Evaluation of an automated auditory brainstem response infant hearing screening method in at risk neonates

An automated auditory brainstem response (ABR) method-the ALGO-1 Plus- has been developed for hearing screening in healthy neonates. The aim of this study was to test the validity of this automated ABR screening method in at-risk neonates in a neonatal intensive care unit. Two hundred and fifty at-risk neonates were selected for screening according to the criteria of the American Joint Commerce on Infant Hearing. All 250 neonates were screened with the ALGO-1 Plus for bilateral hearing loss. When two consecutive screenings pointed to bilateral hearing loss ("refer") further audiological investigations were performed and where necessary therapeutic measures were taken. All children who "passed" the screening unilateral or bilateral enrolled in a nationwide behavioural screening programme at the age of 9 months as well as in a 6-monthly follow up programme documenting speech and language development. A total of 245 (98%) neonates passed the ALGO-1 screening, 230 (92%) at the first attempt and 15 (6%) at the second attempt. Five (2%) were referred with bilateral hearing loss. One of these died of congenital rubella shortly after screening and bilateral congenital hearing loss of > 35 dB was confirmed in the other 4. None of the infants who passed the screening were discovered to have moderate to severe bilateral hearing loss (> 40 dB) with behavioural screening (n = 183/233) or at follow up (n = 233/233). In this study, all at-risk neonates with bilateral congenital hearing loss were detected with ALGO-1 Plus screening. No false-negatives were discovered. CONCLUSION: The ALGO-1 Plus infant hearing screener can be used as a valid automated ABR-screener to detect hearing loss in at-risk neonates in a neonatal intensive care setting.     

Magnesium isotopic abundance measurement in humans: comparison of two mass spectrometric methods

OBJECTIVE: A population of children having bilateral moderate to profound hearing impairment was investigated to find out the number who passed (false negatives) and who failed (true positives) a screening test based on the auditory brain stem response (ABR). DESIGN: This study of the ABR is a parallel report to Lutman, Davis, Fortnum, and Wood (1997), where the transient evoked otoacoustic emission was examined on a similar but not identical population of at-risk neonates. Hearing screening was undertaken in at-risk neonates (targeted screening) at seven hospitals in the UK using the Nottingham ABR Screener. During the period from January 1988 to December 1993, a total of 6983 neonates had been tested. Assessment of audiological records from the participating centers ascertained that 201 children born between January 1988 and December 1993 had hearing threshold levels in both ears of 50 dB or more, averaged over the speech frequencies 0.5, 1, 2, and 4 kHz. Of these, 51 had completed the ABR screening test. Examination of the ABR records, which included both machine and visual scoring, indicated whether they had passed or failed the screening test. RESULTS: Forty-six of the 51 hearing impaired babies failed the neonatal ABR screen either on one or both ears (five false negatives), and 42 failed the test on both ears (nine false negatives). This resulted in field sensitivities of 90% and 82%, respectively. Two cases of suspected progressive hearing loss have been included in the numbers of false negative results. The percentage of babies passing the screen on both ears and subsequently having normal hearing (specificity) is typically 93%. CONCLUSIONS: Neonatal hearing screening in an at-risk population using a highly automated ABR test is a viable and effective tool for identification of hearing impairment. Although the field sensitivity of the test is high, it is unable to identify all babies with a criterion level of hearing loss. There are a number of possible explanations as to the origin of false negative results: configuration of the pure-tone audiogram, a progressive hearing loss, acquired sensorineural or conductive loss, retrocochlear deafness, or an incorrect interpretation of the screening test. Passing a neonatal screening test, therefore, does not exclude the possibility of subsequent hearing impairment and highlights the need for further surveillance.     

Otoacoustic emissions in full-term newborns at risk for hearing loss

Distortion product otoacoustic emissions (DPOEs) and click-evoked otoacoustic emissions (CEOEs) characteristics of the normal newborn population have been previously reported in the literature. There is little information about DPOE evaluations in the newborn population at risk for hearing loss. The authors now report the DPOE and/or CEOE data from six full-term subjects at risk for hearing loss or with highly suspected hearing loss. These subjects were less than 1 year of age and at risk for hearing loss secondary to a history of hereditary hearing loss, meningitis, hyperbilirubinemia, and ototoxic drug exposure. Audiometric evaluation included auditory brainstem responses (ABR), behavioral observation audiometry, and tympanometry. The CEOEs and DPOEs were found to be decreased or absent in the subjects with suspected hearing loss secondary to cochlear pathology; they were found to be normal in a subject with a suspected central hearing loss. This study's data suggest that otoacoustic emissions when combined with ABR can provide a frequency-specific evaluation of cochlear function and help determine the anatomic site of a pathologic lesion.     

