Twenty patients who had undergone open cavity mastoidectomy for chronic middle ear disease were selected for study. Following initial microscopic aural toilet, each cavity was painted in its medial aspect with a marker solution of Bonney's Blue dye. Each patient was assessed at weekly intervals for a minimum of three months following application of dye, and a photographic record made of the cavity. Information was also sought regarding the clinical behaviour of the mastoid cavities, with particular regard to degree of otorrhoea, and accumulation of debris. Migration of epithelium inferred by visible movement of dye occurred in 17 of the 20 mastoid cavities. In all these cases migration occurred in a medial to lateral direction, towards the exterior. In two cases there was no visible migration throughout the period of observation, and inspection after six months revealed the dye to be still present in the original position. Both these cases were of dry trouble-free cavities in which accumulation of debris did not appear to be a problem. This finding calls into question the assumption that clean trouble-free cavities are maintained by a satisfactorily functioning epithelial migration. In the four cavities which suffered from intermittent otorrhoea, epithelial migration occurred at a similar rate to that in dry cavities.

Motorcyclists are known to be exposed to excessive wind noise levels when riding. The potential adverse effects of this exposure on their hearing was investigated. Temporary threshold shift (TTS) was assessed by asking 18 riders to undertake a standard test run of one hour at a steady 80 mph, and performing audiometry before and immediately afterwards. Permanent threshold shift (PTS) was assessed by performing pure-tone audiograms on a highly screened group of 246 motorcyclists and comparing their hearing thresholds with those of an appropriate control group obtained from the MRC National Study of Hearing.

Significant TTS was found at 0.25, 0.5,1 and 2 kHz. The greatest TTS occurred at 1 kHz, with a mean hearing loss of 10.3 dB. The hearing thresholds of the motorcyclists were significantly worse than the controls at 0.25, 0.5, 1 and 2 kHz, andwas most marked at 0.5 and 1 kHz where their hearing loss (PTS) was, respectively, 3.7 and 3.6 dB greater than expected.

These findings demonstrate evidence of both temporary and permanent hearing loss from motorcycling and present a strong argument for the need for some form of remedial action.

A follow-up of seven patients with the autosomal recessive inherited syndrome of distal renal tubular acidosis (RTA) and sensorineural hearing loss is described. Five patients were diagnosed as having primary distal renal tubular acidosis and rickets, four were found to have severe sensorineural hearing loss of over 80 dB: two of which are brothers. Two patients were diagnosed as having secondary distal renal acidosis due to a genetic disorder called osteopetrosis; they are brothers and their audiograms showed a mild conductive hearing loss of an average 35 dB bilaterally. All patients had growth retardation with improvement due to alkaline therapy but their hearing loss was not affected by the medication. The pedigrees of two families with half sibs showed the familial incidence for consanguineous marriage. Consanguinity was found to be positive in five out of the seven patients. The tribal tradition in Saudi Arabia fosters consanguineous marriages for cultural and social reasons and pre-arranged marriages are still seen.

In this study, we investigated the relative localization of some antigenic epitopes in the inner ear. The inner ear protein antigens were extracted from various parts of the guinea pig inner ear. Brain, kidney, lung, heart and liver extracts were also obtained. We found by SDS-polyacrylamide gel electrophoresis that total inner ear extracts separated into three high concentration polypeptide bands with molecular weights of approximately 30, 42, 58 kd and three low density bands of 20,25 and 35 kd. The 30 kd band was found mainly in the extract of the spiral ganglion and the acoustic nerve in the modiolus. The 42 and 58 kd bands were detected in the extract of the spiral ligament and the stria vascularis. The Organ of Corti and the basilar membrane extract gave rise to three bands of 30,42 and 58 kd. Twenty-eight of the 75 sera from patients with inner ear disease reacted with the 30 and 58 kd bands of the inner ear protein extracts by immunoblotting. Sixteen of these 28 positive sera were then used to probe immunoblots of the brain, kidney, lung, heart and liver extracts. The 58 kd band was also found in protein extracts of the brain, the lung and the liver. This study suggests that the 30 kd antigenic epitope may be mainly related to the acoustic nerve and that the 58 kd antigenic epitope is not cochlear specific.

