Phonological awareness, vocabulary, and word reading in children who use cochlear implants: does age of implantation explain individual variability in performance outcomes and growth?

The phonological awareness (PA), vocabulary, and word reading abilities of 19 children with cochlear implants (CI) were assessed. Nine children had an implant early (between 2 and 3.6 years) and 10 had an implant later (between 5 and 7 years). Participants were tested twice over a 12-month period on syllable, rhyme, and phoneme awareness (see James et al., 2005). Performance of Cl users was compared against younger hearing children matched for reading level. Two standardized assessments of vocabulary and single word reading were administered. As a group, the children fitted early had better performance outcomes on PA, vocabulary, and reading compared to hearing benchmark groups. The early group had significant growth on rhyme awareness, whereas the late group showed no significant gains in PA over time. There was wide individual variation in performance and growth in the Cl users. Two participants with the best overall development were both fitted with an implant late in childhoo

Cisplatin Ototoxicity in Children

Cisplatin is a highly effective chemotherapy medicine used in the treatment of many childhood cancers. Like all medications, cisplatin has many side effects and as always the treatment of cancer in children is a balance between the risks of the medications used and their potential benefits. While many side effects of cisplatin chemotherapy are reversible, one major side effect is permanent and irreversible hearing loss (ototoxicity) in both ears which may worsen with time. The severity of cisplatin-related ototoxicity is associated with age and the cumulative dose received: the younger the child and the higher the total dose, the more severe the hearing loss may be. The spectrum of hearing loss varies from mild to moderate high tone hearing loss, to profound loss across the hearing range and permanent deafness. In addition to hearing loss, some children, especially adolescents, also experience tinnitus and vertigo. Cisplatin ototoxicity is one of most important of the many long-term effects experienced by children who are cured of their cancer. The burden of this toxicity may be compounded by other long-term health issues that emerge with time. This chapter will focus on cisplatin-induced hearing loss, its mechanisms, its health impact on the young person and ways to mitigate or reduce the severity of ototoxicity. This chapter has been written by a multi-disciplinary team including paediatric oncologists, audiologists, a psychologist, a health scientist and a parent of a child growing up with high frequency hearing loss

Use of Sodium Thiosulfate as an Otoprotectant in Patients With Cancer Treated With Platinum Compounds: A Review of the Literature

PURPOSE Hearing loss occurs in 50%-70% of children treated with cisplatin. Scientific efforts have led to the recent approval of a pediatric formula of intravenous sodium thiosulfate (STS) for otoprotection by the US Food and Drug Administration, the European Medicines Agency, and the Medicines and Health Regulatory Authority in the United Kingdom. To inform stakeholders regarding the clinical utility of STS, the current review summarizes available literature on the efficacy, pharmacokinetics (PK), and safety of systemic STS to minimize cisplatin-induced hearing loss (CIHL). DESIGN A comprehensive narrative review is presented. RESULTS Thirty-one articles were summarized. Overall, systemic STS effectively reduces CIHL in the preclinical and controlled clinical study settings, in both adults and children with cancer. The extent of CIHL reduction depends on the timing and dosing of STS in relation to cisplatin. Both preclinical and clinical data suggest that systemic STS may affect plasma platinum levels, but studies are inconclusive. Delayed systemic administration of STS, at 6 hours after the cisplatin infusion, does not affect cisplatin-induced inhibition of tumor growth or cellular cytotoxicity in the preclinical setting, nor affect cisplatin efficacy and survival in children with localized disease in the clinical setting. CONCLUSION Systemic administration of STS effectively reduces the development and degree of CIHL in both the preclinical and clinical settings. More studies are needed on the PK of STS and cisplatin drug combinations, the efficacy and safety of STS in patients with disseminated disease, and the ability of STS to prevent further deterioration of pre-established hearing loss

Orthographic influences, vocabulary development and phonological awareness in deaf children who use cochlear implants

In the current study, we explore the influence of orthographic knowledge on phonological awareness in children with cochlear implants and compare developmental associations to those found for hearing children matched for word reading level or chronological age. We show an influence of orthographic knowledge on syllable and phoneme awareness in deaf and hearing children, but no orthographic effect on rhyme awareness. Nonorthographic rhyme awareness was a significant predictor of reading outcomes for all groups. However, whereas receptive vocabulary knowledge was the most important predictor of word reading variance in the cochlear implant group, rhyme awareness was the only important predictor of word reading variance in the reading level matched hearing group. Both vocabulary and rhyme awareness were equally important in predicting reading in the chronological age-matched hearing group. The data suggest that both deaf and hearing children are influenced by orthography when making phonological judgments, and that phonological awareness and vocabulary are both important for reading developmen

Cochlear implantation in individuals with Usher type 1 syndrome

To analyze the occurrence of the Usher type 1 (USH1) gene mutations in cochlear implant recipients with deaf-blind Usher syndrome, and to assess the potential effect of these genes and other factors on the therapeutic outcome. Case series study of nine patients with the phenotypic diagnosis of USH1. Mutation analysis of four USH1 genes ( MYO7A, USH1C, CDH23, and PCDH15) by single strand conformational polymorphism (SSCP) and direct sequencing methods. Pre- and post-implantation audiologic tests including pure tone audiometry, speech perception measures, and qualitative assessment of auditory performance. Nine USH1 patients who received their cochlear implants at the University of Miami Ear Institute, Miami, FL, USA, and at the Department of Cochlear Implants, Great Ormond Street Hospital for Children, London, UK. DNA samples from five of the nine patients were available for mutation analysis. Three of the five patients were found to carry USH1 mutations including two with a truncated mutation in CDH23 and one being a digenic inheritance with mutations in CDH23 and PCDH15. We may have failed to detect mutations in the amplicons analyzed, as neither SSCP nor direct sequencing, even combined, detects all mutations present. Our failure to detect mutations in all five patients may also confirm the genetic heterogeneity of USH1 and additional USH1 loci remain to be mapped. Pre-implantation assessment indicated that all of the subjects were pre-linguistically profoundly deaf, had no consistent response to sound, had varying degrees of auditory-oral habilitation. Age at implantation ranged from 2 to 11 years. There was post-implantation improvement in sound detection and speech recognition measures in closed-set format in all patients. Children implanted at an age of 3 years or less showed good open-set speech perception with lip-reading. All patients are implant users. Those patients who do not show open-set perception still use the cochlear implant as an adjunct of lip-reading or total communication. Testing for mutations in the USH1 genes allows early identification and intervention of children with USH1; timely intervention is important to maximize the development of useful auditory-oral communication skills prior to the onset of the visual impairment