We report a case of a rare cochlear implant complication: the introduction of the electrode array into the superior semicircular canal, with intra-operative measurements of neural response reactions suggesting reasonable functioning of the implant.

A two-year old patient affected by congenital, profound, sensorineural deafness underwent bilateral cochlear implantation at the ENT clinic of the ‘La Sapienza’ University of Rome. Two Clarion 90k devices were implanted, and electrophysiological and radiological checks were performed. After the introduction of the array in the right side, neural response imaging was performed, and a neural potential was found only on two apical electrodes, at a stimulation intensity of 431 clinical units. The situation differed on the left side, where neural response imaging was present at a stimulation intensity of 300 clinical units on the two electrodes tested (one apical electrode (number three), and one middle electrode (number nine)). Intra-operative radiological assessment with a transorbital plain films was performed as usual in order to assess the position of the electrodes inside the cochlea. This radiography showed the electrode array to be in the superior semicircular canal in the right ear.

Intra-operative monitoring tests during cochlear implant surgery play different roles; measurement of impedances and neural response imaging can evaluate the integrity of implant electrodes and the status of the electrode–cochlea interface, but it must not be the sole way in which correct positioning of the array is confirmed. In our opinion, intra-operative radiological assessment is mandatory during cochlear implant surgery.

We report a rare case of giant petrous carotid aneurysm.

Case report and a review of the literature regarding treatment options for such aneurysms.

A 30-year-old man presented with epistaxis, headaches and visual disturbance. Definitive diagnosis was achieved by non-invasive imaging techniques, including magnetic resonance angiography and carotid angiography. Carotid angiography demonstrated a giant petrous carotid aneurysm effacing the petrous apex. The aneurysm was obliterated by internal carotid artery ligation, following successful tolerance of the balloon occlusion test. However, despite internal carotid artery ligation, this patient continued to have minor episodes of epistaxis.

Some aneurysms are too large to be treated with endovascular occlusion techniques; in such cases, ligation of the parent vessel is indicated. However, our patient continued to experience persistent, mild epistaxis despite internal carotid artery ligation, as a result of the reperfusion phenomenon.

The objective of this study was to identify a pattern of signs, symptoms and neuroradiological findings which would assist investigation of vestibular function (especially otolith function) in a group of adult patients with anatomical alterations of the endolymphatic aqueduct and sac.

Fifteen subjects affected by volumetric abnormalities of the vestibular aqueduct were selected from a cohort of patients referred to a tertiary referral neurotological centre between 1 January 2004 and 30 June 2006. All patients underwent accurate clinical history-taking and were evaluated using a standardised set of bedside and instrumental neurotological tests (i.e. audiometry, auditory brainstem response and vestibular evoked myogenic potentials). After these tests, each patient underwent computed tomography and magnetic resonance imaging in order to accurately evaluate the middle ear, labyrinthine capsule and internal auditory canals. These evaluations confirmed clinical suspicion of volumetric abnormalities of the vestibular aqueduct and endolymphatic sac.

All the patients with a defined volumetric alteration in the region of the vestibular aqueduct and endolympatic sac reported a typical pattern of symptoms and signs. The most obvious and frequent symptoms in these patients were migraine-related vertigo (using the Neuhauser criteria, 10 of 15, 66.6 per cent), ‘motion sickness’ (12 of 15, 80 per cent), oscillopsia (nine of 15, 60 per cent) and dizziness (14 of 15, 93.3 per cent). Clinical examination results for the selected patients allowed some useful speculative conclusions. During neurotological evaluation, two instrumental methodologies were especially useful diagnostically: vestibular evoked myogenic potentials of the neck, and the mastoid vibration test at 100 Hz.

Dysfunction of the vestibular aqueduct is suggested by symptomatology characterised by: migraine-related vertigo, unstable or recurring oscillopsia, lowering of the vestibular evoked myogenic potential threshold, hypoacusis, anamnestic report of motion sickness, and nystagmus induced by mastoid vibration and head-shaking. Computed tomography and magnetic resonance imaging are needed in order to confirm clinical suspicions.