The technique of long-term middle ear ventilation using a tympanotomy technique is presented. This has proved successful in the management of persistent middle ear effusion despite numerous previous short- and intermediate-term tympanic membrane ventilation tubes. The technique is particularly appropriate in the presence of severe tympanoscierosis as a result of previous tympanostomy tubes, posterior tympanic membrane collapse and adhesion, and atelectasis.

Sensorineural hearing loss in rheumatoid arthritis (RA) has been reported to be the result of the extraarticular manifestation of the disease (rheumatoid nodular vasculitis) or due to drug ototoxicity.

In an attempt to investigate the presence of sensorineural hearing loss and the possible causes for it we investigated prospectively 45 RA patients (42 female; three male) with a mean age of 52.5±10.7 years and a mean disease duration of 8.5±7.3 years. All patients underwent a complete physical examination and audiological evaluation which included pure tone audiometry and impedance audiometry (tympanogram, static compliance, acoustic reflex, reflex decay, acoustic reflex latency test.

We found a sensorineural hearing loss >20 dB HL in 44.4 per cent (40/90) ears. In all cases the site of hearing loss was the cochlea and in most of them it was bilateral and symmetric (16 patients out of 45 had bilateral sensorineural hearing loss i.e. 35.5 per cent.

There was no correlation between sensorineural hearing loss and age, sex, disease duration, articular and extra-articular manifestations and the presence of autoantibodies in our patients. In addition, no correlation was found between sensorineural hearing loss and drug therapy for one at least of the following drugs: NSAIDs, d-penicillamine, plaquenil and methotrexate.

We noticed a prologation of acoustic reflex latency in five patients (10 per cent) which was found to be correlated with the temporomandibular joint involvement and the presence of rheumatoid factor (RF).

We conclude that inner ear involvement in RA is expressed by: (1) mild symmetric, bilateral sensorineural hearing loss of cochlear type in 35.5 per cent of patients; (2) normal acoustic reflex thresholds; (3) nondecay; and (4) prologation of acoustic reflex latency which appeared in a small number of patients (10 per cent).

Mast cells are a rich source of potent biologically active mediators and are found in connective tissue, associated with blood vessels in many varied inflammatory conditions. Mast cells have been described in nasal polyps and turbinates and in adenoidal tissue in the upper aerodigestive tract. As the middle ear lining is contiguous with the nose and the nasopharynx, the presence of mast cells in aural polyps is interesting.

This preliminary study investigated the presence of mast cells in inflammatory aural polyps using light microscopy. All patients presenting to the department in one year were included. Patients with previous ear disease or surgery and in whom cholesteatoma was suspected were excluded. Except for one patient mast cells were seen in all aural polyps. The implications of these findings is discussed. Further work is needed using electron microscopy.

Vestibular schwannoma occurs both as a sporadic tumour and in the dominantly inherited familial cancer syndrome neurofibromatosis type 2 (NF2). The gene for NF2 has recently beenisolated on chromosome 22, and the demonstration of inactivating germline mutations in NF2 patients and NF2 associated tumours suggests that it acts as a tumour suppressor. The results of recent research in Cambridge suggest that somatic mutations of the NF2 tumour suppressor gene are a critical step in the pathogenesis of both familial and indeed non-familial unilateral sporadic vestibular schwannoma and that the mechanism of tumourigenesis complies with the ‘two-hit’ model. This paper represents a brief review of the current status of molecular biology in relation to vestibular schwannoma in particular and is discussed in relation to the molecular pathology of skull base tumours as a whole.

Forty-eight patients with syphilitic labyrinthitis have been treated and followed up for periods of six months to 25 years; interpretation of the hearing results must be in the light of the natural history of this condition which is perceived to be progression of hearing loss to profound deafness.

