Cochlear implants in children with craniofacial syndromes: assessment and outcomes.

The aim of this retrospective study was to review the outcomes for children with craniofacial syndromes who had received a cochlear implant. The group comprised four children (three girls, one boy) aged between 3.3 and 10.1 years (mean 6.3 years) at time of implantation with the Cochlear CI-22M device. Two children had the CHARGE association. one had Goldenhar's syndrome and one had brachio-oculo-facial syndrome. All had full electrode insertion at time of surgery. At follow-up, three of the children demonstrated benefit in detection, recognition and identification of environmental sounds, and they continued to gain receptive spoken language skills, although none had intelligible speech. The group required careful mapping and higher levels of electrical stimulation of the implant compared to normal child implantees. Stimulation of the facial nerve was a problem with one child. The pre-implantation assessment of these children requires extensive interdisciplinary discussion and careful radiological investigation. Cases should be carefully selected. Parents should receive realistic counselling about outcomes and the time commitment necessary, as habilitation of these children can take twice as long as that of children without additional special needs. Post-implantation, these children continue to require well-coordinated medical and interdisciplinary management.

A study of the application of a frequency transposition hearing system in children.

The aim of this retrospective study was to investigate the use of the frequency transposition Transonic FT 40 system in a group of 36 children with profound sensorineural hearing loss. The group comprised 36 children (11 boys, 25 girls) aged between 2.8 and 15.6 years (mean 7.6 years) at fitting of the FT 40 device. At 48 months post-FT 40 fitting, only 11 children (30%) were still wearing the device. The children discontinued wearing the FT 40 for the following reasons: ergonomic (11%); no perceived benefit from the system (11%); cosmetic (17%); and subsequent cochlear implantation (30%). The performance of the long-term FT 40 users was investigated using the following outcome measures: aided soundfield hearing thresholds: closed set speech tests (the E2L toy test and the Manchester Picture Test discrimination test) and a speech intelligibility rating score. The 11 long-term FT 40 users (three boys, eight girls) were aged from 5.3 to 12.9 years (mean 7.2 years) at the time of initial fitting of the FT 40 device. At time of fitting, the aided soundfield thresholds with the FT 40 were significantly better at 500 Hz (p<0.04), 1 kHz (p<0.019), 2 kHz (p<0.001) and 4 kHz (p <0.001) compared to thresholds with conventional hearing aids. Six of 11 children did not show any change in performance on the closed set speech tests and two children had intelligible speech at 48 months' follow-up. A small subgroup of good performers was identified. These children were younger at age of fitting (mean 6.2 years compared to a mean of 7.7 years for the remainder of the group), were predominantly oral communicators and had identified aetiologies for their deafness. The present study suggests that there is a small subgroup of hearing-impaired children who benefit from frequency transposition hearing systems, and future suggested fitting criteria and outcome measures are listed.

Parental experiences of a paediatric cochlear implant programme.

Although cochlear implantation is a well-established procedure in profoundly deaf children, very little research has investigated whether parents are satisfied with the treatment (including assessment, surgery and rehabilitation) or its outcome, and whether it has met their expectations. In this study, 44 parents of children who had received cochlear implants completed a confidential postal questionnaire. Results indicated that the majority of parents felt that the information they and their child received was both sufficient and appropriate. Many parents experienced more distress than they anticipated, but perceived their child as having experienced less physical discomfort than expected. Some suggestions for improving the service were made, but in general it was felt that little more could be done to inform our 'consumers', or to reduce levels of stress.

Anticonvulsant drugs, growth, and development.

Height and stage of puberty of 67 children with epilepsy were measured before beginning treatment with anticonvulsant drugs and annually to a maximum five years' treatment. Blood concentrations of the drugs used (phenytoin, sodium valproate, carbamazepine, ethosuximide, and phenobarbitone) were monitored throughout. No significant deviation in growth patterns was detected.