Isolated congenital anomalies of the ossicles are rare. The majority of cases of congenital conductive hearing loss secondary to middle-ear anomalies have other associated defects, such as atresia, microtia and craniofacial deformities.

We present a rare case of isolated congenital stapes suprastructure fixation, where a monocrural stapes with a mobile footplate was attached to the promontory by bony synostosis. There was no stapedius muscle nor pyramidal process. The incus and malleus were of normal configuration. Mobilization of the stapes from the promontory resulted in improvement in hearing. We believe this is the first reported case of such an abnormality. A review of the world literature of isolated stapes suprastructure ankylosis and the classifications of minor congenital anomalies are discussed.

The midfacial degloving approach has been available for twenty five years and is slowly increasing in popularity in the management of extensive benign lesions of the sinonasal region, for selected malignancy in this area and to afford access to the nasopharynx and infra-temporal fossa. The advantages, applications and low complication rate are presented in a cohort of 86 patients, ranging from three to 79 years of age with a mean follow-up of 5.5 years. Seventy-seven per cent of the group had benign pathology of which juvenile angiofibroma was the commonest (40 cases). The 20 cases of malignant disease were a heterogeneous group histopathologically including adenoid cystic carcinoma (four cases), malignant schwannoma (two cases), rhabdomyosarcoma (two cases) and squamous cell carcinoma (two cases). Five underwent bilateral radical maxillectomies combined with orbital clearance in one patient. Complications include oro-antral fistula (three cases) and epiphora (three cases) all of which were successfully treated.

Surgeons using the operating microscope are able to make use of numerous landmarks described for the lateral limits of dissection to preserve hearing in acoustic neuroma surgery via the retrosigmoid approach. Similar landmarks for hearing preservation described specifically for the endoscopic-assisted technique, are lacking. By analysing computed tomography (CT) scans of temporal bones, it was observed that to reach within 3 mm of the lateral end of the internal auditory meatus (IAM) via a 3 cm retrosigmoid craniotomy, drilling should be up to about 3 mm medial to the opening of the vestibular aqueduct. It was hypothesized that in surgery, by keeping 3 mm medial to the opening of the vestibular aqueduct, the integrity of inner ear structures would be preserved. This hypothesis was tested in 30 temporal bones and was found to be true. In addition, the lateral end of the IAM up to the transverse crest could be viewed by the 30-degree rigid angled endoscope. This landmark could, therefore, be utilized in the endoscopicassisted technique to predict the optimal amount of bone to be removed at a stage before the internal auditory meatal dura is opened when the intact dura affords added protection to the meatal contents during drilling. Well designed dural flaps on the posterior petrous bone could be created by making a longitudinal incision not more than 7 mm from the superior border of petrous bone and a transverse incision at least 17 mm from sigmoid. These flaps minimize injury to the endolymphatic sac and protect the cochlear nerve and vasculature that when damaged, may result in hearing loss.

This paper describes the development of adult cochlear implantation in Birmingham over its first six years, from 1990 to 1996. During this time 366 patients were referred to the implant programme, female referrals outnumbered males 1.2:1. Eight per cent of referrals were over the age of 70.

The annual rate of referral suggests a fairly steady demand for the service. Approximately one third of patients referred each year were subsequently implanted. The centre is currently performing an average of 2 implants per month. The average time from referral to implant is approximately 19 months.

The greatest number of referrals originated from health districts closest to the implant centre. An examination of patients implanted suggested an imbalance in the uptake of the service amongst the different socio-economic groups.

This paper heralds a series entitled The Future of ORL-HNS and Associated Specialties which the Journal will run through each issue of the year 2000.

