A comparison of the consonant production between Dutch children using cochlear implants and children using hearing aids

Objectives

The main purpose of the present study was to compare the consonant error patterns of Dutch prelingually deaf CI children with prelingually hearing-impaired hearing aid (HA) children. The authors hypothesized that subjects using conventional hearing aids would have poorer consonant production skills. Additionally, the impact of the age at implantation (CI) and the degree of hearing loss (HA) was determined.

Methodology

This is a comparative study of 29 prelingually deaf CI children (m.a. 9;0 y) and 32 prelingually hearing-impaired HA children (m.a. 9;11 y) who received their first hearing aid before the age of 2 years. Nineteen CI children were implanted before the age of 5 years. Nine HA children had thresholds above 90 dB (range: 91-105 dB), 15 between 70 and 90 dB (range: 72-90 dB) and 8 below 70 dB (range: 58-68 dB). Speech samples of all the children were elicited by means of a picture naming test and were video-recorded for further phonetic and phonological analysis.

Results

Considerably more phonetic and phonologic errors were observed in the HA children with thresholds above 70 dB (range: 72-105 dB). No notable differences could be found between deaf CI children and HA children with thresholds below 70 dB. Even children implanted after the age of 5 years showed significantly fewer phonetic and phonological errors than HA children.

Conclusion

The consonant production of implanted children is more adequate than the consonant production of HA children with a hearing loss of 70 dB or more. In addition, the results also indicate that even after the age of 5 years, implantation can still have an advantageous effect on a child's consonant production.     

Parent versus child assessment of quality of life in children using cochlear implants

Objective

Children with hearing loss who use cochlear implants have lower quality of life (QoL) in social situations and lower self-esteem than hearing peers. The child's QoL has been assessed primarily by asking the parent rather than asking the child. This poses a problem because parents have difficulty judging less observable aspects like self-esteem and socio-emotional functioning, the domains most affected by hearing loss.

Methods

This case-control study evaluated QoL in 50 preschoolers using a cochlear implant and their parents with the Kiddy KINDL^®, an established QoL measure. Children's responses were compared to a hearing control group and correlated with demographic variables. We used a questionnaire for parents and a face-to-face interview with children. T-tests were used to compare (a) paired parent-child ratings and (b) children with cochlear implants versus normal hearing. Pearson rank correlations were used to compare QoL with demographic variables.

Results

Children using cochlear implants rated overall QoL significantly more positively than their parents (M_Difference = 4.22, p = .03). Child rating of QoL did not differ significantly by auditory status (cochlear implant (82.8) vs. hearing (80.8), p = .42). Overall QoL correlated inversely with cochlear implant experience and chronologic age, but did not correlate with implantation age.

Conclusions

Preschool children using cochlear implants can assess adequately their own QoL, but parents afford valuable complementary perspective on the child's socio-emotional and physical well-being. Preschool children using cochlear implants rate overall QoL measures similar to hearing peers. A constellation of QoL measures should be collected to yield a better understanding of general QoL as well as specific domains centered on hearing loss.     

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.     

Cochlear involvement in Familial Mediterranean Fever: a new feature of an old disease

Objectives

In this study we first aimed to assess the cochlear functions in children with Familial Mediterranean Fever. The second aim was to investigate the correlation between the hearing levels and some clinical features of Familial Mediterranean Fever including the duration of the disease, age at onset, genetic analysis and colchicine use.

Methods

Thirty-four children with Familial Mediterranean Fever and 27 age matched children were included in the study. Following otologic examination, all children underwent audiometric evaluation, including Pure Tone Average measurements and Distortion Product Otoaoustic Emission testing. Audiological results of the two groups were compared and correlation between the audiologic status and clinical parameters of the disease like the duration of disease, age at onset, mutations and colchicine treatment were studied.

