To evaluate the results of vestibular evoked myogenic potential testing in patients with idiopathic sudden hearing loss, and to correlate these results with the findings of caloric testing, the clinical appearance of vertigo and the influence of age.

Eighty-six patients with unilateral idiopathic sudden hearing loss and 35 healthy controls underwent a standard protocol of neurotological evaluation. Vestibular evoked myogenic potential responses were measured and compared with caloric responses.

On the affected side, 30.2 per cent of patients showed abnormal vestibular evoked myogenic potential responses, while 52.3 per cent had abnormal caloric responses. A statistically significant relationship was found between the results of these two tests. A statistically significant relationship was also found between the type of vestibular lesion and the occurrence of vertigo. Advancing age correlated statistically with more extensive labyrinthic lesions.

A combination of vestibular evoked myogenic potential and electronystagmography testing indicated the existence of vestibular involvement in many patients with idiopathic sudden hearing loss. Both tests are necessary in order to obtain a more thorough and in-depth knowledge of the pathophysiology of idiopathic sudden hearing loss.

Conscious and deceptive exaggeration of hearing loss is termed pseudohypacusis. Even though the Stenger test has been used in the management of pseudohypacusis for almost a century, its sensitivity, specificity and predictive values for unilateral pseudohypacusis have not previously been reported, to our best knowledge. We investigated the efficiency of the Stenger test in detecting unilateral pseudohypacusis, accepting auditory brainstem response testing as the ‘gold standard’.

Candidates with questionable profound or total hearing loss were enrolled in the study. Pure tone audiometry, speech and tonal Stenger tests, and click test auditory brainstem response measurement were performed. Accepting auditory brainstem response testing as the gold standard, the sensitivity, specificity and predictive values of the Stenger test for unilateral, profound pseudohypacusis were assessed.

Two hundred military candidates were enrolled in the study. The sensitivity and specificity of the Stenger test in verifying unilateral, profound hearing loss were 99.4 and 70 per cent, respectively. The positive and negative predictive values of the test were 87.5 and 98.4 per cent, respectively.

The Stenger test is widely used for the evaluation of unilateral or asymmetrical pseudohypacusis. In our opinion, it is a powerfully reliable test. More difficult cases require objective electrophysiological testing to verify functional hearing loss and to exclude specific diagnoses that may imitate pseudohypacusis.

The Avon Area Health Authority was a first-phase site for introduction of universal newborn screening in the UK. The aims of this study were: to review the programme's results to date; to assess the impact screening would have on other services (e.g. the cochlear implant programme); and to assess the longer term outcome for children identified by the screening programme.

All children identified by the Avon universal newborn hearing screening programme between April 2002 and July 2006.

Fifty-four children with a bilateral hearing impairment of worse than 40 dBHL were identified from a screened population of approximately 44 000. Nine of these children were put forward for cochlear implantation, and seven had been implanted at the time of writing. Thirteen of these children were identified with possible auditory neuropathy or dys-synchrony. All the newborn hearing screening programme criteria assessed were met.

The screening programme was effective. Some areas may need review in order to optimise patient care.

Following a report of sudden hearing loss in a patient taking phosphodiesterase type 5 inhibitor, and a Food and Drug Administration announcement concerning this class of drugs, a study was planned to investigate if ototoxicity occurs in patients using phosphodiesterase 5 inhibitor for erectile dysfunction.

Eighteen patients with erectile dysfunction who had been using phosphodiesterase 5 inhibitor were included in the study. Audiometric tests were performed on all patients, between the frequencies 250 and 16 000 Hz, before and 1, 5 and 72 hours after drug ingestion.

Four patients showed a unilateral threshold decrease compatible with ototoxicity criteria; this change was reversible. A statistically significant difference in pre- versus post-drug hearing thresholds was observed in the right ear at 10 000 Hz (p = 0.008). There were no statistically significant hearing threshold differences at any other frequencies (p > 0.05).

Although temporary ototoxicity was noted in four patients, we could not find any permanent, deleterious effect of phosphodiesterase type 5 inhibitor on hearing thresholds.

We report the unique case of a 16-year-old man presenting with sudden and profound left-sided hearing loss following application of a Halo vest.

