Sensorineural hearing loss is a common symptom in patients with myoclonic epilepsy associated with ragged-red fibres (MERRF), one of the mitochondrial encephalomyopathies, although the lesion causing hearing loss in such cases remains unknown. Here we describe the audiological features in three MERRF patients, all of whom exhibited a point mutation in their mitochondrial DNA at nucleotide 8344. Pure-tone threshold audiometry revealed bilateral, sloping-type, sensorineural hearing loss in all three patients. Distortion product otoacoustic emissions, electrocochleography, and auditory brainstem responses were variable, even differing between the right and left ears of the same patient. Taken together, our findings suggest that the primary lesion underlying hearing loss in MERRF patients is in the cochlea, although a retrocochlear lesion may be involved in some patients.

Tympanometry was analysed according to cleft type with respect to age in 239 cleft palate patients (57 with bilateral cleft lip and palate (BCLP)), 122 with unilateral cleft lip and palate (UCLP) and 60 with isolated cleft palate (ICP)). The frequency of type B tympanograms was 46.5 per cent in the BCLP group, 50.6 per cent in the UCLP group and 58.3 per cent in the ICP group. Type A tympanograms were more frequently observed in older patients (median age 11 years) in comparison to type B (median age five years; p<0.001) and type C (median age six years; p<0.001). The total sample showed an age-related decrease in the frequency of type B tympanograms (rs = −0.3942; p<0.001). The frequency of type A tympanograms increased significantly with age (rs = 0.4263; p<0.001), whereas type C was not correlated with age. In the UCLP group, the frequency of type B tympanograms decreased with age (rs = −0.4430; p<0.001), the decrease being faster than in the BCLP group (rs = −0.3186; p = 0.001) and the ICP group (rs = −0.3378; p<0.0001)). Type B tympanograms had the highest correlation with a hearing loss of 21–40 dB at mean hearing level at speech frequencies (MHLSF) (rs = 0.4574; p<0.001), a lower correlation with a hearing loss of 11–20 dB (rs = 0.2184; p = 0.02) and the lowest correlation with hearing loss above 40 dB. At the ages of one to three, the frequency of type B is higher in UCLP patients than in the BCLP and ICP groups, decreasing at seven to 12, increasing again at 13 to 15, and thereafter showing a continuous decrease. In the BCLP group, the frequency of type B increased significantly at the ages of four to six and then decreased continuously from seven to 18. In ICP patients, the changes in the frequency of type B with age are not significant until the age of 15; at the age of seven, it is higher than in the BCLP and ICP groups. The type C tympanogram is not typically found in cleft palate patients and its frequency is not correlated with age.

It can be presumed that each type of cleft lip and palate, due to its characteristic conditions in the epipharynx, will favour a different mode of pathophysiological development of middle-ear disease.

We present the results of the insertion of self-made polyethylene T-tubes for a period of 15–24 months for the treatment of chronic middle-ear effusion. We compare the outcome of our patients to the reported outcome of patients treated with other commonly used ventilation tubes for either shorter or longer periods of time. In a retrospective review of 603 T-tubes inserted in 306 children up to the age of 12 years, charts were reviewed for age, sex, surgical procedure performed, duration of ventilation and complications. In all cases the indication for surgery was chronic middle-ear effusion. The tubes were electively removed by the authors after 15–24 months of ventilation. Spontaneous extrusion was considered a complication. The mean period of ventilation was 20 months. Post-operative otorrhoea was experienced in 6.6 per cent of ears; 4.8 per cent of tubes extruded spontaneously, whereas 3.15 per cent had to be removed earlier than originally planned; 4.9 per cent of ears were re-ventilated at a later date, and 1.49 per cent of ears developed a persistent perforation. We demonstrate that the outcome of patients treated with our self-manufactured tubes for a period of 15–24 months is, in many respects, better or at least comparable to the reported outcome of patients treated with other commonly used ventilation tubes for either shorter or longer periods of time, and that the many complications associated with the conventional T-tube can be reduced. We suggest that our favourable outcome may be due to the duration of ventilation, which was controlled to be shorter than the conventional long-term T-tubes and longer than that of grommets.