Results

Pure tone audiometry hearing levels were within normal levels in both groups. Hearing thresholds of Familial Mediterranean Fever patients were found to be increased at frequencies 8000, 10,000, 12,500 and 16,000 (p < 0.05). In otoacoustic emission evaluation, distortion products and signal-noise ratio of FMF children were lower in the tested frequencies, from 1400 Hz to 4000 Hz (p < 0.05). Interaction of the disease duration and age of disease onset was found to predict hearing levels, distortion products and signal-noise ratios of children with Familial Mediterranean Fever (F value = 2.034; p = 0.033).

Conclusions

To our knowledge this is the first study demonstrating cochlear involvement in children with Familial Mediterranean Fever which showed increased hearing thresholds at higher frequencies in audiometry together with decreased distortion products and signal-noise ratios demonstrated by distortion product otoacoustic emission testing. Similar studies must be carried out on adult patients to see if a clinical hearing impairment develops. The possible mechanisms that cause cochlear involvement and the effect of colchicine treatment on cochlear functions must be enlightened.     

Hearing status in children with congenital cytomegalovirus: up-to-6-years audiological follow-up

Objective

To evaluate the audiological outcome of children with congenital cytomegalovirus infection.

Methods

In a prospective study, the hearing of ninety seven congenitally cytomegalovirus-infected children, born between January 2003 and July 2009, was systematically evaluated until the age of six, applying the Flemish CMV protocol. Depending on the age of the child, the protocol provides hearing evaluation by objective-, play- or conventional audiometry. Symptomatic children with hearing loss at birth were treated with ganciclovir, if parents consented.

Results

Seventy children had a pass on initial screening, 27 had unilateral or bilateral hearing loss. Within the normal hearing group, one asymptomatic and two symptomatic children developed late-onset hearing loss. Within the group with hearing loss, 8 children received ganciclovir, while 8 symptomatic and 11 asymptomatic children did not receive ganciclovir. As for the treated group, 37.5% of the children had stable hearing loss, one child had progressive and one child had fluctuating hearing loss. Improvement of hearing threshold occurred in 37.5% of the children. Among the untreated symptomatic children, hearing loss remained stable in 50%, while progression occurred in 37.5%. In the group of asymptomatic children with hearing loss, hearing loss was most commonly stable (72.7%).Within the group of normal hearing ears at birth (n = 156), there is a significant better progression in pure tone average for ears of asymptomatic subjects in comparison to ears of symptomatic subjects (p ≤ 0.0001). As for the group of ears with hearing loss at birth (n = 38), analysis shows no evidence for a difference in pure tone average progression between the different groups (p = 0.38).

Conclusions

Cytomegalovirus infection may cause hearing loss, in both symptomatic and asymptomatic children. Our data show a significant difference, between both groups, in the progression of pure tone average of normal hearing ears at birth, in favor of the asymptomatic children. This is not the case for ears with hearing loss at birth. However, this may be due to the small number of ears in this group. Our data show the tendency that treatment with ganciclovir increases the likelihood of improvement and reduces the likelihood of deterioration of the hearing.     

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.     

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.     

The Hearing Scale Test for hearing screening of school-age children

Objectives

This study aimed to demonstrate a new modified hearing screening method that can rapidly screen hearing and provide stratified test values for each screened ear of children.

Methods

The proposed Hearing Scale Test (HST) and pure-tone screening (PTS) were applied to 384 school-age children. PTS involved applying four test tones to each tested ear: 500 Hz at 25 dB, and 1000, 2000, 4000 Hz at 20 dB; and classifying the results as “pass” (normal hearing in the ear) or “fail” (possible hearing impairment). The HST employs ten stratified hearing scales from S₁ to S₁₀, with each hearing scale containing four test tones and where adjacent scales differ from each other by 5 dB, ranging from 0 dB (S₁) to 45 dB (S₁₀). The four test tones of hearing scale S₅ are the same hearing criteria and the median reference standard of the stimulus level in the HST. Scales S₁-S₅ on the HST are equivalent to a PTS “pass” result, while S₆-S₁₀ and no response (NR) are equivalent to a PTS “fail” result.

