The extent of hearing impairment amongst Australian Indigenous prisoners in Victoria,and implications for the correctional system

An association between congenital hearing impairmentand deprivation has been suggested, but evidence is limited.We studied children born in Greater Glasgow,1985-94, with bilateral congenital hearing impairment.The children were divided into seven deprivation categoriesusing the Carstairs Deprivation Index. One hundredand twenty-four hearing-impaired children wereborn over the study period, an incidence of 1.18/1000 livebirths. There was a clear association between deprivationcategory and incidence, ranging from 0.47/1000 to1.72/1000. An association with deprivation was seen forchildren with a family history and perinatal problems(such as prematurity and low birth weight). No associationwas found for other aetiological groups such ascraniofacial syndromes or early postnatal infection.Deprivation had no effect on age of diagnosis or hearingaid provision. Deprivation is associated with congenitalhearing impairment, due to more prematurity and lowbirth weight in deprived families, and the fact that familieswith many hearing-impaired members are economicallydisadvantaged. There should be a strong emphasison the needs of the socio-economically disadvantagedwhen planning services for hearing-impaired children.     

A possible mechanism of action of danazol and an ethinylestradiol/ norgestrel combination used as postcoital contraceptive agents

Twenty-seven women requesting postcoital contraception were randomly allocated to take an ethinylestradiol/dl-norgestrel combination or danazol. Urine specimens were assayed for luteinising hormone (LH) and pregnanediol-3-glucuronide (P3G) levels from the day of the postcoital treatment to the next period. In addition, the urine samples of these recruits and 12 additional women were assayed for the Beta-subunit of human chorionic gonadotropin (B-hCG). A consistent pattern of alteration in urinary steroids was lacking, indicating a heterogenous effect on ovarian function. There was no evidence of early pregnancy in successfully treated cases. We suggest that the main mechanism of action of these drugs is at the endometrial level.     

A case of a nasal dermoid presenting as a median upper lip sinus

Nasal dermoids are uncommon developmental abnormalities. Median upper lip fistulas are even rarer and recognized as a separate pathology. The authors present the unusual case of a child with a sinus on the philtrum of the upper lip and a sinus tract passing all the way to the anterior skull base through the nasal septum. The authors propose that nasal dermoids and medial upper lip fistulas have a common aetiology, and may be the same entity. The authors suggest they should all be managed like nasal dermoids and have MRI scanning prior to theatre. If the lesion extends into the nose, excision by external rhinoplasty is the preferred option.     

The morphological effects of dexamethasone on the pituitary-adrenal axis of the rat--a quantitative study

Adult Sprague-Dawley rats received a daily intraperitoneal injection of dexamethasone (100 or 200 micrograms/kg) for 2 or 6 weeks. Quantitative assessment of the anterior pituitary corticotroph population performed using the stereological measurement of volume density (Vv). An indirect assessment of corticotroph function was also made, based on adrenal weight and histology. Adrenal changes were consistent with dose-related inhibition of corticotroph function in all dexamethasone-treated animals. Immunopositive corticotrophs were easily identified and in no treatment group was corticotroph Vv significantly less than in the appropriate vehicle-injected control group. In male animals, there was a significant increase (P less than 0.05) in Vv with 100 micrograms/kg at both 2 and 6 weeks. These results suggest that it is possible to produce inhibition of corticotroph function by the administration of such doses of dexamethasone without causing a significant reduction in the anterior pituitary corticotroph population. This may be related to the mode of negative feedback of such a regime.     

An update in the palliative management of malignant dysphagia.

BACKGROUND: Oesophageal cancer is generally associated with late presentation and poor prognosis. Therefore palliative surgery has been largely superseded by less invasive non-surgical techniques. Once palliation is indicated, the aims of the management should be: the maintenance of oral intake, minimizing hospital stay, relief of pain, elimination of reflux and regurgitation and the prevention of aspiration. METHODS: This study was a review of all published English language data on the palliation of malignant dysphagia between 1994-1999. The Medline and Bids databases were searched and other references were derived from the material perused. Results and CONCLUSIONS: Palliative treatment for oesophageal cancer should be individualized and relate to tumour stage, size and location, the patient's medical condition and his/her personal wishes. The palliative treatment largely includes self-expanding metal stents (SEMS), laser (including photodynamic therapy (PDT)) or a combination of the two to relieve symptoms, this may be employed with or without other treatments such as radiotherapy/chemotherapy (RT/CT) with the aim of reducing tumour bulk and possibly prolonging survival. A multi-disciplinary approach is vital in patients with advanced oesophageal cancer.     

