Newborn hearing concurrent gene screening can improve care for hearing loss: a study on 14,913 Chinese newborns

Objective

Newborn hearing screening has been widely adopted and made an achievement to some degree. Current screening protocols rely solely on detecting existing auditory disorders at the time of screening and are unable to identify individuals susceptible to auditory disorders in later life. Even if the hearing loss newborn is referred, most cases could not be diagnosed until 6-12 months old with no etiology being elucidated. This study reports the first effort to combine traditional hearing screening with genetic screening to improve the efficacy of newborn hearing screening.

Methods

This study was undertaken in 12 regional hospitals located in 11 provinces of China. 14,913 newborn babies received hearing concurrent genetic screening. The hearing screening was performed with OAE or AABR. Blood sample was collected with a universal newborn genetic screening card. And three common gene, mtDNA 12S rRNA, GJB2 and SLC26A4 were screened with standard protocol.

Results

Among all the 14,913 newborns, 86.1% (12,837/14,913) individuals passed the first-step hearing screening, 7.8% (1168/14,913) babies passed only one side, and the other 6.1% (908/14,913) were bilaterally referred. Gene screening found 306 individuals had one or two mutant alleles, the carrier rate is 2.05% (306/14,913) among the entire newborn population. The risk for hearing loss was 100% (7/7) for those newborns carrying causative GJB2 or SLC26A4 mutations (homozygotes or compound heterozygotes), 14.4% (23/160) for GJB2 heterozygote carriers, 12.3% (15/122) for SLC26A2 heterozygous carriers, and the total prevalence of referral hearing screening was approximately 14.7% (45/306). However, 85.3% (261/306) newborns passed hearing screening among these carriers including 18 newborns with 12S rRNA mt.1555A>G pathogenic mutation, who would suffer from sudden hearing loss once applying aminoglycoside drugs.

Conclusion

The cohort studies provided the essential population parameters for developing effective programs for hearing care of newborns in China. Hearing concurrent gene screening in newborns may confirm the abnormal results from hearing screening tests, help to find the etiologic of the hearing loss, and better recognize infants at risk for late-onset hearing loss occurring prior to speech and language development. In conclusion, a survey on 14,913 Chinese newborns proved that concurrent genetic screening could improve newborn hearing screening for hearing defects.     

Etiological diagnosis in the hearing impaired newborn: proposal of a flow chart

Objective

Most industrialized countries have introduced some form of universal newborn hearing screening program. Both identification and rehabilitation of hearing loss in newborns have evolved to an acceptable standard and the need for a standardized etiological protocol is emerging.

Methods

Extensive literature search to determine which investigations can help identifying the cause of congenital hearing loss and how to limit extensive testing in these children by taking into account the most prevalent causes.

Findings

A stepwise approach to detect the cause of hearing loss in children with congenital sensorineural hearing loss was developed.

Conclusion

In general it is advised to first rule out Cx26/Cx30 and infectious causes (cytomegalovirus and, if indicated, toxoplasmosis and rubella), and to preserve more extensive investigations for those children in whom these causes do not explain the hearing loss.     

Universal newborn hearing screening in the Champagne-Ardenne region: A 4-year follow-up after early diagnosis of hearing impairment

Introduction

Permanent congenital hearing loss is one of the most frequent congenital anomaly at birth. Universal newborn hearing screening (UNHS) was introduced in numerous countries in order to allow an early diagnosis and intervention for congenital hearing impairment.

Objective

First aim of this study is to evaluate the accuracy of early diagnosis of hearing impairment after UNHS. Second aim is to discuss the auditory intervention proposed after this diagnosis. Last aim is to evaluate the relevance of UNHS for early diagnosis and intervention.

Materials and methods

Prospective study. UNHS program was introduced in the entire French region of Champagne-Ardenne in January 2004. Forty-one children have benefited of an early diagnosis of hearing impairment until June 2007. They were included in an intervention program consisting of an audiometric follow-up and an auditory intervention. This program was conducted until June 2008.