A total of 305 children, five to 16 months of age, were treated from 1983–1984 with ventilation tubes – Shah vent Teflon tube – inserted under local anaesthesia for recurrent acute otitis media (RAOM) or otitis media with effusion (OME). The final study group comprised 281 children (92.1 per cent) monitored prospectively for five years, 185 in the OME-group and 96 in the RAOM-group. For the first insertion of tubes the average ventilation period was 15.4 months. Re-tympanostomy, with adenoidectomy simultaneously at the first time was performed in 99 ears (35.2 per cent); once in 27.0 per cent, twice in five per cent, and three times in 3.2 per cent. Mastoidectomy due to otorrhoea was performed in three ears (1.1 per cent). The children in the OME-group were at higher risk of repeated post-tympanostomy otorrhoea episodes than children in the RAOM-group. These episodes of otorrhoea during the first insertion of ventilation tubes significantly increased both the tube extrusion rate and the need for subsequent re-tympanostomies. No major complications were caused by the tympanostomy procedure as such. It is concluded that early tympanostomy is a safe procedure in young children with RAOM or OME. However, parents should be carefully informed of risks of post-tympanostomy otorrhoea and recurrent disease after insertion of ventilation tubes necessitating subsequent tube insertion, especially in children with OME.

Thirty-four ears with conductive hearing loss due to otosclerosis were operated upon using the laser stapedotomy technique. Audiological results were compared with the results of 316 non-laser stapedotomies. The post-operative air-bone gap, calculated as the difference between the postoperative air and bone conduction levels, was smaller with the laser stapedotomy group. Also, the bone conduction showed significant improvement with the use of laser.

Significant sensorineural hearing loss was not found in any of the laser-treated patients. According to our results, we concluded that laser is of benefit in stapes surgery for improving the hearing results and minimizing the inner ear trauma.

Neurogenic inflammation may play a role in the aetiology of secretory otitis media (SOM). The strongest candidate that initiates the characteristic symptoms of neurogenic inflammation is supposed to be substance P. Capsaicin is a specific antagonist of substance P. The effects of capsaicin on middle ear mucosa have not been studied yet. In an attempt to investigate the effect of pre-treatment with capsaicin on the development of SOM an experimental study was performed. Fourteen Wistar rats were divided into two groups. Seven rats were pre-treated with capsaicin (Group 1) and the others were administered isotonic saline solution (Group 2). Seven days after the third injection rats were operated on and the right tympanal orifice of the Eustachian tube was obstructed. Animals were sacrificed seven days after the operation. Their bullas were excised bilaterally and were studied by light microscopic technique. In Group 1 there was no effusion except for one case. The subepithelial layer was thickened by fibroblast proliferation. Capillary proliferation and some glandular atrophy were observed. In Group 2 the middle ear lumens were filled with effusion. Oedema with dilatation in capillaries and medium-sized vessels of lamina propria was observed as a common feature of the group. Subepithelial fibrosis was found in one case. Capsaicin pre-treatment prevented the formation of effusion in the middle ear lumen in spite of tuba occlusion. The results of this preliminary study lead us to consider that an imbalance in the autonomic innervation of the mucosa of the middle ear may play a role in the aetiology of SOM as in vasomotor rhinitis, and capsaicin may be an alternative in the treatment.

Hearing loss has long been associated with diabetes mellitus. Microangiopathy, associated with thickening of the basement membranes of small vessels, has been implicated as a major source of multiple system organ disease.

Objective This study was designed to evaluate changes in basement membrane thickness in the inner ear of laboratory animals suffering from non-insulin-dependent diabetes mellitus (NIDDM) with, and without, exposure to moderate intensity noise exposure in an attempt to extrapolate the same disease process in humans.

Design Spontaneously hypertensive-corpulent non-insulin-dependent rats (SHR/N-cp) were selected as a genetic model for the above study. Both lean and obese rats were used in this study. A genetically similar control group of animals (LA/N-cp) were used as controls. These animals express both the lean and obese phenotypes, but they lack the NIDDM gene. Forty-eight animals in each group were sacrificed at the end of the study. The cochleas were dissected and fixed. The basement membrane of the stria vascularis was examined using transmission electron microscopy.

Setting This study was a laboratory-based, standard animal study.

Main outcome This study was designed to show microangiography of the inner ear as related to NIDDM with, and without, obesity and noise exposure.

