Auditory neuropathy: unexpectedly common in a screened newborn population.

Auditory neuropathy, or dyssynchrony, is defined by an abnormal or absent auditory brainstem response but intact otoacoustic emissions or cochlear microphonics. It is associated with impaired hearing on behavioural pure-tone audiometry, absent acoustic reflexes, and poor speech perception, particularly in noisy environments. These results suggest a disorder of inner hair-cell and or eighth-nerve function.

Paediatric cochlear implant durability: the Nottingham experience.

Objective: To determine durability of cochlear implant devices in a large paediatric cohort.

Design: Retrospective review of database records of children consecutively implanted between 1989 and March 2002.

Methods: The records of 363 children were studied.

Case report: cochlear implantation in Mondini dysplasia with congenital footplate defect--implications for meningitis risks during implantation.

Following the reports of a cluster of meningitis cases in recently implanted patients the FDA issued cautionary advice relating to the risk of meningitis after cochlear implantation (US Food and Drug Administration, 2002). Similar advice and a national reporting call has been issued by the Department of Health in the UK (Medical Devices Agency, 2002) and universal prophylactic pneumococcal vaccination started. We present a case of bilateral Mondini-type dysplasia associated with a defective stapes footplate and highlight the need for surgical vigilance to reduce the risks of meningitis from undiagnosed anatomical defects.

Infections following cochlear implantation.

Cochlear implantation has become routine in the management of children and adults with profound sensorineural hearing loss. In rare cases postoperative infections necessitate removal of the implant. We present six such cases that have been managed within our programme.

Cochlear implantation--United Kingdom and Ireland surgical survey.

Cochlear implantation has now become a routine procedure in the management of severe to profound deafness. An initial survey was carried out in late 1997 of the surgeons carrying out implantation in the United Kingdom and Ireland, requesting details of medical and surgical aspects of cochlear implantation. A follow-up survey was conducted in early 2002 to evaluate any changes in clinical practice.

Retrospective study of intra-operative testing with the Nucleus CI24M cochlear implant and use of the back-up device.

The combined use of integrity testing (IT) and impedance telemetry (ImTe) intra-operatively is evaluated. One hundred and fifty children implanted with the Nucleus device were studied. In 81% of patients, normal results were obtained on all electrodes from both ImTe and IT.

Tracheal agenesis with unique anatomy.

A premature infant with a unique form of tracheal agenesis is described. The combination of difficulty in intubation, abnormal course of the nasogastric tube on plain x-ray, and gastric perforation raised the suspicion of an upper airway malformation. Tracheal agenesis is an extremely rare, typically fatal, congenital anomaly with scattered case reports of its successful management.

Surgical complications and their management in a series of 300 consecutive pediatric cochlear implantations.

Objective: To report the short- and long-term complications encountered in a large number of consecutive children undergoing implantation in a single center. The current study also describes the management and sequelae of each complication.

Study Design: Prospective study assessing the surgical findings and complications of deaf children undergoing implantation.

Congenital midnasal stenosis in an infant.

Neonates and infants are obligate nasal breathers and nasal obstruction in this age group can be a life threatening emergency. Even though the commonest cause for nasal obstruction is nasal oedema, bony stenosis of the posterior choanae or pyriform aperture is quite common and may be seen on CT scans. We describe a case of mid-nasal stenosis in a neonate and discuss the aetiology and management of nasal obstruction in this age group.

Predicting speech perception outcomes following cochlear implantation using Nottingham children's implant profile (NChIP).

Background/objective: Nottingham children's implant profile (NChIP) is a systematic framework to assess deaf children before implantation taking into account not only the well-known factors (age, duration of deafness) but also family and support services, expectations, children's cognitive abilities and learning style. The aim of the present paper is to assess the predictive value of the NChIP in the long-term outcomes of implanted children.

Patients: This prospective and longitudinal study involved 51 profoundly deaf children, implanted within the same paediatric cochlear implant programme.

Assessing candidate children for cochlear implantation with the Nottingham Children's Implant Profile (NChIP): the first 200 children.

Background/objective: Nottingham Children's Implant Profile (NChIP) is a profile designed to assess candidate children for cochlear implantation. It includes the demographic details of the children (chronological age and duration of deafness), medical and radiological conditions, the outcomes of audiological assessments, language and speech abilities, multiple handicaps or disabilities, family structure and support, educational environment, the availability of support services, expectations of the family and deaf child, cognitive abilities, and learning style. The aim of the present study is to present the NChIP data obtained on the first 200 children implanted in the same cochlear implant programme and evaluate NChIP's use in the selection phase of cochlear implantation.