Case report and review of the world literature concerning spinal realignment procedures, spinal manipulation and Halo vest application.

A 16-year-old man presented to the ENT clinic with sudden and profound left-sided hearing loss after undergoing posterior release of a fixed flexion extension deformity of the cervical spine and Halo vest application. The hearing loss slowly improved until the patient disrupted the Halo vest while boarding a bus. Regular audiometry documented the progress of the patient's hearing loss.

Procedures that significantly alter the established bony anatomy of the neck can be associated with profound audiological deficit through disturbance of the vertebrobasilar arterial system. Such a phenomenon may be associated with application or disruption of a Halo vest.

To evaluate hearing results following intratympanic gentamicin perfusion in patients with Ménière's disease.

Middle-ear perfusion of 0.4 ml of gentamicin 40 mg/ml solution was performed in Ménière's disease patients who had previously failed to respond to other medical treatment.

Between May 1999 and November 2005, 22 patients (mean age 58.5 years) underwent intratympanic gentamicin perfusion. Mean follow up was 30 months. At the first post-perfusion visit (four to six weeks following perfusion), three of the 22 patients (13.63 per cent) had a pure tone average improvement of at least 10 dB, 15 (68.18 per cent) showed no change and four (18.18 per cent) demonstrated a decrease in hearing of more than 10 dB. Regarding speech discrimination scores, one patient (4.54 per cent) exhibited an improvement of at least 15 per cent, 15 (68.18 per cent) showed no change and six (27.27 per cent) showed a decrease of at least 15 per cent. After long-term follow up (12–40 months following perfusion), 10 patients (45.45 per cent) showed stable hearing, and 12 (54.54 per cent) exhibited a pure tone average decrease of more than 10 dB. Six patients (27.27 per cent) showed a speech discrimination score decrease of at least 15 per cent, while 16 (72.72 per cent) had no change. Complete cessation of vertigo was reported by 20 of the 22 patients (90.9 per cent), while two (9.09 per cent) reported episodic vertigo spells.

Intratympanic gentamicin perfusion provides effective control of vertigo in patients with Ménière's disease. However, significant hearing loss may occur immediately after perfusion; therefore, this treatment should be considered only for patients whose hearing has already been affected by the disease.

This case is reported in order to demonstrate the importance of detailed clinical analysis, including evaluation of personal and family history, in the differential diagnosis of sudden sensorineural hearing loss.

A 50-year-old woman presented with a sudden onset of sensorineural hearing loss in her right ear. She had experienced three previous episodes of sudden sensorineural hearing loss in her left ear, at the ages of 35, 48 and 50 years. She also reported suffering two strokes with left hemiparesis due to fibromuscular dysplasia of her right internal carotid artery. A positive family history of stroke among maternal relatives suggested autosomal dominant inheritance. The patient's personal and family history suggested a rare cause of sudden sensorineural hearing loss, for which alternative therapeutic modalities may be applicable in selected cases.

Careful follow up of any patient with sudden sensorineural hearing loss and evaluation of their personal and family history is essential, in order to uncover evidence of rare underlying causes of sudden sensorineural hearing loss. For patients with such rare diagnoses, alternative therapy and surveillance modalities may be useful in disease management, depending on pre-existing pathology. Those patients should be managed via a multidisciplinary approach, including genetic counselling, in order to achieve the best possible outcome.

Congenital cytomegalovirus infection is the leading identified nongenetic cause of congenital sensorineural hearing loss. Most of the infections are asymptomatic but may be detected from umbilical cord vein and/or newborn serum positivity for human cytomegalovirus immunoglobulin M, and from urine positivity (on polymerase chain reaction) for human cytomegalovirus deoxyribonucleic acid in the newborn period. Children infected by cytomegalovirus may later develop sensorineural hearing loss. In symptomatically infected infants, ganciclovir therapy administered in the neonatal period prevents hearing deterioration. However, preventative therapy of asymptomatic congenital cytomegalovirus disease with ganciclovir is controversial, as side effects such as severe neutropenia may occur during treatment.