Results

The two screening methods produced comparable “pass” and “fail” results. In the HST, the “pass” results were further stratified as S₁ in 4 ears, S₂ in 52 ears, S₃ in 226 ears, S₄ in 272 ears, and S₅ in 169 ears, while the “fail” results were stratified as S₆ in 23 ears, S₇ in 12 ears, S₈ in 1 ear, S₉ in 2 ears, S₁₀ in 5 ears, and no response (NR) in 2 ears. The hearing screening results of the HST are interpreted as follows: scales S₁-S₅ indicate normal hearing, scales S₆ and S₇ indicate possible hearing impairment, and scales S₈-S₁₀ and NR indicate confirmed hearing impairment.

Conclusions

Conventional PTS only gives a pass/fail result for each screened ear, lacks hearing status assessment, and lacks stratified test values to be recorded for follow-up. In contrast, the HST has stratified hearing scales for each screened ear, which reflects the current hearing status and provides test values that can be recorded for follow-up.     

Investigating child raising attitudes of fathers having or not having a child with hearing loss

Aim

The main goal of this study is investigating child raising attitudes of fathers having or not having a child with hearing loss.

Material and method

The fathers of 20 children with hearing loss who attend the Training Unit of Hearing and Speaking Abilities in Hacettepe University Department of Audiology and Speech Defects and 20 fathers having children who have a normal hearing have participated in this study. Demographic Information Form and P.A.R.I (Parental Attitude Research Instrument) have been used as devices for data collecting.

Results

Sub dimension of overprotection scores (42.76 ± 5.59) of the fathers having a child with hearing loss are found higher on a statistically meaningful level than the scores (40.16 ± 6.55) of fathers who do not have a handicapped child (p < 0.01). Together with this, the fathers having a child with hearing loss get lower scores from the democratic/equality and strict discipline sub dimensions as compared to the fathers who do not have a handicapped child, and this situation is found meaningful statistically (p < 0.01).

Conclusion

It is very important that the father's having enough information and support help the father to be encouraged and feel himself enough for this issue. Moreover, the wrong attitudes and behaviors towards the child can be corrected upon noticing them.     

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.     

A comparison of the speech recognition and pitch ranking abilities of children using a unilateral cochlear implant, bimodal stimulation or bilateral hearing aids

Objective

The present study compared the speech recognition and pitch ranking abilities of normally hearing children (n = 15) to children using a cochlear implant (CI) alone (n = 8), bilateral hearing aids (HAs) (n = 6), or bimodal stimulation (BMS) (n = 9). It was hypothesised that users of BMS would score higher on tasks of speech and pitch perception than children using a CI alone, but not children using HAs.

Methods

Participants were assessed on tasks of monosyllabic word recognition in quiet, sentence recognition in quiet and noise (10 dB signal-to-noise ratio), and a pitch ranking task using pairs of sung vowels one, half, and a quarter of an octave apart.

Results

There were no significant differences between the mean percentage-correct scores of the four participant groups for either words in quiet or sentences in quiet and noise. However, the proportion of bimodal users who scored >80% correct (80%) was significantly greater than the proportion of high-scoring unilateral CI (25%) or bilateral HA users (17%). Contrary to expectations, there was also no significant difference between the pitch ranking scores of users of BMS and users of a CI alone for all three interval sizes (p < 0.05, RM-ANOVA). However participants using only acoustic hearing (i.e. the NH and HA groups) scored significantly higher than participants using electrical stimulation (i.e. the CI and BMS groups) on the pitch ranking task (p < 0.05; RM-ANOVA).

Conclusions

Contrary to findings in postlingually deafened adults, we found no significant bimodal advantage for pitch perception in prelingually deafened children. However, the performance of children using electrical stimulation was significantly poorer than children using only acoustic stimulation. Further research is required to investigate the contribution of the non-implanted ears of users of BMS to pitch perception, and the effect of hearing loss on the development of pitch perception in children.     

The effect of mild hearing loss on academic performance in primary school children

Objectives

To determine the prevalence of mild hearing loss and its association with academic performance among primary school children.

