To present results for the auditory rehabilitation of patients with Treacher Collins syndrome with bilateral osseous atresia, using middle-ear implantation with a Vibrant Soundbridge.

Three patients underwent vibroplasty for aural atresia with moderate to severe conductive hearing loss. The pre-operative Jahrsdoerfer radiological score was 4 for all patients. Patients underwent active middle-ear implantation of a Vibrant Soundbridge implant (coupling the floating mass transducer to the rudimentary stapes or footplate distally, and positioning it adjacent to the round window membrane proximally), with audiological analysis as follow up.

After implant activation, the mean air conduction threshold ± standard deviation decreased to 22.8 ± 5.5 dB HL, representing a mean functional gain of 44.5 dB. The mean word recognition score (for bisyllabic words at 65 dB SPL) increased from 0 to 97 per cent.

Vibrant Soundbridge implantation is an effective hearing rehabilitation procedure in patients with Treacher Collins syndrome with bilateral osseous atresia. This is a versatile implant which can achieve coupling even in cases of severe middle-ear malformation.

To explain a clinical observation: a notch in the stimulus spectrum during transient evoked otoacoustic emission measurement in ears with secretory otitis media.

The effects of tympanic under-pressure were investigated using a pressure chamber. A model of the ear canal was also studied.

Tympanic membrane reflectance increased as a consequence of increased stiffness, causing a notch in the stimulus spectrum. In an adult, the notch could be clearly distinguished at an under-pressure of approximately −185 daPa. The sound frequency of the notch corresponded to a wavelength four times the ear canal length. The ear canal of infants was too short to cause a notch within the displayed frequency range. The notch was demonstrated using both Otodynamics and Madsen equipment.

A notch in the otoacoustic emission stimulus spectrum can be caused by increased stiffness of the tympanic membrane, raising suspicion of low middle-ear pressure or secretory otitis media. This finding is not applicable to infants.

To determine the feasibility and safety of transtympanic balloon dilatation of the eustachian tube.

Transtympanic eustachian tube dilatation was performed on six cadaver heads using balloon catheters. Catheters were placed in each eustachian tube and the head scanned by computed tomography. Randomised, blinded dilatation of one balloon in each head was performed, followed again by a second computed tomography scan. The scans were reviewed by a neurotologist and neuroradiologist who were blinded to previous treatment, and measurable dilatation and incidental damage noted.

There was adequate placement of the balloon catheter beyond the bony isthmus in 6 of 10 eustachian tubes. There was one insufficient catheter placement and three adverse placements (one into the petrous carotid canal and two into the vidian canal). Only one dilated tube showed a measurable increase in diameter.

This experiment revealed serious safety issues with transtympanic eustachian tube dilatation. Therefore, this approach should not be considered feasible at this time.

The aggressiveness of cholesteatoma in children compared with adults is well known. However, the factors influencing the poorer prognosis of paediatric cholesteatoma are not well understood. This study compared the proliferative potential of paediatric cholesteatoma with that of adult cholesteatoma, using Ki-67 as a proliferation marker.

A prospective study of 67 patients with aural cholesteatoma was performed. Thirty-eight adult and 29 paediatric cases were evaluated using clinical parameters including bone erosion, complications and extent of disease. A surgical specimen underwent histological evaluation and measurement of the proliferation index using Ki-67 labelling. Normal epithelium from a control group was also examined.

Cholesteatoma epithelium has a greater rate of proliferation than normal skin. There were however no statistical differences between the paediatric and adult cholesteatoma groups in terms of clinical behaviour or proliferation potential. Paediatric cholesteatoma was similar to adult cholesteatoma in terms of complications, bone erosion and disease spread.

Cholesteatoma is a disorder of epithelial proliferation. Although postulated to be more aggressive in children than adults, this study found no clinicopathological differences between paediatric and adult cases.

