No abstract

Branchio-oto-renal syndrome is a rare autosomal dominant condition characterized by hearing loss, branchial arch abnormalities and renal tract malformations. We present the first reported case of branchio-oto-renal syndrome associated with bilateral congenital cholesteatoma and ossicular chain abnormalities. The pathogenesis of this syndrome is described and the literature is reviewed.

The incidence of barotitis in this study of aircraft passengers was 14%. This figure could be reduced to 6% in passengers who performed nasal balloon inflation during descent. We recommend nasal balloon autoinflation in aircraft passengers who have difficulty clearing their ears during and after flying.

Objectives:  Selecting patients with asymmetrical sensorineural hearing loss for further investigation continues to pose clinical and medicolegal challenges, given the disparity between the number of symptomatic patients, and the low incidence of vestibular schwannoma as the underlying cause. We developed and validated a diagnostic model using a generalisation of neural networks, for detecting vestibular schwannomas from clinical and audiological data, and compared its performance with six previously published clinical and audiological decision‐support screening protocols. Design:  Probabilistic complex data classification using a neural network generalization. Settings:  Tertiary referral lateral skull base and a computational neuroscience unit. Participants:  Clinical and audiometric details of 129 patients with, and as many age and sex‐matched patients without vestibular schwannomas, as determined with magnetic resonance imaging. Main outcome measures:  The ability to diagnose a patient as having or not having vestibular schwannoma. Results:  A Gaussian Process Ordinal Regression Classifier was trained and cross‐validated to classify cases as ‘with’ or ‘without’ vestibular schwannoma, and its diagnostic performance was assessed using receiver operator characteristic plots. It proved possible to pre‐select sensitivity and specificity, with an area under the curve of 0.8025. At 95% sensitivity, the trained system had a specificity of 56%, 30% better than audiological protocols with closest sensitivities. The sensitivities of previously‐published audiological protocols ranged between 82–97%, and their specificities ranged between 15–61%. Discussion:  The Gaussian Process ORdinal Regression Classifier increased the flexibility and specificity of the screening process for vestibular schwannoma when applied to a sample of matched patients with and without this condition. If applied prospectively, it could reduce the number of ‘normal’ magnetic resonance (MR) scans by as much as 30% without reducing detection sensitivity. Performance can be further improed through incorporating additional data domains. Current findings need to be reproduced using a larger dataset.

Barotitis is an acute or chronic inflammation caused by environmental pressure changes. The most common cause is the pressure change during descent in civil aviation. To prevent barotitis the middle ear pressure has to be equalised several times during descent. This can be achieved by performing the Valsalva manoeuvre, but for children, many of whom have a dysfunction of the Eustachian tube, this is difficult to perform and they are therefore at high risk of developing barotitis during flight. The traditional treatment modalities of barotitis are inflation by a Politzer balloon, myringotomy or prophylactic grommet insertion. An alternative treatment or prophylactic measure is autoinflation using the Otovent® treatment set. This prophylaxis/treatment can be performed by the child with assistance from its parents as soon as possible or rather before the descent has started. The prevalence of barotitis amongst transit passengers was found to be highest in young children, 25 per cent, compared with adults, five per cent. Only 21 percent of the youngest children with negative middle ear pressure after flight managed a successful Valsalva's manoeuvre, whereas 82 per cent could increase the middle ear pressure inflating the Otovent® set. In conclusion we recommend autoinflation using the Otovent® set by children and adults with problems clearing the ears during flight.