Measuring communicative performance with the German version of the FAPCI-instrument: normative data and longitudinal results

Objective

The German version of the Functioning after Paediatric Cochlear Implantation (FAPCI) inventory was designed to capture the communicative performance of cochlear implanted children. In order to be able to compare cochlear-implanted children to their normal hearing peers, normative growth curves were designed. Furthermore it was of interest how the communicative performance develops over time and whether it is influenced by age at implantation.

Method

A polynomial regression curve was fit to the data of 133 normal hearing children. This normative curve was compared to individual growth curves of 90 cochlear implanted children. The cochlear-implanted study sample was split up into four groups depending on the age at implantation.

Results

The normative growth curve increases from 1 year of age until saturation is reached with 3 years of age. The individual FAPCI trajectories of cochlear implanted children are heterogeneous, but in general they are delayed in comparison to the normative growth curve. “Early implanted children” follow the development of their normal hearing peers more closely than “later-implanted children”.

Conclusion

The German version of the FAPCI parental questionnaire constitutes an additional instrument in monitoring the communicative performance development of cochlear implanted children and allows for comparison to normal hearing peers.     

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.     

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.     

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.     

Prospective double blind randomized clinical trial comparing 75% versus 95% silver nitrate cauterization in the management of idiopathic childhood epistaxis

Objectives/hypothesis

Primary outcome measure: to evaluate which concentration of silver nitrate cauterization was more efficacious in the management of idiopathic childhood epistaxis. Secondary outcome measures: to evaluate side effects and pain scores of the differing concentrations.

Study design

Prospective double blind randomized clinical trial.

Participants and setting

All children 16 years of age or younger referred by the accident and emergency department or general practitioner, with recurrent idiopathic epistaxis, who met the inclusion criteria entered the trial.Patients were randomized to receive either the 75% or 95% silver nitrate cauterization. Patients were reviewed at two weeks and eight weeks post cauterization. Pain scores, side effects and success of each treatments were recorded at the follow up clinic.

Results

101 patients completed the trial. 52 patients were randomized to receive the 95% concentration, and 49 patients were randomized to receive the 75% cauterization. In the 75% concentration group, 98% of patients had total resolution of their symptoms at the eight-week follow up. Mean pain scores in this group was 1 out of 10. In the 95% group, 90% had total resolution of their symptoms at the eight-week follow up. Mean pain scores in this group was 5 out of 10. There was a statistical difference in efficacy and pain scores (0.01 and 0.001).

Conclusion

We would recommend the use of 75% silver nitrate cauterization in the management of childhood epistaxis, it appears to be more efficacious, has fewer side effects and is better tolerated.     

Articulation skills in Turkish speaking children with cochlear implant

Objective

The benefits of using cochlear implant (CI) on speech perception and production have been documented. The aim of this study is to investigate the effect of age of cochlear implantation performed and length of auditory experience with CI on the development of articulation skills in prelingual children with CI.

Methods

For this purpose, 14 children with CI were administered through the standardized articulation test named AAT (Ankara Articulation Test). In order to evaluate the development of articulation skills, AAT was applied to each child with CI at the first and the fourth years of CI. The test group was selected among those congenitally hearing impaired children who had used hearing aids bilaterally before the age of one and a half, and received intervention after fitting hearing aids. The test group was divided into two subgroups: Group 1 consisted of the children implanted at and below the age of 3 and Group 2 consisted of the children implanted after the age of 3. Evaluations of articulation skills between groups and within groups were performed in the first and fourth years of implantation. In the study, nonparametric statistics have been used to compare the test scores. Mann-Whitney U-test and Wilcoxon test have been used in the comparisons made between groups and within groups, respectively.

Results

While there has not been observed any statistically significant difference between the first year articulation skills of children with CI at and below the age of three and children with CI above the age of 3, for the fourth year this difference has been found to be statistically significant.

Conclusions

The results demonstrate the importance of the early application of CI and length of auditory experience with CI in the development of articulation skills.     