The study population consisted of 23 asymptomatic children with congenital cytomegalovirus infection. Twelve children were treated just after diagnosis of cytomegalovirus infection in the newborn period, with ganciclovir 10 mg/kg bodyweight for 21 days. The other 11 children were observed without therapy. Over a four to 10 year follow-up period, we evaluated all the children's hearing status using pure tone audiometry.

All 23 children had normal sensorineural hearing at one year follow up. Five of the 23 children (21.7 per cent) were lost to follow up over the four to 11 year follow-up period. Of the remaining 18 children, sensorineural hearing loss occurred in two (11.1 per cent). Neither child had been treated with ganciclovir in the newborn period. An eight-year-old boy showed bilateral high frequency loss and a 10-year-old girl showed severe unilateral sensorineural hearing loss. In the ganciclovir-treated group (nine children), none showed sensorineural hearing loss. During ganciclovir therapy, moderate neutropenia occurred as a side effect in two out of 12 (16.6 per cent) treated children. Speech and general development were normal in all children.

Asymptomatic congenital cytomegalovirus infection is likely to be a leading cause of sensorineural hearing loss in young children. Intravenous ganciclovir therapy seems to offer a medical option to prevent subsequent sensorineural hearing loss. Further studies including a greater number of children are needed. Cytomegalovirus screening models are mandatory if medical therapy is to be implemented in time.

Deafness is the hidden disability of childhood, and leads to poor educational and employment prospects. There is little published information on deafness in Pakistan. Profound hearing impairment is more prevalent in countries where consanguineous marriages are common, such as Pakistan. This study aimed to assess causes of childhood deafness and association with parental consanguinity, within deaf and hearing children in the Peshawar district of Pukhtoonkhwa Province, Pakistan.

One hundred and forty deaf children were identified from two schools for deaf children within the Peshawar district. These children were assessed via audiology, otoscopic examination, case note review and parental history, in order to attempt to ascertain the cause of their deafness. Two hundred and twenty-one attendees at a local immunisation clinic (taken as representative of the local childhood population) were also screened for hearing impairment. Parents of both groups of children were assessed by interview and questionnaire in order to ascertain the mother and father's family relationship (i.e. whether cousins or unrelated).

Of the 140 deaf school pupils, 92.1 per cent were profoundly hearing impaired and 7.9 per cent were severely hearing impaired. All these children had bilateral sensorineural hearing loss. A possible cause of deafness was identified in only six of these children. Parental consanguinity (i.e. first or second cousins) was established for 86.4 per cent of deaf school pupils and 59.7 per cent of immunisation clinic attendees. None of the control children were identified as having a hearing problem.

The prevalence of parental consanguinity was significantly higher in deaf children compared with non-hearing impaired children. However, the study also confirmed a high rate of consanguinity within the general Peshawar community. In this setting, prevention of consanguineous unions is the only means of reducing levels of congenital hearing impairment. The current levels of hearing disability represent both a prominent public health problem and an important, potentially preventable childhood disability.

Previously, we have mapped the DFNA52 (Online Mendelian Inheritance in Man (OMIM) 607683) locus, using an 8.8-cM interval on the human chromosome 5q31.1-q32, in a large, consanguineous Chinese family with congenital sensorineural hearing loss. In order to identify the responsible pathogenic mutation within the DFNA52 locus, we set out to identify candidate disease genes within that region and to sequentially analyse these candidate genes.

Using bioinformatics analysis, 52 candidate disease genes were identified based on gene expression data, deafness phenotype, and findings from a mouse model and from the literature (including two mouse deafness genes NEUROG1 and SMAD5). Mutation detection was performed for the 52 candidate genes, in patients from the pedigree.

In these patients, we found no disease-causing mutations in the coding and splice site regions of these genes, which segregated with the disease. However, 108 single nucleotide polymorphisms were identified, of which 15 were novel. Eleven of these 108 single nucleotide polymorphisms altered the encoded amino acid.

Although we identified a number of nucleotide changes in the affected patients, by analysis of coding and splice site regions of the genes, none of these changes are likely to be pathogenic mutations segregating with the disease. The result implies that the genes studied are unlikely to be a cause of DFNA52-linked sensorineural hearing loss.

To characterise the hearing loss, and the frequency of the mitochondrial deoxyribonucleic acid 12S ribosomal ribonucleic acid A1555G mutation, in a large pedigree of aminoglycoside-induced deafness.