Results

There were 28 males patients and 13 females patients. The diagnosis of hearing aid impairment was carried at an average age of 3.2-month. The auditory follow-up allowed confirming the initial diagnosis of deafness for the majority of the children as for their degree of hearing loss. Auditory intervention was heterogeneous depending on degree of hearing loss of the children.

Conclusion

This UNHS program demonstrates its validity and feasibility for early diagnosis and intervention of congenital hearing impairment. It brought a major impact on the management of congenital hearing impairment in Champagne-Ardenne.     

Status of newborn hearing screening program in India

Objectives

The present study is aimed at figuring out the status of new-born hearing screening program being conducted in India, estimate the use of different screening instruments used and tests practiced, study the role of various professionals involved and document the current practices of audiologists in the country.

Method

A questionnaire on “Newborn Hearing Screening Survey” was sent to 185 institutions (165 medical colleges and 20 Speech and hearing centers) all over India and the information gathered was subjected to appropriate analyses.

Results

On a 16.75% return rate of the questionnaire, almost half of the colleges have their annual birthing census more than 2000. The majority of sites (57.13%) report an average length of stay for a vaginal delivery to be more than 24 hours with 78.94% also reporting of a NICU of Level II/III type. Only 38.09% of the medical colleges have a universal Newborn Hearing screening program (NBHS) in comparison to 80% of the Speech and Hearing centers. Again 43.8% of the medical colleges who conduct NBHS program have an audiologist and majority of them work towards screening. Almost 63% of the speech and hearing centers use physiological tests like ABR, OAE or their combination to screen newborns. Both medical and speech and hearing organizations prefer that the audiologist inform and even give a written material regarding the results of the tests to the parents. Only 62.65% of the institutions refer less than 11% of their clients for further testing at the time of discharge. In 50% of the medical colleges the results of a test are reported to the parents and primary care physicians/doctors and in 37.5% of the speech and hearing centers the results are informed to the parents.

Conclusions

While keeping in mind the incidence of hearing impairment in the neonatal population of India, the results of this survey warrant the need for an urgent implementation of universal neonatal hearing screening in all the health care facilities in the country, at large.     

Screening for delayed-onset hearing loss in preschool children who previously passed the newborn hearing screening

Objective

The goal of this study was to investigate the prevalence of delayed-onset hearing loss in preschool children who previously passed newborn hearing screening in Shanghai, China.

Methods

Between October 2009 and September 2010, 21,427 preschool children were enrolled from five areas of Shanghai, who had passed newborn hearing screening. Children were screened for delayed-onset hearing loss with pediatric audiometers. Children with positive results in initial and re-screening tests were assessed audiologically and for risk indicators according to the Year 2007 Statement of the American Joint Committee on Infant Hearing (JCIH).

Results

During the study period, 445 children (2.08%) were referred for audiologic assessment; 16 (0.75/1000, 95% CI 0.38-1.12) had permanent delayed-onset hearing loss. Of these, five (0.23/1000) had bilateral moderate hearing loss; seven (0.33/1000) had mild bilateral hearing loss; and four (0.19/1000) had unilateral moderate or mild hearing loss. JCIH risk indicators were established for six children (6/16, 37.5%), including parental concern regarding speech and language developmental delay, neonatal intensive care unit with assisted ventilation or hyperbilirubinemia, recurrent otitis media with effusion, craniofacial malformation, and family history (n = 1 for each). The remaining ten (62.5%) had no related risk factors.

Conclusions

A significant proportion of preschool children have undiagnosed delayed-onset hearing loss. Hearing screening in preschool is recommended for an early detection.     

The economics of screening infants at risk of hearing impairment: an international analysis

Objective

Hearing impairment in children across the world constitutes a particularly serious obstacle to their optimal development and education, including language acquisition. Around 0.5-6 in every 1000 neonates and infants have congenital or early childhood onset sensorineural deafness or severe-to-profound hearing impairment, with significant consequences. Therefore, early detection is a vitally important element in providing appropriate support for deaf and hearing-impaired babies that will help them enjoy equal opportunities in society alongside all other children. This analysis estimates the costs and effectiveness of various interventions to screen infants at risk of hearing impairment.