To determine the frequency and type of gap junction protein beta-2 gene mutations in Malay patients with autosomal recessive, non-syndromic hearing loss.

A total of 33 Malay patients with autosomal recessive, non-syndromic hearing loss were screened for mutations in the Cx26 coding region. Deoxyribonucleic acid was extracted from buccal swab samples and subjected to polymerase chain reaction. Slow-reannealing was performed, followed by screening using denaturing high performance liquid chromatography.

Eight of the samples (24.2 per cent) showed heterozygous peaks, and further sequencing of these samples revealed four patients (50.0 per cent) with the W24X mutation, two (25.0 per cent) with the V37I mutation and another two (25.0 per cent) with the G4D mutation.

Analysis of buccal swab samples by denaturing high performance liquid chromatography is noninvasive and suitable for rapid and reliable screening of gap junction protein beta-2 gene mutations in patients with autosomal recessive, non-syndromic hearing loss. Malay patients with autosomal recessive, non-syndromic hearing loss have different kinds of gap junction protein beta-2 gene mutations which are rarely found in other populations.

In developing countries, the rate of complications from chronic suppurative otitis media is still high, due to factors associated with poverty. This study aimed to determine the prevalence of chronic suppurative otitis media and associated complications.

We prospectively studied all patients presenting with chronic suppurative otitis media to the ear, nose and throat unit of the Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria, over a two-year period.

One hundred and sixty new cases of chronic suppurative otitis media were seen. These constituted 11.1 per cent of all new cases and 21.5 per cent of all otological cases seen. About half (51.7 per cent) of these patients were younger than 15 years. Most patients were from rural areas and had been previously managed by unlicensed, untrained practitioners. Complications were mostly seen in children. Intracranial complications were seen in 10 (6.3 per cent) cases; this was associated with a high mortality rate.

This study found that, in Nigeria, a developing country, ‘safe’ chronic suppurative otitis media was not without serious problems. Provision of adequate health facilities may reduce such problems.

To determine the expansile and absorptive properties of the expandable ear wick when tested with commonly prescribed ear drop preparations.

This in vitro study assessed wick expansion and absorption of drops when different ear drop preparations were applied. Nine commonly used ear drop preparations were tested (Gentisone HC, Sofradex, Otomize, Betnesol, Exocin, Canesten, Locorten-Vioform, EarCalm and ichthammol glycerin) with a standard expandable ear wick.

Weight, length and width of otowick following exposure to ear drops.

There was a marked difference in the absorption and expansion of the otowick when different drops were used. This corresponded to the aqueous or oil base of each preparation. All aqueous ear drop preparations (i.e. Gentisone HC, Sofradex, Otomize, Betnesol, Exocin and EarCalm) produced similar rates of absorption into, and expansion of, the otowick. The oil-based ear drop preparations (i.e. Canesten, Locorten-Vioform and ichthammol glycerin) failed to expand the wick and showed poor rates of absorption into the wick.

Expandable ear wicks should only be used in conjunction with aqueous ear drops. When using expandable otowicks as an adjunct to treating fungal otitis externa, acetic acid ear drops should be prescribed, as all other anti-fungal drops are oil-based.

To develop an easy method of performing myringotomy and grommet insertion, using minimal instruments.

An ear speculum and a branula were used.

This method was found to be useful.

An easy method of performing myringotomy and grommet insertion is proposed.

We report a histologically proved case of lymphangiomatosis of the skull, involving the temporal bone and presenting as multiple lytic bone lesions.

A case report and a review of the literature concerning lymphangiomatosis are presented.

Lymphangiomatosis affecting bones is a rare disorder caused by a congenital malformation of the lymphatic system, resulting in diffuse proliferation of the lymphatic channels and involving bones, parenchymal organs and soft tissue. Involved bones show massive osteolysis and progressive, localised bone resorption.

Lymphangiomatosis should be kept in mind in the differential diagnosis of lytic lesions of the skull.

The receptive (aural) vocabulary development of children with binaural-aided residual hearing was investigated in a prospective longitudinal analysis (repeated measures).