The role of dyslipidemia in sensorineural hearing loss in children

Objective

The relationship between dyslipidemia and hearing is controversial; especially in children, the available evidences are scarce. This study is designed to determine whether dyslipidemia is associated with sensorineural hearing loss in a group of 5-18-year-old children and adolescents.

Methods

Through an analytic cross-sectional study, records of all 5-18-year-old children who attended the pediatric endocrinology clinic of Loghman Hospital in Tehran, Iran, between April 2007 and April 2009 were reviewed. Records with a lipid profile were determined. Lipid profiles were repeated for eligible cases; they were enrolled if the results were the same as before (normal or dyslipidemic). Hearing loss, speech discrimination scores and lipid profiles were analyzed.

Results

When controlled for age and sex, no association between dyslipidemia and sensorineural hearing loss were found. There was also no statistically significant relationship between dyslipidemia and sensorineural hearing loss in different age groups.

Conclusions

Dyslipidemia seems to have no association with sensorineural hearing loss in 5-18 years old children according to this study.     

Language development in children after receiving bilateral cochlear implants between 5 and 18 months

Objective

The objective of this study was to examine receptive and expressive language development in children who received simultaneous bilateral cochlear implants (CIs) between 5 and 18 months of age and to compare the results with language development in chronologically age-matched children with normal hearing.

Methods

The study used a prospective, longitudinal matched-group design. Data were collected in a clinical setting at postoperative cochlear implant check-ups after 3, 6, 9, 12, 18, 24, 36, and 48 months of implant use. The sample included 42 children: 21 cochlear implant users and 21 with normal hearing, matched pairwise according to gender and chronological age. Communication assessments included the LittlEARS questionnaire, the Mullen Scale of Early Learning, and the Minnesota Child Development Inventory.

Results

The cochlear implant users’ hearing function according to LittlEARS was comparable to that of normal-hearing children within 9 months post-implantation. The mean scores after 9 and 12 months were 31 and 33, respectively in the prelingually deaf versus 31 and 34 in the normal-hearing children. The children's receptive and expressive language scores showed that after 12-48 months with cochlear implants, 81% had receptive language skills within the normative range and 57% had expressive language skills within the normative range. The number of children who scored within the normal range increased with increasing CI experience.

Conclusions

The present study showed that prelingually deaf children's ability to develop complex expressive and receptive spoken language after early bilateral implantation appears promising.The majority of the children developed language skills at a faster pace than their hearing ages would suggest and over time achieved expressive and receptive language skills within the normative range.     

Prevalence of c.35delG and p.M34T mutations in the GJB2 gene in Estonia

Objective

The purpose of this study was to determine the prevalence of c.35delG and p.M34T mutations in the GJB2 gene among children with early onset hearing loss and within a general population of Estonia.

Methods

Using an arrayed primer extension assay, we screened 233 probands with early childhood onset hearing loss for 107 different mutations in the GJB2 gene. We then looked for the two most common mutations, c.35delG and p.M34T, in a population of 998 consecutively born Estonian neonates to determine the frequency of these mutations in the general population.

Results

In 115 (49%) of the patients with early onset hearing loss, we found a mutation in at least one allele of the GJB2 gene. Seventy-three (31%) were homozygous for the c.35delG mutation, seven (3%) were homozygous for the p.M34T mutation, and five (2%) had c35delG/p.M34T compound heterozygosity. Other six identified mutations in GJB2 gene occurred rarely. Among the 998 anonymous newborn samples, we detected 45 who were heterozygous for c.35delG, 2 individuals homozygous for c.35delG, and 58 who were heterozygous for p.M34T. Additionally, we detected two c.35delG/p.M34T compound heterozygotes.

Conclusion

The most common GJB2 gene mutations in Estonian children with early onset hearing loss were c.35delG and p.M34T, with c.35delG accounting for 75% of GJB2 alleles. The carrier frequency for c.35delG and p.M34T in a general population of Estonia was 1 in 22 and 1 in 17, respectively, and was higher than in most other countries.     

