Validation of a family-centred outcome questionnaire for pinnaplasty: a cross-sectional pilot study.

OBJECTIVES: Comparison of pinnaplasty techniques is difficult due to variation in the reporting of outcomes. We aimed to develop a family-centred outcome questionnaire for use after pinnaplasty and assess it for reliability and validity. DESIGN: Cross-sectional study. SETTING: Tertiary referral paediatric otolaryngology centre. PARTICIPANTS: 20 prospective and 71 retrospective pinnaplasty patients and their parent(s). METHODS: The Post-Operative Pinnaplasty Questionnaire (POPQ) was developed after semi-structured interview with families of children undergoing pinnaplasty. Children aged 4-16 were recruited. Three different ear measurements (auriculocephalic angle, helix-mastoid distance and Walker's ratio) were performed pre-operatively. Children were reviewed 3 months post-operatively and asked to complete a POPQ and Glasgow Children's Benefit Inventory with their parent(s). Ear measurements were repeated and data collected on surgical technique and complications. 200 retrospective pinnaplasty patients were posted a POPQ and Glasgow Children's Benefit Inventory and data collected on surgical technique, complication rate and Walker's angle. MAIN OUTCOMES MEASURES: Validity and reliability of the POPQ. RESULTS: Age range was 4-16 (median 12). POPQ correlated well with Glasgow Children's Benefit Inventory (Spearman's rho = 0.776, P < 0.001). There was no correlation of POPQ scores with age, sex, complication or surgical technique. POPQ score was not associated with severe prominence pre-op nor change or degree of prominence post-op. POPQ displayed good internal consistency (Cronbach's alpha 0.850) and ease-of-use scores. CONCLUSIONS: We have developed a family-focused outcome tool for pinnaplasty that displays good face validity, internal consistency and correlation with health-related quality of life and that is simple and easy to use. No correlation was seen with more traditional measures of outcome such as complications or ear measurements. Further refinement and testing of validity and reliability on a larger sample is planned.

Anatomical variations of the temporal bone on high-resolution computed tomography imaging: how common are they?

OBJECTIVES: This study aimed to evaluate the prevalence of normal variations of temporal bone anatomy on high-resolution computed tomography imaging and report their clinical importance. METHODS: A retrospective review was conducted of high-resolution temporal bone computed tomography imaging performed at NHS Greater Glasgow and Clyde over an eight-year period. The presence of five variants was determined. These variants were: a high dehiscent jugular bulb, an anteriorly located sigmoid sinus, a deep sinus tympani, an enlarged cochlear aqueduct and a large internal auditory meatus. RESULTS: A total of 339 temporal bones were examined. The incidences of a high dehiscent jugular bulb, anteriorly located sigmoid sinus, deep sinus tympani, enlarged cochlear aqueduct and an enlarged internal auditory meatus were 2.76 per cent, 2.94 per cent, 5.01 per cent, 0.58 per cent and 1.76 per cent respectively. CONCLUSION: Anatomical variations of the temporal bone are not uncommon and it is important for the investigating otologist to be aware of such variations prior to undertaking surgery.

Cholesteatoma has a high prevalence in Turner syndrome, highlighting the need for earlier diagnosis and the potential benefits of otoscopy training for paediatricians.

AIM: Girls with Turner syndrome are prone to cholesteatoma, a serious suppurative middle ear disease. We aimed to confirm its high prevalence in Turner syndrome, identify risk factors and suggest possible strategies for earlier detection. METHODS: We reviewed 179 girls with Turner syndrome between 1989 and 2012 to identify cases of cholesteatoma. RESULTS: Seven girls (3.9%) had cholesteatoma (index girls) and each was compared with three age-matched girls without cholesteatoma (comparison girls). All the index girls had either the 45,X or 45,X/46X,i(Xq) karyotypes. Nine ears were initially affected, with three recurrences in two girls. Median age at first cholesteatoma presentation was 11.9 years (range: 7.5-15.2), with otorrhoea for three (range: one to seven) months in all 12 affected ears. Index girls had a significantly higher proportion of previous recurrent acute (p = 0.007) and chronic otitis media (p = 0.008), chronic perforation (p = 0.038) aural polyps (p < 0.0001) and tympanic membrane retraction (p = 0.0001) than comparison girls. CONCLUSION: Cholesteatoma has a high prevalence in Turner syndrome. Risk factors include 45,X and 46,XiXq karyotypes; a history of chronic otitis media, tympanic membrane retraction and persistent otorrhoea; and older age. Earlier recognition of ear disease is needed and otoscopy training for paediatricians caring for Turner syndrome patients may be beneficial.

Cholesteatoma surgery in children: 10-year retrospective review.

OBJECTIVE: To review outcomes following paediatric cholesteatoma surgery performed between 1999 and 2009 in a tertiary paediatric ENT unit. STUDY DESIGN: Retrospective case note review. RESULTS: A total of 137 mastoid procedures were recorded. Fifty-four per cent of children were observed to have disease involving the entire middle-ear cleft and mastoid complex. The revision rate was 25 per cent. Time to recurrence was one to three years in 17 patients, three to six years in five patients, and six to nine years in three cases. Eight of 25 revision cases demonstrated spontaneous improvement in air conduction thresholds following primary surgery. A high facial ridge and inadequate meatoplasty correlated highly with disease recurrence. CONCLUSION: Children tend to present with aggressive disease. Disease extent and ossicular chain involvement are associated with a higher risk of recurrent disease. Spontaneous improvement in hearing thresholds following cholesteatoma surgery should alert the clinician to recurrent disease.