes intermittent doses of albuterol (Ventolin) and neither currently requires supplemental oxygen. This study of 27 infants with severe persistent pulmonary hypertension of the newborn suggests that conservative management without induced alkalosis or respiratory paralysis is accompanied by no sensorineural hearing loss and a good neurologic outcome.     

What is the role of the distraction test of hearing?

The use of the distraction test as a screen for hearing loss in infancy was reviewed in the Bristol and District Health Authority. Inaccuracies in data recording were found which challenged the screening and referral rates produced by the National Child Health Computer. Representative samples of children failing the distraction test and children needing hearing aids were followed up through the screening process. Of 130 children failing the test, only 46 (35%) had moderate conductive hearing loss and none had a severe loss. Of 34 children with sensorineural hearing loss (SNHL) requiring hearing aids, seven (21%) were identified by the test and five (15%) presented late after having been screened. The distraction test as now used in Bristol identifies large numbers of children with minor hearing loss. The referral of these children to the secondary services leads to delays for those children with more significant loss. The test is no longer the main method for identifying children with SNHL.     

What is the role of the distraction test of hearing?

The use of the distraction test as a screen for hearing loss in infancy was reviewed in the Bristol and District Health Authority. Inaccuracies in data recording were found which challenged the screening and referral rates produced by the National Child Health Computer. Representative samples of children failing the distraction test and children needing hearing aids were followed up through the screening process. Of 130 children failing the test, only 46 (35%) had moderate conductive hearing loss and none had a severe loss. Of 34 children with sensorineural hearing loss (SNHL) requiring hearing aids, seven (21%) were identified by the test and five (15%) presented late after having been screened. The distraction test as now used in Bristol identifies large numbers of children with minor hearing loss. The referral of these children to the secondary services leads to delays for those children with more significant loss. The test is no longer the main method for identifying children with SNHL.     

Early nasal continuous positive airway pressure in a cohort of the smallest infants in Denmark: neurodevelopmental outcome at five years of age

AIM: To evaluate neurodevelopmental outcome at age 5 y of age in a cohort of preterm children treated mainly with nasal continuous positive airway pressure (CPAP) in the neonatal period. METHODS: A national prospective observational study was conducted in Denmark which included all 269 surviving children with a birthweight below 1000 g or a gestational age below 28 wk born in Denmark in between 1994 and 1995. A total of 164 children (61%) had been treated with nasal CPAP only in the neonatal period. A reference group (n = 76) of term children was studied in parallel. RESULTS: Of the 269 surviving children, 252 (94%) were examined. Twenty-four children (10%) had cerebral palsy, and three children were blind. No case of hearing impairment was detected. Nineteen percent of the index children had an IQ score <-- 2 SD and 42% had an IQ score <-- 1 SD of the mean score of the reference group. CONCLUSIONS: The intellectual development of children in this cohort treated with early nasal CPAP did not suggest a higher proportion of adverse effects on the brain compared to the published follow-up studies of preterm children treated with mechanical ventilation. In our population-based cohort, however, the survival rate for infants below 25 wk of gestation was relatively low and this may indicate a limit for the use of early nasal CPAP.     

Auditory profile in children recovering from bacterial meningitis

In the present study BERA profile of 30 post-meningitic children was compared with 15 normal children of the same age and it was observed that 36.6% children in the age range of 6 months to 36 months were found to have varying degree of sensorineural deafness. Severe bilateral sensorineural hearing loss (>80 dB) was observed in 6.6% children and moderate (40–80 dB) hearing loss in 30% of children. Abnormalities were bilateral in both the samples of children with severe hearing loss (>80 dB) whereas among 9 children who had moderate hearing loss abnormalities were bilateral in one patient and unilateral in the remaining 8 children. A relationship between higher incidence of sensorineural deafness and younger age of children, and occurence of seizures during meningitis were noted. But no relationship was observed with either sex, hydrocephalus, subdural effusion or with low CSF sugar and high CSF proteins.     