Sensorineural deafness in Tanzanian children—Is ototoxicity a significant cause? A pilot study

Objectives

9 years experience working in Tanzania revealed a surprisingly large population of profoundly deaf children. The object was to identify the cause.

Methods

200 children between the ages of 5-12 with significant sensorineural hearing loss were identified in Stone town, Zanzibar. These were aged and neighbourhood matched with 218 normal hearing children.A parental questionnaire administered by health workers from the Zanzibar Outreach Programme was used to interview parents of the matched group of deaf and normal hearing children. Outcome measures included the genetic history especially first cousin marriage (common in Zanzibar), rubella, head injury, fever admission, and drug treatment of fever.The limitations were lack of hospital records and it was a retrospective study. The results were analysed from Excel spreadsheets.

Results

36% of the deaf group had an infant fever admission history compared with 4% of the normal hearing group. Nearly all had i.m. quinine and/or gentamicin. The genetic history was equal in both groups, and rubella was rare. Most fevers were assumed to be malaria or pneumonia which are unlikely causes of deafness on their own.

Conclusions

Ototoxic drugs given to sick infants with “fever”, without weight recording or gentamicin level monitoring, was the probable cause of a third of all severe/profound deafened Zanzibarian children.The Ministry of Health and Social Welfare in Zanzibar have reacted by issuing posters to all health facilities advising hearing testing in all children three months after discharge following “fever” admission.This is a pilot study and the apparent findings need to be confirmed by a well designed prospective study as soon as possible.     

Evolution of communication abilities after cochlear implantation in prelingually deaf children

Objective

This study tries to evaluate different factors on communication ability outcomes in cochlear implanted children.

Methods

Communication abilities are studied using the validated APCEI-scale based on five components of the language: cochlear implant acceptance, perceptive language performance, comprehension of the oral orders, expressive language and speech intelligibility.APCEI-scores were calculated every 6 months for the first 2 years, then yearly.The studied variables were: gender, social origin, preoperative residual hearing, age, aetiology of hearing loss, and associated disabilities.

Results

Communication ability scores increased with high socioeconomic level, presence of residual hearing, younger patients when no residual hearing, connexin mutation related deafness, and absence of associated disabilities. No significant difference has been noted between both sexes.

Conclusion

Many different factors influence the evolution of communication abilities of cochlear implanted children.Investigating the cause of hearing loss, presence of associated disabilities and residual hearing before surgery may help to predict outcome and plan appropriate care to those children with negative predictive factors.     

Evaluation of auditory development in infants and toddlers who received cochlear implants under the age of 24 months with the LittlEARS Auditory Questionnaire

Background and Aims

Newborn hearing screening and early intervention for congenital hearing loss have created a need for tools assessing the hearing development of very young children. A multidisciplinary evaluation of children's development is now becoming standard in clinical practice, though not many reliable diagnostic instruments exist. For this reason, the LittlEARS^® Auditory Questionnaire (LEAQ) was created to assess the auditory skills of a growing population of infants and toddlers who receive hearing instruments. The LEAQ relies on parent report, which has been shown to be a reliable way of assessing child development. Results with this tool in a group of children who received very early cochlear implantation are presented.

Methods

The LEAQ is the first module of the LittlEARS^® comprehensive test battery for children under the age of two who have normal hearing (NH), cochlear implants (CIs) or hearing aids (HAs). The LEAQ is a parent questionnaire comprised of 35 “yes/no” questions which can be completed by parents in less than 10 min. Sixty-three children who received unilateral CIs at a young age were assessed longitudinally and their performance was compared to that of a NH group.

Results

All CI children reached the maximum possible score on the LEAQ on average by 22 months of hearing age, i.e. 38 months of chronological age. In comparison, the NH group reached the maximum score by 24 months of age demonstrating that auditory skills of CI children often develop quicker than those of NH children. In the two comparison groups of children aged (a) younger and older than 12 months, and (b) between 6-9 and 21-24 months at first fitting, the early implanted children reached the highest scores faster than the later implanted children. Furthermore, three children with additional needs were tested. They showed slower growth over time but also received benefits from early implantation.