Methods

The economic analysis used a decision tree approach to determine the cost-effectiveness of newborn hearing screening strategies. Two unique models were built to capture different strategic screening decisions. Firstly, the cost-effectiveness of universal newborn hearing screening (UNHS) was compared to selective screening of newborns with risk factors. Secondly, the cost-effectiveness of providing a one-stage screening process vs. a two-stage screening process was investigated.

Results

Two countries, the United Kingdom and India, were used as case studies to illustrate the likely cost outcomes associated with the various strategies to diagnose hearing loss in infants. In the UK, the universal strategy incurs a further cost of approximately £2.3 million but detected an extra 63 cases. An incremental cost per case detected of £36,181 was estimated. The estimated economic burden was substantially higher in India when adopting a universal strategy due to the higher baseline prevalence of hearing loss. The one-stage screening strategy accumulated an additional 13,480 and 13,432 extra cases of false-positives, in the UK and India respectively when compared to a two-stage screening strategy. This represented increased costs by approximately £1.3 million and INR 34.6 million.

Conclusions

The cost-effectiveness of a screening intervention was largely dependent upon two key factors. As would be expected, the cost (per patient) of the intervention drives the model substantially, with higher costs leading to higher cost-effectiveness ratios. Likewise, the baseline prevalence (risk) of hearing impairment also affected the results. In scenarios where the baseline risk was low, the intervention was less likely to be cost-effective compared to when the baseline risk was high.     

A comparison of audiometric and objective methods in hearing screening of school children. A preliminary study

Objective

In newborn hearing screening, one exclusively applies objective hearing testing methods - based on evoked potentials and/or on otoacoustic emissions. However, when testing school children, one can consider both audiometric and electrophysiological methods. The choice of methods is determined by the aims of the program. If one wants to detect conductive hearing losses, impedance audiometry seems to be the method of choice.

Methods

The aim of this study was to compare test performance measures from audiometric and objective methods (OAEs and impedance audiometry), in the hearing screening of school children. Screening protocols were applied on a group of 190 children of about 12 years of age (6th grade of primary school).

Results

For a single application of a screening procedure, the best performance was observed in the automated four-tone audiometry, followed by the tympanometry and the TEOAE-based procedures. Screening performance was enhanced using a combination of automated and impedance audiometry. A four-tone audiometry test combined with tympanometry gives a sensitivity of 65%, and the PPV of 46%, which are reasonable values, acceptable for practical use. The use of a TEOAE protocol degrades the overall performance of screening.

Conclusions

Screening of school children is feasible with a combination of automated audiometry and tympanometry with time requirements equal to 3 min per subject.     

Qualitative analysis of parents’ experience with receiving the news of the detection of their child's hearing loss

Objective

Despite the fact that clinicians are responsible for delivering bad news, they have been shown to lack both confidence and skill in performing this basic task. The time immediately after the detection of childhood hearing loss is perceived as stressful. We conducted a qualitative study to assess parents’ experiences with receiving the bad news of the detection of their child's hearing loss.

Study design

Semi-structured interviews were conducted with families of children with hearing loss identified during early childhood.

Setting

A rehabilitation center treating pre-school children in the north of Israel.

Patients

14 families/parents of children diagnosed prior to the implementation of a universal screening program.

Main outcome measure

Parents’ perceptions of the manner in which the information regarding the detection of their child's hearing loss was given, and what their feelings were at the time.

Results

50% of parents expressed dissatisfaction with the process of the breaking of the bad news. ABR is perceived by parents and health professionals alike as the definitive moment of diagnosis. The emotions experienced by parents at the moment of the breaking of the bad news were predominantly shock and upset. The meaning of the news was perceived differently under different circumstances. Information given bluntly, without empathy, was a frequent complaint. Parents repeatedly stated the importance of the formulation of a plan for the future.

