We report an extremely rare case of congenital cholesteatoma of mastoid origin.

A male patient was admitted with a one-month history of dizziness and headache, plus tinnitus in the right ear. Computed tomography scanning of the temporal bone showed destruction of the posterior wall of the external auditory canal by a lesion of soft tissue density in the right mastoid cavity, and also destruction of the bony plates of the posterior fossa and the sigmoid sinus, and of the mastoid tegmen. During surgery, a huge cholesteatoma sac was observed in the mastoid cavity, containing a large amount of keratinous material. The tegmen mastoideum and the bony plates of the posterior fossa and the sigmoid sinus were also observed to be destroyed. The skin and the tympanic membrane of the external auditory canal were intact, and the middle ear and aditus ad antrum mucosa were normal. The huge cholesteatoma sac was completely excised via a partial translabyrinthine approach, eradicating the superior and posterior semicircular canals.

This case of congenital cholesteatoma of mastoid origin was diagnosed by clinical examination, radiological evaluation and surgical findings.

The relationship of pars flaccida retraction with epitympanic aeration and mastoid size is ill-defined. Both pars flaccida retraction and small mastoid size are traits of nearly all clinical entities of otitis media.

To determine, in clinically normal specimens, the relationship of pars flaccida retraction with mastoid pneumatisation and epitympanic aeration.

Post-mortem anatomical dissection of 41 bequeathed adult crania without clinical otitis.

Pars flaccida retractions were viewed with an operating microscope and judged using Sade's classification. Mastoid sizes were determined radiographically (by plain Law lateral images). Ten crania, five with the largest mastoids and five with the smallest mastoids, were studied by computed tomography.

No specimen had a retraction worse than Sade grade two (i.e. retracted onto the neck of the malleus). Retractions were unrelated to the extent of mastoid pneumatisation. All epitympani were found to be normal on both anatomical dissection and computed tomography imaging.

Pars flaccida retraction is probably related to prior, presumably transient, non-aeration of Prussak's space.

This study aimed to investigate the validity of adjusting computed tomography thresholds in order to replicate a temporal bone model suitable for dissection training and education.

A simulated three-dimensional model of a human temporal bone was prototyped using selective laser sintering. The powder layers were laser-fused, based on detailed computed tomography data, and accumulated to create a three-dimensional structure. The computed tomography threshold value of the stapes was modified on standard triangular language file in order to replicate the stapes. The intensity value was determined to select the fluid lumen of the inner ear and the bone surface, in order to replicate the inner ear.

The model could be shaved, using surgical instruments, in the same manner as during real surgery. The stapes could be reproduced, making this model even more realistic than a previous version. The inner ear was recreated, along with the surrounding bony wall and the ossicles.

This model facilitates dissection training and easy understanding of the relation between the labyrinth and the surrounding structures.

We aimed to study the influence of age, in normal hearing individuals, on: the masking level difference test, the speech recognition in noise test, the transient evoked otoacoustic emissions test, and the contralateral transient evoked otoacoustic emission suppression test. We also aimed to research the effect of age when using these tests to evaluate the central auditory nervous system.

Transient evoked otoacoustic emissions and contralateral transient evoked otoacoustic emission suppression were measured in all subjects. Subjects also underwent masking level difference and speech recognition in noise tests.

We found a decrease in transient evoked otoacoustic emission amplitudes, speech recognition in noise scores and hearing thresholds with age. We also found that higher masking level difference values were associated with lower speech recognition in noise scores and contralateral transient evoked otoacoustic emission suppression values.

We conclude that decreasing speech recognition in noise scores are associated with decreasing contralateral transient evoked otoacoustic emission supression values. This effect may be related to medial efferent system dysfunction.

To define the impact of patient-related and audiovestibular parameters on the prognosis of sudden hearing loss.

Eighty-three patients were included in this retrospective study. All were treated medically. We recorded the patients' demographic parameters, systemic diseases, time elapsed between onset of sudden hearing loss and initiation of treatment, tinnitus, vestibular symptoms, type of initial audiogram, pure tone averages and speech discrimination scores. For all patients, audiological measurements were performed on initial admission and at the completion of treatment on the 10th day.

There was no correlation between the hearing gain and recovery rate scores and patients' gender or age (p>0.05). However, a correlation was found between gender and relative hearing gain. Vertigo was not correlated with hearing gain and recovery rate scores (p<0.05). However, relative hearing gain correlated negatively with the presence of vertigo (−r=0.05, 81 degrees of freedom, p=0.043). Patients with <40 dB hearing loss on admission showed a better relative hearing gain (r=0.55, 81 degrees of freedom, p=0.03). Relative hearing gain correlated positively with better pre-treatment speech discrimination scores (r=0.82, 81 degrees of freedom, p=0.009) and negatively with poorer pre-treatment pure tone averages (−r=0.082, 81 degrees of freedom, p=0.009). There was no correlation between the scores for hearing gain, relative hearing gain and recovery rate and: systemic diseases (p>0.05); time elapsed between onset of sudden hearing loss and initiation of treatment (p>0.05); type of audiogram on initial admission (p>0.05), except for midfrequency type of audiogram; and tinnitus (p>0.05).