Thirty-three children with sensorineural hearing loss, without major co-morbidities (mean age at the first testing point, 56.2 months, standard deviation 19.0 months) were recruited from the 1994 German Goettinger Hearing Language Register. Their receptive vocabulary was measured using standardised tests (with reference to normal hearing child performance) on three separate test points (mean follow up, 18.7 months, standard deviation 5.0 months).

On average, the study group scored below the normal range at the first and second tests and made a significant improvement at the last test. The degree of hearing loss was found to be significantly correlated to vocabulary performance at all time points, with increasing correlation coefficients. At the third test, significant predictive variables for successful receptive vocabulary outcome were found to be: degree of hearing loss (t = 5.43; p < 0.0001); age at diagnosis (t = 2.29; p = 0.03); and nonverbal intelligence (t = 2.82; p = 0.009).

If permanent childhood hearing impairment is mild and/or is detected early, and if the child grows up in a monolingual environment, the development of receptive vocabulary within the normal range is possible.

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.

We report a rare case of otological presentation of craniopharyngioma.

Case report and review of world literature concerning presentations of craniopharyngioma.

A six-year-old girl was referred to our department with unilateral hearing loss. This appeared to be a complete sensorineural hearing loss. Otoacoustic emissions were reproducible on both sides. Magnetic resonance scanning revealed a massive, cystic craniopharyngioma exerting pressure on the patient's ventricular system and brainstem and also invading the internal acoustic canal. The mass was resected via a craniotomy. The patient's hearing loss completely recovered, and she experienced no neurological or endocrinological side effects of the treatment. Craniopharyngioma have a prevalence of 0.13–2:100 000.

Craniopharyngioma is a rare disease. First presentation with otological symptoms is extremely rare. Otoacoustic emissions can differentiate between cochlear and retrocochlear causes of sensorineural hearing loss.

To evaluate the reliability of transcranial magnetic stimulation in eliciting admittance changes due to activation of middle-ear muscles.

Admittance changes induced by transcranial magnetic stimulation at the inion were evaluated in eight normal subjects, two subjects with prelingual deafness and 22 patients suffering from other otological disorders characterised by absence of acoustic reflex.

Responses showed a predominant negative peak in normal ears. Two small positive components, one preceding and the other following the negative deflection, were less consistently elicited. Only a positive wave was detected in otosclerotic subjects. Patients with tympanic membrane perforation or previous tympanoplasty with ossicular discontinuity did not show any response.

Transcranial magnetic stimulation is able to activate both stapedius and tensor tympani muscles. In conjunction with admittance audiometry, it may represent a method of exploring the mechanics of the middle ear when acoustic reflex testing is not reliable. It can be helpful in the confirmation of stapes fixation when a severe to profound hearing loss is present.

To investigate the use of a new technique to close persistent tympanic membrane perforations under general anaesthesia, in patients in whom this has previously been considered impractical.

Twenty patients aged 50 years and over were recruited. All had a persistent, symptomatic tympanic membrane perforation in at least one ear. Pre-operatively, an audiogram was performed and the tympanic membrane was assessed in order to establish the site and size of the perforation. Under general anaesthesia, the edges of the perforation were freshened and a sheet of Epifilm, trimmed to a size roughly 2 mm larger in diameter than the perforation, was tucked through the perforation. The ear was dressed appropriately. Patients were seen two weeks post-operatively in the out-patients department and reassessed.

The study was based at Poole General Hospital, a District General Hospital.

Twenty patients were recruited from the out-patients clinics of Poole Hospital ENT department.

(1) closure of the perforation, as a percentage of the original size; (2) improvement of hearing, as an average across all frequencies, expressed in dB hearing level; and (3) presence or absence of discharge.

Five patients were operated upon and assessed six weeks post-operatively. The Epifilm had dissolved but the perforations remained the same size in all five patients. There were no other adverse effects. Pure tone audiometry at this stage was redundant. The study was subsequently aborted.

The authors conclude that repair of tympanic membrane perforations with hyaluronic acid ester films is not to be recommended.