Hearing loss was clinically assessed. Blood samples were collected from 27 family members (19 matrilinear and eight non-matrilinear) and leukocyte deoxyribonucleic acid was extracted. Mitochondrial deoxyribonucleic acid fragments, spanning the 1555 location, were amplified by polymerase chain reaction. Polymerase chain reaction products were analysed by restriction fragment length polymorphism and deoxyribonucleic acid sequencing.

We detected the A1555G mutation in all 19 matrilinear relatives. Of these 19, two exhibited congenital deafness, four had no hearing deficits and the remaining 13 suffered mild to profound hearing loss.

We confirmed that the A1555G mutation is a ‘hot spot’ associated with non-syndromic, inherited hearing loss. This mutation may play a vital role in the pathogenesis of hearing impairment, and can result in various grades of deafness.

The aim of this study was to investigate the differences between pre- and post-operative hearing thresholds, measured by high frequency audiometry, in patients undergoing open heart surgery.

Pre- and post-operative audiometric assessments were performed in 20 patients undergoing open heart surgery. Pure tone audiometry testing was performed at 500, 1000, 2000, 3000, 4000, 6000, 8000, 10 000, 12 000 and 14 000 Hz. We also evaluated: patients' clinical parameters (i.e. age, sex, diabetes mellitus, hypertension, hypercholesterolaemia, history of myocardial infarction, and whether undergoing coronary artery bypass surgery or valve surgery); various operative details (operative temperatures, cardiopulmonary bypass time and cross-clamp time); and post-operative clinical progress and its effect on hearing loss.

Patients' pre- and post-operative pure tone audiometric results were significantly different at some frequencies (p < 0.05). In addition, there was a significant impact of hypertension, hypercholesterolaemia, history of myocardial infarction and cross-clamp time.

This study shows that open heart surgery using cardiopulmonary bypass can lead to significant post-operative changes in hearing levels at some frequencies. Some additional patient parameters may influence this process. It seems possible that the risk of such hearing loss could be reduced; further studies may be able to define the significance of patients' concomitant disorders.

To quantitatively evaluate the diagnostic accuracy of diagnostic tests for immunomediated hearing loss.

We searched Medline and the Cochrane Database of Systematic Reviews for potentially relevant studies.

Twenty-five studies met the inclusion criteria of this systematic review. The diagnosis of immunomediated hearing loss was based on the clinical presentation and the response to corticosteroid administration.

The following data were extracted from the selected studies and entered into a standardised database: population demographics; exclusion and inclusion criteria; diagnostic tests; sensitivity; specificity; the number of true positive, true negative, false positive and false negative values; therapy used, including dose and duration; and delay between symptom onset and therapy commencement.

This systematic review combined data from 679 patients with immunomediated hearing loss, reported by 22 research teams. Substantial heterogeneity was found among the included studies; for this reason, summary sensitivity and specificity values were not computed.

The results of diagnostic tests for immunomediated hearing loss depend on many factors, and there is a risk of potential bias. This is the first time that such a systematic review has been presented; such a review is a more rigorous method of demonstrating the utility of the available diagnostic tests.

Despite multiple systemic manifestations, sudden sensorineural hearing loss in a patient with antiphospholipid syndrome is rarely reported.

A 46-year-old man with primary antiphospholipid syndrome had a sudden onset of hearing loss and tinnitus in the right ear in December 2005, because he discontinued use of warfarin and acetylsalicylic acid for a few days.

Audiometry revealed saucer-type sensorineural hearing loss with a pure tone average of 73 dB in the right ear, and flat-type hearing loss with a pure tone average of 25 dB in the left ear. Electronystagmography displayed multiple central signs and bilateral canal paresis, while a vestibular evoked myogenic potential test revealed bilateral delayed responses. After admission, the patient was re-treated with warfarin and acetylsalicylic acid. Follow-up audiometry showed recovery of right-sided hearing, with a pure tone average of 12 dB, three days after presentation.

Consensus exists on the effectiveness of anticoagulant agents in aiding a favourable outcome of sudden sensorineural hearing loss in patients with antiphospholipid syndrome.

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.