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.     

Comparison of two hearing screening programs in the same population: Oto-acoustic emissions (OAE) screening in newborns and behavioral screening when infants

Objective

Hearing screening programs in infancy should identify hearing impairment as early as possible. The two common programs utilize either objective neonatal tests (oto-acoustic emissions (OAE) or automatic auditory brainstem responses (aABR)) or behavioral screening at 7-9 months of age. Most countries employ only one of these options. The uniqueness of this study is the comparison of both hearing screening programs on the same group of children.

Methods

The study was conducted on 1545 children born between the years 1999 and 2003 who were followed up in public well baby clinics in the Jerusalem district. The children were tested with transient oto-acoustic emissions (TEOAE) before discharge from the neonatal ward, and later, at the age of 7-9 months, underwent a behavioral hearing screening test in a public well baby clinic. The results of both hearing screening programs were compared.

Results

The compliance rates were 99.7% for the neonatal testing and 83% for the 7-9 months behavioral testing (p = 0.0001).The failure rate was 4-6% in both screening programs; failure of OAE testing was unilateral in 65% of newborns; at 7-9 months bilateral failure was more common (56%).There was an 11.2% disagreement (kappa coefficient 0.03) between the outcomes of both tests.In another group of 49 known hearing-impaired children, 27 who had undergone newborn screening were diagnosed before the age of behavioral testing. Twelve children had failed either both tests or the only test they underwent. In nine cases, the children had passed one of the hearing screening tests and had failed the other, and one child had passed both tests.

Conclusions

Newborn hearing screening has the advantages of objectivity, early identification, and higher compliance. The major advantage of the later behavioral test is identification of later onset or progressive hearing impairment as well as auditory neuropathy spectrum disorder. Each screening test is testing different entities; hence they are complementary and not interchangeable or superfluous.We recommend a comprehensive two-step hearing screening plan (newborn and later behavioral) with close cooperation between the health care providers involved.     

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.     

The use of a bone-anchored hearing aid (Baha) in children with severe behavioural problems—The Birmingham Baha programme experience

Objective

A trial of a bone conductor is traditionally used to determine whether a bone-anchored hearing aid (Baha) will be beneficial to a child. However there is a subgroup of children where a Baha assessment is not possible due to severe behavioural/mental/sensory disorders. We describe our experience in a small series of such children.

Method

Retrospective case series review of four children at the severe end of the spectrum of behavioural difficulties who underwent Baha implantation where no formal preoperative hearing aid assessment was possible. The Glasgow Children's Benefit Inventory and a visual analogue scale assessing health status were used to determine the benefit of Baha implantation in this group.

Results

There was no surgical morbidity in this group although a more intensive postoperative follow up was required. All four children wore their hearing aids at least 8 h a day. Parents reported a positive impact of the Baha on the behaviour and mood of their children. The Baha showed a positive benefit when assessed using the Glasgow Children's Benefit Inventory and showed a positive change in health status.

Conclusions

We feel that our early experience with Baha in children with severe behavioural difficulties has been positive to date. Multidisciplinary teams should not dismiss these children even if a trial of a bone conductor is not possible. We feel that the bone-anchored hearing aid has been successful in our cases because the children do not physically feel the presence of the hearing aid.     

Hearing aid functioning in the preschool setting: stepping back in time?

Purpose

In order for children with hearing impairment to receive maximum benefit from preschool programs, their amplification devices must be functioning appropriately. The purpose of this study, therefore, was to investigate the functionality of hearing aids in a preschool setting.

Methods

Forty-four hearing aids worn by 24 preschool children, whose ages ranged from three to five years, (mean age = 45.2 months, median age = 48 months) from four state funded aural/oral habilitation preschools were evaluated using listening checks and visual examinations. Hearing aids passing both evaluations were given a pass rating and those failing one or more of the evaluations were given a fail rating. Causes of fail rates were documented.

Results

Over half of the hearing aids tested were determined to have at least one or more problems (n = 27). These problems were detected using listening and visual checks.

Conclusions

Failure rates based on basic listening and visual checks among the hearing aids examined in this study demonstrated no improvement over those reported in the 1970's. The results re-emphasize the need for a national dialogue on this topic.     

Characteristics of hearing impairment in Yemeni children with chronic suppurative otitis media: A case-control study

Background

Chronic suppurative otitis media (CSOM) is a serious disorder particularly in low resource settings. It can lead to disabling hearing impairment and sometimes life-threatening infective complications.

Objective

The aim of the present study was to describe the characteristics of hearing impairment associated with CSOM in Yemeni children.

Methods

A case-control study of 75 children with CSOM and 74 healthy controls. Hearing was assessed by behavioural testing and audiometry.

Results

Cases had lower academic performance than controls (OR 15.31, 95% CI 1.99-322.14, p < 0.001). Disabling hearing impairment >30 dB was present in 51.5% (right ear) and 66.7% (left ear) of children with CSOM.

