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.     

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.     

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.     

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.     

Appropriate hearing screening in the pediatric patient with head trauma

Purpose

To develop an algorithm for the appropriate audiologic screening of in children with head trauma.

Methods

Participants were the first consecutive 50 children admitted to a children's hospital trauma service after October 1, 2005, whose injuries resulted in a Glasgow Coma Scale (GCS) score ≤13 and/or loss of consciousness (LOC) but no history of hearing loss. Screening tympanometry, otoacoustic emissions testing, and/or routine audiometric evaluation were performed as soon as possible after admission. Age, GCS score, Pediatric Trauma Score, Injury Severity Score, presence of head and neck soft tissue injury, temporal bone fracture, skull fracture not involving the temporal bone, midface/mandible fractures, intracranial abnormality on computed tomography, and cervical fracture were analyzed as risk factors for hearing loss.

Results

Seventeen (34%) of the 50 children had abnormal hearing test results. Fischer's exact test showed abnormal test results were most strongly related to temporal bone fracture (p = 0.0041), non-temporal bone skull fracture (p = 0.0211) and younger age (p = 0.0638).

Conclusions

Any child with head trauma and clinical or radiologic evidence of temporal bone fracture should have early hearing evaluation. Using the proposed algorithm to test children with head trauma and GCS ≤13 and/or LOC and age <3 years or any type of skull fracture may help identify children with hearing loss in a more cost effective manner.     

Findings from multidisciplinary evaluation of children with permanent hearing loss

Objectives

To describe clinical findings from a multidisciplinary program for children with permanent hearing loss (PHL).

Methods

Retrospective chart review at a tertiary care children's hospital. Patients: Two hundred patients charts were selected from the population of 260 children with permanent hearing loss presenting between July 2005 and December 2006. Main outcome measures: PHL etiology; radiographic findings; clinical findings by genetics, ophthalmology, developmental pediatrics, speech pathology, and aural rehabilitation.

Results

Etiology of hearing loss was determined in 60% of subjects. Genetic causes of hearing loss were identified or presumed (positive history of first degree relative with hearing loss) in 27% of the children. Structural ear anomalies were found in 20% of children. Among the 36% of children with CNS imaging, abnormal findings were noted in 32%. There were a high rate of ophthalmological findings (53%) among children seen by ophthalmology (n = 105). Neurodevelopmental evaluations were completed in 58% of subjects and clinically significant findings were noted in 68%. Of the 61% of children who receiving received speech/language evaluations, 77% required intervention. Over half of the 40% of subjects who had an aural rehabilitation evaluation needed therapy. There were not significant differences in rates of findings for children with mild or unilateral hearing loss as compared to children with more severe degrees of hearing loss.

Conclusions

Interdisciplinary medical evaluation of children with PHL allows for the identification and treatment of clinically significant ophthalmologic, neurodevelopmental, genetic, and speech/language disorders. A high rate of CNS and temporal bone abnormalities were identified. These findings provide an understanding of the importance of considering thorough medical and developmental evaluations among children who are deaf/hard of hearing.     

Microarray-based mutation detection of pediatric sporadic nonsyndromic hearing loss in China

Objective

To investigate the molecular etiologic causes of sporadic nonsyndromic hearing loss in Chinese children.

Methods

179 sporadic nonsyndromic hearing loss children were subjected to microarray-based mutation detection for nine hot spot mutations in four of the most common deafness-related genes, including GJB2, SLC26A4, GJB3, and 12s rRNA.

Results

The incidence of positive genetic errors was 43.58% with the current set of target genes in sporadic nonsyndromic hearing loss children. Among them, 25.14% of cases had genetic defects in GJB2, 16.76% of cases had pathogenic mutations in SLC26A4, 1.12% of cases were caused by 12s rRNA mutations, and GJB3 mutation was detected in 0.56% of this group of patients.

Conclusions

Our results demonstrated that genetic factors were important causes for sporadic nonsyndromic hearing loss in Chinese pediatric cases. Mutations of GJB2 and SLC26A4 are two major genetic causes, whereas mutations of GJB3 and 12s rRNA result in the development of hearing loss in a small percentage of sporadic nonsyndromic hearing loss cases. Microarray testing is a helpful and instrumental screening method in the diagnosis of genetic hearing loss.     