Conclusions

Qualitative enquiry provided valuable information. Effective strategies for the breaking of bad news should become an integral part of universal neonatal screening programs.     

Subjective tinnitus and hearing problems in adolescents

Objectives

We investigated the hearing problems and tinnitus frequencies in adolescents at three public primary and two high schools.

Methods

This study was carried out at three public primary and two high schools. 428 Turkish school children (244 girls, 184 boys) were asked to voluntarily answer a set of questionnaires in their classrooms at the beginning of the training program. There were 250 students (105 male, 145 female) in Primary School and 178 (79 male, 99 female) students in High School. We used questionnaire to evaluate subjective tinnitus and hearing problems. Walkman usage, listening loud and noisy music, intra-familial physical trauma, concentration difficulty in class and school success were also evaluated.

Results

In age-related groups (Group 1 = 11-13 years; Group 2 = 13-15 years; Group 3 = 16-18 years), hearing loss was present in 32.1% of Group 1, 19% of Group 2 and 28.3% of Group 3. Listening loud and noisy music was reported in 81.8% of Group 1, 95.4% of Group 2 and 87% of Group 3. Tinnitus was present 36.8% in Group 2, 33.5% in Group 1 and 31.5% in Group 3. Tinnitus after listening loud music was present in 42.7% of Group 2, 36.1% of Group 3 and 25.6% of Group 1. Among all students with tinnitus, 19.5% considered their school success as very good, 41.1% as good and 39.4% as bad. In students, using Walkman, tinnitus was seen both in the right and left ears.

Conclusion

Tinnitus may be seen in adolescents at primary and high schools. Listening loud and noisy music and Walkman usage may cause an increase in the frequency of tinnitus manifestation. Adolescents should be educated about the hazardous effects of loud music. Education should include families, teachers, students, and whole community. These issues should be taken into public health policy of the countries.     

The effectiveness of the promotion of newborn hearing screening in Taiwan

Objective

Hearing is a critical ability for the development of a child's speech and language. Many studies in different countries have shown the universal newborn hearing screening and early intervention has greatly reduced the negative impact caused by congenital hearing loss. The first universal newborn hearing screening program in Taiwan took place in MacKay Memorial Hospital in 1998 and was subsequently endorsed by the government. The incidence of bilateral congenital hearing impairment in Taiwan is approximately 2.6 per 1000 live birth. The aim of this paper is to analyze the age of diagnosis, hearing aid fitting, and intervention of congenitally hearing impaired children with and without hearing screening after public awareness and government endorsement of newborn hearing screening.

Materials and methods

There were 263 hearing impaired children participated in this study, receiving their auditory habilitation therapy at Children's Hearing Foundation from 2006 to 2010. 114 of those children went through newborn hearing screening and 149 without it. The age of diagnosis, hearing aid fitting, and auditory intervention were compared between these two groups. The age of diagnosis and intervention of congenitally hearing impaired children among different years were analyzed too.

Results

The average age of diagnosis was 8.7 months, the age of hearing aid fitting was 12.4 months and age of auditory intervention was 18.8 months for the group of hearing impaired children with newborn hearing screening. For hearing impaired children without newborn screening, their average age of diagnosis was 27.5 months; age of hearing aid fitting was 31.3 months and age of auditory intervention was 40.5 months. There were significant differences in the age of diagnosis, hearing aid fitting and auditory intervention between congenitally hearing impaired children with and without hearing screening.

Conclusions

This research indicates that newborn hearing screening facilitates early identification, diagnosis and intervention of congenitally hearing impaired children in Taiwan. The age of identification, diagnosis and intervention of congenital hearing impaired children has also been reduced gradually over the years after government endorsement of newborn hearing screening in Taiwan.     

Screening for pre-school and school-age hearing problems: European Consensus Statement

Objectives

To formulate consensus statement and policies on structured hearing screening programs in pre-school and school-age children in Europe. This consensus will be brought before the European Union's Member States as a working and effective program with recommendations for adoption.