Conclusion

Disabling hearing impairment was identified as a major health problem in these Yemeni children with CSOM. There is a need for investment to reduce the burden of CSOM and its complications in these communities. Greater attention to the chronic disabling effects of CSOM in children is required in poor communities and low resource settings.     

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.     

Neonatal hearing screening in Benin City

Objective

Congenital hearing loss is a major health care problem that tends to retard the developmental milestones of children. It takes early detection and intervention to avoid a permanent loss in acquisition of speech and cognitive functions. Hence, the importance of hearing screening in all newborns especially in the developing world for accurate statistics and early intervention. Therefore, this work was aimed at detecting the crude prevalence of congenital hearing loss among the newborns in Benin City.

Methodology

Consecutive neonates at designated immunization centers in Benin City metropolis were screened for hearing loss via the detection and analysis of distortion product otoacoustic emissions from both ears. The handheld otoacoustic machine model MAICO 8172 was employed and the outcome results presented in tables. Statistical analysis was performed using SPSS 11.

Results

A total of 400 neonates (218 males and 182 females) were screened for the presence of otoacoustic emission in both ears. Ninety neonates (22.5%) p < 0.05 were referred. Bilateral hearing loss was seen in 26 (6.5%) whereas 64 (16%) had unilateral loss.

Conclusions

The screening tests suggest a high crude prevalence (6.5%) of bilateral neonatal hearing impairment in Benin City necessitating confirmation and intervention. The study fortifies the need for hearing screening among all new born in developing countries.     

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.     

GJB2 and mitochondrial DNA 1555A>G mutations in students with hearing loss in the Hubei Province of China

Objectives

The GJB2 and MTRNR1 1555A > G mutations are the prevalent causes of hearing loss worldwide. However, the mutation profiles of the two genes are dependent on the ethnic or geographic origins. Therefore, this study was to characterize the forms and frequencies of the two genes in 813 students with hearing loss in Hubei province, Central China.

Methods

Blood samples from 813 students were obtained with informed consent. Genomic DNA was extracted from peripheral blood leukocytes. The target fragments were amplified by polymerase chain reaction (PCR). Sequencing (or enzyme digestion) was applied to identify sequence variations.

Results

Ten different mutations were identified in GJB2 in 146 of the 813 (17.96%) patients and 11.81% (96/813) patients had homoplasmic mtDNA 1555A > G mutation.

Conclusions

This study demonstrated the high prevalence of GJB2 and mtDNA 1555A > G mutations in Central Chinese population. Therefore, it will be effective to perform GJB2 and mtDNA 1555A > G mutation analysis for genetic screening for hearing loss in this population.     

Maturation of auditory function related to hearing threshold estimations using the auditory brainstem response during infancy

Objective

The objective of this study was to quantify the maturation of the Auditory Brainstem Response (ABR) at discrete periods during infancy and to provide a means to appropriately estimate hearing thresholds when the ABR is immature.

Methods

A longitudinal study was designed to measure the in situ ABR thresholds of infants using air-conduction tone bursts of 500, 2000 and 4000 Hz. Thresholds were measured using an eardrum-level microphone to eliminate the bias related to coupler-referenced scales such as the dB nHL scale used for adult assessments.

Results

The study found that the in situ thresholds of a sample of normally developing infants decreased significantly during the first 6 months of life. A comparison of these in situ thresholds with those of normal-hearing adults revealed that the ABR response reached maturity in these infants between 4 and 6 months of age for the frequencies 500 and 2000 Hz but remained immature, or elevated, at 6 months of age for 4000 Hz.

Conclusions

The maturation of the ABR should be considered during the estimation of an infant's audiogram and subsequent diagnosis.     

The audiological characteristics of a hereditary Y-linked hearing loss in a Chinese ethnic Tujia pedigree

Objective

To investigate audiometric characteristics of hearing loss in a large Chinese ethnic Tujia family and determine its hereditary type.

Methods

Total 76 live individuals were investigated in the notable 84 members of this family. The detailed audiometric evaluations were undertaken for the proband and his 47 family members. The degrees of sensorineural hearing impairment were defined as an air/bone gap <15 dB hearing loss averaged over 0.5, 1 and 2 kHz. The severity of hearing loss was established based on the hearing ability of the better ear, averaged over 0.5, 1, 2 and 4 kHz, and classified into four categories: mild, moderate, severe and profound.

Results

Nineteen patrilineal relatives of the 76 live members had hearing impairment. The age of onset ranged from 7 to 21 years old with the average of 13.2 years. The audiometric defect was described by auditory curves of a high frequency in 47% of the patients. Affected members in this family demonstrated a non-syndromic, late onset, bilateral, symmetrical, postlingual and sensorineural hearing loss.