School performance of survivors of neonatal encephalopathy associated with birth asphyxia at term

School performance testing was completed at 8 years of age on 145 children who had had neonatal encephalopathy associated with birth asphyxia as term infants and on a comparison peer group of 155 children. The prospectively identified clinical categories of encephalopathy for the neonates were 56 mild (hyperalertness, hyperexcitability), 84 moderate (lethargy, hypotonia, suppressed primitive reflexes), and 5 severe (stupor, flaccidity, absent primitive reflexes). The mortality rate to 8 years of age was 13%. The incidence of impairment, which included cerebral palsy, blindness, cognitive delay, convulsive disorder, and severe hearing loss, was 16% among those assessed at 8 years (75% of survivors). Intellectual, visual-motor integration, and receptive vocabulary scores, as well as reading, spelling, and arithmetic grade levels for those with moderate or severe encephalopathy, were significantly below (p less than 0.01) those in the mild encephalopathy or peer comparison groups. Predictors of reading performance for the study group included category of encephalopathy, birth weight for gestational age, native language, and mother's educational level (multiple R = 0.58). Nonimpaired survivors of moderate encephalopathy were more likely to be more than one grade level delayed than were children from the peer group (reading 35% vs 15%, spelling 18% vs 8%, arithmetic 20% vs 12%, p less than 0.01). Thus children who had moderate and severe neonatal encephalopathy are at risk for physical and mental impairment and reduced school performance. Children with mild encephalopathy had school performance scores similar to those of their peers.     

Prevalence, aetiology, and care of severe and profound hearing loss

Severe and profound hearing loss (> 70 dB) were analysed in a retrospective study of 226 children, born between 1976 and 1985, and recruited from three French administrative departments. The prevalence was 0.54 per 1000 children under 9 years old, with no decrease over the study period. A hereditary origin was identified in 20.8% of cases and an infectious origin in 11.5%. Perinatal risk factors were present in 11.5%, while the aetiology was undetermined in more than half the cases. In 85.8% of the children there was no other severe impairment. Marked learning difficulties were observed: 36% of the children were two years behind their age group and 28% were more than two years behind. The age of initial care decreased over the study period but is still too advanced. Systematic neonatal screening would enable earlier care, which should limit the social and educational impact of hearing loss.     

Early hearing detection and intervention in children with prelingual deafness, effects on language development

AIM: The purpose of this study was to assess, the cognitive and receptive language abilities in children with prelingual hearing impairment, in relation to the age of enrollment in the intervention program and examine the related variables. METHODS: Seventy children with congenital prelingual deafness were divided into 2 groups based on their age at the start of the intervention program: 17 children enrolled between 0-12 months of age, 53 children enrolled after the age of 12 months. The age of intervention is defined as the identification and confirmation of hearing loss, adaptation of hearing aids, and enrollment in the program of special education at the Orthophonological Institute of Rome. Assessments were carried out at 5 years of age. The receptive language abilities were measured using the Peabody picture vocabulary test (PPVT), while the cognitive abilities used the Raven standard progressive matrices test. The material was administered by staff skilled in assessing children with hearing loss. The assessment of language score tests (PPVT and Raven progressive matrix test) of samples of children with hearing loss was compared with normal standardized scores of hearing peers at 5 years of age. Mean group differences were compared using t-tests. The results were considered statistically significant for a P-value less than or equal to 0.05. RESULTS: A progressive decline in the mean PPVT score with increasing ages of enrollment was present. The mean receptive language score of the children enrolled within the first 12 months was significantly better (P<0.001) compared to those over 13 months. The nonverbal IQ, determined by Raven's standard progressive matrices, showed no statistically significant differences in IQ scores (P = 0.083) between children with early and late age of enrollment. Our data revealed that language abilities are significantly affected by the degree of hearing loss (P<0.001 Children with very severe hearing loss, find it more difficult to achieve adequate language abilities than children with moderate and severe hearing. CONCLUSION: According to previous studies on the matter, our data suggest that identification of hearing loss at early age associated with early hearing aid fitting, and enrollment in early intervention within the first 12 months of age, may help to obtain good results in the receptive language skills performance. The early identification of prelingual hearing loss at birth through the neonatal screening must therefore be, , considered the primary step for accessing a quality intervention.     

Hearing assessment by brainstem auditory evoked responses (BAER) in neonates at risk.

In the present study, brainstorm auditory evoked responses (BAER) were recorded in 68 at risk neonates discharged from the neonatal ICU of Safdarjung Hospital. The high risk group of 35 neonates included 13 neonates with multiple (3-4) risk factors and 22 neonates with single risk factors, viz., prematurity (less than 32 weeks) low birth weight (LBW) (less than 1500 g), hyperbilirubinemia requiring exchange transfusion, severe birth asphyxia, craniofacial malformations and sepsis with meningitis treated with amikacin for 3 weeks. The remaining 33 neonates were grouped in the low risk category who had only one of the following factors: prematurity (33-36 weeks)/LBW (1500-2000 g), hyperbilirubinemia requiring phototherapy, mild/moderate birth asphyxia, or sepsis treated with amikacin for 2 weeks. The test was performed at the mean conceptional age of 40.2 weeks (range 34-44 weeks) and involved determination of threshold of hearing as per presence of wave V. A normal response had wave V at 30 dB hearing level click stimulus at 50/sec from both ears or to 30 dB hearing level from one ear and 45 dB hearing level from the other ear. Thirteen neonates of the high risk group failed to produce a normal response (5 failed at 30 dB, 6 failed at 45 dB, and 2 failed at 75 dB hearing level). Forty six per cent of them had multiple high risk factors. All the low risk group neonates had normal threshold of 30 dB hearing level in the initial screening.(ABSTRACT TRUNCATED AT 250 WORDS)     