Symptomatic treatment of neurolathyrism with tolperisone HCL (Mydocalm): a randomized double blind and placebo controlled drug trial

Birth hypoxia, asphyxia and ischemia have often been thought to be major causes of early hearing loss or deafness. The purpose of the present review is to focus on the role of these particular factors for perinatal auditory disorders. On the whole, only a small proportion of neonatal hearing loss is caused by perinatal factors. The exact etiology of neonatal hearing loss in children with complicated deliveries is difficult to evaluate due to the large number of causative factors that might be involved. After reviewing the literature covering the past 15-20 years, it is not possible to say that we understand the relative importance of different factors and their interactions. However, in the majority of studies, birth asphyxia is not correlated with hearing loss in babies with complicated deliveries Prolonged artificial ventilation, the presence of severe hypoxic ischemic encephalopathy or persistent pulmonary hypertension are important factors. The brain is more susceptible to anoxia than the ear and both are more likely to be damaged after prolonged pre-, peri- and postnatal hypoxia-ischemia than pure hypoxia during delivery. Perinatal hypoxia is more likely to cause a temporary hearing loss than a permanent one. Preterm babies are more vulnerable than term babies. The total number of risk factors, e.g. medicated by total length of stay in the neonatal intensive care unit and length of artificial ventilation, is the best predictor of risk for hearing loss of perinatal origin. The similarities between hearing loss and cerebral palsy are pointed out; only 8% of the cases of cerebral palsy are considered to be caused by conditions during delivery.     

The effects of cochlear hearing loss, age and sex on the auditory brainstem response

This study analyzed the effects of auditory impairment, age and sex on the auditory brainstem response (ABR) wave latencies. ABR wave I, wave V and I-V interval measures were extracted from the clinical records of 201 patients with cochlear hearing loss. Females had consistently earlier wave V latencies and shorter I-V intervals than males. No age effects were observed. Degree of impairment had a systematic effect on ABR wave latencies and I-V intervals. Wave I displayed latency extension with increasing levels of high-frequency hearing loss, whilst for wave V increases in latency were dependent upon both degree and slope of the hearing loss. Present results suggest that many of the previously reported sex differences and variable interactions seen for the ABR can be accounted for by differences in the underlying distribution of audiogram shapes within and between study populations. Different audiometric configurations were found to produce consistent differential effects on both wave I and wave V latency and thus influence the I-V interval. This study underlines the need to develop a more detailed model of impairment effects if correction factors are to be employed more effectively in ABR testing for retrocochlear pathology.     

Effects of muscle electrical activity on the transmission of developing neuromuscular junction

A prospective cohort study in a neonatal intensive care unit (ICU) was carried out to evaluate whether the incidence of infection in neonates receiving intestinal decolonization was reduced in comparison to those who did not. This study was performed after controling possible confounding infection risk factors. A total of 536 babies were screened in our ICU during the 27-month study period. Neonates were admitted to the ICU for different reasons: low weight, respiratory distress syndrome, acute fetal suffering, surgery, etc. The doctor in charge decided whether the baby should be decolonized or not, so this experimental study was non-random. Thus more of the babies with a greater risk of infection were decolonized more often than the other babies who were not so much at risk. In this study, babies were classified by type of decolonization given: a well-performed Selective Intestinal Decolonization (SID) was done (early and with three oral drugs: E polymyxin, tobramycin and nystatin): 10.8% of the babies; Incorrect SID (was begun late and/or less than three drugs were used): 16.7% of the babies; and Without SID (72.9%). Total nosocomial infection (NI) was 11.2%, catheter-associated sepsis was 42% of the total NI. When the NI incidence was directly compared among groups, it was lower in the group without SID, but infants with decolonization initially had more infection risk factor than the first group. For this reason, multiple logistic regression was used in order to stratify factors by infection probability, and correcting the existing bias.(ABSTRACT TRUNCATED AT 250 WORDS)     

