Background:

Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle-Wells syndrome. Previous reports have described the hearing loss to be progressive in nature. Method: To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle-Wells syndrome.

Results:

The patient underwent a cochlear implantation with a modest outcome.

Conclusion:

Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

Objective:

To assess the efficacy of dynamic slow motion video endoscopy as a test of eustachian tube function based on its correlation with the eustachian tube swallow test. Method: The eustachian tube swallow test and dynamic slow motion video endoscopy were performed on 100 clinically normal middle ears of adults undergoing rigid nasal endoscopy for various indications. The dynamic slow motion video endoscopy findings were interpreted by three observers who were blind to the results of the eustachian tube swallow test, and the findings of both techniques were compared.

Results:

There was a statistically significant correlation between the dynamic slow motion video endoscopy and eustachian tube swallow test results for some of the more lenient criteria. Five of the 100 eustachian tubes had a floppy medial cartilaginous lamina which appeared to contribute to the eustachian tube dysfunction.

Conclusion:

Dynamic slow motion video endoscopy appeared to over-diagnose eustachian tube dysfunction when used as a standalone test of eustachian tube function. However, when used in combination with other tests of eustachian tube function, it can provide valuable information regarding the structural and functional status of the pharyngeal end of the eustachian tube.

Background:

We report the case of an unusual late presentation of congenital tracheal stenosis in a 13-year-old boy. He was treated with minimally invasive Coblation resection of the stenotic segment, avoiding a major open tracheal resection and reconstruction. This case report is the first to document the use of an ultra-fine Coblation wand in the treatment of congenital tracheal stenosis.

Results:

The case proceeded well, without any complications. The patient had a fully healed and patent trachea at 12-week post-operative review.

Conclusion:

Complex cases of congenital stenosis should be managed with a multidisciplinary approach. Different and novel treatment options should be explored to find one that suits the individual patient. Minimally invasive Coblation technology can offer less invasive treatment with quicker recovery and shorter hospitalisation.

Background:

Osteonecrosis is a benign condition characterised by necrotic exposed bone, and is associated with bisphosphonate use. Osteonecrosis of the external auditory canal is rare, with only a few reported cases. Method: Two case reports of temporal bone osteonecrosis are presented.

Results:

A 64-year-old man with a history of immunoglobulin G kappa multiple myeloma developed a right external auditory canal ulcer 6 years after commencement on clodronate. A 72-year-old woman taking alendronate for osteoporosis, initially diagnosed and treated for right-sided otitis externa, was found to have underlying exposed bone in the right external auditory canal, with a computed tomography scan confirming destruction of the temporal bone.

Conclusion:

With increasing use of both oral and intravenous bisphosphonates in the community for benign conditions such as osteoporosis and for malignant conditions such as breast cancer and multiple myeloma, the diagnosis of bisphosphonate-associated osteonecrosis should always be considered in patients with a temporal bone lesion, and a relevant drug history taken.

Objective:

This paper reports a case of Dirofilaria repens infection in a patient who presented with an anterolateral neck swelling. Dirofilaria repens infection of the neck region is rare even in countries where dirofilarial infestation is endemic. The diagnosis is made by identifying the worm in surgical or pathological specimens. Case report: A 47-year-old man presented with an 8-week history of non-tender, right-sided, lower anterolateral neck swelling and weight loss. An ultrasound scan showed a cystic lesion containing a living worm. The cyst was excised and the patient showed full recovery at follow up.

Conclusion:

To our knowledge, there has been no previous report of an anterolateral neck swelling secondary to Dirofilaria repens infection in Europe. Our case is unusual because of the rarity of Dirofilaria repens presenting as a neck swelling.

Objective:

To evaluate residual tumour occurrence after vestibular schwannoma surgery, based on intra-operative registration and magnetic resonance imaging one year post-operatively.

Methods:

Patients undergoing translabyrinthine surgery for vestibular schwannoma in Denmark between 1976 and 2008 were registered in a national database covering 5.5 million inhabitants.