The authors report a prospective study in which the aim was to analyse the usefulness of different criteria in optimizing the diagnosis of allergic fungal rhinosinusitis. From 1995 to 1998, 165 patients were operated on for chronic rhinosinusitis. Investigations used in this study for the diagnosis of allergic Aspergillus rhinosinusitis consisted of an analysis of clinical, radiological, immuno-allergic criteria. Fourteen patients presented with allergic Aspergillus rhinosinusitis. One hundred and fifty-one patients did not present any of the necessary criteria for the diagnosis of allergic Aspergillus rhinosinusitis. The results show that the characteristic macroscopic appearance, the maxillary sinus localization, and the presence of positive specific IgE to Aspergillus fumigatus are arguments that reinforce the diagnostic certitude of allergic fungal sinusitis. No specific clinical or radiological criteria orients a diagnosis of chronic rhinosinusitis toward that of allergic fungal rhinosinusitis. The other immuno-allergic tests do not contribute to the diagnosis of allergic fungal rhinosinusitis. pathological, mycological, and

Twenty-two cases of perforated tympanic membrane due to fungal otitis externa were observed over a five-year period.

The diagnosis of fungal otitis externa was made on clinical grounds due to the obvious presence of fungal bloom in the external ear canal. Some perforations were noted at the first treatment after the fungal debris had been removed from the external ear canal using a microscope. Other perforations were observed to develop over a few days. Initially, a discrete area of the tympanic membrane appeared white and opaque. As time progressed the white area disintegrated, forming a perforation. Once the otitis externa had resolved most perforations healed spontaneously. Two that were observed to develop during treatment required a myringoplasty. Another one closed significantly but a tiny persistent perforation required cauterization with trichloracetic acid to encourage it to close over completely. The only residual hearing loss was in a case with almost total disintegration of the tympanic membrane requiring a myringoplasty.

Treatment of fungal otitis externa for the patients in this series was aural toilet using suction under a microscope and insertion of a gauze wick saturated in a combination of hydrocortisone, clotrimazole, framycetin and gramicidin.

Five patients of various ages with difficulty in speech discrimination were evaluated. All showed evidence of abnormal auditory brainstem responses (ABRs) beginning with the VIIIth cranial nerve. Broad summating potentials were evoked on their electrocochleograms (EcochGs) and they all exhibited almost normal cochlear outer hair cell function by otoacoustic emissions (OAEs) recordings. Behavioural audiometric testing revealed a mild to moderate elevation of pure-tone threshold in all patients. The shape of their pure-tone losses varied, being predominantly low-frequency in four patients (rising slope pattern) and flat across all frequencies in one patient. Speech intelligibility scores of all patients were poor and out of proportion to what would have been expected if threshold elevation of pure-tone was of cochlear origin (i.e. markedly poor scores on the speech audiogram with good scores on the auditory comprehension test). Patients were neurologically normal when the hearing impairment was first manifested. We suggest that this type of hearing impairment is due to an isolated and sporadic disorder of auditory nerve function. It occurs in isolation and does not seem to be part of a generalized neuropathological process.

High resolution T2-weighted magnetic resonance (MR) imaging has been proposed as a rapid, inexpensive means of investigating patients with sensorineural deafness, particularly to exclude vestibular schwannomas. Whether the accepted ‘gold standard’ of contrast-enhanced T1-weighted images can be omitted, however, remains controversial. Over a 22-month period the use of axial turbo-spin echo T2-weighted images (T2W) were prospectively compared with contrast-enhanced T1-weighted spin echo scans in the evaluation of 513 patients presenting with audiovestibular symptoms. A 2-D T2W turbo spin echo (TSE) sequence with 3 mm slices was used in 340 patients while a 3-D sequence with overlapping 1 mm slices was used in 173 patients. The T2-weighted image findings were documented and subsequently compared with contrast-enhanced images. With the 2-D sequence 24 patients (25 lesions) had internal auditory meatus (IAM)/cerebello-pontine angle (CPA) masses identified by contrast-enhanced T1-weighted images, all of which were seen on the T2-weighted TSE sequence; there was one false positive ‘mass’ on the T2-weighted scans and one false negative case of IAM dural enhancement on T1-weighted imaging; six were considered normal initially on the T2-weighted images although three were subtly abnormal in retrospect. With the 3-D sequence three acoustic neuromas were all identified correctly with no false positive and only one false negative result (labyrinthitis). The 2-D and 3-D images were judged technically inadequate for clinical assessment in 15 and nine per cent respectively. We conclude that mass lesions of the IAM/CPA can be reliably identified on T2W TSE imaging but labyrinthine lesions may be missed without contrast enhancement. This is of particular importance in planning the management of neurofibromatosis type 2. Non-neoplastic disorders of the inner ear are also likely to be missed.

