Hereditary sensorineural hearing loss is the most frequently occurring birth defect. It has profound effects for the individual and is a substantial burden on society. Insight into disease mechanisms can help to broaden therapeutic options and considerably lower lifetime social costs. In the past few decades, the identification of genes that can cause this type of hearing loss has developed rapidly.

This paper provides a concise overview of the currently known genes involved in non-syndromic hereditary hearing loss and their function in the inner ear.

The main purpose of this study was to investigate the psychological and functional impact attributed to acoustic neuroma symptoms.

A sample of 207 acoustic neuroma patients completed a study-specific questionnaire about the severity, frequency, and psychological and functional impact of 9 acoustic neuroma symptoms.

The survey response rate was 56.4 per cent. All symptoms had some degree of psychological impact for the majority of participants; hearing loss was the symptom most often reported to have a severe psychological impact. The majority of respondents reported functional impact attributed to hearing loss, balance disturbance, dizziness, eye problems, headache and fatigue; balance disturbance was the symptom most often reported to have a severe functional impact. For most symptoms, psychological and functional impact were related to severity and frequency.

Of the acoustic neuroma symptoms investigated, hearing loss and balance disturbance were the most likely to have a severe psychological and functional impact, respectively.

To determine the efficacy of intratympanic methylprednisolone injections for treating sudden sensorineural hearing loss.

A retrospective chart review was performed to identify patients suffering from sudden sensorineural hearing loss with no recovery after oral steroids. Patients were given up to three intratympanic methylprednisolone injections at one-week intervals. They were classified according to their functional hearing class, remission was monitored and potential factors affecting prognosis were analysed.

Intratympanic injections provide effective salvage therapy for sudden sensorineural hearing loss (p = 0.039). Changes in pure tone average and speech discrimination score were analysed following intratympanic methylprednisolone injections. The pure tone average reached a plateau after the second injection; however, the speech discrimination score improved until after the third injection. Hearing improvement after intratympanic injections mainly occurred at low frequencies. The interval between symptoms appearing and intratympanic injections starting was not significantly associated with remission (p = 0.680).

A delay between symptom onset and the first intratympanic methylprednisolone injection does not seem to affect prognosis.

To create a system for the uniform registration and classification of complications and failures in cochlear implant surgery, and apply it to all the patients who underwent implantation in our clinic.

The definition of a medical complication was established, and data for all cochlear implantations performed between 1987 and 2012 were entered into a custom-made database system.

One or more medical complications were registered in 19.0 per cent of 1003 cochlear implantations. The incidence of ‘hard failure’ was 2.3 per cent. Findings revealed a decrease in device failures over the years; the rate of medical complications remained constant.

Our database system, which is available free of charge, enables fast and accurate data entry. There were a relatively high number of (minor) complications in our series, but comparison with the relevant literature is difficult. This emphasises the need for a uniform definition of ‘complication’ as it relates to cochlear implant surgery, and an appropriate classification system for such complications.

The loading of bone-anchored hearing system sound processors usually occurs two to three months after surgical implant. This study examined a new bone-anchored hearing system coupling mechanism that permits loading at two weeks post-implantation without compromising osseointegration.

Twenty implants were implanted into 15 patients. The interval between operation and time of processor loading was recorded, along with the cause of any delay and any late complications.

Two patients were fitted with implants at seven and nine weeks. The delay was a result of administrative errors; the patients reported no skin problems. Of the remaining 17 implants, 8 processors were fitted at 2 weeks, 1 at 3 weeks, 4 at 4 weeks, 3 at 7 weeks and 1 at 8 weeks. For those nine implants fitted later than two weeks, the delay was because of incomplete skin healing.

The Oticon Medical Xpress system allowed processor loading at two weeks post-operatively, providing skin healing was adequate. Early loading occurred in approximately half of the patients. All patients were fitted within the two to three months traditionally allowed. Prolonged skin healing time was the main reason for the delayed fitting of sound processors.

Otitis media represents a major health concern in Australian Indigenous children (‘Indigenous children’), which has persisted, despite public health measures, for over 30 years.

Global searches were performed to retrieve peer-reviewed and ‘grey’ literature investigating the epidemiology of and risk factors for otitis media in Indigenous children, published between 1985 and 2012.