This paper attempts to review changes in the lives of hearing-impaired patients within the developing world, brought about by globalisation and development. The paper also explores limitations to improved care and addresses the collective moral responsibility of developed nations.

Analysis of literature.

Within developing nations, large populations have emerged with a similar pattern of problems, access to information and aspirations as those living in developed nations. However, marked differences in income have persisted. These trends have resulted in a relative increase in the proportion of the hearing-impaired population in need of cochlear implantation, while at the same time restricting their access to such treatment.

The emergence of global markets and media and a shared sense of destiny amongst the people of this planet should translate into a concerted, worldwide effort to assist the deaf in developing countries. Much more can be done within existing resources and frameworks to improve the quality of these peoples' lives.

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.

To investigate vestibular function in human immunodeficiency virus positive subjects.

We studied vestibular function in 60 human immunodeficiency virus positive subjects reporting dizziness. All three Center for Disease Control and Prevention categories of human immunodeficiency virus infection were represented in the study group (30 patients in class A, 20 in class B and 10 in class C). Subjects had had no previous history of acute vertigo. All subjects underwent: neurotological screening for spontaneous, positional and positioning nystagmus, using head-shaking and head-thrust (Halmagyi) tests; audiometrical examination; and electronystagmography with bithermal stimulation (Freyss' method). The results of the 30 class A subjects were compared with those of 30 human immunodeficiency virus negative patients reporting dizziness.

Abnormal otoneurological findings increased progressively from the A to C categories, particularly regarding increased central damage (3.3 per cent of class A, 35 per cent of class B and 100 per cent of class C subjects). In contrast, the incidence of peripheral vestibular disorders remained almost the same, comparing the three categories (33.3 per cent in class A and 50 per cent in classes B and C subjects). Moreover, a higher number of human immunodeficiency virus positive subjects showed abnormal otoneurological findings, compared with the dizzy, human immunodeficiency virus negative subjects.

In our opinion, a vestibular disorder may occur in human immunodeficiency virus positive patients as a result of direct viral damage, even in the early phase of infection. Central vestibular damage may be established later on, and may be linked to different causes (e.g. superinfections, vascular causes and drug toxicity).

Otitis media with effusion is a common condition of childhood. The development of an information leaflet for parents of children with the condition, and its impact on clinical management, have not previously been examined.

Eighteen doctors and 38 parents assessed the content of an information leaflet on otitis media with effusion, by applying two rounds of the modified Delphi technique. A qualitative assessment of content items was also performed.

From the 23-item list used in the first assessment round, four items had a low doctor–parent agreement and seven were excluded. Differences were also noticed in comments on the value of such leaflets, with parents being more positive about the value of leaflet distribution.

During the consultation, doctors may not tell parents what they want to know, especially regarding daily care of their child. An information leaflet, developed using the Delphi technique, can help reduce this discrepancy and increase parents' satisfaction.

To evaluate quality of life after surgery for benign neoplastic disease of the parotid gland.

A quality of life questionnaire, which was created from the Hebrew version of the University of Washington Quality of Life prototype, was applied to 55 patients who underwent surgery for benign neoplastic parotid disease. All patients were examined in Baskent University Adana Teaching and Medical Research Center, where all except 10 subjects (who responded by phone) completed the quality of life questionnaire.

The highest overall score was 96.3 indicating no salivary fistula. Only one patient complained of salivary secretion through the wound scar. The lowest overall score was 59.5 indicating loss of sensation. Although no post-surgical pain was reported by 32 (58 per cent) patients, 16 (29 per cent) patients reported a post-surgical change in their appearance. Scarring and surgical site depression were reported by 26 (47 per cent) and 17 patients (30 per cent), respectively. Facial nerve impairment was reported by seven patients (13 per cent) during the early post-operative period; these patients recovered from that impairment. The only significant statistical correlations were noted between general health and gender, and between post-surgical pain and gender. There was not any statistical correlation between all data and age, tumour type and education level.

The general status of patients who have undergone surgery for a benign parotid neoplasm can be assessed with a quality of life questionnaire. Patients' post-surgical quality of life can be improved by the correct choice of surgical approach and reconstructive method.