Simulators are becoming an increasingly important part of surgical training. Temporal bone surgery is one area in which simulators, such as the Voxel-Man TempoSurg simulator, are likely to play a significant role in training. We present learning curve data from novice trainees using this simulator to learn cortical mastoidectomy, exposure of the sigmoid sinus, and exposure of the short process of the incus.

We measured the time taken to perform the procedures, the volume of reference bone removed, and the structures damaged during dissection.

We found improvement in a number of parameters over the course of the study. The overall scores, structural damage scores and time taken improved, to differing degrees, for each task. The volume of reference bone removed remained constant.

These results indicate that the trainees' efficiency improved as they became more proficient at removing a given volume of reference bone.

This study aimed to determine the prevalence of nasal polyps in patients with allergic rhinitis and with asthma, and also to assess the impact of this condition on these disorders.

Cross-sectional study.

The presence of nasal polyps was assessed by rhinoscopy and endoscopic examination.

Nasal polyps were detected in 60 out of 250 patients (24 per cent) with documented asthma or allergic rhinitis. There was a statistically significant correlation between asthma severity and nasal polyposis prevalence (p = 0.007), but not between allergic rhinitis severity and nasal polyposis prevalence (p = 0.081). The prevalence of nasal polyps increased significantly with increasing patient age and rhinitis or asthma duration.

The prevalence of nasal polyps in patients with allergic rhinitis or asthma was higher than previously reported. Given this high prevalence of nasal polyposis, nasal examination and concomitant treatment of this disorder are recommended.

The temporal bone may be the first involved site in cases of systemic disease, and may even present with acute, mastoiditis-like symptomatology. This study aimed to evaluate the incidence of such non-infectious ‘acute mastoiditis’ in children.

Retrospective chart review of 73 children admitted to a tertiary referral centre for acute mastoiditis.

In 71 cases (97.3 per cent), an infectious basis was identified. In the majority of cases (33 of 73; 45 per cent), the responsible bacteria was Streptococcus pneumoniae. However, histopathological studies revealed a non-infectious underlying disease (myelocytic leukaemia or Langerhans' cell histiocytosis) in two atypical cases (2.7 per cent).

‘Acute mastoiditis’ of non-infectious aetiology is a rare but real threat for children, and a challenging diagnosis for otologists. A non-infectious basis should be suspected in every atypical, persistent or recurrent case of acute mastoiditis.

Image guidance surgery is an emerging technology that may allow more efficient treatment of sinus disease. This retrospective study examines National Health Service and military patients who underwent procedures using image guidance surgery during the period 2001–2009.

Medical records were reviewed in terms of indications for surgery, incidence of major complications and need for revision following image guidance surgery. An attempt was also made to determine the cost-effectiveness of purchasing this navigational system.

A total of 132 patients underwent 147 procedures using image guidance surgery over the 8-year period. The indications for surgery ranged from severe nasal polyposis and chronic rhinosinusitis to malignant tumours in the paranasal sinus and skull base region. Average length of follow up was 17.6 months. Four patients had a major complication. Fourteen patients underwent revision surgery. The cost of providing an image guidance surgery service was estimated to be £110,000–120,000 during the study period. The economic model for the subgroup of nineteen military patients (with non-polypoid chronic rhinosinusitis) suggests that use of this technology will reduce overall costs by approximately £70,000 when compared with conventional sinus surgery.

This study provides some evidence that image-guided sinus surgery is cost effective, safe and may decrease surgical revision rates.

To discuss the minimally invasive treatment and prognosis of juvenile nasopharyngeal angiofibroma involving the eye and optic nerve.

Retrospective analysis of clinical data for 18 large juvenile nasopharyngeal angiofibromas, with reports of three typical cases.

The tumour invaded the orbit, eye, optic nerve and optic chiasm in 18, 9, 8 and 5 patients, respectively. Twelve patients were cured after surgery, with the affected eye and vision essentially returning to normal. In six patients, tumour residue was found in the middle cranial fossa; two of these six did not return for follow up. Four patients underwent radiotherapy; all four regained placement of the eye in a normal or near-normal position. One of these four patients regained normal visual acuity, two experienced no improvement in visual acuity, and one became blind.