Results:

Translabyrinthine surgery was undertaken on 1143 patients. Of these, 978 had total, 140 near-total and 25 subtotal tumour excision, as assessed intra-operatively by the surgeon. One year after surgery, 65 per cent of small tumour remnants and 11 per cent of large tumour remnants were not visible on magnetic resonance imaging. The mean pre-operative size was significantly smaller for totally excised tumours, compared with near-totally and subtotally excised tumours. Revision surgery was performed for 14 patients (1.2 per cent), of whom 2 had received total, 5 near-total and 6 subtotal excisions initially.

Conclusion:

Most residual tumours disappear spontaneously, probably due to devascularisation. Few patients with a small residual vestibular schwannoma will require revision surgery or secondary radiotherapy.

Background:

Cholesteatoma is considered a benign, gradually expanding and destructive epithelial lesion of the temporal bone. The pathogenesis of different classifications of cholesteatoma is marked by similar underlying cellular and molecular processes. Stepwise explanations of the histopathogenesis have been described previously. The current paper focuses on expounding the molecular events of cholesteatoma. Method and results: Cholesteatoma pathogenesis encompasses a complex network of signalling pathways during: epidermal hyperplasia, perimatrix-matrix interactions and mucosal disease. This paper presents a review of the molecular events driven by inflammatory mediators and enzymes during: cholesteatoma growth (cell proliferation and apoptosis); maintenance and deterioration (angiogenesis and hypoxia, oxidative stress and toxicity); and complications (bone erosion and hearing loss). The cascade of molecular events applicable to atelectasis and cholesteatoma that coexist with chronic otitis media and bone erosion as sequelae is summarised.

Conclusion:

The role of lipids in this disease is relatively unexplored, but there is evidence in support of fatty acid role-players that needs confirmation. Future directions in lipid research to delineate molecular mechanisms are proposed.

Introduction: Cisplatin is an effective chemotherapeutic agent commonly used in the treatment of malignant tumours, but ototoxicity is a significant side effect.

Objectives:

To discuss the mechanisms of cisplatin ototoxicity and subsequent cell death, and to present the results of experimental studies. Material and methods: We conducted a systematic search for data published in national and international journals and books, using the Medline, SciELO, Bireme, LILACS and PubMed databases.

Results:

The nicotinamide adenine dinucleotide phosphate oxidase 3 isoform (also termed NOX3) seems to be the main source of reactive oxygen species in the cochlea. These reactive oxygen species react with other molecules and trigger processes such as lipid peroxidation of the plasma membrane and increases in expression of the transient vanilloid receptor potential 1 ion channel.

Conclusion:

Cisplatin ototoxicity proceeds via the formation of reactive oxygen species in cochlear tissue, with apoptotic cell death as a consequence.

Background:

Numerous techniques have been described to manage the skin and other soft tissues during bone-anchored hearing aid insertion. Previously, generally accepted techniques have sometimes led to distressing alopecia and soft tissue defects. Now, some surgeons are rejecting the originally described split skin flap in favour of a less invasive approach.

Objective:

To investigate bone-anchored hearing aid placement utilising a single, linear incision with either no or minimal underlying soft tissue reduction. Patients and methods: Thirty-four adults were prospectively enrolled to undergo single-stage bone-anchored hearing aid placement with this modified technique. A small, linear incision was used at the standard position and carried down through the periosteum. Standard technique was then followed with placement of an extended length abutment. Patients were reviewed regularly to assess wound healing, including evaluation with Holgers' scale.

Results:

Only 14.7 per cent of patients had a reaction score of 2 or higher. Most complications were limited to minor skin reactions that settled with silver nitrate cautery and/or antibiotics. None required revision surgery for tissue overgrowth, and there were no implant failures.

Conclusion:

Our results suggest this to be a simple and effective insertion technique with favourable cosmesis and patient satisfaction.