Conclusions

The LEAQ is a quick and effective tool for assessing auditory skills of very young children with or without hearing loss. In our study, the auditory skills of children with CI progressed very quickly after implantation and were comparable with those of NH peers.     

Auditory evoked magnetic fields in children with functional hearing loss

Objectives

Abnormal cortical responses in patients with functional hearing loss were evaluated by magnetoencephalography, which can better separate bihemispherical activity than electroencephalography.

Methods

Auditory evoked fields in response to 1 kHz or 2 kHz tone bursts at 80 dB sound pressure level were measured by a helmet-shaped magnetoencephalography system in 22 patients with functional hearing loss (18 females, mean age 13.2 years) as well as 5 control subjects under 10 years old. Waveform, latency, and equivalent current dipole of N100m responses were used to evaluate activity in the bilateral auditory cortices.

Results

Abnormal N100m of the contralateral response to the stimulated ear, either absence or delayed latency in comparison to normal adult subjects, was found in 6 of the 7 patients with functional hearing loss aged 9 years or younger, but in only 3 of the 15 patients aged 10 years or older. However, such abnormalities were also observed in younger control subjects.

Conclusion

Auditory evoked field may be applied to objectively evaluate cortical auditory function in patients with functional hearing loss, but the normal findings for young children have not yet been established.     

The development of auditory skills in infants with isolated Large Vestibular Aqueduct Syndrome after cochlear implantation

Objective

The purpose of this study was to investigate the auditory performance of infants with isolated Large Vestibular Aqueduct Syndrome (LVAS) after cochlear implantation, compare their performance with those of infants with a normal inner ear, and establish a database of auditory development.

Method

435 infants with congenital severe to profound hearing loss participated in this study. 62 infants in group A were diagnosed with isolated LVAS. 373 infants in group B had a normal inner ear. Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to evaluate the development of auditory skills.

Results

The mean scores for auditory ability showed no significant difference between groups A and B. The mean scores for the three different auditory skills increased significantly over time. The differences were statistically significant in mean scores among the three different auditory skills for group B.

Conclusion

Auditory skills of infants with isolated LVAS developed rapidly after cochlear implantation, in a similar manner to those of infants with a normal inner ear. Cochlear implantation is an effective interventional approach and an established therapeutic option for infants with isolated LVAS.     

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.     

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.     

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.     

The importance of acoustic reflex for communication

Purpose

The purpose of the study was to compare the speech recognition capacity between listeners with and without acoustic reflex using different types of noises and intensities.

Materials and methods

We studied 18 women allocated to 2 groups: acoustic reflex present (20 ears) and absent (16 ears). They were presented with 180 disyllable words (90 to each ear), emitted randomly at a fixed intensity of 40 dB above the pure tone average hearing level. At the same time, 3 types of noises were presented ipsilaterally (white, pink, and speech), one at a time, at 3 intensities: 40, 50, and 60 dB above the pure tone average hearing level.

Results

The ages and auditory thresholds were statistically equal between the groups. There was a significant difference in mean number of hits between the 2 groups for the 3 types of noises used. There was also a significant difference in mean number of hits for noise type and intensity when white and pink noise was used at 40 and 50 dB and for all the intensities when speech was used.

Conclusion

Acoustic reflex helps communication in high-noise environments and is more efficient for speech sounds.     

External validation of the LittlEARS® Auditory Questionnaire with English-speaking families of Canadian children with normal hearing

Objective

To examine the external validity of the United Kingdom English version of the LittlEARS^® Auditory Questionnaire with English-speaking families of Canadian children with normal hearing.

Methods

The United Kingdom English version of the LittlEARS was administered to English-speaking families of 130 children with normal hearing in Ontario, Canada. Total scores for these children were compared to German-derived normative values.

Results

There was no significant difference between Canadian and German norms when using the United Kingdom English version of the LittlEARS Auditory Questionnaire.

Conclusions

The United Kingdom English version of the LittlEARS Auditory Questionnaire is appropriate for use with English-speaking families of normal hearing Canadian children.