Patients and methods

A comparative cross sectional study was conducted on standard (grade) five school children in a state in Malaysia. Five schools were selected by using simple random sampling. There were three classes (A, B and C) of standard five in each school. Student's selection into these classes was made by respective school administration based on their final term examination in standard four. Class A was for the best academic achievers while class C was for the poorest. Ear examinations were done followed by pure tone audiometry for confirmation of the hearing status. In this study, mild hearing loss was defined as an average threshold of 20-39 dB at 0.5, 1, 2 and 4 kHz. Data analysis was done using SPSS Version 12.0 software. Chi-square test was used to determine the association between mild hearing loss and academic performance. The significant level was set at p = 0.05.

Results

From a total of 257 students, 234 have completed the examinations (response rate = 91.0%). Fifty-three percent of them were boys while 47% were girls. A hundred and forty-nine (63.7%) of them were from class A while 85 (36.3%) were from class C. The prevalence of hearing loss in our school population was 15% (95% CI: 11.0-20.0%). Thirty-two (88.9%) of them were having conductive type. Out of the total, 38.9% were having problems in both ears. The students who have poor academic performance have been shown to be significantly associated with mild hearing loss (p < 0.001).

Conclusions

There was a high prevalence of mild hearing loss among primary school children. This problem could affect their academic performance. Therefore, hearing assessment is highly recommended to be done on every child especially to those who have poor academic achievement.     

Cochlear implant outcomes in children with motor developmental delay

Introduction

Multiple handicapped children and children with syndromes and conditions resulting additional disabilities such as cerebral palsy, global developmental delay and autistic spectrum disorder, are now not routinely precluded from receiving a cochlear implant. The primary focus of this study was to determine the effect of cochlear implants on the speech perception and intelligibility of deaf children with and without motor development delay.

Method

In a cohort study, we compared cochlear implant outcomes in two groups of deaf children with or without motor developmental delay (MDD). Among 262 children with pre-lingual profound hearing loss, 28 (10%) had a motor delay based on Gross Motor Function Classification (GMFC). Children with severe motor delays (classification scale levels 4 and 5) and cognitive delays were excluded. All children completed the Categories of Auditory Perception Scales (CAP) and Speech Intelligibility Rating (SIR) prior to surgery and 24 months after the device was activated.

Result

The mean age for the study population was 4.09 ± 1.86 years. In all 262 patients the mean CAP score after surgery (5.38 ± 0.043) had a marked difference in comparison with the mean score before surgery (0.482 ± 0.018) (P = 0.001). The mean CAP score after surgery for MDD children was 5.03, and was 5.77 for normal motor development children (NMD). The mean SIR score after surgery for MDD children was 2.53, and was 2.66 for NMD children. The final results of CAP and SIR did not have significant difference between NMD children versus MDD children (P > 0.05).

Conclusion

Regarding to the result, we concluded that children with hearing loss and concomitant MDD as an additional disabilities can benefit from cochlear implantation similar to those of NMD.     

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.     

Communicative abilities in toddlers and in early school age children with cleft palate

Objectives

Evaluation of improvement in communicative abilities in children with nonsyndromic cleft palate.

Methods

Longitudinal retrospective case history study. Out of 117 children with cleft lip and/or cleft palate born in 1998, 1999 and 2000 and enrolled in the cleft palate team of the University Medical Centre Groningen (UMCG), 63 children were included in the study; 29 (46%) boys and 34 (54%) girls. From these 63 Dutch speaking children communicative abilities were measured when toddlers and at early school age. Cleft types were cleft lip with or without cleft alveolus (CL ± A; n = 10, 5%), unilateral cleft lip and palate (UCLP; n = 23, 37%), bilateral cleft lip and palate (BCLP; n = 9, 14%) and isolated cleft palate (CP; n = 21, 33%). The percentage of problems in language comprehension, language production, articulation, hearing and hypernasality, present when toddlers, were compared with the percentage of problems found at early school age. The treatments executed were also analysed.