Methods

A distinguished panel of experts discussed hearing screening of pre-school and school-age children during the 10th Congress of European Federation of Audiology Societies (EFAS), held in Warsaw, Poland, on June 22, 2011. The panel included experts in audiology, otolaryngology, communication disorders, speech language pathology, education and biomedical engineering.

Results

Consensus was reached on thirteen points. Key elements of the consensus, as described herein, are: (1) defining the role of pre-school and school screening programs in the identification and treatment of hearing problems; (2) identifying the target population; (3) recognizing the need for a quality control system in screening programs.

Conclusions

The European Consensus Statement on Hearing Screening of Pre-school and School-age Children will encourage the appropriate authorities of the various countries involved to initiate hearing screening programs of pre-school and school-age children.     

Treatment of persistent middle ear effusion in cleft palate patients

Objectives

The goals of the research project are to learn how to individualize otologic care for cleft palate patients and to be able to counsel families of children with cleft palate on the benefit of tympanostomy tubes, hearing issues and risks of multiple sets of tubes.

Methods

The study is a retrospective chart review. Patients with a cleft palate with or without a cleft lip born between 1 January 2000 and 31 December 2005 referred to the Connecticut Children's Medical Center Craniofacial Department were included in the study. The patients were offered individualized ear surgery (PE tube placement) only if persistent middle ear fluid was present for over 3 months with a conductive hearing impairment. The primary outcome measures included the newborn hearing screening results, number of ear tube surgeries, and complications of PE tube insertion.

Results

There were 86 patients with cleft palate spectrum with or without cleft lip (45 females and 41 males). Twelve had undocumented newborn hearing evaluations. Of the 74 evaluable results, 61 (82%) passed the newborn hearing screening, 8 (11%) failed and 5 (7%) were inconclusive. By 5 years old, 84 (98%) patients received at least one set of ear tubes for persistent middle ear fluid with conductive hearing impairment, while 2 received no tubes (2%). Of those who received ear tubes, the range was 1-6 with a mean of 1.7. Twelve patients (14%) had tympanosclerosis. Eight patients (9%) had eardrum perforation. One patient had myringoincudopexy. Of the 86 patients, 12 had undocumented newborn hearing evaluations. Of the 74 evaluable results, 61 (82%) passed the newborn hearing screening, 8 (11%) failed and 5 (7%) were inconclusive.

Conclusions

(1) The majority of children born with cleft palate do not have middle ear fluid at birth. (2) Most children with cleft palate will likely develop persistent middle ear fluid with conductive hearing loss. Risks of complications from ear tubes in cleft palate patients are few and manageable using standard sized ear tubes.     

The universal newborn hearing screening program of Taipei City

Objectives

To establish a hearing screening program with high coverage, low referral rate, high follow-up rate, and early intervention in Taipei City.

Methods

From September 2009 to December 2010, 85% delivery units in Taipei City, which includes 20 hospitals and 14 obstetrics clinics, were recruited into the screening program in two stages. A total of 15,930 babies were born in these participating hospitals and clinics during the program period. Among these neonates, 15,790 underwent hearing screening test with automatic auditory brainstem response (AABR). The screening was free of charge to the parents. The hearing screening examination was performed 24–36 h after birth. The same test was repeated between 36 and 60 h of age if the baby failed the first hearing test. The neonate was referred to the diagnostic hospitals for further investigations if he failed the second test.

Results

The screening coverage rate was 99.1% (15,790/15,930). The incidence of bilateral moderate to severe and unilateral hearing loss was 1.4 per 1000 (22/15,790) and 1.5 per 1000 (24/15,790), respectively. Four percent (626/15,790) of newborns failed to pass the initial screening test and 1.0% of newborns failed to pass the second screening test. Therefore, 1.0% newborns were referred for diagnostic assessments. The follow-up rate was 94.4% (151/160). Sixty-four percent (14/22) of babies with bilateral hearing loss completed the full diagnostic hearing tests within 3 months of birth.