In patients with this tumour, surgery may return the eye to the normal position and may restore visual acuity if the optic fundus, macula, retina and optic nerve are only mildly affected. Modern radiotherapy can treat the intracranial residue effectively. A combined approach via the antrum, infratemporal fossa and nasal cavity, using a Caldwell–Luc incision, provides minimally invasive surgical access.

Klotho protein is involved in insulin-signalling and ageing. Klotho mutation causes premature ageing and significantly shortens the lifespan. The anti-neoplastic drug cisplatin promotes ototoxicity at higher doses by inducing apoptosis. This study aimed to clarify the effect of klotho expression on cisplatin ototoxicity, using an auditory cell line.

Expressions of klotho messenger RNA and protein were analysed by reverse-transcription polymerase chain reaction and western blotting. Auditory cells (HEI-OC1 line) were pretreated with 2 nM klotho protein for 2 hours; 15 µM cisplatin was then applied. After 48 hours incubation, assessment of cell viability (via 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazoliumbromide assay), apoptosis (via Hoechst 33258 staining) and reactive oxygen species was performed.

Klotho protein expression increased in cisplatin-treated auditory cells. Cells treated with both klotho protein and cisplatin showed a viability of 67.7 per cent, versus 59.4 per cent in cisplatin-treated cells. Klotho significantly attenuated the cisplatin-induced increase in reactive oxygen species, and increased the viability of cells with cisplatin-induced cytotoxicity.

Klotho protein is protective against cisplatin-induced auditory cell cytotoxicity; inhibition of reactive oxygen species may be the main mechanism.

To characterise balance disorders occurring after head trauma, using videonystagmography, and to test the efficiency of videonystagmography as a diagnostic and monitoring tool.

Prospective, cohort analysis of 126 head trauma patients managed with vestibular evaluation, monitoring and treatment, in a tertiary referral centre. Analytical parameters included: head injury severity; balance disorder type, severity and time of onset; and patient recovery and outcome.

Head trauma was minor in 31.7 per cent, mild in 36.6 per cent, moderate in 19 per cent and severe in 12.7 per cent. Balance disorder symptoms included vertigo in 42.9 per cent, unsteadiness in 15.9 per cent, dizziness in 9.5 per cent and none in 31.7 per cent. Videonystagmographic balance disorder diagnosis type was peripheral vestibular in 23.8 per cent, central in 7.9 per cent, mixed in 12.7 per cent, benign paroxysmal positional vertigo in 4.8 per cent and no findings in 50.8 per cent. Balance disorder was immediate in 47.6 per cent (this included all moderate and severe trauma cases). Benign paroxysmal positional vertigo developed within the first week in two-thirds of cases. More severe trauma cases had longer recovery times. Peripheral, mixed and central balance disorders recovered within the first three months. Early rehabilitation of acute balance disorders led to early recovery regardless of diagnosis.

Videonystagmography enables precise, simple, cost-effective monitoring of balance disorders after head trauma, and improves care and outcomes.

To review the genotype and cochlear implantation outcome of patients with nonsyndromic hearing loss and enlarged vestibular aqueduct.

Twenty-one Chinese children with nonsyndromic hearing loss and enlarged vestibular aqueduct underwent genetic examination. A DNA microarray was used to screen for the IVS7-2A>G and H723R mutations. Any DNA samples with one or none of the two mutant alleles were sequenced to detect other mutations in the SLC26A4 and FOXI1 genes.

Twelve SLC26A4 mutations were detected, including three novel mutations. The most common mutations detected were IVS7-2A>G and H723R. Twelve patients received cochlear implants, and subsequently demonstrated excellent speech perception.