The role of removable prostheses and implants in the restoration of the worn dentition

Newborns are often discharged from hospital at the age of about 48 hours. At this age, transient evoked otoacoustic emissions (TEOAEs) are not necessarily recordable in all healthy newborns. In order to determine the factors which would enable the successful recording of TEOAEs before discharge to facilitate screening for hearing, 65 fullterm newborns under 48 hours of age were tested, the youngest being 10 hours old. The ears of those neonates in whom TEOAEs could not be obtained (N = 7 neonates bilaterally, 6 unilaterally) were examined otoscopically, cleaned of vernix and retested for TEOAEs. We were thus able to record in at least one ear in all neonates tested, if the ears were clean, if they were asleep and if the testing room was quiet.     

Transitory evoked and distortion products of otoacoustic emissions in absent auditory evoked potentials

Transient click-evoked otoacoustic emissions (TEOAE) and distortion-product otoacoustic emissions (DPOAE) are produced by an active biomechanical process in the cochlea, presumably related to outer hair-cell activity. Although it is generally accepted that in most cases of hearing loss with absent auditory evoked potentials neither TEOAE nor DPOAE can be found, some cases with such a constellation have been described. Here we report another four cases of children with severe to profound hearing loss where we discovered reproducible TEOAE and DPAOE, whereas auditory evoked potentials were missing. TEOAE and DPOAE recordings in these cases indicate substantially preserved outer hair-cell function independent of profound pre-sensineural hearing loss. Since the incidence of children with preserved otoacoustic emissions together with impairment of synaptic or postsynaptic function of the first neuron is not known, the unconditioned use of TEOAE nor DPOAE as a screening instrument must be seriously questioned. Secondly, in conjunction with subjective audiometry and brain-stem-evoked potentials, emission recordings is an indispensable measurement prior to cochlear implantation and use of high-power hearing aids.     

Prostate cancer and prostate bed SPECT imaging with ProstaScint: semiquantitative correlation with prostatic biopsy results

The last (may 1995) NHI Consensus Development Conference on cochlear implant recommends to extend the use of cochlear implant for adult patient suffering from bilateral acquired severe hearing impairment. Its indications are a severe-to-profound sensorineural hearing loss bilaterally presenting an open-set sentence recognition scores less than or equal to 30 percent under best aided conditions. We report the results of our 4 first implantees responding to these criteria, and discuss the mechanisms of the speech intelligibility improvement which has been obtained.     

Aided speech recognition abilities of adults with a severe or severe-to-profound hearing loss

Adults with severe or severe-to-profound hearing losses constitute between 11% and 13.5% of the hearing-impaired population. A detailed investigation of the speech recognition of adults with severe (n = 20) or severe-to-profound (n = 14) hearing loss was conducted at The University of Melbourne. Each participant took part in a series of speech recognition tasks while wearing his or her currently fitted hearing aid(s). The assessments included closed-set tests of consonant recognition and vowel recognition, combined with open-set tests of monosyllabic word recognition and sentence recognition. Sentences were presented in quiet and in noise at +10 dB SNR to replicate an environment more typical of everyday listening conditions. Although the results demonstrated wide variability in performance, some general trends were observed. As expected vowels were generally well perceived compared with consonants. Monosyllabic word recognition scores for both the adults with a severe hearing impairment (M = 67.2%) and the adults with a severe-to-profound hearing impairment (M = 38.6%) could be predicted from the segmental tests, with an allowance for lexical effects. Scores for sentences presented in quiet showed additional linguistic effects and a significant decrease in performance with the addition of background noise (from 82.9% to 74.1% for adults with a severe hearing loss and from 55.8% to 34.2% for adults with a severe-to-profound hearing loss). Comparisons were made between the participants and a group of adults using a multiple-channel cochlear implant. This comparison indicated that some adults with a severe or severe-to-profound hearing loss may benefit from the use of a cochlear implant. The results of this study support the contention that cochlear implant candidacy should not rely solely on audiometric thresholds.     

