The Development of a Paediatric Phoneme Discrimination Test for Arabic Phonemic Contrasts.

Objective: The aim of this project was to develop the Arabic CAPT (A-CAPT), a Standard Arabic version of the CHEAR auditory perception test (CAPT) that assesses consonant perception ability in children. Method: This closed-set test was evaluated with normal-hearing children aged 5 to 11 years. Development and validation of the speech materials were accomplished in two experimental phases. Twenty-six children participated in phase I, where the test materials were piloted to ensure that the selected words were age appropriate and that the form of Arabic used was familiar to the children. Sixteen children participated in phase II where test-retest reliability, age effects, and critical differences were measured. A computerized implementation was used to present stimuli and collect responses. Children selected one of four response options displayed on a screen for each trial. Results: Two lists of 32 words were developed with two levels of difficulty, easy and hard. Assessment of test-retest reliability for the final version of the lists showed a strong agreement. A within-subject ANOVA showed no significant difference between test and retest sessions. Performance improved with increasing age. Critical difference values were similar to the British English version of the CAPT. Conclusions: The A-CAPT is an appropriate speech perception test for assessing Arabic-speaking children as young as 5 years old. This test can reliably assess consonant perception ability and monitor changes over time or after an intervention.

Ototoxicity-induced hearing loss and quality of life in survivors of paediatric cancer.

INTRODUCTION: Hearing loss is a permanent and debilitating side-effect of a range of interventions commonly used in the treatment of childhood cancers, primarily ototoxic medications such as cisplatin. The purpose of this study was to examine the impact of hearing loss, in a population already at risk of reduced quality of life due to the cancer and treatment-related factors. METHODS: This study used a questionnaire specifically designed to tap issues relevant to children with hearing loss, the Paediatric Audiology Quality of Life questionnaire (PAQL). Parents of 78 children treated for a wide range of solid tumours and leukaemias completed the PAQL, 41 of whom had sensorineural hearing loss as a result of the cancer treatment. RESULTS: Significant differences between those children with hearing loss and those whose hearing remained normal were found on all four scales of the questionnaire. Children affected by ototoxicity were rated as having poorer quality of life in terms of their ability to communicate with family and peers, their independence, interactions with peers and emotional well-being. CONCLUSION: These results highlight the impact of acquired hearing loss and reinforce the importance of assessing quality of life with a measure capable of tapping the issues of consequence to the population under investigation. They also have implications for the clinical management of children during and following treatment for a wide range of cancers: medically, audiologically, psychologically and educationally.

Systematic Review of Auditory Training in Pediatric Cochlear Implant Recipients.

Objective The purpose of this systematic review is to evaluate the published research in auditory training (AT) for pediatric cochlear implant (CI) recipients. This review investigates whether AT in children with CIs leads to improvements in speech and language development, cognition, and/or quality of life and whether improvements, if any, remain over time post AT intervention. Method A systematic search of 7 databases identified 96 review articles published up until January 2017, 9 of which met the inclusion criteria. Data were extracted and independently assessed for risk of bias and quality of study against a PICOS (participants, intervention, control, outcomes, and study) framework. Results All studies reported improvements in trained AT tasks, including speech discrimination/identification and working memory. Retention of improvements over time was found whenever it was assessed. Transfer of learning was measured in 4 of 6 studies, which assessed generalization. Quality of life was not assessed. Overall, evidence for the included studies was deemed to be of low quality. Conclusion Benefits of AT were illustrated through the improvement in trained tasks, and this was observed in all reviewed studies. Transfer of improvement to other domains and also retention of benefits post AT were evident when assessed, although rarely done. However, higher quality evidence to further examine outcomes of AT in pediatric CI recipients is needed.

Preliminary assessment of the feasibility of using AB words to assess candidacy in adults.

BACKGROUND: Adult cochlear implant (CI) candidacy is assessed in part by the use of speech perception measures. In the United Kingdom the current cut-off point to fall within the CI candidacy range is a score of less than 50% on the BKB sentences presented in quiet (presented at 70 dBSPL). GOAL: The specific goal of this article was to review the benefit of adding the AB word test to the assessment test battery for candidacy. RESULTS: The AB word test scores showed good sensitivity and specificity when calculated based on both word and phoneme scores. The word score equivalent for 50% correct on the BKB sentences was 18.5% and it was 34.5% when the phoneme score was calculated; these scores are in line with those used in centres in Wales (15% AB word score). CONCLUSION: The goal of the British Cochlear Implant Group (BCIG) service evaluation was to determine if the pre-implant assessment measures are appropriate and set at the correct level for determining candidacy, the future analyses will determine whether the speech perception cut-off point for candidacy should be adjusted and whether other more challenging measures should be used in the candidacy evaluation.

High-frequency audiometry reveals high prevalence of aminoglycoside ototoxicity in children with cystic fibrosis.