Conclusions

The universal newborn hearing screening program is an adequate program for Taipei City with high coverage, low referral rate, and good follow-up rate. Screening fees covered by third parties, two-stage screening steps with AABR strategy, and the stringent monitoring system proved to be effective.

Level of evidence

2b, individual cohort study     

Prevalence of A1555G mitochondrial mutation in Chinese newborns and the correlation with neonatal hearing screening

Objective

To investigate the feasibility of genetic screening for deafness causative genes in the process of newborn hearing screening in China.

Methods

Total 865 newborn babies between November 2009 and March 2010 were enrolled for the simultaneous hearing and deafness causative gene screening in Tongji Hospital, Wuhan, China. Hearing screening followed a two-stage strategy with transient evoked otoacoustic emissions. Infants referred after the second-stage screening were tested by diagnostic auditory brainstem response (ABR). Genomic DNA was extracted from heel blood of newborns, and the mitochondrial 12S rRNA A1555G mutation was detected by polymerase chain reaction (PCR) based restriction fragment length polymorphism and confirmed by DNA sequencing.

Results

In hearing screening, 134 out of the 865 newborns (15.5%) were referred after the first-stage screening and 86.6% (116/134) of them returned for the second stage. After the second-stage screening, 15 who were still referred were tested by diagnostic ABR and 3 of them failed the test. On the other hand, gene screening identified 6 of the 865 newborns (0.7%) harbored homoplasmic 12S rRNA A1555G mutation although they passed the hearing screening.

Conclusion

It might be practical and effective to complement routine hearing screening in newborns with gene screening for the purpose of early diagnosis and discovery of the late-onset hearing loss.     

Newborn hearing screening on infants at risk

Objectives

This article presents the results of newborn hearing screenings on infants at risk of hearing impairment at the French University Hospital of Besançon from 2001 to 2007.

Materials and methods

All newborns at risk of hearing impairment were tested according to the method recommended by the Joint Committee on Infant Hearing (JCIH): a two-step automated oto-acoustic emissions (AOAE) program, completed by an auditory brainstem response (ABR) for the positive diagnosis of hearing impairment. The screening started with AOAE on the third day of life, at the earliest. If one or both ears did not have AOAE, the infant was re-tested at which time, should the AOAE again be positive, ABR was performed. When the ABR threshold was 40 dB or more, the infant was referred to an audiologist specialized in infant deafness for diagnosis confirmation and management.

Results

Over the period, 1461 infants were screened, among whom 4.55% were diagnosed as deaf or hard of hearing. Nearly 10% of the infants were lost to follow up. Forty-six children had a sensorineural hearing impairment, of which 34 were bilateral and were managed before the age of 6 months. The risk factors for sensorineural hearing loss were (in order of statistical significance): severe birth asphyxia; neurological disorder; syndromes known to be associated with hearing loss; TORCH (toxoplasmosis, rubella, cytomegalovirus, herpes) infections; family history of deafness; age at the time of screening; and the association of 2 or more risk factors. However, birth weight inferior to 1500 g and premature birth before the 34th week of pregnancy did not show a statistically significant influence on sensorineural hearing loss. Craniofacial anomalies (mostly cleft palate and ear aplasia) were a significant factor for conductive hearing loss.

Conclusion

Our selected hearing screening on infants at risk allowed 60 deaf children access to early management. However, too many children were lost to follow up; which revealed that better information regarding risk of hearing loss must be provided to parents and paramedics and universal newborn screening needs to be performed. The most important result of this study is that in a population of hearing impaired children, with an impairment incidence close to what is commonly reported, the association of several risk factors proves to be a significant additional risk factor for hearing impairment.     

National Newborn Hearing Screening Program in Turkey: Struggles and implementations between 2004 and 2008

Objective

In this review, we have presented the data of our National Newborn Hearing Screening Program (NNHSP) with total 764,352 newborns those screened in last five years.