The outcome of sudden hearing loss was unaffected by systemic disease, tinnitus or type of audiogram (except for midfrequency type). The following were poor prognostic factors in the outcome of sudden hearing loss: female gender, presence of vertigo, initiation of treatment more than seven days after onset of hearing loss, and >40 dB hearing loss on admission.

We report our experience with bilateral, simultaneous tympanomastoidectomies and the results of an opinion survey of otologists.

A chart review of 116 tympanomastoidectomies revealed 12 patients who underwent bilateral, simultaneous tympanomastoidectomies. An opinion survey generated 121 responses.

Of the 12 patients, none suffered any outcome which would have been avoided by staging the procedures. Twenty-three of 24 operated ears had the same or better hearing post-operatively. Of the survey respondents, 74 felt that performing bilateral, simultaneous tympanomastoidectomies was unsafe, largely because of the risk of bilateral sensorineural hearing loss.

Although bilateral, simultaneous tympanomastoidectomies carry double the risk of unilateral sensorineural hearing loss, compared with the unilateral procedure, the risk of bilateral sensorineural hearing loss is only 0.006–0.2 per cent, as derived mathematically from historical data. Respondents to the survey were mostly opposed to bilateral, simultaneous tympanomastoidectomies, but even those opposed gave indications for simultaneous procedures. This finding probably reflects an ambivalence about the theoretical risks of the operation versus the potential patient benefits. A decision tree for proceeding to the second case is presented.

To assess changes in the prevalence of otitis media, associated with the use of highly active antiretroviral therapy, in Brazilian human immunodeficiency virus (HIV) infected children.

Division of otorhinolaryngology, Hospital das Clínicas, Sao Paulo University Medical School, Brazil.

A cohort of 459 HIV-infected children aged below 13 years.

The prevalence of otitis media and the serum cluster of differentiation four glycoprotein T lymphocyte count were compared for children receiving highly active antiretroviral therapy (with protease inhibitors) and those receiving standard antiretroviral therapy (without protease inhibitors).

Otitis media was present in 33.1 per cent of the children. Children aged from zero years to five years 11 months receiving highly active antiretroviral therapy had a higher prevalence of acute otitis media (p=0.02) and a lower prevalence of chronic otitis media (p=0.02). Children who were receiving highly active antiretroviral therapy had a mean serum cluster of differentiation four glycoprotein T lymphocyte count greater than that of those who were receiving standard antiretroviral therapy (p<0.001).

The use of highly active antiretroviral therapy in Brazilian HIV-infected children was associated with a lower prevalence of chronic otitis media.

To hypothesise the probable pathophysiological mechanism responsible for visual loss in allergic fungal sinusitis, other than direct compression.

Retrospective, non-randomised case series. Out of 274 cases of allergic fungal sinusitis, four cases with sudden visual loss were enrolled into the study. The fourth case had visual loss on the contralateral side to bony erosion of the lateral wall of the sphenoid sinus.

All four cases were evaluated with fungal smear, immunoglobulin (Ig) E titres, visual evoked potentials, non-contrast computed tomography and magnetic resonance imaging of the paranasal sinuses, and fundus examination. They then underwent endoscopic sinus debridement followed by intravenous methylprednisolone.

Improvement in vision.

All four cases experienced an improvement in vision: full recovery in three cases and partial improvement in one case.

In view of the operative, radiological and laboratory findings for case four, with the suggestion of a hyperimmune response to fungal antigens (in the form of raised IgE titre and positive fungal serology), we suggest that a local immunological reaction to fungal antigens might be responsible for the observed visual loss in cases of allergic fungal sinusitis, in addition to mechanical compression of the optic nerve.

To assess the change in health-related quality of life following septal surgery.

Post-intervention, health-related quality of life questionnaire.

Adult patients undergoing septal surgery, recruited from two sources (the Scottish ENT outcomes study and the North Glasgow National Health Service Trust) over an 18-month period.

Glasgow benefit inventory score.

A total of 149 Glasgow benefit inventories were returned. Of these, 13 were incomplete and were thus excluded, leaving 136 to be analysed. The response rate from the Scottish ENT outcomes study patients was 55 per cent and the response rate from the North Glasgow National Health Service Trust patients was 41 per cent, giving an overall response rate of 49 per cent. The mean Glasgow benefit inventory score was 11.3 (standard deviation 20, 95 per cent confidence interval 7.8 to 14.7). This result was similar to those of other studies using the Glasgow benefit inventory to assess septal surgery outcomes.