BACKGROUND: Intravenous aminoglycoside (IV AG) antibiotics, widely used in patients with cystic fibrosis (CF), are known to have ototoxic complications. Despite this, audiological monitoring is not commonly performed and if performed, uses only standard pure-tone audiometry (PTA). The aim of this study was to investigate ototoxicity in CF children, to determine the most appropriate audiological tests and to identify possible risk factors. METHODS: Auditory assessment was performed in CF children using standard pure tone audiometry (PTA), extended high-frequency (EHF) audiometry and distortion-product otoacoustic emissions (DPOAE). RESULTS: 70 CF children, mean (SD) age 10.7 (3.5) years, were recruited. Of the 63 children who received IV AG, 15 (24%) children had ototoxicity detected by EHF audiometry and DPOAE. Standard PTA only detected ototoxicity in 13 children. Eleven of these children had received at least 10 courses of IV AG courses. A 25 to 85 dBHL hearing loss (mean±SD: 57.5±25.7 dBHL) across all EHF frequencies and a significant drop in DPOAE amplitudes at frequencies 4 to 8 kHz were detected. However, standard PTA detected a significant hearing loss (>20 dBHL) only at 8 kHz in 5 of these 15 children and none in 2 subjects who had significantly elevated EHF thresholds. The number of courses of IV AG received, age and lower lung function were shown to be risk factors for ototoxicity. CONCLUSIONS: CF children who had received at least 10 courses of IV AG had a higher risk of ototoxicity. EHF audiometry identified 2 more children with ototoxicity than standard PTA and depending on facilities available, should be the test of choice for detecting ototoxicity in children with CF receiving IV AG.

Normal hearing in a child with the m.1555A>G mutation despite repeated exposure to aminoglycosides. Has the penetrance of this pharmacogenetic interaction been overestimated?

The mtDNA m.1555A>G mutation causes increased susceptibility to aminoglycoside ototoxicity resulting in significant hearing loss in 100% of reported exposed cases. Genetic and audiological assessments were conducted in a sample of 59 children with cystic fibrosis (CF) undergoing aminoglycoside treatment. Of the two m.1555G patients identified one had severe-profound deafness. Surprisingly, the second m.1555G patient exhibited well-preserved hearing despite repeated exposure. This may be a rare case of intact hearing in an m.1555G individual with aminoglycoside use. Alternatively, its penetrance may have been previously overestimated due to recruitment bias. Further studies are required to determine the true penetrance to inform m.1555A>G genetic testing in similar clinical scenarios.

Aminoglycoside antibiotics cochleotoxicity in paediatric cystic fibrosis (CF) patients: A study using extended high-frequency audiometry and distortion product otoacoustic emissions.

UNLABELLED: Despite known ototoxic effects of aminoglycoside (AG) antibiotics, audiological assessment is not routinely undertaken in UK CF patients. Consequently, the incidence of hearing loss is not well established. OBJECTIVE: To document the incidence of hearing loss in cystic fibrosis (CF) children. DESIGN: Hearing function of 45 children from Great Ormond Street Hospital was assessed using pure-tone audiometry up to 20kHz and DPOAEs up to 8kHz. STUDY SAMPLE: 39/45 of participants had received intravenous (IV) AGs, 23 of which received repeated IV AGs every 3 months. RESULTS: In this high exposure group, 8 (21%) had clear signs of ototoxicity; average 8-20kHz thresholds were elevated by ∼50dB and DPOAE amplitudes were >10dB lower at f2 3.2-6.3 kHz. The remaining 31/39 (79%) of AG exposed patients had normal, even exceptionally good hearing. The 21% incidence of ototoxicity we observed is substantial and higher than previously reported. However, our finding of normal hearing in children with equal AG exposure strongly suggests that other unknown factors, possibly genetic susceptibility, influence this outcome. CONCLUSIONS: We recommend comparable auditory testing in all CF patients with high AG exposures. Genetic analysis may help explain the dichotomy in response to AGs found.

Bilateral sequential cochlear implantation in the congenitally deaf child: evidence to support the concept of a 'critical age' after which the second ear is less likely to provide an adequate level of speech perception on its own.

This study attempts to answer the question of whether there is a 'critical age' after which a second contralateral cochlear implant is less likely to provide enough speech perception to be of practical use. The study was not designed to predict factors that determine successful binaural implant use, but to see if there was evidence to help determine the latest age at which the second ear can usefully be implanted, should the first side fail and become unusable.Outcome data, in the form of speech perception test results, were collected from 11 cochlear implant programmes in the UK and one centre in Australia. Forty-seven congenitally bilaterally deaf subjects who received bilateral sequential implants were recruited to the study. The study also included four subjects with congenital unilateral profound deafness who had lost all hearing in their only hearing ear and received a cochlear implant in their unilaterally congenitally deaf ear. Of those 34 subjects for whom complete sets of data were available, the majority (72%) of those receiving their second (or unilateral) implant up to the age of 13 years scored 60 per cent or above in the Bamford Kowal Bench (BKB) sentence test, or equivalent. In contrast, of those nine receiving their second or unilateral implant at the age of 15 or above, none achieved adequate levels of speech perception on formal testing: two scored 29 per cent and 30 per cent, respectively, and the rest seven per cent or less.A discriminant function analysis performed on the data suggests that it is unlikely that a second contralateral implant received after the age of 16 to 18 years will, on its own, provide adequate levels of speech perception. As more children receive sequential bilateral cochlear implants and the pool of data enlarges the situation is likely to become clearer.The results provide support for the concept of a 'critical age' for implanting the second ear in successful congenitally deaf unilateral cochlear implant users. This would argue against 'preserving' the second ear beyond a certain age, in order to use newer models of cochlear implant or for the purpose of hair cell regeneration and similar procedures in the future. The results suggest a new and more absolute reason for bilateral implantation of congenitally deaf children at an early age.