Neurodevelopmental outcome of very low birth weight babies admitted to a Malaysian nursery

OBJECTIVE: To examine the prevalence and pattern of neurodevelopmental handicap at 2 years of age in very low birth weight infants (VLBW) admitted in 1993 to a level 3 Malaysian nursery. METHODS: All VLBW babies born in the hospital or referred for neonatal care during 1993 were enrolled prospectively in the study. At 2 years of age development was assessed using the Griffiths mental scales. Neurological, hearing and visual assessments were graded into five groups according to functional handicap. Control infants were randomly selected during attendance at a primary health care clinic. RESULTS: One hundred and fifty VLBW infants were admitted and 82 (54.6%) survived to 2 years, of whom 77 (93.9%) were assessed. The mean General Quotient (GQ) on the Griffiths Scales was 94 (15.7) for the study group and 104 (8.3) for the 60 controls. For GQ, 21 (27.3%) of the study population were 1 or more SD below the mean (18 between 1 and 2 SD and 3 > 2 SD) compared with 1 (1.6%) of the controls who was 1-2 SD below the mean. Visual impairment occurred in 2 study infants and none of the controls. There was no hearing impairment in either group. Cerebral palsy occurred in 3 (1 mild and 2 moderate-severe) of the study group and none of the controls. Functionally 18 (23.3%) of the study group had mild handicap, 1 (1.3%) moderate, 2 (2.5%) severe, 2 (2.5%) multiply severe and 54 (70.2%) were normal. CONCLUSION: Although survival was low, overall rates of functional handicap were similar to those reported in developed countries but the proportion with moderate or severe handicap was low.     

Congenital cytomegalovirus infection - a common cause of hearing loss of unknown aetiology

Aim: The aim of this study was to investigate the role of congenital cytomegalovirus (CMV) infection as a cause of various types of sensorineural hearing loss (SNHL) in a group of nonsyndromic children with otherwise unknown aetiology of hearing loss. Furthermore, the occurrence of combined congenital CMV infection and connexin 26 (Cx26) mutations was investigated. Methods: The dried blood spot (DBS) cards of 45 children with various degrees of hearing deficits and 46 children with severe/profound hearing loss were tested for CMV DNA with polymerase chain reaction (PCR) technique. The DBS cards of the 46 children with severe/profound hearing loss were also analysed for Cx26 mutations. Results: Of the 45 children with various degrees of hearing loss, nine were positive for CMV DNA (20%). The nine children represented severe/profound, mild and unilateral hearing loss. From the 46 children with severe/profound hearing loss, nine of 46 (20%) were positive for CMV DNA. In addition, three of the CMV DNA‐positive children were carriers of mutations of Cx26. Conclusion: Congenital CMV infection is a high risk factor in hearing impairment among children.     

Auditory Function after Continuous Infusion of Gentamicin to High-risk Newborns

ABSTRACT. Audiometry was performed at four years of age in 69 of 105 surviving children who had received continuous intravenous infusion of gentamicin during neonatal intensive care. A hearing loss of 20 dB was found in 2 of them (3 %), corresponding to that shown in other studies of survivors following neonatal intensive care. Free field audiometry performed in another 7 children and questionnaires returned from 13 of the remaining 29 gave no suspicion of hearing loss. Thus there is no indication that continuous 24 hours intravenous infusion of gentamicin causes more hearing impairment than intermittent intravenous or intramuscular administration.     

Auditory function after continuous infusion of gentamicin to high-risk newborns

Audiometry was performed at four years of age in 69 of 105 surviving children who had received continuous intravenous infusion of gentamicin during neonatal intensive care. A hearing loss of 20 dB was found in 2 of them (3%), corresponding to that shown in other studies of survivors following neonatal intensive care. Free field audiometry performed in another 7 children and questionnaires returned from 13 of the remaining 29 gave no suspicion of hearing loss. Thus there is no indication that continuous 24 hours intravenous infusion of gentamicin causes more hearing impairment than intermittent intravenous or intramuscular administration.