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.     

Risk factors for sensorineural hearing loss in NICU infants compared to normal hearing NICU controls

Objectives

To evaluate independent etiologic factors associated with sensorineural hearing loss in infants who have been admitted to the neonatal intensive care unit compared to normal hearing controls.

Method

Between 2004 and 2009, 3366 infants were admitted to the neonatal intensive care unit of Sophia Children's Hospital, of which 3316 were screened with AABR. A total of 103 infants were referred for auditory brainstem response analysis after failure on neonatal hearing screening. We included all infants diagnosed with sensorineural hearing loss. Each patient was matched with two normal hearing controls from the neonatal intensive care unit of the same gender and postconceptional age.The following risk factors were studied: birth weight, dysmorphic features, APGAR scores (at 1, 5 and 10 min), respiratory distress (IRDS), CMV infection, sepsis, meningitis, cerebral bleeding, cerebral infarction, hyperbilirubinemia requiring phototherapy, peak total bilirubin level, furosemide, dexamethason, vancomycin, gentamycin and tobramycin administration.

Results

Fifty-eight infants were diagnosed with sensorineural hearing loss: 26 girls and 32 boys. The incidence of dysmorphic features (P = 0.000), low APGAR score (1 min) (P = 0.01), sepsis (P = 0.003), meningitis (P = 0.013), cerebral bleeding (P = 0.016) and cerebral infarction (P = 0.000) were significantly increased in infants with sensorineural hearing loss compared to normal hearing controls (n = 116).

Conclusion

Dysmorphic features, low APGAR scores at 1 min, sepsis, meningitis, cerebral bleeding and cerebral infarction are associated with sensorineural hearing loss independent of neonatal intensive care unit admittance.     

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.     

Non-organic hearing loss in childhood

Objective

The present study aimed to investigate the etiology, symptoms, diagnosis and prognosis of pediatric patients with non-organic hearing loss (NOHL), and to heighten awareness of this disorder among physicians.

Methods

Between January 2000 and July 2009, we retrospectively reviewed the medical records of 47 pediatric patients (aged 6-18 years of age) diagnosed with NOHL. The diagnosis was made when there were audiometric discrepancies between the subjective and objective hearing thresholds of the patient in the absence of any organic disease.

Results

Eighteen patients presented with unilateral hearing loss, and 29 showed bilateral hearing loss. Five patients received steroid treatment before the correct diagnosis was made, and six had secretory otitis media and underwent a tympanostomy tube placement.

Conclusion

If physicians are unaware of the possibility of NOHL; they may misdiagnose children with idiopathic sudden sensorineural hearing loss and administer high-dose steroid treatments or exploratory tympanotomies. Otoacoustic emissions are abolished when NOHL patients have secretory otitis media. In these cases, after tympanostomy tube placement, they should undergo objective electrophysiologic examinations to reevaluate NOHL.     

A new hearing screening system for preschool children

Objectives

This study aimed to investigate the practical application of Smart Hearing, a new hearing screening system for preschool children.

Methods

The screening system was applied to 6288 preschool children. The system auto-tested hearing thresholds at three frequencies: 1 kHz, 2 kHz, and 4 kHz; a 30 dB hearing level (HL) was the critical intensity for passing. Children with positive results were referred for audiological evaluation (pure tone audiometry, tympanometry and distortion product otoacoustic emissions assessment, etc.). To evaluate the test accuracy, 312 children (5%) were randomly selected to receive audiology assessment.

Results

In this study, 582 children (9.3%) tested positive in the screening, and the referral rate of the four age groups from 3 to 6 years old was 18.8%, 11.9%, 6.5% and 4.0%, respectively. A total of 463 children underwent audiological assessment, of which 12 cases (1.91‰; 95% CI: 0.83‰, 2.99‰) were diagnosed with permanent hearing loss, and 75 cases (1.19%; 95% CI: 0.92%, 1.46%) were diagnosed with temporary conductive hearing loss. No mixed hearing loss was found in this study. The specificity of the system was 92.6% and the sensitivity was only 37.5%.

Conclusions

This screening system is suitable for the universal hearing screening of preschool children above 4 years old, and further improvements of the system are needed to increase its sensitivity.     

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.     

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.     

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.     

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.