Patients reported minimal improvement in their health-related quality of life following septal surgery.

To analyse and compare chest radiographs and cervicothoracic computed tomography scans taken prior to thyroid surgery, in order to locate and predict thoracic extension of substernal goitres.

This eight-year, retrospective study included 223 patients who had undergone total thyroidectomy for benign, substernal, multinodular goitres. Chest radiographs and computed tomography scans were reviewed by two physicians (a senior ENT surgeon and a senior radiologist).

The most frequent mode of extension was anterior or prevascular, seen in 76 per cent of cases, especially on the left side. Fifty-nine per cent of goitres did not reach the aortic arch and 11 per cent extended beyond the aortic arch. A cervical surgical approach was performed in approximately 99 per cent of cases.

Thorough examination of computed tomography scans is essential in order to identify and classify substernal goitres and to decide the best surgical procedure. A cervical surgical procedure is almost always sufficient and safe for the surgical management of substernal goitres.

Computed tomography appears to be the best imaging modality for identifying and characterising substernal goitres. Surgical management is almost always via a cervical approach and does not differ from that used for benign cervical goitres. In the future, magnetic resonance imaging may be used; its results would need to be compared with those of computed tomography.

To explore whether the incidence of post-tonsillectomy haemorrhage requiring a return to theatre demonstrates a monthly or seasonal variation.

Retrospective, cross-sectional study including all 256 799 tonsillectomies performed between April 2000 and March 2005 in NHS trusts in England.

Return-to-theatre rates for each calendar month and each three-month ‘season’, over the five-year study period.

A statistically significant association was found between the calendar month and the proportion of patients requiring a return to theatre for post-tonsillectomy haemorrhage (p=0.005). There was a bimodal seasonal distribution peaking in the winter and summer months, with winter rates significantly higher than spring rates (p=0.0104).

The incidence of severe post-tonsillectomy haemorrhage showed monthly and seasonal variations. This is clearly a multi-factorial problem. However, in patients at high risk of bleeding or of the complications of bleeding, consideration should be given to performing tonsillectomy at a time of year of lower incidence.

Ear lobule deformities can occur as a result of trauma. Surgical reconstruction can be challenging. There does not appear to be a consensus on the best form of repair. We report the case of a 27-year-old woman who suffered traumatic loss of the ear lobule during childhood. A novel technique is described using a posterior based bi-lobar flap to completely reconstruct the ear lobule in two stages.

This form of repair has the advantage of a well-hidden scar, with the donor site providing skin of similar thickness and pigmentation.

To determine if chondrodermatitis nodularis chronica helicis can be treated conservatively using a doughnut-shaped pillow.

Prospective study in Wrexham Maelor Hospital, which is a district general hospital.

All the patients clinically diagnosed with chondrodermatitis nodularis chronica helicis between September 2003 and September 2004. All such patients were seen by an ENT consultant with a special interest in facial lesions. Patients who could not be clinically diagnosed with chondrodermatitis nodularis chronica helicis were excluded from the study.

Pain relief following the use of a doughnut pillow was used as the main outcome measure.

Twenty-three (14 female and 9 male) patients were treated with a special doughnut-shaped pillow designed to relieve the pressure on the affected ear. Of the 23 lesions treated 13 remained pain free after a follow-up period of one year.

Conservative treatment of chondrodermatitis nodularis chronica helicis using a doughnut pillow is very cost effective.

Mucormycosis is an opportunistic, fulminating fungal infection of the sino-nasal region. It tends to affect people with immune suppression. The hard palate is a rare site of the disease and few cases have been reported in the literature. This report discusses the nature of hard palate mucormycosis, with the aim of outlining possible explanations and reviewing associated characteristics.

This was a prospective analysis of four cases of hard palate mucormycosis. The patients were referred from the medical department, with uncontrolled diabetes mellitus. An aggressive treatment protocol was used, i.e. an intensive course of antifungal drugs and frequent debridement of dead tissue.

A painful, dusky red swelling was noticed at the early stages of the hemi-palate lesion. It was eventually followed by deep ulceration and even big perforation. Orbit and intracranial extension was the preceding sign in all instances. The progression of the perforation was not controlled by a full course of treatment. There were two deaths during the treatment course.

Mucormycosis of the hard palate is an ominous sign. Although rare, the lesion is progressive in nature and barely controlled by treatment. Clinicians should maintain a high degree of clinical suspicion in the management of patients with palatal ulcer and debilitating illness.