Methods

National Newborn Hearing Screening Program (NNHSP) has been conducted in Turkey since the year 2003. National Newborn Hearing Screening Program (NNHSP) had begun at the end of 2003 only in 1 center. After birth, in the third day, Transient Evoked Otoacoustic Emissions (TEOAEs) test criteria and if necessary, auditory brain response (ABR) testing evaluation methods were applied to newborn. The children diagnosed with hearing loss were further referred for advanced treatment and rehabilitation to advanced audiologic centers.

Results

After five years of carrying out the program (between 2004 and 2008) a total number of 764,352 newborns were screened for hearing impairment. In the year 2008, National Newborn Hearing Screening Program (NNHSP) had given the chance for 2136 children with various types of hearing loss (320 with unilateral and 417 with bilateral hearing loss) to detect and refer to more experienced centers for further treatment.

Conclusions

Our results indicate that the necessity of newborn hearing screening is an indispensable issue. We have been targeted to develop National Newborn Hearing Screening Program (NNHSP) till given chance to access for every newborn in Turkey in next five years.     

Newborn hearing screening in a developing country: Results of a pilot study in Abidjan, Côte d’ivoire

Objective

To investigate the feasibility of neonatal hearing impairment in newborn babies in Abidjan, Côte d’Ivoire.

Methods

It is a cross-sectional study in which all infants aged from 3 to 28 days, attending for Bacille Calmette-Guerin (BCG) immunization in primary care centers or hospitalized in neonatal intensive care units (NICU), between July 2007 and March 2008, were included. Screening followed a two-stage strategy with transient evoked otoacoustic emissions (TEOAE). Infants referred after the second-stage screening were scheduled for diagnostic evaluation by diagnostic auditory brainstem response (ABR). The variables analyzed were: screening coverage, referral rates; return rates for second-stage screening and diagnostic evaluation, incidence of permanent hearing loss and age at diagnosis.

Results

1306 newborns, of a total of 1495, were successfully screened, giving a screening coverage of 87.4%. The average age was 4.5 days (S.D.: 2.7), with 5.85 days (S.D.: 3.17) for the immunization group and 3.20 days (S.D.: 0.40) for the neonatal unit group. In total, 286 out of the 1306 infants (21.9%) were referred after the first-stage screening; out of which 193 (67.5%) return for the second stage. After the second-stage screening, 48 (16.8%) were scheduled for diagnostic evaluation (45 from NICU and 3 from primary care centers). The overall referral rate for diagnostic evaluation was 3.7% (48/1306). Only 18.75% of those referred (9/48) returned for evaluation, and seven of them (77.8%) were confirmed with hearing loss (2 from immunization group and 5 from neonatal unit group). The prevalence of permanent hearing loss in this screened population was 5.96 per 1000 (7/1174 babies who completed the screening) [95% I.C.: 5.62-6.30 per 1000]. The mean age at diagnosis was 22 weeks (S.D.: 8.3). The reasons for non-completed screening were, according to 62 mothers: no financial means, absence of hearing loss, fear of spouse reactions, lack of information about this test and deafness.

Conclusion

The incidence of permanent and early hearing impairment identified by this screening program was about 6 per 1000. Routine hearing screening of infants for the early detection of hearing loss is necessary in Côte d’Ivoire. It is possible to implement such a hearing screening, targeting all newborns, in primary health care centers and neonatal intensive care units.     

A questionnaire-based study on parental satisfaction with a universal newborn hearing screening program in Kuala Lumpur,Malaysia

Objectives

The present study aimed to determine levels of parents’ satisfaction associated with the universal newborn hearing screening process in a university hospital setting in Kuala Lumpur, Malaysia.

Methods

Parents whose babies had undergone a hearing screening test at the Pusat Perubatan Universiti Kebangsaan (Universiti Kebangsaan Malaysia Medical Center), Kuala Lumpur, Malaysia, participated in this study. In this study, the original English version of the Parent Satisfaction Questionnaire with Neonatal Hearing Screening Program (PSQ-NHSP) was translated and adapted into Malay language. Thus, this self-administered Malay version of PSQ-NHSP was used to measure parents’ satisfaction on information of newborn hearing screening program, personnel in charge of the hearing testing, hearing screening activities, and overall satisfaction.