At the beginning of the series treatment was with ampicillin alone and on this treatment 11 ears with useful hearing were maintained at this level. The group with combined ampicillin and prednisone treatment, which included some failures from ampicillin alone, maintained useful hearing in 28 out of 29 ears. Those treated initially with ampicillin and prednisone and later also given regular injections of ACTH, maintained hearing in 16 out of 17 ears.

At the outset, there were 29 profoundly deaf ears. One of these recovered some useful hearing but two ears with useful hearing at the outset became profoundly deaf so that at the end there were 30 profoundly deaf ears. However, all patients as opposed to ears, with useful hearing at the outset were left with some hearing; no patient became profoundly deaf.

Total surgical decompression of the facial nerve can be offered to patients severely affected by Bell's palsy whether via the transattical or middle fossa approach. We prefer, when feasible, the transattical approach because it does not violate the cranial vault. The purpose of this study was to find the anatomical parameter of the temporal bone, measured by means of computed tomography (CT scan) and to decide which of these approaches should be offered. Sixty temporal bones were studied by CT scan, and then dissected in order to perform total facial nerve decompression via the transattical approach. Correlation between the two studies was established. Results suggest that measurement by CT scan of the attical area in the axial plane (AAA) may determine those patients for whom the transattical approach to facial nerve decompression should be undertaken.

The term sialectasis refers to dilatation of the salivary ducts. Parotid sialectasis presents itself as recurrent painful swellings during mastication and/or swallowing. Our results with 10 cases treated by tympanic neurectomy over a period of nine years, from 1983 to 1992, were encouraging as seven cases had improvement of their symptoms.

Anatomy of the tympanic plexus and surgical procedure are discussed as well as a review of the literature.

We recommend extensive interruption of the secreto-motor nerve fibres by thoroughly drilling into the hypotympanum and below the basal turn of the cochlea. We also recommend that this be carried out by an experienced otologist to avoid possible complications.

Facial pain is a common symptom. Patients with this symptom without any overt disease of nose and paranasal sinuses are often diagnosed as having chronic rhinosinusitis. Thirty-six cases were seen and treated surgically by middle turbinectomy or septal surgery in this series. The xylocaine (10 per cent) test seemed to be a good prognostic aid for the surgical procedures. Medical treatment did not seem to relieve the symptoms. Larger series with control trials are recommended in order to establish this practice.

The mucociliary escalator is the first line of defence of the upper and lower respiratory tracts (Greenstone and Cole, 1985; Sleigh et al., 1988). Failure of mucociliary clearance is associated with chronic or recurrent respiratory tract infection. Ten patients with chronic rhinosinusitis underwent nasal brushings for the assessment of ciliary beat frequency. In two no beating cilia were seen; in the remainder the mean value was 9.3 Hz ± 2.3; range 6.1–12.8 Hz (n = 8).

Following three months continuous oral antibiotic therapy repeat nasal brushings demonstrated increased ciliary beat frequencies in all patients, mean value 13.7 Hz ± 1.6; range 11.5–16.3 Hz (n = 10); (p<0.001 paired t-test).

Depression of mucociliary clearance can occur secondarily to chronic infection and is improved by prolonged antibiotics.

The records of 94 consecutive developmentally retarded children with speech retardation and suspected hearing loss who underwent auditory assessment by both conventional behavioural observation audiometry (BOA) and brain stem evoked response audiometry (BERA) were analysed. In 54 children (57.4 percent) there was good agreement between the results of both techniques leading to a clearcut diagnosis. In 22 children a diagnosis was possible only by the results of BERA as the results of BOA were inconclusive. Of the remaining 18 children, two groups could be identified whose results posed a dilemma. Group 1 (n = 7) consisted of children whose BOA test results differed considerably from their BERA results. Group 2 (n = 11) consisted of children in whom there was no discernible response by BERA while the response by BOA was either inconsistent (n = 5) or not elicitable (n = 6). The specific strategies to be adopted for hearing assessment in these situations are discussed.