Children with a tracheostomy: experience of their carers in school.

Objective: Tracheostomies in children are increasingly performed for chronic medical conditions. There are no published studies reporting the experience of children with a tracheostomy in school. Such information would be valuable in planning the care and education of these children.

Screening and follow up assessment in three cases of auditory neuropathy.

Three children with auditory neuropathy are described. Two were detected via a targeted neonatal hearing screening programme based on auditory brain stem response testing, and one via the routine Health Visitor Distraction Test. Auditory neuropathy is an important but poorly understood disorder which has implications on planning future hearing screening policy and management of hearing impairment.

Use of vocalic information in the identification of /s/ and /sh/ by children with cochlear implants.

Objective: When a syllable such as "sea" or "she" is spoken, listeners with normal hearing extract evidence of the fricative consonant from both the fricative noise and the following vocalic segment. If the fricative noise is made ambiguous, listeners may still perceive "s" or "sh" categorically, depending on information in the vocalic segment. Do children whose auditory experience comes from electrical stimulation also display this effect, in which a subsequent segment of speech disambiguates an earlier segment?

Design: Unambiguous vowels were appended to ambiguous fricative noises to form tokens of the words "she," "sea," "shoe," and "Sue.

Surgery and functional outcomes in deaf children receiving cochlear implants before age 2 years.

Objective: To examine the feasibility of cochlear implantation in children younger than 2 years regarding surgery and functional outcomes.

Design: Prospective study.

Setting: Tertiary pediatric cochlear implant center.

Intra-operative recordings of electrically evoked auditory nerve action potentials in young children by use of neural response telemetry with the nucleus C124M cochlear implant.

The electrically evoked action potential (EAP) was recorded intra-operatively by use of neural response telemetry (NRT) on the Nucleus C124M cochlear implant. The aim of the present study was to investigate the EAP in young children immediately following implant surgery and whilst the children were still anaesthetized. The effect of data collection parameters on the reliability of the EAP was assessed and the relationships of the EAP findings to the intra-operative electrical auditory brainstem response (EABR) and early behavioural threshold levels (T-levels) were also investigated.

Functionally equivalent ages and hearing levels of children with cochlear implants measured with pre-recorded stimuli.

Three groups of children undertook an interactive computer-based closed-set test of the ability to identify pre-recorded spoken words presented acoustically. The test was completed by 31/39 children with profound hearing loss who had used the Nucleus Spectra-22 cochlear-implant system for at least one year (Group A); by 30 children with normal hearing (Group B); and by 22 children with severe-profound hearing loss who used acoustic hearing aids (Group C). Among the implanted children, those who were younger when implanted and who had used their devices for longer produced higher scores (multiple-r = 0.

Cochlear implantation in children with large vestibular aqueduct syndrome and a review of the syndrome.

Objective: Children with Large Vestibular Aqueduct Syndrome (LVAS) frequently develop speech and language skills prior to deterioration of their hearing. Operations designed to halt the progression of hearing loss have largely failed so the question of Cochlear Implantation in these children has arisen. It had been suggested that there would be technical difficulties in implanting these patients and, therefore, there had been an initial reluctance to proceed to implantation.

Cochlear implants in children.

Cochlear implants are electronic prostheses that provide a high quality sense of hearing to severely and profoundly deaf children and adults. As improvements in surgical technique and device performance have occurred, indications for implantation have expanded.

Assessment of the functioning of peripheral auditory pathways after cochlear re-implantation in young children using intra-operative objective measures.

The intra-operative electrical auditory brainstem response (EABR), electrical stapedius reflex threshold (ESRT) and the early post-operative behavioural threshold level (T-level) were recorded in five children undergoing cochlear re-implantation. The aim of the study was to assess objectively the effect of re-implantation on intra-operative objective measures and to investigate neuronal function. The children were aged between 2.

The prognostic value of promontory electric auditory brain stem response in pediatric cochlear implantation.

Objective: To test the hypothesis that children with clear promontory electrically evoked auditory brain stem responses (prom-EABRs) would outperform, after cochlear implantation, children who had no prom-EABR preoperatively.

Design: A prospective study was undertaken on 47 implanted children assigning them to two groups (group A: 35 children with a clear wave e-V in the preoperative prom-EABR and group B: 12 children with no prom-EABR). Speech perception and speech intelligibility were assessed annually up to 3 yr after implantation with the IOWA sentence test (level A and level B), Connected Discourse Tracking, Categories of Auditory Performance, and Speech Intelligibility Rating.