Role of tonsillectomy in histology for adults with unilateral tonsillar enlargement

Unilateral tonsillar enlargement (UTE) may indicate malignancy. The purpose of this study was to evaluate the necessity of tonsillectomy for histological examination as a routine practice for every such case. We attempted to identify every adult who had tonsillectomy in our unit from January 1991 to January 2000 in whom the indication for operation was unilateral tonsillar enlargement. The case notes and pathology records were reviewed. One hundred and seven cases were identified but 9 case records could not be found, leaving 98 cases for analysis. Forty-eight were men and 50 were women, aged 16-85 (median age 32). Twenty-three malignancies were identified (23%). A high degree of clinical suspicion was noted pre-operatively in 37 cases, which included all 23 malignancies. Malignancy was more common in the presence of ulceration, male sex, lymphadenopathy, and age 45 years or more, and when it was the patient who first noticed the tonsillar enlargement. Current smoking and persistent pain were not significantly associated with malignancy. Night sweats and weight loss were uncommon. In many cases the apparent tonsillar enlargement was spurious due to asymmetry of the tonsillar pillars.     

Outcomes of surgery for laryngotracheal stenosis-the parents perspective

Reconstructive laryngeal surgery has been very successful in the treatment of laryngotracheal stenosis. Traditionally authors have used survival and decannulation rates as markers of success.

Objective

To evaluate the outcome of paediatric laryngotracheal surgery from the parents perspective.

Method

Retrospective audit of all children undergoing laryngotracheal reconstruction or cricotracheal resection for laryngotracheal stenosis in a three year period at the Royal Hospital for Sick Children, Glasgow. An open-ended questionnaire was used to assess parental concerns regarding their child's airway, post-operative results and the current status of their voice, breathing and swallowing.

Results

30 patients were eligible for the inclusion of which there were 17 responses (56%). The most common concerns pre-operatively were difficulty breathing, tracheostomy and survival. Post-operatively parents felt that breathing, voice and a reduction in the number of lower respiratory tract infections were the main improvements. We also identified the high level of importance that the parents assigned to their child's independence and ability to lead a normal life.

Conclusion

This study has given us a unique insight into the concerns of parents who have children with laryngotracheal stenosis. It has also allowed us to evaluate our post-operative results from a qualitative perspective through the eyes of the parents.     

Otolaryngological presentations of Cornelia de Lange syndrome

Aim

Children with Cornelia de Lange syndrome frequently present to otolaryngology services with hearing problems. Airway problems have not previously been reported. We wish to describe our experience of the overall management in a series of children with Cornelia de Lange syndrome.

Methods

Retrospective case note review of children diagnosed with Cornelia de Lange syndrome presenting to our department between 2005 and 2014.

Results

Six patients were seen. Airway problems consisted of laryngeal overspill with severe gastroesophageal dysmotility and reflux despite structurally normal airway (1 case), laryngomalacia requiring supraglottoplasty (2 cases), reflux laryngitis with secondary laryngomalacia and coincidental tracheal diverticulum (1 case) choanal atresia requiring stents (1 case) and obstructive sleep apnoea (1 case). Supraglottoplasty produced a dramatic improvement in feeding and breathing in both children who underwent the procedure. Two children had palatal anomalies and one underwent cochlear implantation for a profound sensorineural hearing loss.

Conclusion

Children with Cornelia de Lange syndrome have multifaceted ENT problems. Airway pathology has not previously been described in Cornelia de Lange syndrome but has been common in our experience. We wish to highlight that laryngomalacia in Cornelia de Lange syndrome responds well to supraglottoplasty.     

Risk factors for failure of supraglottoplasty

Objectives

To assess for identifiable risk factors for failed surgical intervention in children with laryngomalacia.

Methods

Retrospective case note review between September 2007 and March 2012. Case notes were reviewed for demographic data, symptoms, co-morbidities, operative technique, postoperative recovery, complications, length of hospital stay including intensive care unit (ICU) care, and resolution of symptoms.

Results

148 children underwent supraglottoplasty. Case notes were available for 115 (78%) patients. 35% (41/115) of cases were females and 65% (74/115) were male. A bimodal age distribution was observed with peaks at 3 months and 3.5 years. Those over one year of age were more likely to have complications (p = 0.035). There was no significant difference in outcomes for age (p > 0.05). In patients less than one year, reflux symptoms were significantly associated with a higher likelihood of failure of the operation (p = 0.013). Patients under one year with pre-operatively identified comorbid conditions were less likely to have an improvement in breathing (p = 0.002). Cold steel was used in 55% (63/115) of cases, laser only in 17% (20/115) cases and a combination of the two techniques in 28%, (32/115). There was no association between the surgical technique used and complications (p = 0.558). There was no association between improvement in symptoms and surgical technique used (p = 0.560). There was a significant association between delayed post-operative neurological diagnosis and failure of the operation (p < 0.001). 21 (18%) patients required a second procedure.

Conclusions

Pre-operative predictors of failure were patients with reflux symptoms (p = 0.013). Patients that required a second procedure were 37 times more likely to have a delayed diagnosis of an underlying neurological condition. Failure of symptoms to improve after supraglottoplasty should alert the clinician to the possibility of an underlying neurological disorder.