Long-term outcome of children undergoing surgery for suspected perilymph fistula.

UNLABELLED: A number of authors have suggested that surgery for suspected perilymph fistula is effective in preventing deterioration of hearing and in improving hearing in some cases in the short term. We present long-term hearing outcome data from 35 children who underwent exploration for presumed perilymph fistula at The Children's Hospital, Sydney, Australia, between 1985 and 1992. METHODS: The pre-operative audiological data (mean of 500, 1000, 2000 and 4000 Hz results) were compared with the most recently available data (range two to 15 years) and the six-month post-operative data. RESULTS: The short-term results showed no significant change in hearing at six months, with a subsequent, statistically significant progression of hearing loss in both operated and non-operated ears (Wilcoxon signed rank test: operated ear, p < 0.017; non-operated ear, p < 0.009). CONCLUSION: In this case series, exploratory surgery for correction of suspected perilymph fistula did not prevent progression of long-term hearing loss.

The use of diagnostic sleep nasendoscopy in the management of children with complex upper airway obstruction.

In this paper we present a prospective study of the role of diagnostic sleep nasendoscopy in the investigation and treatment of 20 children referred to a tertiary children's hospital with obstructive awake apnoea or severe obstructive sleep apnoea (apnoea/hypopnoea index greater than 30/h). We describe the technique of diagnostic sleep nasendoscopy and provide a classification system for documenting the findings. We demonstrate sleep nasendoscopy is an invaluable tool in the management of children who continue to have upper airway obstruction despite previous adenotonsillectomy and in children with airway obstruction due to cerebral palsy, syndromes and craniofacial malformations. With the use of pre- and post-intervention sleep studies we have shown that sleep nasendoscopy is accurate in deciding the most appropriate mode of intervention in the individual child with obstructive breathing, from (continuous positive airway pressure) CPAP to site-specific surgery. We conclude that sleep nasendoscopy combined with rigid laryngo-bronchoscopy should be standard practice in the evaluation of children with complex upper airway obstruction.

Lymphangioma of the tongue presenting as Ludwig's angina.

In this paper we present a case of a 6-year-old child who presented with Ludwig's angina caused by an infected tongue base lymphangioma. We provide a literature review of tongue lymphangioma and Ludwig's angina in the paediatric population. Both Ludwig's angina and tongue base lymphangioma are rare conditions in their own right and a combination of the two has not previously been described in the literature.

A middle ear teratoma causing acute airway obstruction.

A rare benign teratoma of the middle ear, eustachian tube and nasopharynx presenting in an 8-month-old infant, is reported. The true nature of the tumour was not appreciated until after partial surgical resection from the middle ear. This procedure was complicated 3 days later by life-threatening airway obstruction, caused by the tumour slipping from the eustachian tube and obstructing the larynx. This unusual presentation and the pathology of the teratoma are discussed. The case demonstrates the importance of nasopharyngeal examination where middle ear lesions distort the eustachian tube.

When the diagnosis of deafness is wrong.

OBJECTIVE: To describe three children in whom there had been major errors in the diagnosis of hearing loss. CLINICAL FEATURES: In three children (two developmentally delayed, one not developmentally delayed) hearing thresholds obtained by behavioural testing were later proven wrong. This resulted in significant family distress and inappropriate educational approaches. INTERVENTION AND OUTCOME: Electrocochleography and brainstem audiometry were performed, demonstrating normal cochlear function. Simultaneous microinspection of the ears gave information about current or old middle ear disease and the likelihood of past conductive hearing loss. In each case hearing aids could be discarded, enabling parents and teachers to concentrate on one rather than multiple problems. CONCLUSION: Electrocochleography and brainstem audiometry should be used more frequently to check the diagnosis of hearing loss in children who are developmentally delayed, hyperactive or autistic and who do not give consistent responses to behavioural testing. It should also be considered if parents are firmly convinced that the diagnosis of deafness is wrong.

Velocardiofacial (Shprintzen) syndrome: an important syndrome for the dysmorphologist to recognise.

We report the dysmorphological, genetic, and speech therapy aspects of 38 cases of velocardiofacial syndrome presenting to a craniofacial clinic and a specialised children's hospital, to indicate a relatively low incidence of clefting, good response to pharyngoplasty, considerable variability of the syndrome, and two further familial cases. We emphasise the low index of suspicion by paediatricians and paediatric subspecialists which resulted in delayed diagnosis and delayed treatment for the hypernasal speech and velopharyngeal insufficiency for periods of four months to seven years.