Canalplasty is the surgical procedure whereby the external auditory meatus is widened. The indications include exostoses, stenosing external otitis and widening for surgical access. One hundred consecutive ears operated on by one surgeon are reported. The surgical technique is described in detail, paying particular attention to bone removal from the anterior canal wall. In this paper the majority of cases were occasioned by soft tissue rather than bony stenosis. The re-stenosis rate was four per cent and in each case this was associated with the use of a middle temporal artery flap. Partial, transient, delayed facial palsy occurred in two per cent, probably relating to thermal injury transmitted from the burr. A full, spontaneous recovery of facial function occurred in each case. This is a safe and effective technique for canal widening.

Oto-endoscopes allow surgeons to control blind pockets within the middle ear. Many surgeons credit the reduction of residual cholesteatoma in their practice to the use of oto-endoscopes. Since 1988, the author has been using rigid endoscopes in combination with the operating microscope in cholesteatoma surgery. Between 1988 and 1999, 231 primary cholesteatoma operations were performed. The operations included closed cavity mastoidectomy (53 patients), small cavity mastoidectomy (115 patients), open mastoidectomy with primary canal wall reconstruction (44 patients) or mastoid obliteration (19 patients). The median follow-up period was 6.5 years. The incidence of residual cholesteatoma is closed cavity mastoidectomy (9.4 per cent) was found to be similar to that of open cavity mastoidectomy (8.7 per cent). Site analysis of the residual cholesteatoma revealed an incidence of 3.6 per cent in the epitympanum, 10.5 per cent in the sinus tympani and 0.7 per cent in the mastoid bowl. This study showed that residual cholesteatoma has not been eliminated with the use of middle-ear endoscopy, although the incidence has reduced the figures for closed cavity mastoidectomy to single figures.

A prospective controlled clinical study was carried out at the Department of Ophthalmology and ENT, Inönü University Medical Faculty, Turgut Ozal Medical Center, Research Hospital, to evaluate the audio-vestibular involvement in patients with Behçet's syndrome compared with controls.

Twenty-five consecutive patients with Behçet's syndrome (mean age ± SD, 34.96 ± 8.50) and 20 age- and sex-matched healthy volunteers (hospital staff) as control subjects (mean age ± SD, 34.45 ± 9.16) were included in this study. Behçet's patients were divided into two groups according to the number of criteria, complete (all four major criteria) and incomplete (three major criteria without ocular involvement). The groups were compared with each other or controls regarding inner ear involvement. Audiometric pure-tone thresholds at 125 to 8000 Hz were obtained in all subjects in both groups, and pure tone average (PTA) hearing thresholds were calculated for the middle, high and low frequencies. In addition, short increment sensitivity index (SISI), tone decay and BERA examinations were performed in all Behçet's patients.

Sensorineural hearing loss (SNHL) was present in six of 25 patients with Behçet's syndrome. Two Behçet's patients had unilateral total SNHL, two had bilateral moderate level SNHL, one had bilateral low-frequency SNHL and one bilateral high frequency SNHL. In two, BERA, and in five SISI, examination disclosed inner ear involvement. In control subjects, the past medical history was normal and there was no consistent audio-vestibular complaint. Their PTA thresholds were all in the normal range. Otoscopic examination findings were normal, with intact, mobile tympanic membranes in both groups.

The present study showed that audio-vestibular involvement is not infrequent in Behçet's syndrome compared with age- and sex-matched healthy controls, and it is under-estimated. All Behçet's patients should regularly be followed by an otolaryngologists and be given information about the possibility of inner ear involvement. According to our results, hearing loss occurs more often in older patients and also in the complete form of Behçet's syndrome.