Prematurity, incubator noise and hearing disturbances (author's transl)

1. An audiologic follow-up study was done on 62 children, ages 10-11 years. All children had been pre-term infants, with a gestational age between 29 and 35 weeks. They had been maintained in an incubator for a period of 21-61 days. The children had frequently received ototoxic antibiotics in the newborn period, and had been subject to many perinatal risk factors with a high "non-optimal score." 2. 44 children (71%) were found to be audiologically normal, whereas 8 (13%) had minor auditory defects and 10 (16%) had major hearing defects. 3. There was a significant correlation between hearing defects and the "non-optimal score": children with the most difficult perinatal course were more likely to develop hearing difficulties. 4. This study was unable to support the assumption that the hearing loss found was due to high incubator noise levels: (a) The incubator noise level was slightly below the probably dangerous limit for noise exposure. (b) There was no correlation between the hearing impairment and the length of incubator stay. (c) The type of hearing defect was not generally consistent with that associated with noise pollution.     

Electrophysiological studies in the follow-up of children with perinatal asphyxia history

OBJECTIVES: The objective of this study was to analyze the usefulness of electrophysiological studies [electroencephalogram (EEG) and auditory-evoked potential (AEP)] during the follow-up of children with perinatal asphyxia antecedents. PATIENTS AND METHODS: A prospective epidemiological study of perinatal asphyxia in term neonates born at the University Hospital San Juan (Alicante, Spain) between November 1991 and February 1995 was performed. Perinatal asphyxia was graded as non-severe (1 minute Apgar score < or = 6 and/or umbilical artery pH < 7.20, with abnormal fetal heart patterns and/or meconium-stained amniotic fluid and the need for immediate neonatal resuscitation) and severe (1 minute Apgar score < or = 3 and umbilical artery pH < 7.10). The incidence of hypoxic-ischemic encephalopathy (classification of Levene and Sarnat & Sarnat) during the neonatal period and neurological sequelae (classification of Finer and Amiel-Tison) during the follow-up period were studied. Electrophysiological studies (EEG and AEP) were made mainly between 12 and 18 months of life. RESULTS: During the study period there were 156 cases of perinatal asphyxia in full-term live births (31 severe and 125 non-severe). Hypoxic-ischemic encephalopathy was present in 25.6% of asphyxiated newborn infants, being mild in 30 cases, moderate in 5 and severe in 5. The incidence of neurological sequelae in 115 asphyxiated newborns followed for 24 month was 16.5%. This included mainly motor disabilities. We did not find any case of epilepsy, but there were 4 children with febrile seizures and one case of benign myoclonic seizures. EEG was performed in 88 cases during follow-up, and only was abnormal in two infants without seizures. AEP was performed in 82 cases during follow-up and hearing loss was detected in 4 children with neurosensorial hypoacusia (3 unilateral and 1 bilateral). CONCLUSIONS: Rutinary EEG is not useful during follow-up of children with antecedents of perinatal asphyxia. However, AEP is a hearing screening procedure for infants at risk of deafness, such as in perinatal asphyxia, and the cases of neurosensorial hearing loss detected by AEP in our population were clinically unapparent.     

A critical review of the role of neonatal hearing screening in the detection of congenital hearing impairment

BACKGROUND: This review was commissioned because of the increasing doubt about the ability of existing screening programmes (mainly the health visitor distraction test (HVDT) at 7-8 months) to identify children with congenital hearing impairment, and technological advances which have made neonatal hearing screening an alternative option. OBJECTIVES: To review the available literature on the screening of permanent childhood hearing impairment. To provide commissioners and providers of health care with information about how to deliver a more uniform service, better outcomes, and more cost-effective screening. To identify areas for further research and service development. HOW THE RESEARCH WAS CONDUCTED: The research involved a review of the available published and unpublished literature, and a comprehensive survey of current pre-school hearing screening provision in the UK coupled with a health economics study of hearing screening costs. The research also included a number of focus groups and visits to key centres in the UK and North America. RESEARCH FINDINGS: EPIDEMIOLOGY OF PERMANENT CHILDHOOD HEARING IMPAIRMENT: There are approximately 840 children a year born in the UK with significant permanent hearing impairment likely to affect their own and their family's quality of life. Present services will miss about 400 of these children by 1 1/2 years of age, and about 200 of these children by 3 1/2 years of age. Such late identification of hearing impairment greatly reduces the responsiveness of the services for individual children. EVIDENCE FOR IMPROVED OUTCOMES WITH EARLIER IDENTIFICATION: Hearing-impaired children identified late are at risk of substantial delay in their acquisition of language and communication skills, with consequent longer-term risk to education achievement, mental health and quality of life. Theoretical arguments on neural development support the limited evidence here for the increased benefit for child and family associated with very early identification. In general, parents and professionals want very early identification, which, if implemented properly, does not cause undue anxiety. CURRENT UK PRACTICE: The survey of current practice indicated a major problem with poor information systems. This problem was further highlighted as a major concern by the multi-disciplinary focus groups. Practice varies. There are two District-wide programmes in which all newborn babies are neonatally screened, a large number of ad hoc programmes for neonatal screening of 'at-risk' babies, a variety of early surveillance programmes, and widespread use of the HVDT. Intervention and habilitation for the majority of those screened neonatally is routinely undertaken within 6 months of birth. For those screened only by the health visitor, identification was on average at about 26 months of age with intervention at about 32 months on average. (ABSTRACT TRUNCATED)     

