In the management of sensorineural hearing loss, effective therapy for degenerated hair cells, third order neurons, ganglions, dendrites and synaptic areas of the vestibulo-cochleo-cerebral pathway remains an enigma. Transplantation of stem and progenitor cells appears to be an emerging potential solution, and is the focus of this review.

To review recent developments in the management of sensorineural hearing loss in the field of stem cell research.

A systematic review of the English language literature included all experimental and non-experimental studies with a Jadad score of three or more, published between 2000 and 2010 and included in the following databases: Cochrane Library Ear, Nose and Throat Disorders; Medline; Google Scholar; Hinari; and the Online Library of Toronto University.

Of the 455 and 29 600 articles identified from Medline and Google Scholar, respectively, 48 met the inclusion criteria. These were independently reviewed and jointly analysed.

Although there is not yet any evidence from successful human studies, stem cell and ‘alternative stem cell’ technology seems to represent the future of sensorineural hearing loss management.

To identify the incidence of vestibular schwannoma amongst patients referred from a direct referral audiology clinic, and also the number of incidental findings, as seen on magnetic resonance imaging or computed tomography scans.

Prospective data collection for patients referred from a direct referral audiology clinic due to audiological evidence of asymmetrical hearing loss. The audiograms, magnetic resonance imaging and computed tomography scans of patients with diagnosed vestibular schwannoma were subsequently reviewed.

A total of 4100 patients were seen during the study period, with 396 scans performed. Six (1.5 per cent) patients had vestibular schwannoma, while 12 (3 per cent) had significant incidental findings.

Patients referred from the direct referral audiology clinic had a low incidence of vestibular schwannoma detection. Their detection rate for significant incidental findings was similar to previous reports. If the current protocol had not been in place, over 300 patients would have been needlessly added to the ENT clinic list. Thus, general practitioner referral to direct referral audiology clinics provides a cost-effective way of managing asymmetrical sensorineural hearing loss in older patients. The number of such clinics could be expanded.

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.

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.

To report the outcome of posterior semicircular canal occlusion surgery for intractable benign positional vertigo, regarding vertigo cure rate and hearing and balance outcomes.

Retrospective review of 53 patients presenting with benign positional vertigo, unresponsive to repositioning manoeuvres, who eventually underwent posterior canal occlusion, over a 20 year period.

From 1991 to 2011, 5364 benign positional vertigo patients were treated in our balance disorders clinic; 53 of those who failed to respond to repositioning underwent posterior canal occlusion. All 53 were cured of their benign positional vertigo. Nine suffered some symptomatic permanent hearing loss (>20 dB at low and >25 dB at high frequencies). Ten patients suffered caloric vestibular function deterioration, with mild but permanent subjective imbalance in five; a further 10 patients with no post-operative caloric test changes also had some permanent imbalance. Benign positional vertigo later developed in the operated ear lateral canal in two patients and in the opposite ear posterior canal in eight patients. Two patients needed bilateral sequential posterior canal occlusion.

Posterior canal occlusion is a highly effective treatment for intractable benign positional vertigo, with what is probably an acceptable risk to hearing and balance: five of six patients will have no hearing problem and nine of 10 no balance problem after surgery.

Previous evidence shows that the n10 component of the ocular vestibular evoked myogenic potential indicates utricular function, while the p13 component of the cervical vestibular evoked myogenic potential indicates saccular function. This study aimed to assess the possibility of differential utricular and saccular function testing in the clinic, and whether loss of saccular function affects utricular response.

Following vibration conduction from the mid-forehead at the hairline, the ocular n10 component was recorded by surface electromyograph electrodes beneath both eyes, while the cervical p13–n23 component was recorded by surface electrodes over the tensed sternocleidomastoid muscles.

Fifty-nine patients were diagnosed with probable inferior vestibular neuritis, as their cervical p13–n23 component was asymmetrical (i.e. reduced or absent on the ipsilesional side), while their ocular n10 component was symmetrical (i.e. normal beneath the contralesional eye).

The sense organ responsible for the cervical and the ocular vestibular evoked myogenic potentials cannot be the same, as one response was normal while the other was not. Reduced or absent saccular function has no detectable effect on the ocular n10 component. On vibration stimulation, the ocular n10 component indicates utricular function and the cervical p13–n23 component indicates saccular function.

Olfactory dysfunction is common. The reliability of self-assessment tools for smell testing is still controversial. This study aimed to provide new data about the accuracy of olfactory self-assessment compared with a standardised smell test.

Prospective, controlled, cohort study of patients with olfactory disorders and healthy controls.

Ninety-six patients with a smell deficit and 71 controls were asked to rate their sense of smell on a visual analogue scale. Their olfactory abilities were also evaluated with the Sniffin' Sticks tests.