Three novel mutations were detected in Chinese patients with nonsyndromic hearing loss and enlarged vestibular aqueduct. The SLC26A4 mutation spectrum in the Chinese population is similar to that in other East Asian populations. Cochlear implantation is a safe and effective treatment in patients with enlarged vestibular aqueduct.

To evaluate the diagnostic value of echo-planar diffusion-weighted magnetic resonance imaging in pre-operative detection of suspected primary acquired, residual and/or recurrent cholesteatoma.

Fifty-eight chronic otitis media patients with suspected cholesteatoma were thus evaluated two weeks pre-operatively, and divided into group one (41 patients, no previous surgery, suspected primary acquired cholesteatoma) and group two (17 patients, previous surgery, scheduled ‘second-look’ or revision surgery for suspected residual or recurrent cholesteatoma). Patients' operative, histopathology and radiological findings were compared.

Cholesteatoma was found in 63 per cent of group one patients and 58 per cent of group two patients at surgery. Histopathological examination of surgical specimens indicated that imaging accurately predicted the presence or absence of cholesteatoma in 90 per cent of group one (37/41; 23 true positives, 14 true negatives) and 76 per cent of group two (13/17; seven true positives, six true negatives). Three patients in both groups were false negative diagnoses and one patient in both groups was a false positive. The sensitivity, specificity, and positive and negative predictive values of echo-planar diffusion-weighted magnetic resonance imaging of cholesteatoma were respectively 88, 93, 95 and 82 per cent in group one and 70, 85, 87 and 66 per cent in group two.

Echo-planar diffusion-weighted magnetic resonance imaging is a valuable technique with high sensitivity and specificity for cholesteatoma imaging.

This experimental study aimed to investigate the effects of vitamins E, B and C and l-carnitine in preventing cisplatin-induced ototoxicity.

Twenty-five adult, male, Wistar albino rats were randomly allocated to receive intraperitoneal cisplatin either alone or preceded by vitamins B, E or C or l-carnitine. Auditory brainstem response (i.e. hearing thresholds and wave I–IV intervals) and distortion product otoacoustic emissions (i.e. signal-to-noise ratios) were recorded before and 72 hours after cisplatin administration.

The following statistically significant differences were seen: control group pre- vs post-treatment wave I–IV interval values (p < 0.05); control vs vitamin E and B groups' I–IV interval values (p < 0.05); control vs other groups' hearing thresholds; vitamin E vs vitamin B and C and l-carnitine groups' hearing thresholds (p < 0.05); and vitamin B vs vitamin C and l-carnitine groups' hearing thresholds (p < 0.05). Statistically significant decreases were seen when comparing the initial and final signal-to-noise ratios in the control, vitamin B and l-carnitine groups (2000 and 3000 Hz; p < 0.01), and the initial and final signal-to-noise ratios in the control group (at 4000 Hz; p < 0.01).

Vitamins B, E and C and l-carnitine appear to reduce cisplatin-induced ototoxicity in rats. The use of such additional treatments to decrease cisplatin-induced ototoxicity in humans is still under discussion.

Earlier studies have shown that individuals with thalidomide embryopathy can have skeletal deformities, ear and eye aberrations, and facial palsy. This study aimed to survey the frequency and characteristics of facial palsy in this group of individuals.

Thirty-one individuals with thalidomide embryopathy (age range, 45–47 years) and 25 healthy adults (age range, 41–56 years; mean age ± standard deviation, 49 ± 4.2 years).

Voluntary facial movements, lip force and three-dimensional lip motion analysis.

Four of the thalidomide embryopathy individuals (13 per cent) had congenital facial palsy. All four had eye aberrations, three had ear anomalies and one had a limb anomaly. Individuals with thalidomide embryopathy without a clinical diagnosis of facial impairment had significantly weaker lips and more restricted lip mobility than healthy controls. This study contributes to the overall knowledge of thalidomide embryopathy by adding a description of how facial expression can be affected in this condition.