Type 2 neurofibnormatosis is a dominantly inherited disorder in which the great majority of sufferes develop bilateral vestibular schwannomas. In a UK study of 183 individuals from 112 families we have previously shown a fairly similare disease course within families, but quite marked inter-familial variation. We have confirmed an increase in severity when the gene is inherited from an affected mother, but evidence that women are more severely affected than men is lacking. Age at onset of symptoms, of deafness and at diagnosis are idential for the entire dataset and for a comparison of 10 male/female sibling paries. Only three out of 42 pregnancies in symptomatic women were accompanied by reversible worsening in symptoms due to vistibular schwannomas. Of 328 consecutive cases of unilateral vesitibular schwannoma, there was no significant difference in the sex ratio or size. There now appears to be little evidence for a female hormonal effect on vestibular schwannomas. However, females with type 2 neurofibromatosis have significantly more meningfiomas.

Central electrical stimulation of the auditory pathway can allow hearing in patients sufferingfrom deafness localized in the auditory nerve. Developments in a multi-channel auditory brainstem implant based on the Nucleus Mini 22 Cochlear implant with transcutaneous signal transmission is discussed. The devices have been implanted in nine European patients suffering from Neurofibromatosis Type 2. Preliminary speech perception results and patient satisfaction are encouraging, and the data presented include some limited open speech recognition.

A prospective study on the dynamics of tympanic membrane atelectasis during the treatment for glue ear was performed in a sample of 115 ears of 83 children aged between one and 11 years. The progression in the degree of pars tensa atelectasis was analysed in relation to six potentially relevant factors. Multivariate analysis showed that the factor with the most predictive value on the progression of the pars tensa retraction was the grade of atelectasis at initial detection (p<0.0001). The use of grommets did not have any significant influence on the outcome grade of atelectasis. There was an association between previous grommet insertion and localized retractions in the inferior segment of the pars tensa (P<0.0001). However, localized retractions in the postero-superior quadrant were not associated with previous grommet insertion (P<0.02). Although the hearing thresholds of atelectatic ears were significantly worse than normal ears especially at 4 kHz (p<0.006), the difference was less than 5 dB.

High-resolution, tympano-cochlear scintigraphy (TCS) is a useful tool for visualizing changes in labyrinthine bone metabolism in active otosclerosis in vivo. But until now, the activity patterns have mostly been rather imprecisely ascribed to the labyrinthine structures; more exactly by means of high- resolution CT (HR-CT). Experimental studies on TCS using a human temporal bone model revealed that correlative imaging of X-ray photographs and the scintigrams or superimposition with masks of the temporal bone drawn from the X-rays can facilitate the localization of small foci of about 0.5–1 mm.

Clinical applications of the visualization technique, combining functional with structural images, confirmed the benefit of this method, improving the accuracy in detection and localization of focal activity enrichment of the petrous bone in cases of active otosclerosis by means of TCS.

The use of tissue autofluorescence for the detection and localization of cancer of the larynx is described. In this pilot study, eight patients with probable carcinoma of the vocal folds underwent laryngoscopy in which the tissue autofluorescence spectra of normal and pathologically confirmed tumour tissue were acquired in vivo. Fluorescence images of the suspect areas were also acquired using the LIFE system (Xillix Technologies Corp.). The results suggest that the autofluorescence properties of laryngeal tissue, under 442 nm illumination, are similar to those of bronchial tissue and that the LIFE system has the potential to increase the accuracy of staging of cancer of the larynx and also to allow earlier diagnosis of tumours and theirrecurrence

The air conduction thresholds in the right and left ears, and the interaural asymmetry of thresholds at 0.5, 1, 2, 3, 4 and 6 kHz were measured in a group of 225 soldiers exposed to a variety of weapon noise who were referred for assessment because of a deterioration in hearing on routine testing. At 0.5 and I kHz the threshold levels rarely exceeded 25 dB and the interaural asymmetry was 10 dB or less in 90 per cent of cases. The degree of hearing loss and interaural asymmetry increased as the frequency increased, with the average loss being significantly greater in the left ear at 2, 3, 4 and 6 kHz.

Recommendations are made for the selection of cases of asymmetrical hearing loss exposed to weapon noise which require further investigation to exclude a retrocochlear cause or to define spurious hearing threshold levels.