Results

Except for hearing problems, problems in all other communicative fields improved significantly. In the total group language comprehension problems decreased from 23% to 2% (p = 0.00), language production problems from 21% to 6% (p = 0.01), articulation problems from 57% to 25% (p = 0.00) and hypernasality from 38% to 10% (p = 0.04). Hearing problems appeared more difficult to treat effectively, they decreased from 42% to 31% (p = 0.29). Children with BCLP appeared to have the most problems, followed by children with UCLP and then children with CP. Children with CL ± A show the least problems. In the intervening period, often a combination of treatments was performed. Pharyngoplasty appeared to be very successful in treating hypernasality, with a success rate of 86%.

Conclusions

At early school age, in children with clefts, speech and language problems were significantly improved following a multidisciplinary approach to treatment and resemble their peers without clefts. Hearing problems were more difficult to treat.     

Progressive hearing loss after completion of cisplatin chemotherapy is common and more pronounced in children without spontaneous otoacoustic emissions before chemotherapy

Objective

High frequency hearing loss following cisplatin chemotherapy is frequent in children and often necessitates the fitting of hearing aids. During therapy, hearing is usually monitored. Post-therapeutic follow-up does not routinely include monitoring of hearing, although there are indications that hearing thresholds can decline after therapy.

Methods

Pure-tone audiograms taken from 27 children (17 males, 10 females) treated with cisplatin at Muenster university hospital (mean age 9.84 years, standard deviation 3.67 years) including an audiological follow-up at least 6 months after therapy, were analyzed retrospectively.

Results

In follow-up tests after completion of therapy, 24.1% of all ears showed an increase in mean high frequency hearing thresholds (4-8 kHz). Post-therapeutic hearing deterioration was significant at 4 kHz and significantly more pronounced in children without measurable spontaneous otoacoustic emissions (SOAE) before therapy. Post-therapeutic hearing deterioration did not occur in ears with normal pure tone thresholds (≤10 dB at all frequencies) after cisplatin therapy. No correlation was found between post-therapeutic hearing deterioration and cranial irradiation.

Conclusions

Cisplatin chemotherapy follow-up should include audiological monitoring in all children with elevated pure tone thresholds after therapy. Routine SOAE measurements taken as part of baseline audiometry before the start of chemotherapy can be taken into consideration.     

BAHA results in children at one year follow-up: A prospective longitudinal study

Objective

To evaluate audiometric and clinical results of children fitted with a bone-anchored hearing aid with specific emphasis on speech discrimination in different sound environments after one year of use.

Methods

We performed a prospective longitudinal study. Seventeen patients between the ages of 5 and 18 years old were included. All patients underwent a complete tonal and vocal evaluation at four pre-determined intervals between the pre-operative period and one-year of bone-anchored hearing aid (BAHA) use. Basic pure-tone average and speech reception threshold were measured in different sound environments. Speech discrimination improvement was tested with the voice originating from the side of the BAHA-fitted ear and with the voice originating from a source directly in front of the patient. These measures were repeated with confounding noise facing the patient then from the side of the affected ear. All tonal and vocal evaluations were performed pre-operatively, the day of processor insertion, 6 months and 12 months after processor insertion.A variance analysis was performed to compare differences in hearing gain with BAHA over time.

Results

Hearing gain with BAHA was clinically and statistically significant at all intervals. Conventional tonal evaluation revealed significantly improved hearing gain after BAHA insertion compared with pre-operative testing with BAHA (26.3 dB vs. 17.3 dB), and this improvement was maintained at one year (27.9 dB). Speech discrimination gain at one year was better than immediately post-insertion (21.9% vs. 11.7%). Maximal gain with BAHA was found with the voice originating from the side of the affected ear and with confounding noise facing the patient (27.1% at one year), whereas the least gain was found in a silent room with the voice coming from straight ahead (11.9% at one year).

Conclusions

Pure-tone average gain at one year post-insertion was similar to immediate post-insertion gain. BAHA aids speech discrimination most when the voice originates from the side of the affected ear with confounding noise facing the patient. Speech discrimination gain improves with time, suggesting an underlying learning process. The best BAHA gain in speech discrimination occurred with background noise.     