A postal survey was carried out to determine the current clinical practice amongst consultant otolaryngologists in the UK, regarding re-insertion of ventilation tubes or recommendation of hearing aids in cases of recurrence of otitis media with effusion (OME) after ventilation tube extrusion.

Amongst the 319 respondents, 15 (4.70 per cent) routinely, 146 (45.77 per cent) sometimes, and 158 (49.53 per cent) either never, or very rarely, recommend hearing aids. Hearing aids and ventilation tubes were both suggested to be equally good options by some consultants but they preferred surgery for a number of reasons.

There were inconsistencies in practice and some of the reasons for re-inserting ventilation tubes are not evidence-based. A hearing aid is a non-invasive option and this survey shows a need for a randomized control trial of hearing aids and ventilation tubes in the management of persistent and recurrent OME.

An ossicular prosthesis produced by making a mould from a cadaveric incus and injecting ionomeric cement into it (incus replica prosthesis, IRP) has been cemented to the malleus head and stapes head and evaluated in fresh human temporal bones using a laser vibrometer. Stapes velocity was recorded under acoustic stimulation and foot plate displacements were derived. Fourteen frequencies between 125 Hz and 8000 Hz have been used. Measurements were made with the ossicular chain intact, following removal of the incus, with the prosthesis cemented to the malleus, but not the stapes, and with the incudo-stapedial joint cemented. In a second series of experiments the performance of the IRP was compared with that of a Causse partial ossicular replacement prosthesis, using the same experimental set-up. The results indicate that the prosthesis performs almost as well as the intact ossicular chain and that cementing the incudostapedial joint gives better sound transmission than leaving it uncemented. In addition the IRP outperforms the conventional partial ossicular replacement prosthesis.

In the present work we describe the management of hypertrophied inferior turbinates using CO2 laser in the UniPulse mode. The laser beam is delivered through the nasal probe delivery system, and the procedure is performed using the 0° endoscope. The UniPulse mode allowed fine-tuned, char-free tissue ablation, and the nasal probes allowed delivery of laser energy to the posterior parts of the inferior turbinates.

The procedure was performed on 149 patients. The one-year post-operative follow-up period revealed good functional results in 131 patients (88 per cent). The minimal nasal cross sectional area increased significantly from 0.52 cm2 to 0.81 cm2. The average decongestive effect, on the other hand, decreased from 0.26 cm2 to 0.07 cm2 indicating significant laser-induced fibrosis. The operation can be performed as an out-patient procedure with little or no morbidity.

The surgical closure of a nasal septal perforation is recognized as being particularly challenging. A series of 11 consecutive patients who underwent closure of a septal perforation using a mucosal flap/composite conchal cartilage graft technique are reviewed, and the surgical technique described. The size of the perforation repaired varied, with eight cases being 2 cm or more in diameter. There was no significant graft donor site morbidity and complete perforation closure was achieved in eight cases after a mean observation time of 19.8 months. These results suggest that this is a suitable technique for closing nasal septal perforation.

Telomerase maintains the length of telomeres in immortal cells and is also often associated with cell proliferation. Cholesteatoma epithelium is characterized by a dysregulation with hyperproliferative growth. The study evaluated the telomerase activity in cholesteatoma and normal retro-auricular skin to discover the relationship between telomerase expression and clinical findings. Twenty-two samples of cholesteatoma and 15 samples of retro-auricular skin were obtained from patients undergoing middle-ear surgery. The telomerase activity was detected by the telomerase repeat amplification protocol (TRAP) assay method. Seventeen of the 22 (77.3 per cent) cholesteatoma cases expressed telomerase activity, whereas none of the 15 retro-auricular normal skin (0 per cent) detected telomerase activity. There was no significant difference between telomerase expressions and clinical findings, including hearing level, duration of disease, and the degree of extension (p>0.05). The high expression of telomerase in cholesteatoma suggests that the activation of telomerase may be related to the proliferative nature of cholesteatoma.

Two hundred and seventy-one Japanese patients who underwent surgical removal of neuroma from the cerebello-pontine angle using the translabyrinthine approach were retrospectively reviewed in order to investigate the nerve origin on an anatomical basis. In 269 out of the 271 cases, the origin of the neuromas was identified. Among these cases, 84.8 per cent of tumours originated from the inferior vestibular nerve (IVN), followed by the superior vestibular nerve (SVN) (8.9 per cent). Of the 5.6 per cent of tumours arising from the vestibular nerve, however, it could not be determined whether they originated from the SVN or IVN. Two cases of neuromas originating from the facial nerve were seen (0.7 per cent). No neuroma arose from the cochlear nerve.