Conclusions

The audiometric configuration in males of the pedigree is consistent with the hereditary Y-linked hearing loss. Thus we speculate that a putative gene on the Y chromosome could contribute to the cause of the disease.     

Measuring communicative performance with the FAPCI instrument: preliminary results from normal hearing and cochlear implanted children

Objective

: To develop preliminary “growth curves” of Functioning after Pediatric Cochlear Implantation (FAPCI) scores using a cross-sectional sample of normal hearing children and to compare these curves to trajectories of FAPCI scores in children receiving cochlear implants.

Methods

: Quantile regression was used to develop growth curves from the FAPCI scores of a cross-sectional sample of 82 normal hearing children (age range 7 months-5 years). Trajectories of FAPCI scores from a longitudinal cohort of 75 children with cochlear implants (age range 1-5 years) were compared to these growth curves.

Results

: FAPCI scores were positively associated with increasing age in normal hearing children with a rapid increase in scores observed at earlier ages followed by a plateau at age 3 years. FAPCI trajectories for cochlear-implanted children varied with age at implantation and did not reach a plateau until age 5-6 years.

Conclusion

: Normal hearing children demonstrated increasing FAPCI scores with age, and these preliminary growth curves allow for the interpretation of a cochlear-implanted child's FAPCI scores in comparison to normal hearing children. Additional research using a larger, longitudinal cohort of normal hearing children will be needed to develop definitive normative FAPCI trajectories.     

Implementation of neonatal screening for hearing impairment: Influence on pediatric otitis media surgery in The Netherlands

Objective

Screening for hearing impairment in the Netherlands took place at the age of 9 months for many years but was recently moved to the neonatal period. Since otitis media has its highest prevalence at the age of 9 months, it might be that screening for hearing impairment at this age is linked to treatment of otitis media. We were interested in what the impact would be on the number of children treated with ventilation tubes or adenoidectomy when they were no longer screened at the age of 9 months.

Methods

National birth rates, data regarding screening for hearing impairment at the age of 9 months and at the neonatal period, as well as data regarding adenoidectomy and tube placement were used to study treatment of otitis media in children aged 0-2 years in the Netherlands.

Results

The percentage of children treated with ventilation tubes after implementation of neonatal screening for hearing impairment did not decline (OR 1.198-1.112), and more children were treated at a younger age. The number of children treated with adenoidectomy did however decline (OR 0.724-0.890).

Conclusion

There seems to be an association between the implementation of neonatal screening for hearing impairment and the treatment of otitis media. During the whole studied period there was a slight but statistical significant increase in the odds for tube placement in all children aged 0-23 months. After implementation of the neonatal screen there was a distinct increase in the number of children aged 6-11 months treated with tubes. In the same period a statistical significant decline in the odds of undergoing adenoidectomy was observed.     

Exploring factors that affect the age of cochlear implantation in children

Objective

Early access to sound through early cochlear implantation has been widely advocated for children who do not derive sufficient benefit from acoustic amplification. Early identification through newborn hearing screening should lead to earlier intervention including earlier cochlear implantation when appropriate. Despite earlier diagnosis and the trend towards early implantation, many children are still implanted well into their preschool years. The purpose of this study was to examine the factors that affected late cochlear implantation in children with early onset permanent sensorineural hearing loss.

Methods

Data were examined for 43 children with cochlear implants who were part of a group of 71 children with hearing loss enrolled in a Canadian outcomes study. Eighteen (41.9%) of the 43 children were identified through newborn screening and 25 (58.1%) through medical referral to audiology. Medical chart data were examined to determine age of hearing loss diagnosis, age at cochlear implant candidacy, and age at cochlear implantation. Detailed reviews were conducted to identify the factors that resulted in implantation more than 12 months after hearing loss confirmation.

Results

The median age of diagnosis of hearing loss for all 43 children was 9.0 (IQR: 5.1, 15.8) months and a median of 9.1 (IQR: 5.6, 26.8) months elapsed between diagnosis and unilateral cochlear implantation. The median age at identification for the screened groups was 3.3 months (IQR: 1.4, 7.1) but age at implantation (median 15.8 months: IQR: 5.6, 37.1) was highly variable. Eighteen of 43 children (41.9%) received a cochlear implant more than 12 months after initial hearing loss diagnosis. For many children, diagnosis of hearing loss was not equivalent to the determination of cochlear implant candidacy. Detailed reviews of audiologic profiles and study data indicated that late implantation could be accounted for primarily by progressive hearing loss (11 children), complex medical conditions (4 children) and other miscellaneous factors (3 children).

Conclusions

This study suggests that a substantial number of children will continue to receive cochlear implants well beyond their first birthday primarily due to progressive hearing loss. In addition, other medical conditions may contribute to delayed decisions in pediatric cochlear implantation.     

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.     

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.     

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.