The genetics of ovarian cancer: concepts in testing and counseling

This article describes a screening program for hearing loss among infants. The program, designed according to ASHA recommendations, was implemented in a public health district of the province of Quebec. In the first stage of the program all infants born between April 1, 1990 and March 30, 1992 were assessed for risk factors for deafness. In the second stage, those infants considered at risk were given an audiological examination at six months of age. There were 3,944 births during the study period and of these, 8.4% of the infants had at least one risk factor for hearing impairment, the most frequent of which was a family history of hearing impairment at an early age, followed by: hyperbilirubinemia, admission to a high risk neonatal unit, consumption of ototoxic medications, anatomical malformations, perinatal infections and insufficient birthweight. Of the infants at risk, 54.2% were given the audiological examination at six months and one case of deafness was diagnosed.     

Early diagnosis of congenital hearing loss. Use of oto-acoustic emission tests in newborn infants with increased risk of hearing impairment

With the use of today's screening programme children with congenital hearing losses (prevalence about 1/1,000) get their diagnosis unacceptably late (median age 28 months). Newer screening methods as oto-acoustic emissions and auditory brain stem responses have been in use, separately or as combined tests. The methods are used both as universal screening and as screening of selected children with increased risk of congenital hearing impairment. On the basis of ten risk factors for congenital hearing loss, we present our results from screening in the neonatal period. 283 out of 8,980 children (3.2%) born in Ostfold county over a period of three years have been examined with the use of oto-acoustic emissions. 16 children had pathological emission tests bilaterally on repeated testing and underwent further examination in the ear-nose-and-throat department. One of these children has turned out to have hearing loss.     

Paediatric hearing loss in rural Pakistan

OBJECTIVE: The present study was designed to generate population-based data on the prevalence and causes of hearing loss in rural Pakistani children. A community screening programme was utilized to identify and evaluate the hearing impaired. METHODS: The study was performed in conjunction with the Ministry of Population Welfare in Sialkot District, Punjab Province. School-aged children between the ages of 5 and 15 years were screened and examined for hearing impairment according to World Health Organization (WHO) protocols. Case-control analysis of audiometric, physical examination, and risk factors for hearing loss were performed, followed by chi-square analyses. RESULTS: A total of 607 children comprised the study population, with an overall point prevalence of hearing impairment of 7.9%. Fifty percent of all hearing loss was conductive in nature, amenable to either medical or surgical therapy. The risk factors most associated with conductive hearing loss were otorrhea and multiple ear infections greater than 5. In cases of severe hearing loss, 70% were the result of consanguinous marriages. Almost no cases of hearing loss were attributable to measles, mumps, rubella, and the TORCH infections. CONCLUSION: This study has generated some badly needed population-based data on the magnitude of the problem of hearing loss in rural Pakistan. It is hoped that the results of this work will stress the importance of hearing health in Pakistan and to encourage other professionals to pursue similar projects.     

