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. We describe a case-note survey of patients with and without auditory neuropathy, using data from the local newborn hearing screening programme collected prospectively from 2002 to 2007. During this period, 45 050 infants were screened with otoacoustic emissions, 30 patients were diagnosed with suspected severe to profound hearing loss (16 males, 14 females), and 12 of those 30 had auditory neuropathy (six males, six females). Mean gestational age was 33 weeks 1 day in the auditory neuropathy group and 35 weeks in the non-auditory neuropathy group. The most significant risk factors for auditory neuropathy were hyperbilirubinaemia (p=0.018), sepsis (p=0.024), and gentamicin exposure (p=0.024). Children with auditory neuropathy comprise a subgroup of patients with hearing impairment involving different pathologies most commonly associated with the risk factors related to admission to neonatal intensive care units. Improvement is possible with maturity, at least in a minority.

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. On many occasions, the diagnosis is a retrospective one at postmortem examination. The possibility of surgical correction rests on early diagnosis, anatomy, birth weight, and associated anomalies. Early recognition is mandatory, but in our case, the low birth weight precluded consideration for reconstructive surgery.

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. SETTING: Pediatric tertiary referral center for cochlear implantation. PATIENTS: The present study includes 300 consecutive children undergoing implantation, with a mean age at implantation of 5.1 years, ranging from 1.3 to 16.9 years. Of these children, 196 (65%) had congenital deafness of unknown cause. The commonest known cause was meningitis (73 of 300 [24%]) followed by congenital cytomegalovirus infection (17 of 300 [6%]). Children have been followed up regularly after implantation, typically at yearly intervals after the first year. The mean duration of follow-up at the time of the study was 4 years (range, 0.1-14 yr). RESULTS: There were no major perioperative (within 1 d after surgery) or major early postoperative (within 1 wk after surgery) complications. In the same periods, there were 19 and 15 minor complications, respectively. These complications (e.g., eardrum perforation, hematoma, flap swelling, wound infection, temporary facial weakness) settled with conservative treatment or minor intervention. With regard to the late surgical complications (>1 wk after surgery), there were 7 major (e.g., severe flap infection requiring explantation, cholesteatoma, persistent eardrum perforation) and 14 minor complications (e.g., mild flap infection, flap swelling, hematoma). A number of complications were encountered even 14 years after the original operation, and some of them needed repeated interventions, highlighting the importance of long-term follow-up. However, most of the complications occurred very close to the surgical procedure (<1 yr). CONCLUSION: An overall rate of 2.3% for major surgical complications and an overall rate of 16% for minor surgical complications suggest that cochlear implantation is a relatively safe surgical operation in experienced centers. Most surgical complications are minor and can be managed with conservative treatment or minor surgical intervention. However, meticulous attention to surgical detail, especially handling soft tissues and leaving the posterior canal wall intact, and long-term follow-up are of paramount importance in minimizing the incidence of surgical complications.

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. All children were pre-lingually deaf with age at onset of deafness <2 years and age at implantation <6 years. Three and 4 years following implantation all children were assessed using two measures of speech perception. No child was lost to follow-up and no child was excluded from the study for any reason. RESULTS: The most constant predictor of the outcomes was children's learning style explaining upto 29% of the variance. Other significant predictors of the outcome were shorter duration of deafness, young age at implantation and family structure/support. CONCLUSIONS: Intrinsic factors in children and how well they communicate with their environment are of paramount importance to the outcome following implantation. The predictive value of NChIP has been demonstrated and the most important predictors of the outcome were children's learning style, short duration of deafness, young age at implantation, and family structure/support.