Currently, the diagnosis of acoustic neuromas is best made with Gd-enhanced magnetic resonance imaging (MRI). However, our data indicate that the development of a functional test of the individual IVN can be useful for screening most cases of acoustic neuroma and in facilitating their early diagnosis.

This paper attempts to define and categorize the vertigo associated with migraine. A retrospective chart review of 344 cases of vertigo identified 19 cases with headaches characteristic of migraine as per strictly defined criteria (International Headache Society, 1988).Four distinct types of vertiginous syndromes were noted. The commonest syndrome (Group I) manifested transient episodes of imbalance with additional momentary subjective rotary vertigo worsened by movement. The attacks lasted a few hours and evaluation in the inter-episode interval demonstrated no vestibular deficit. Group II manifested transient objective rotatory vertigo of from 10 minutes to a few hours but no demonstrable permanent vestibular deficit. Group III displayed symptoms and signs characteristic of benign paroxysmal positional vertigo (BPPV) and Group IV manifested a permanent unilateral labyrinthine weakness. Causation of vertigo by migraine was implied in 10 of 19 cases where the headache and vertigo occurred simultaneously and in two other cases where the vertigo improved with anti-migraine prophylactic treatment.

Four distinct and characteristic vertigo syndromes have been noted with migraine. Their spectrum ranges from a transient reversible dysfunction to a more permanent destruction, and includes involvement of both the peripheral and the central vestibular systems.

Patients with advanced and recurrent laryngeal and hypopharyngeal cancer often need combined therapy, which is associated with greater morbidity than single modality treatment. The aim of this study was to assess the quality of life in laryngectomees and to assess whether differences exist between the irradiated and the non-irradiated patients. The University of Michigan Head and Neck Quality of Life questionnaire, a validated instrument for assessing the head and neck cancer related functional status and well-being, was used for this purpose. Subjects for the study included patients who had undergone total laryngectomy for laryngeal or hypophyarngeal squamous cancer. No significant difference between the groups was evident in the various domain scores, although a trend towards higher scores was seen in the combined therapy group. This study suggests that long-term side effects induced by radiotherapy do not adversely affect the quality of life in laryngectomised patients.

Cosmetic outcome of the columellar incision closure in external rhinoplasty patients has been a subject of discussion. This study was conducted to assess whether tissue adhesives provide an alternative option for sutureless closure of columellar skin incisions for cases utilizing open technique rhinoplastic surgery. One hundred and one patients undergoing external rhinoplasty were randomized to either topical application of butylcyanoacrylate or polypropylene sutures for columellar skin closure. The majority of tension on the wound edges was taken up using 5-0 chromic catgut. Cosmetic outcomes were evaluated by two otolaryngologists independently using visual analogue and Hollander wound evaluation scales in a blinded manner. There was no statistically significant difference in cosmesis between the surgeons’ evaluation scores for either type or repair of the columellar incision. Since the tissue adhesive forms its own protective barrier, post-operative care is simplified. Closure with adhesives eliminates the need for post-operative suture removal requiring an extra visit that should lead to more efficient use of physician and patient time. Butylcyanoacrylate performs cosmetically as well as standard suture closure of columellar skin incision used for external rhinoplasty.

Otolaryngologists and general practitioners commonly prescribed intranasal corticosteroid drops for rhinitis. Compliance in real patients has not previously been studied, but is generally believed to be poor. Recent concerns over systemic adverse effects of topical corticosteroids have highlighted the risks of overdosing. Fifty patients, who were prescribed betamethasone, were prospectively studied for accuracy of compliance using a weighed dose study. Patients consistently administered inaccurate quantities of nasal corticosteroid drops, with a marked tendency to overdose up to four times the recommended daily dose (RDD) in some cases. The mean dose administered was 200 per cent of the RDD. Of the 50 patients, only seven (14 per cent) administered the correct dose. The introduction of metered-dose delivery systems should be considered to reduce the risk of inadvertent overdosing.