Results/Conclusions NIDDM alone does not cause statistically significant basement membrane thickening; however, NIDDM in combination with obesity and/or noise exposure did show significant thickening and the combination of all three showed the greatest thickening. NIDDM appeared to be the greatest contributing factor.

Nasal continuous positive airway pressure (nCPAP) is now the treatment of choice for patients with sleep apnoea syndrome. Side-effects and adverse reactions have been described in isolated reports with this device. We have, therefore, systematically studied the side-effects of nCPAP therapy in 300 consecutive patients referred to the London Chest Hospital Ventilatory Support Unit. Ninety-six per cent of patients complained of at least one side-effect resulting from the therapy, with 45 per cent complaining of a side-effect specific to the nasal mask. There was no correlation between the side-effects and level of pressure used during nCPAP. The rate of compliance remained high in spite of the side-effects, with a daily use of 7.8 hours (SD 8.05) and with 83 per cent of the patients using the device every night. Although nCPAP treatment remains acceptable to most patients there exists a high incidence of adverse effects.

Recent developments in our understanding of prion diseases have raised concerns for the public health. There is now compelling evidence that the transmissible agent for variant Creutzfeldt-Jakob disease (vCJD) in affected individuals is accumulated in lymphoreticular tissues such as the appendix and tonsils. This agent demonstrates a remarkable resistance to standard methods of sterilisation used in hospital sterile services departments. The possible implications this has on the safety of surgical instruments in ENT and other surgical practice is discussed. This review also outlines the history of our understanding of prion diseases and describes the development of a diagnostic test for vCJD in the living patient by pharyngeal tonsil biopsy.

Factors affecting auditory improvement after stapes surgery were investigated retrospectively on a study group of 106 otosclerotic ears (86 subjects). While the closure of the air-bone (A-B) gap after surgery was good at 2 kHz and 4 kHz, it was poor at 8 kHz and at frequencies lower than 1 kHz. Under 1 kHz, the lower the frequency, the worse the A-B gap after surgery. Stapedotomy and partial stapedectomy showed better post-operative hearing gain at 4 kHz than total stapedectomy. Total stapedectomy scored significantly better at 250 Hz and 500 Hz than stapedotomy. There was a close relationship between the pre-operative and post-operative A-B gap at frequencies under 1 kHz. The smaller the pre-operative A-B gap, the better the closure of the post-operative A-B gap at frequencies under 1 kHz. The smaller the pre-operative A-B gap, the better the closure of the post-operative A-B gap at these frequencies. It was speculated that otosclerotic ears with a larger pre-operative airbone gap might have another lesion in the middle ear other than the oval window.

Conservative treatment for otitis media with effusion (OME) led us to consider the use of hearing aids as a way of managing the associated hearing loss. This study aimed to assess the compliance of patients and acceptance of hearing aids for the management of children with OME.

Thirty-nine children who had been given binaural hearing aids to manage OME were assessed at routine follow-up after six months. A clinician who did not prescribe the aid administered a questionnaire to assess compliance, change in symptoms and acceptance of the aids.

Thirty-eight parents thought the aids were easy to use and 25 (66 per cent) were completely satisfied with the management. Aided hearing improved by a mean of 17 dB (Range 10–30) over three frequencies, 0.5, 1.0, 2.0 Khz and all parents reported subjective hearing improvement in their children. The stigma of an aid was reported as minimal under the age of seven.

Hearing aids provide a non-invasive way of managing the problems associated with OME which is acceptable to certain parents and children. Long-term effects of using aids need to be evaluated before they can be recommended.

Tumours of the neuroendocrine system in the head and neck region are mostly paragangliomas of the glomus tympanicum or jugulare, or of the carotid body. The majority of these tumours are benign, and the coexistence of multiple paragangliomas seems to be rare. Pre-operative embolization and surgery are regarded as primary therapy for these tumours. The treatment regimen in any patient depends on age, general health, hearing status and the function of the lower cranial nerves. Several presentations are possible in which paragangliomas occur as systemic disease.

