Functional outcomes in the Cleft Care UK study--Part 3: oral health and audiology.

OBJECTIVES: To compare oral health and hearing outcomes from the Clinical Standards Advisory Group (CSAG, 1998) and the Cleft Care UK (CCUK, 2013) studies. SETTING AND SAMPLE POPULATION: Two UK-based cross-sectional studies of 5-year-olds born with non-syndromic unilateral cleft lip and palate undertaken 15 years apart. CSAG children were treated in a dispersed model of care with low-volume operators. CCUK children were treated in a centralized, high volume operator system. MATERIALS AND METHODS: Oral health data were collected using a standardized proforma. Hearing was assessed using pure tone audiometry and middle ear status by otoscopy and tympanometry. ENT and hearing history were collected from medical notes and parental report. RESULTS: Oral health was assessed in 264 of 268 children (98.5%). The mean dmft was 2.3, 48% were caries free, and 44.7% had untreated caries. There was no evidence this had changed since the CSAG survey. Oral hygiene was generally good, 96% were enrolled with a dentist. Audiology was assessed in 227 of 268 children (84.7%). Forty-three per cent of children received at least one set of grommets--a 17.6% reduction compared to CSAG. Abnormal middle ear status was apparent in 50.7% of children. There was no change in hearing levels, but more children with hearing loss were managed with hearing aids. CONCLUSIONS: Outcomes for dental caries and hearing were no better in CCUK than in CSAG, although there was reduced use of grommets and increased use of hearing aids. The service specifications and recommendations should be scrutinized and implemented.

Evaluation of hearing thresholds in 3-month-old children with a cleft palate: the basis for a selective policy for ventilation tube insertion at time of palate repair.

Hearing thresholds in children with a cleft palate prior to cleft palate repair are not widely documented, and audiological criteria for short-term ventilation tube insertion do not exist. The aims of this prospective study are to estimate hearing thresholds in 40 children with a cleft palate by 3-month developmental age with auditory brainstem responses (ABRs) under natural sleep and to estimate a hearing threshold guideline for short-term ventilation tube insertion. Our results show a wide range of air conduction hearing thresholds using click ABRs (2-4 Hz), which ranged from 25 to 102 dBnHL in the left ear and from 25 to 80 dBnHL in the right ear with means of 53 and 49 and standard deviations of 17 and 13 respectively. The bone conduction thresholds ranged from 0 to 55 dBnHL with a mean of 26 and a standard deviation of 13. Eighty-three per cent of children had flat, type B, on high-frequency tympanograms, indicative of middle ear effusion. Thirty per cent of the infants had a cleft palate associated with a known syndrome. Currently, it is the authors' practice to use short-term ventilation tubes on a selective basis at the time of cleft palate repair when there is a conductive hearing loss of more than 55 dBnHL in the better ear as determined by ABR with type B high-frequency tympanograms. This threshold level takes into account electrophysiological and auditory pathway maturation discrepancies. With this as the guideline, between 28% and 35% of the children in this study would be eligible for surgery. This criterion still requires further validation.

Cochlear implantation in Jervell and Lange-Nielsen syndrome.

A case of familial prolonged QT interval and congenital sensorineural hearing loss is described emphasising the diagnostic and management implications. Jervell and Lange-Nielsen syndrome is important because of its potential association with sudden death in children with congenital sensorineural deafness. It is known to be associated with mutations of the genes KCNQ1 (KVQTI) and KCNE1 (Isk). The underlying molecular abnormality leads to cardiac and cochlear dysfunction through a potassium channel defect. All children with congenital sensorineural hearing loss who have suffered unexplained syncopal attacks or convulsions should be screened for this syndrome. There is also a strong case for including a 12 lead ECG as part of the investigative work up of all children with congenital sensorineural deafness in whom a firm aetiology has not been established.

The difficulties encountered in completing an audit cycle in a district general hospital.

Clinical audit has brought about tremendous improvements in the quality of clinical practice in all aspects of health care. However, completing an audit cycle presents many obstacles. We present our experience in the difficulties encountered in implementing changes to current practice. The factors involved are discussed.