In Indigenous children, the prevalence of otitis media subtypes is 7.1–12.8 per cent for acute otitis media, 10.5–30.3 per cent for active chronic otitis media and 31–50 per cent for tympanic membrane perforation. The initial onset of otitis media in Indigenous children occurs earlier and persists for longer after the first year of life, compared with non-Indigenous children. Indigenous children are colonised by otopathogens more frequently, at younger ages and with a higher bacterial load. Poor community and domestic infrastructure, overcrowding and exposure to tobacco smoke increase the risk of otitis media in Indigenous children; however, the availability of swimming pools plays no role in the prevention or management of otitis media.

Despite awareness of the epidemiological burden of otitis media and its risk factors in Indigenous children, studies undertaken since 1985 demonstrate that otitis media remains a significant public health concern in this population.

Low-dose clarithromycin has been recommended for the treatment of chronic rhinosinusitis without nasal polyps. However, it is uncertain whether a high dose of clarithromycin is more effective than a low dose.

Forty-three chronic rhinosinusitis patients were randomised to low-dose or high-dose clarithromycin groups, and clinical efficacy was evaluated. Pre- and post-treatment measures included: nasal symptom assessment, endoscopic inspection (Lund–Kennedy system), a quality of life questionnaire (the Sino-Nasal Outcome Test 20) and examination of cytokine levels (interleukin-5 and -8) in nasal secretions.

The high dose of clarithromycin was significantly better in terms of clinical efficacy than the low dose for the treatment of chronic rhinosinusitis (p < 0.025). Significant differences in nasal cytokine levels (interleukin-5 and -8) were also observed between the low-dose and high-dose groups after short-term clarithromycin treatment (p < 0.025).

Short-term, high-dose clarithromycin appears to be more effective for the treatment of chronic rhinosinusitis than low-dose clarithromycin.

To determine the incidence of high jugular bulb in a group of patients with definite Ménière's disease, and to investigate whether the position or size of the jugular bulb is significantly different in the affected ear than in the unaffected ear.

Retrospective review of patient charts, audiograms, and computed tomography scans to determine the position and size of the jugular bulb in the affected and contralateral ears, as well as other abnormalities.

High jugular bulb was found in 57.1 per cent of affected ears. Encroachment of the cochlear and vestibular aqueducts was apparent in 39.3 per cent and 35.7 per cent, respectively, of affected ears. Diverticulum and dehiscence were observed in 28.6 per cent of affected ears. High jugular bulb was significantly associated with encroachment of the cochlear aqueduct (p = 0.003).

The mediolateral and anteroposterior position of the jugular bulb determines encroachment of the surrounding structures. An abnormal position is postulated to contribute to the development of Ménière's disease.

To determine factors affecting facial nerve outcome of vestibular schwannoma surgery.

This retrospective cohort study comprised 652 patients. The outcome measure was House–Brackmann classification at two years post-operatively. Univariate and multivariate analyses were carried out to determine the factors affecting facial nerve outcome. The incidence rates of hemifacial spasm, metallic taste and crocodile tear syndrome were recorded.

For tumours less than 1.5 cm, 95 per cent of outcomes were normal, 100 per cent were satisfactory (House–Brackmann grades I–III) and 0 per cent were unsatisfactory (grades IV–VI). For tumours 1.5–2.4 cm, 83 per cent of outcomes were normal, 99 per cent were satisfactory and 1 per cent were unsatisfactory. For tumours 2.5–3.4 cm, 68 per cent of outcomes were normal, 96 per cent were satisfactory and 4 per cent were unsatisfactory. For tumours 3.5–4.4 cm, 52 per cent of outcomes were normal, 80 per cent were satisfactory and 20 per cent were unsatisfactory. For tumours larger than 4.4 cm, 50 per cent of outcomes were normal, 72 per cent were satisfactory and 28 per cent were unsatisfactory.

Tumour size and operation year were significant predictors of facial nerve outcome. The surgical learning curve was steepest for the first 50 patients.

To compare chronic suppurative otitis media prevalence in rural and urban primary school children in Bangladesh, and to determine its relationship with specific sociodemographic factors.

In this cross-sectional survey, 681 rural primary school children from Shibpur upazilla, Narsingdi district, and 964 urban primary school children from Dhaka Metropolitan City, underwent an ENT check-up by doctors trained in ENT. Their parents or guardians were interviewed with regard to their sociodemographic status and other related issues using a pre-tested protocol.