1. Paragangliomas may occur bilaterally, or, in rare cases, in multiple areas. Pre-operative bilateral angiography is of utmost importance. In case of multicentricity, it might be necessary to proceed without, or just with, unilateral surgery for preservation of adjacent structures. In surgery of jugular vein paraganglioma, we usually perform a modified transmastoidal and transcervical approach with preservation of middle-ear structures and the ossicles. As an alternative or supplement to surgery, radiotherapy or definitive embolization may be used in the treatment of paragangliomas.

2. Paragangliomas may occur as multiple endocrine neoplasia (MEN) syndrome combined with medullary thyroid gland carcinoma, and, facultatively, pheochromocytoma In these cases, endocrinological examination and magnetic resonance imaging (MRI) of the adrenal region, the thorax and the neck are required for an adequate therapeutic strategy. As MEN may be inherited, family history should be evaluated.

3. Paragangliomas can became malignant and metastasize. Thus, cervical lymph node metastases or distant metastases may occur. We recommend the removal of all ipsilateral lymph nodes and their histological examination.

We present the outcome of implantation in the first 100 adult patients treated under the Midland Cochlear Implant Programme. All patients were post-lingually deaf with profound or total hearing loss. Performance was tested in lip-reading, implant only and combined lip-reading and implant modes using BKB sentences, connected discourse tracking (CDT) and environmental sound recognition. Assessments were made at nine and 18 months post-implant.

The dominant aetiologies were idiopathic and meningitis. Meningitis was associated with the greatest numbers of ossified cochleas. Forty-three per cent of cases of partial ossification were identified only at surgery. Four per cent of patients became non-users of their devices, however the majority used their implants for more than 12 hours each day. The mean scores at nine months post-implant, in the implant only mode, were for environmental sound recognition 56.7 per cent, for BKB sentences 46.6 per cent (80 per cent of patients scored above 0 per cent) and for CDT 31.2 words per minute (w.p.m.) (62 per cent scored above zero per cent). In the combined lip reading and implant mode the mean scores, at nine months, were for BKB sentences 81.5 per cent and for CDT 65.8 w.p.m. All results were sustained at 18 months.

Patients reported that implantation significantly reduced their hearing handicap. Pre-operative measures of depression were also significantly reduced at nine months post-implant. Results were sustained at 18 months.

Post-operative audiological outcomes in the electrical stimulation only mode correlated significantly with length of profound deafness. Results suggest that performance outcome is also related to the number of active electrodes.

It has been reported that the galvanic body sway test does not correlate with the caloric test. We evaluated the galvanic body sway test in patients with vestibular schwannomas using three parameters: the angle of deviation response onset, the maximum value of the deviation response, and the area of deviation. These parameters reflect velocity, position, and locus of the centre of pressure respectively. Among these parameters, only the angle of deviation response onset showed unilateral weakness of the response correlating with the canal paresis value, which indicates that velocity is responsible for conduction in the vestibular nerve. However, the galvanic body sway test is apt to be preferred to the caloric test. This might be attributed to the decreased sensitivity of this test.

Much debate exists over the management of mucosal chronic suppurative otitis media in children, with the majority of it centred around the correct timing to perform either a myringoplasty (an operative repair of the tympanic membrane) or type I tympanoplasty (reconstruction of the tympanic membrane when there is an intact and mobile ossicular chain). Further discussion will use the term tympanoplasty to mean both of the above definitions. We present the findings of a recent survey of UK ENT consultants questioning their opinions on various management aspects of mucosal CSOM in the paediatric population. We also present an extensive review of the literature to provide us with published evidence in order to analyse the results of the questionnaire.

A new cochlear implant (CI–24M) has recently been released by Cochlear Ltd. The shape and size of the receiver-stimulator differs from that of the CI–22M. Infants as young as one year of age are now receiving cochlear implants. We have examined the likely effect of skull growth following the implantation of a CI-24M cochlear implant in an infant of this age.