Spatial hearing of normally hearing and cochlear implanted children

Objective

Spatial hearing uses both monaural and binaural mechanisms that require sensitive hearing for normal function. Deaf children using either bilateral (BCI) or unilateral (UCI) cochlear implants would thus be expected to have poorer spatial hearing than normally hearing (NH) children. However, the relationship between spatial hearing in these various listener groups has not previously been extensively tested under ecologically valid conditions using a homogeneous group of children who are UCI users. We predicted that NH listeners would outperform BCI listeners who would, in turn, outperform UCI listeners.

Methods

We tested two methods of spatial hearing to provide norms for NH and UCI using children and preliminary data for BCI users. NH children (n = 40) were age matched (6-15 years) to UCI (n = 12) and BCI (n = 6) listeners. Testing used a horizontal ring of loudspeakers within a booth in a hospital outpatient clinic. In a ‘lateral release’ task, single nouns were presented frontally, and masking noises were presented frontally, or 90° left or right. In a ‘localization’ task, allowing head movements, nouns were presented from loudspeakers separated by 30°, 60° or 120° about the midline.

Results

Normally hearing children improved with age in speech detection in noise, but not in quiet or in lateral release. Implant users performed more poorly on all tasks. For frontal signals and noise, UCI and BCI listeners did not differ. For lateral noise, BCI listeners performed better on both sides (within ∼2 dB of NH), whereas UCI listeners benefited only when the noise was opposite the unimplanted ear. Both the BCI and, surprisingly, the UCI listeners performed better than chance at all loudspeaker separations on the ecologically valid, localization task. However, the BCI listeners performed about twice as well and, in two cases, approached the performance of NH children.

Conclusion

Children using either UCI or BCI have useful spatial hearing. BCI listeners gain benefits on both sides, and localize better, but not as well as NH listeners.     

Identification of novel variants in the TMIE gene of patients with nonsyndromic hearing loss

Objective

To determine whether variants of the TMIE gene are causes of nonsyndromic deafness in Taiwan.

Methods

A genetic survey was made from 370 individuals, with 250 nonsyndromic hearing loss and 120 normal hearing individuals. Genomic DNA was extracted from peripheral blood leukocytes and then subjected to PCR to amplify selected exons and flanking introns of the TMIE gene; the amplified products were screened for base variants by autosequence. Data from the two groups were then compared using Fisher's two-tailed exact test and Armitage's trend test.

Results

The analysis revealed 7 novel variants in the TMIE gene. Of the 7 variants, 5 variants were found in both nonsyndromic hearing loss and normal hearing group. Both allelic and genotype frequencies of these sequence changes did not differ significantly between patients and controls (P > 0.05). However, a missense variant (c.257G > A) and one promoter variant (g.1-219A > T) were found in two patients with nonsyndromic hearing loss. Family study and microsatellite analysis found that c.257G > A variant is not inherited from his parents. The c.257G > A variant encodes a protein with glutamine at position 86 instead of arginine (p.R86Q), a residue that is conserved in mammals but different in fish, and predicted to be extracellular.

Conclusions

Despite the fact that the frequency of TMIE variants in our study subjects was low, we suggested that c.257G > A (p.R86Q) variant is a de novo and may be as a risk factor for the development of hearing loss in Taiwanese.     

Children with cochlear implants: Cognitive skills,adaptive behaviors,social and emotional skills

Objective

The aim of this study is to examine cognitive skills, adaptive behavior, social and emotional skills in deaf children with cochlear implant (CI) compared to normal hearing children.

Methods

The study included twenty children affected by profound hearing loss implanted with a CI compared to 20 healthy children matched to chronological age and gender.

Results

Results of this study indicated that 55% of children with CI showed a score in the normal range of nonverbal intelligence (IQ > 84), 40% in the borderline range (71 < IQ < 84) and 5% were in mild range (50 < IQ < 70). No significant differences were found after comparison with normal hearing children. Children with CI reported more abnormalities in emotional symptoms (p = .018) and peer problems (p = .037) than children with normal hearing. Age of CI was negatively correlated with IQ (p = .002), positively correlated with emotional symptoms (p = .04) and with peer problems (p = .02).

Conclusions

CI has a positive effect on the lives of deaf children, especially if it is implanted in much earlier ages.