In this study, 6.02 per cent of the rural primary school children and 2.07 per cent of the urban primary school children had chronic suppurative otitis media. (Overall, 3.71 per cent of the children had the disorder.) The disorder was slightly more prevalent among girls than boys in both rural (6.05 vs 5.98 per cent) and urban (2.33 vs 1.82 per cent) communities. There was a significant association between the presence of chronic suppurative otitis media in children and: parents' or guardians' occupation and their annual income, housing type, family size, maternal education, and bathing habit.

Improvement of associated sociodemographic factors would reduce the prevalence and resultant complications of chronic suppurative otitis media in primary school children in developing countries.

To evaluate the long-term stability of intratympanic steroids and investigate the ‘real’ impact of sudden sensorineural hearing loss on patients.

A total of 14 patients treated with intratympanic steroids were evaluated by audiometric and vestibular examinations. The modified Glasgow Benefit Inventory was used to evaluate quality of life changes after intratympanic steroid treatment.

There was no significant difference between pure tone average post-intratympanic steroids and at follow up. The general Glasgow Benefit Inventory score was not significantly associated with the presence of tinnitus or dizziness, or with patient age. The change in pure tone average after intratympanic steroid treatment did not correlate with social or physical scores, but correlated strongly with the general Glasgow Benefit Inventory score (p = 0.0023). Intratympanic steroid administration led to a stable improvement in hearing. Quality of life assessment showed that patients can feel satisfaction regardless of the hearing outcome. Patients who regained a social hearing level expressed greater satisfaction than patients without serviceable hearing. Overall, quality of life improvement was not related to hearing improvement.

Sudden sensorineural hearing loss is devastating. Considering the audiological effects alone ignores the ‘human’ perspective. Audiological success can correlate with poor quality of life outcome.

To observe the long-term outcome of ossiculoplasty using autogenous mastoid cortical bone in chronic otitis media in-patients.

Sixty-one ears of 57 in-patients with chronic otitis media, with or without cholesteatoma, underwent type III tympanoplasty using autogenous mastoid cortical bone as the prosthetic material. Twenty-one ears were treated by canal wall down mastoidectomy and 40 ears by canal wall up mastoidectomy. The follow-up period was 3 to 6 years (average 4.2 years). Pure tone averages for thresholds at 0.5, 1, 2 and 3 kHz were calculated using standard conventional audiometry.

The pre-operative mean air–bone gap of 31.6 dB, for all ears, was reduced to 20.3 dB post-operatively. For the 40 canal wall up ears, this value decreased from 30.8 dB to 19.9 dB, and for the 21 canal wall down ears it decreased from 33.0 dB to 21.0 dB. The differences between the pre- and post-operative mean air–bone gap values were significant.

No cases of extrusion, necrosis or resorption were exhibited for the autogenous mastoid cortical bone prosthesis. A significant hearing improvement was obtained in the majority of cases and this remained stable over time.

Inexperienced otologists require training on the temporal bone drilling process, prior to any surgical activity. The shortage of cadaveric temporal bones exerts pressure to create realistic physical prototypes. This paper describes the evaluation by otology experts of a specially developed temporal bone resin model.

Computed tomography images were transformed into digital files, and anatomically identical right temporal bone models were created using stereolithography. These hand-painted resin prototypes were sent to 25 otologists, accompanied by a 20-item questionnaire.

Satisfaction rate was 92 per cent. The overall prototype score was 48.87 out of 60. Average scores were: 12.63 out of 15 for anatomy-morphology, 6.98 out of 9 for quality of drilling, 16.74 out of 21 for identification of anatomical elements and 7.41 out of 9 for stages of drilling. Limitations of the model included an excessively vivid facial nerve colour and difficulty in identifying the posterior semicircular canal. Disadvantages related to the thickness of the resin and its residues were identified.

The prototype appears to provide an attractive solution to the shortage of cadaveric temporal bones. However, interest in the model for drilling technique training for inexperienced otologists has not yet been assessed.

To determine the prevalence and characteristics of various diagnostic groups amongst patients referred to ENT practices with the primary complaint of dizziness.

A prospective, observational, multicentre study.

Consecutive patients presenting with dizziness to the participating ENT practices were enrolled. Seven ENT specialists at three clinics participated.

Benign paroxysmal positional vertigo was diagnosed in 53.3 per cent of the 1034 study patients. Fifty-nine per cent of these experienced night-time awakening with dizziness, which was a significant proportion in comparison to the other diagnostic groups. Benign paroxysmal positional vertigo was the most frequent diagnosis in all age groups, including those over 70 years.