The whole cohort showed a significant correlation between visual analogue scale smell scores and Sniffin' Sticks total scores. This correlation was also significant in the patient group, but not in the control group. These results were independent of olfactory deficit aetiology and subject age.

Self-assessment of olfaction is only a reliable indicator in smell-impaired patients, not in healthy controls. For an accurate assessment of olfaction, reliable, standardised tests are needed.

Amiodarone can induce severe hyperthyroidism that justifies its withdrawal and the introduction of antithyroid drugs. Continuing amiodarone use, failure to control hyperthyroidism and poor clinical progress may require thyroidectomy. This study aimed to evaluate patients’ post-operative development and mid-term outcome after thyroidectomy for amiodarone-associated thyrotoxicosis.

Prospective case series.

Tertiary care centre.

We prospectively collected cases of amiodarone-associated thyrotoxicosis requiring thyroidectomy due to failure of antithyroid treatment, despite amiodarone discontinuation. Post-thyroidectomy complications were compared immediately, 30 days and one year post-operatively, and also for scheduled versus emergency surgery cases.

Of 11 total cases, nine scheduled thyroidectomy cases had no morbidity after elective surgery. Two cases required emergency surgery for multiple organ failure and cardiac problems. Immediate post-operative complications (mostly haemodynamic) occurred in both cases (emergency vs routine surgery, p = 0.018).

In such cases, pre-operative medical treatment is vital to limit peri- and post-operative complications, but surgery should not be delayed if the haemodynamic status deteriorates. Surgery, with careful anaesthesia, is the cornerstone of the treatment.

The differential diagnosis of endolaryngeal mesenchymal neoplasms includes a wide spectrum of benign and malignant pathologies, which have been rarely photo-documented and assessed as a group.

Non-epithelial neoplasms of the endolarynx seen at our centre from 2002 to 2011 (n = 38; 36 treated at our institution) were retrospectively reviewed, with attention to clinical presentation, radiographic imaging, operative management, histology, and pre- and post-operative endoscopy. Submucosal squamous cell carcinomas, mucosal cysts, amyloid and Teflon granulomas were excluded.

Twenty-three of a total of 36 patients underwent definitive endoscopic surgical treatment. Supraglottic pathologies included lymphoma, lipoma, neuroendocrine carcinoma, lymphangioma, oncocytoma, haemangioma, synovial cell sarcoma and benign spindle cell neoplasm. Transglottic pathologies included synovial cell sarcoma and granular cell tumour. Glottic pathologies included granular cell tumour, osteoma, rhabdomyoma, rhabdomycosarcoma and myofibroblastic sarcoma. Subglottic pathologies included chondrosarcoma, neurofibroma, adenoid cystic carcinoma and vascular malformation.

The site of origin, degree of malignant behaviour and sensitivity to adjuvant treatment determined the course of surgical management, i.e. endolaryngeal versus transcervical, and limited removal versus wider resection.

Syringing of the ear is one of the most common procedures performed for cleaning cerumen from the external auditory canal. Common complications following syringing are pain, external auditory canal trauma and otitis externa. Hearing and vestibular loss have also been reported as complications. However, we are unaware of any report of facial nerve palsy as a complication of ear syringing. Such a case is reported.

We describe a case of facial nerve palsy as a complication of syringing, which demonstrates the dramatic presentation of this condition and emphasises the need for great care while syringing the ears.

It is important to be aware of this unusual complication with its distinctive presentation. Surgical intervention should be undertaken at the earliest opportunity, for favourable results. A risk-minimising strategy for ear syringing is recommended.

We report a case of bilateral pneumolabyrinth presenting as sudden, bilateral deafness, without temporal bone fracture, after a fall.

A 49-year-old man presented with sudden, bilateral deafness and whirling vertigo, without any other neurological manifestations. Temporal bone computed tomography clearly demonstrated the presence of air in the vestibule and cochlea on both sides. However, there was no definite fracture line, ossicular chain anomaly or soft tissue density in the temporal bone or middle-ear cavity. The patient was treated conservatively. Unfortunately, there was no improvement in his hearing.

Pneumolabyrinth is an uncommon condition in which air is present in the vestibule or cochlea. It is rarely found, even with fractures violating the otic capsule or with transverse fractures of the temporal bone. In addition, its bilateral occurrence is extremely rare. In this article, we describe a case of bilateral pneumolabyrinth presenting as sudden, bilateral deafness, without temporal bone fracture, an occurrence which has not previously been reported.

We describe the first reported case of a phosphaturic mesenchymal tumour, mixed connective tissue variant, invading the temporal bone.