Multi-variant analysis of otoacoustic emissions and estimation of hearing thresholds: transient evoked otoacoustic emissions

Evaluation of cochlear hearing loss by means of transiently evoked otoacoustic emissions is already established in clinical practice. However, accurate prediction of pure-tone thresholds is still questioned and is still regarded as troublesome. Both click- and tone-burst-evoked otoacoustic emissions at several intensity levels were measured and analysed in 157 ears from normally hearing and 432 ears from patients with different degrees of pure sensory hearing loss using the ILO88/92 equipment. Results of otoacoustic emissions (OAE), elicited by clicks and tone-bursts at centre frequencies from 1 to 5 kHz, were analysed using two different statistical methods. Both multivariate discriminant analysis and forward multiple regression analysis were used to determine which OAE variables were most discriminating and best at predicting hearing thresholds. We found that a limited set of variables obtained from both tone-burst and click measurements can accurately predict and categorize hearing loss levels up to a limit of 60 dB HL. We found correct classification scores of pure-tone thresholds between 500 and 4000 Hz up to 100 per cent when using combined click and tone-burst otoacoustic measurements. Prediction of pure-tone thresholds was correct with a maximum estimation error of 10 dB for audiometric octave frequencies between 500 and 4000 Hz. Measurements of multiple tone-bursts OAEs have a significant clinical advantage over the use of clicks alone for clinical applications, and a good classification and prediction of pure-tone thresholds with otoacoustic emissions is possible.     

Influence of respiratory distress syndrome on body composition after preterm birth

AIM: To observe changes in body composition during the first week after birth, in preterm neonates with and without respiratory distress syndrome (RDS), so as to be able to provide optimal fluid and energy intake. METHODS: Twenty four babies with RDS and 19 healthy preterm babies, with gestational ages ranging from 26-36 weeks, were studied daily for the first week after birth. Total body water (TBW) was measured using bioelectrical impedance analysis. The babies were weighed daily and a record made of fluid and energy intake. Body solids were calculated as the difference between body weight and TBW. RESULTS: There was a highly significant reduction in body weight by the end of the week, with the RDS babies losing more than the healthy babies (RDS 7.6%; non-RDS 3.7%). There was no significant difference in the amount of TBW at birth in the babies with and without RDS (RDS 85.1%; non-RDS 85.5%) and both groups lost the same amount of body water (RDS 10.9%; non-RDS 9.9%) by the end of the first week. The amount of total body water lost was unrelated to the volume of fluid administered. There was a loss of body solids during the first day in the RDS group, but, overall, there was a highly significant increase in both groups between birth and day 7, which was greater in the healthy babies (RDS 13.0%; non-RDS 42.7%). CONCLUSIONS: Loss of body water after birth occurs to the same extent in healthy preterm neonates and in babies with RDS and is unrelated to the volume of fluid administered. Given adequate nutritional support, an increase in body solids can accompany early postnatal weight loss and begins almost immediately after birth, in both healthy preterm babies and babies with RDS.     

Treatment of spondylolysis with external electrical stimulation and bracing in adolescent athletes: a report of two cases

BACKGROUND: An epidemic of bilateral optic neuropathy is affecting large numbers of people aged between 10 and 40 in Dar es Salaam, the capital city of Tanzania. The disease is characterised by acute onset of bilateral visual impairment, bilateral impairment of colour vision, and a characteristic temporal pallor of the optic discs. The disease often occurs in association with peripheral neuropathy and sensorineural hearing loss. This report presents the first data on disease prevalence in adolescents, based on a rapid assessment of schoolchildren. METHODS: Three schools in Dar es Salaam were visited and all children aged between 10 and 16 were screened for the disease. RESULTS: The prevalence of bilateral optic neuropathy among the schoolchildren is estimated to be 1.0% (95% CI 0.5-1.4%). This suggests that 5000 children (95% CI 2600-7300) aged 10-16 in Dar es Salaam may have the disease. CONCLUSION: The results of this rapid assessment indicate this epidemic is a major public health problem. The prevalence of the disease in the community is likely to be far higher than found in this survey because children with the disease may have withdrawn from school. As the disease predominantly affects young adults, resulting in impaired vision and hearing, the economic and social consequences are enormous.     

Cochlear implantation. Practical information for the generalist

The diagnosis and investigation of a patient with bilateral, severe-to-profound sensorineural hearing loss is presented. The steps toward cochlear implantation, including tests of audition, balance, radiographic imaging, and the pre- and postrehabilitation period, are reviewed. The two major cochlear implants available today, and future developments within the cochlear implant, are presented.