Results

Of the 200 questionnaires distributed, 119 parents (59.5%) responded. Overall, more than 80% of parents were satisfied with the program. The highest percentage of satisfaction (95.6%) was related to the contents of an information leaflet. However, parents were not satisfied with items measuring communication aspect of personnel in charge of the hearing screening. In specific, 38.1% of parents were not satisfied with the explanations and information provided by the screeners on the test procedures, while 26.1% of parents found that the information they received on the test results was insufficient.

Conclusions

The findings of the present study revealed that parents were generally satisfied with the UNHS program. However, further intervention is required to improve the communication aspects of the personnel in charge of the hearing testing. Results suggest that the questionnaire is easily employed and effective tool for assessing parental satisfaction with newborn hearing screening programs. Additionally, this study has demonstrated the survey tool to be useful in identifying areas that need changes or improvements.     

Children with hearing impairment--living with cochlear implants or hearing aids

Objective

The aim of this study was to enhance knowledge about the life circumstances of children with cochlear implants or hearing aids, regarding daily functioning and attitude to the impairment.

Methods

Data were obtained from 36 children with cochlear implants and 38 children with hearing aids via study-specific questionnaires with fixed answer alternatives. The questions covered (1) usage of aids and related factors, (2) hearing in different everyday situations, (3) thoughts about the children's own hearing and others’ attitudes to it, and (4) choice of language. The data were analyzed using SPSS, and presented via the theoretical frame of the International Classification of Functioning, Disability and Health, Child and Youth version (ICF-CY).

Results

Children with CI and HA functioned equally well in daily life, but there were also certain differences. Symptoms from neck and shoulders were more common among children with hearing aids than among children with cochlear implants (p < .001). Children with hearing aids used their aids significantly less often than those with cochlear implants (p < .001). The participation variables showed that children with hearing aids had significantly more hearing problems in team sports (p = .033) and outdoor activities (p = .019), in comparison to children with cochlear implants. The two groups had similar thoughts regarding their own hearing, mostly considering it not to be a problem. They also did not generally think that other people found their hearing to be a problem.

Conclusions

Children with cochlear implants and children with hearing aids have, in some aspects, equally good functioning in everyday life situations. However, certain differences were found in dimensions of functioning, regarding neck and shoulder pain, usage of aids and sign language, and hearing problems in some activities.     

An initial overestimation of sensorineural hearing loss in NICU infants after failure on neonatal hearing screening

Objective

Infants admitted to neonatal intensive care units have a higher incidence of significant congenital hearing loss. We classified audiologic diagnoses and follow-up in infants who had been admitted to our neonatal intensive care unit.

Methods

We included all infants admitted to the neonatal intensive care unit at Sophia Children's Hospital between 2004 and 2009 who had been referred for auditory brainstem response measurement after failing neonatal hearing screening with automated auditory brainstem response. We retrospectively analyzed the results of auditory brainstem response measurement.

Results

Between 2004 and 2009 3316 infants admitted to our neonatal intensive care unit had neonatal hearing screening. 103 infants failed neonatal hearing screening: 46 girls and 57 boys. After first auditory brainstem response measurement we found 18% had normal hearing or a minimal hearing loss. The remainder had a type of hearing loss, distributed as follows: 15% conductive, 32% symmetric sensorineural, 14% asymmetric sensorineural, and 21% absent auditory brainstem responses. Repeated auditory brainstem response measurement showed a shift in hearing outcome. The main difference was an improvement from symmetric sensorineural hearing loss to normal hearing. However, in a small percentage of children, the hearing deteriorated.

Conclusions

As many as 58% of infants in this high-risk population who failed the neonatal hearing screening were diagnosed with sensorineural hearing loss or absent auditory brainstem responses. An initial overestimation of sensorineural hearing loss of about 10% was seen at first auditory brainstem response measurement. This may be partially explained by a conductive component that has resolved. Finally, in a small percentage of children the hearing deteriorated.