In this study of patients referred to ENT for dizziness, benign paroxysmal positional vertigo was the dominant diagnostic entity, in all age groups and overall. All clinicians in contact with dizzy patients must consider benign paroxysmal positional vertigo, especially in the elderly.

There has been no previous research to demonstrate the risk of noise-induced hearing loss in industry in Nepal. Limited research on occupational noise-induced hearing loss has been conducted within small-scale industry worldwide, despite it being a substantial and growing cause of deafness in the developing world.

The study involved a cross-sectional audiometric assessment, with questionnaire-based examinations of noise and occupational history, and workplace noise level assessment.

A total of 115 metal workers and 123 hotel workers (control subjects) were recruited. Noise-induced hearing loss prevalence was 30.4 per cent in metal workers and 4.1 per cent in hotel workers, with a significant odds ratio of 10.3. Except for age and time in occupation, none of the demographic factors were significant in predicting outcomes in regression analyses. When adjusted for this finding, and previous noise-exposed occupations, the odds ratio was 13.8. Workplace noise was significantly different between the groups, ranging from 65.3 to 84.7 dBA in metal worker sites, and from 51.4 to 68.6 dBA in the control sites.

Metal workers appear to have a greater risk of noise-induced hearing loss than controls. Additional research on occupational noise-induced hearing loss in Nepal and small-scale industry globally is needed.

Peripheral vestibular function is commonly assessed using the Unterberger test. Patients are asked to march on the spot and their extent of rotation is recorded. The sensitivity of this test depends on an assessor accurately estimating the degree of rotation. This study therefore aimed to compare observer estimates with a smartphone application (DplusR Balance) that accurately records rotation.

Twenty-five participants were asked to estimate the degree of rotation in 10 successive Unterberger tests performed by a volunteer.

The average difference between estimated and application recorded extent of rotation was 30°.

Assessors poorly estimate the degree of rotation in this clinical test, to an extent sufficient to affect clinical interpretation and diagnosis. We recommend the use of this application or alternative methods to record the degree of rotation in patients.

We aimed to evaluate the clinical efficacy of ciprofloxacin plus fluocinolone acetonide (antibiotic plus corticosteroid) ear drops compared to ciprofloxacin (antibiotic) ear drops in diffuse otitis externa.

This was a multicentre, randomised, parallel-group, double-blind study involving 590 patients of both sexes aged 7 years or older.

The rate of clinical cure was higher (p = 0.01) with ciprofloxacin plus fluocinolone acetonide than with ciprofloxacin alone. The mean total symptom score was lower with ciprofloxacin plus fluocinolone acetonide (p = 0.005). No differences were found in the percentage of patients reporting resolution of otalgia between patients receiving ciprofloxacin plus fluocinolone acetonide and patients receiving only ciprofloxacin. Resolution of oedema and otorrhoea (p = 0.003 and p = 0.002, respectively) was higher with ciprofloxacin plus fluocinolone acetonide, as was eradication or presumed eradication (p = 0.003). There were eight mild adverse events, three with the ciprofloxacin plus fluocinolone acetonide combination (not related to the treatment) and five when ciprofloxacin was administered alone (directly related to the treatment).

Ciprofloxacin plus fluocinolone acetonide is a more effective treatment for diffuse otitis externa than ciprofloxacin alone. The ciprofloxacin plus fluocinolone acetonide combination also has an excellent safety profile.

This study describes the clinical features of up-beating vertical nystagmus observed during the seated–supine positional (straight head-hanging) test in patients with benign paroxysmal positional vertigo.

A total of 190 patients with benign paroxysmal positional vertigo symptoms who had presented between 2009 and 2012 were enrolled for this retrospective case series. Twelve patients with positional up-beating vertical nystagmus, as confirmed by video-nystagmography during the seated–supine positional test, were selected.

The incidence and duration of symptoms of multiple canal benign paroxysmal positional vertigo were significantly lower compared with the other types of benign paroxysmal positional vertigo (p = 0.029 and p = 0.048 respectively). Trauma was the leading aetiological factor in those patients (p = 0.012). The average number of therapeutic manoeuvres required for the relief of symptoms in patients with multiple canal involvement was significantly higher than in the other groups (p = 0.041).

In patients with benign paroxysmal positional vertigo, the presence of vertical up-beating nystagmus while lying down is a unique peripheral sign and could indicate multiple canal involvement. Therefore, the seated–supine positional test should always be included in the test battery.