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. PATIENTS: The study assessed 200 profoundly deaf children. Fifty-six children (28%) were deafened by meningitis, 132 (66%) were born deaf, and 12 (6%) were deafened from other causes (head injury, viral infection, progressive deafness). RESULTS: Chronological age at the time of assessment (before implantation) was the most common factor of major concern (9% of the children studied) and the pre-implant language and speech abilities of the children was the most common factor of mild to moderate concern, affecting 63% of the children. The second most common factor of mild to moderate concern was duration of deafness (37%) and the third was the learning style of the children (33%). Availability of support services was the least factor of concern as 179 children (90%) did not have any problems in this area. CONCLUSIONS: Earlier identification of deafness (by universal neonatal hearing screening programs) may reduce age at implantation and duration of deafness as areas of concern in the future. However, the pre-implant language and speech abilities of the candidate children, the gap between chronological and language age, and the learning style of children are now emerging as key areas of research in the field. NChIP was found to be a very useful casework tool in the initial evaluation of the deaf children promoting and enhancing interdisciplinary teamwork across the different professionals. NChIP was also used as part of the decision-making process by the cochlear implant programme professionals and as a counselling tool for the parents. Finally, NChIP has helped to identify those children and families who need additional support. In the case of recently established paediatric cochlear implant programmes it would be a 'neutral' yet sensitive way of promoting good interdisciplinary collaboration and also peer support within team discussions when selecting children for cochlear implantation.

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. The reported results show a low surgical complication rate in both children and adults. Reasons for this are discussed.

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. Extensive infected granulation tissue was found around the implant at exploration despite prolonged intravenous treatment with appropriate antimicrobial agents. All devices were explanted and three have been reimplanted at our unit. We discuss our management of these cases, the need for explantation, consideration for reimplantation and their functional outcome following reimplantation. We also highlight how systemic inflammatory markers can be unhelpful in detecting significant infection surrounding a cochlear implant.

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." A four-choice identification test was undertaken by children with normal hearing (N = 29), prelingually deaf children with the Nucleus Spectra-22 implant system using the SPEAK coding strategy (N = 13), postlingually deafened adults with the same implant system (N = 26), and adults with normal hearing (N = 10). The last group undertook the test before and after the stimuli were processed to simulate the transformations introduced by the SPEAK coding strategy. RESULTS: All four groups made use of vocalic information. Simulated processing reduced the use made by normal-hearing adults. Implanted subjects made less use than the other groups, with no significant difference between implanted children and implanted adults. The highest levels of use by implanted subjects were within one standard deviation of the mean level displayed when normal-hearing adults listened to processed stimuli. Analyses showed that the SPEAK strategy distorted formant contours in the vocalic segments of the stimuli in ways that are compatible with the errors of identification made by implanted subjects. CONCLUSIONS: Some children with implants can extract information from a following vowel to disambiguate a preceding fricative noise. The upper limit on this ability may be set by distortions introduced by the implant processor, rather than by the auditory experience of the child.

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. PATIENTS: A consecutive sample of 12 children younger than 2 years at the time of cochlear implantation (8 boys and 4 girls). The cause of hearing loss was meningitis in 6 children and congenital in 6. INTERVENTIONS: Multichannel cochlear implantation using the Nucleus C124M (Cochlear Co, Sydney, Australia) device. Functional outcome was assessed using the Listening Progress Profile and the Categories of Auditory Performance. MAIN OUTCOME MEASURES: Perioperative and postoperative surgical complications and functional outcome. RESULTS: Eight children had a completely patent cochlea. Four children required a 3- to 5-mm drilling to reach the scala tympani because of ossification after meningitis. Full insertion was achieved in 11 patients; the other child received 18 electrodes. One patient had temporary facial nerve weakness; 2 others had wound edema and serous discharge that resolved with conservative management. In the longer term, 1 child experienced a single episode of acute otitis media; another had recurrent episodes of otitis media. Mean Listening Progress Profile scores increased from 1 to 42 and median Categories of Auditory Performance scores increased from 0 to 5 at 2 years postsurgery. Comparison with the scores in the 2- to 5-year group showed no significant differences. No significant tuning difficulties were experienced with all children. CONCLUSIONS: Cochlear implantation is feasible in children younger than 2 years without significant surgical complications or particular tuning difficulties. Functional results 2 years after implantation were as good as or better than those of children who underwent implantation between ages 2 and 5 years.