A female patient presented with increasing deafness. On examination there appeared to be a mass behind an intact tympanic membrane. Further radiological investigation showed a vascular mass occupying the middle ear, mastoid and internal auditory meatus. This was surgically resected and revealed to be a benign phosphaturic mesenchymal tumour, mixed connective tissue variant. The tumour recurred a year later, presenting as facial nerve palsy. A revision procedure was carried out; the tumour was excised with the sacrifice of a segment of the facial nerve, and a facial-hypoglossal nerve anastomosis was performed.

This case report highlights the occurrence of this benign but sometimes aggressive tumour, of which both clinicians and pathologists should be aware. Early recognition of the condition remains of utmost importance to minimise the debilitating consequences of long-term osteomalacia in affected patients, and to prevent extracranial and intracranial complications caused by the tumour.

We report a case of progressive facial hemiatrophy with cervical sympathetic hyperactivity as a possible underlying aetiology, based on clinical findings, three-dimensional computed tomography and thermographic imaging.

We present a case report in which we describe the investigation and clinical course of progressive facial hemiatrophy, and we also review the world literature on this condition.

To our knowledge, this is the first report in the world literature of progressive facial hemiatrophy with cervical sympathetic hyperactivity indicated as a possible underlying aetiology, based on clinical findings, three-dimensional computed tomography and thermographic imaging.

This syndrome may lead to atrophy of the subcutaneous adipose tissue with hyperfunction of the vegetative system. Although this is a rare syndrome, otolaryngologists should be aware of its symptoms, aetiology and treatment.

We report two extremely rare cases of polyps from unilateral accessory middle turbinates, one of which coexisted with a polyp from an inferomedially projecting, pneumatised, secondary middle turbinate.

Case report and literature review concerning accessory middle turbinate and secondary middle turbinate.

Two patients presented with unilateral nasal obstruction. In both patients, nasal endoscopy revealed polypoid masses originating from the middle meatus. Paranasal sinus computed tomography and histopathological analysis confirmed the presence of polyps originating from an accessory middle turbinate and secondary middle turbinate, which were resected uneventfully via endoscopic sinus surgery.

To our knowledge, this is the first report in the world literature of polyps originating from a unilateral accessory middle turbinate and secondary middle turbinate. Pre-operative recognition of these rare anatomical variations is of particular importance in avoiding intra-operative complication.

Spontaneous pneumocephalus is a rare condition that has been reported infrequently. Alien limb syndrome is an uncommon phenomenon most often seen in patients with frontal and callosal lesions.

Case report of a patient with pneumocephalus presenting with alien limb syndrome. The patient underwent successful surgical management. A literature review and discussion of aspects of this presentation are also included.

In this case, a spontaneous pneumocephalus has formed a frontal space-occupying lesion and presented with alien limb phenomena.

Abnormalities of the third branchial arch are less common than those of the second arch and usually present with left thyroid lobe inflammation. This paper describes 15 cases of pyriform sinus fistulae of third branchial arch origin usually presenting as recurrent thyroid abscess on the left side.

A retrospective review of 15 cases of third arch fistulae managed 2000 and 2008, diagnosed based on histopathology and radiological evidence of a fistulous tract, and treated with fistulectomy with left hemithyroidectomy.

All patients (six boys and nine girls, aged three to 15 years) presented with recurrent low neck inflammation. Pre-operative ultrasound, computed tomography fistulography and barium swallow demonstrated a third arch fistulous tract, left-sided in all cases. The fistula was detected intra-operatively and pathologically in all cases. Surgery (successful in all cases) emphasised complete recurrent laryngeal nerve and ipsilateral pyriform sinus exposure, to facilitate tract excision, with left hemithyroidectomy. There was no recurrence over three to five years' follow up.

Paediatric recurrent low neck inflammatory episodes, due to thyroidal abscess, especially left-sided, should raise suspicion of pyriform sinus fistulae.

We describe the first reported case of a malignant glomus tumour of the larynx.

Case report and review of the world literature concerning malignant glomus tumours.

A 37-year-old man presented with progressive hoarseness and dyspnoea. A smooth, right-sided laryngeal mass was found on flexible nasolaryngoscopy. Initial biopsy of this lesion was reported as a true ‘glomus tumour’, which is a benign lesion. Wide local excision was performed. The final histology of this specimen showed it to be a ‘malignant glomus tumour’. Immunohistochemistry was positive for smooth muscle actin, collagen type IV, vimentin and cluster of differentiation 34 glycoprotein. Total laryngectomy was then performed as a definitive curative procedure.

Glomus tumour is rarely found in visceral locations. This tumour and its malignant counterpart are rare differential diagnoses of laryngeal masses.