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.

Our aim was to examine the presentation, complications and hospital course in this patient population, and to ascertain whether these patients had long term otological problems.

We retrospectively reviewed the medical records of all patients presenting with acute mastoiditis between January 1990 and December 2005. Patients' parents were contacted by telephone and questioned about further otological problems.

Twenty-nine patients were included in the study, and 27 of these patients' parents were contactable to complete the telephone questionnaire. Sixty-nine per cent of children had no previous history of acute otitis media prior to presentation. Forty-five per cent of patients had received oral antibiotics prior to presentation. Sixty-two per cent of patients developed complications, i.e. a subperiosteal abscess or failure to respond to medical therapy, resulting in the need for surgical intervention (in the form of incision and drainage of periosteal abscess, cortical mastoidectomy, or grommet insertion). Mean follow up of patients was eight years and one month; five (17 per cent) patients had been followed up for less than one year. Two (7 per cent) patients developed a further episode of mastoiditis within six weeks of initial presentation, both of whom required cortical mastoidectomy. Three (10 per cent) patients had further problems with recurrent acute otitis media, requiring tympanostomy tube insertion. One patient required a modified radical mastoidectomy for cholesteatoma (15 years later). Twenty-two patients (91 per cent) had been followed up for longer than one year; these patients had subjectively normal hearing and were asymptomatic at the time of study.

The majority of patients who had suffered an episode of acute mastoiditis had no adverse long term otological sequelae.

To evaluate the effects of antituberculosis treatment on the voice quality of laryngeal tuberculosis patients, measured by patient self-assessment, perceptual analysis and acoustic analysis.

A total of 14 laryngeal tuberculosis patients were enrolled. Laryngeal tuberculosis was established either by biopsy and histopathological examination or by rapid regression of the laryngeal lesions after antituberculosis medication. Before and after treatment, all patients were evaluated perceptually (on a scale of zero to three), and 12 assessed their own voices using the voice handicap index-10 scale. Acoustic analysis was performed to allow objective evaluation.

Patients' ages ranged from 21 to 72 years (mean, 41). The male to female ratio was 12:2. Eight patients (57 per cent) had tuberculous involvement of the epiglottis, four (28 per cent) had involvement of the aryepiglottic fold and eight (57 per cent) had involvement of the false vocal folds. The glottis was the less commonly involved part of the larynx, including true vocal folds (28 per cent, n = 4) and posterior commissure (14 per cent, n = 2). Perceptual evaluation, on a scale of zero to three, gave the patients a median score of six; after commencement of treatment, the median score decreased to two. The mean voice handicap index-10 score decreased from 24 to 12 after treatment. An obvious improvement in acoustic analytical parameters was also found following treatment.

Antituberculosis treatment clearly improved the voice outcomes of laryngeal tuberculosis patients, according to self-assessment, perceptual analysis and acoustic analysis.

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 identify regional surgical referral patterns for adenotonsillectomy in children with obstructive sleep apnoea to our tertiary centre with paediatric intensive care unit facilities and to establish guidelines for elective paediatric intensive care unit referral and admission.

Two methods were used. A questionnaire was sent to ENT consultants in five surrounding hospitals with no in-house paediatric intensive care facilities. The second was a prospective observational study undertaken in our tertiary centre for a sub-set of patients undergoing obstructive sleep apnoea adenotonsillectomy between January 2002 and February 2005. These children were considered high risk as judged clinically by an ENT surgeon. Most had obstructive sleep apnoea and a co-morbidity. Otherwise healthy children with simple obstructive sleep apnoea were excluded.

15 out of 20 consultants responded to the questionnaire. Four referred on the grounds of clinical history, five referred based on pulse oximetry, nine referred syndromal children and four did not refer electively. Of the 49 high risk patients operated on, only 12 required paediatric intensive care admission with no emergency paediatric intensive care admissions. No otherwise healthy children with uncomplicated obstructive sleep apnoea symptoms required paediatric intensive care admission during the study period.

There was no regional consensus regarding paediatric intensive care unit referral for obstructive sleep apnoea adenotonsillectomy. Clinical judgement without complex sleep studies by those experienced in this area was sufficient to detect complicated cases of obstructive sleep apnoea with co-morbidity requiring paediatric intensive care.

Paediatric neck abscesses remain common problems which are sometimes difficult to manage.

We conducted a retrospective study of 64 children who underwent incision and drainage of neck abscesses at Leeds General Infirmary from 1 February 2002 to 31 July 2006. The aim of this study was to identify the presenting symptoms in children, the appropriateness of prescribed antibiotics and the role of atypical mycobacteria in neck infections. The outcome measure was clinical resolution of the abscess.

The mean presenting age was 44.2 months (3.68 years). The commonest sign and symptom was neck mass (96.9 per cent). The mean period of hospitalisation was 3.7 (± standard deviation of 1.9) days. Staphylococcus aureus (48.4 per cent) was the commonest organism cultured. Atypical mycobacteria were found in only 4.7 per cent of the specimens. Flucloxacillin was the most common antibiotic used (57.8 per cent), often in combination with other antimicrobials. The abscess recurrence rate was 4.7 per cent. No fatalities occurred in this series of patients.

Appropriately prescribed intravenous antibiotics and surgical drainage remain the central core of treatment. Atypical mycobacterial infection is an important differential diagnosis of a painless, cervico-facial mass. An algorithm for the management of paediatric neck abscesses is proposed.

The objective was to assess the presence of Ki-67, and oestrogen and progesterone hormone receptors as well as their clinical correlates in acoustic neuroma.

Medical records of 59 patients who were operated on for acoustic neuroma between 1995 and 2003 were evaluated retrospectively. Formaldehyde-fixed paraffin-embedded archival acoustic neuroma specimens of the patients were used for immunohistochemical assessments of oestrogen and progesterone hormone receptors, and Ki-67 proliferative marker.

Tumour sizes were small (<19 mm), medium (20–39 mm) and large (>40 mm) in 21, 35 and 3 patients, respectively. On immunohistochemistry, all samples were (+) for progesterone receptor and (–) for oestrogen receptor staining. Ki-67 staining was encountered in 34 of 59 (57.6 per cent) patients, and Ki-67 values ranged from 0 per cent to 10.9 per cent (mean 1.36 per cent). There was no correlation between Ki-67, gender, tumour size and symptoms of the patients (p > 0.05).

Oestrogen is not an important hormone in acoustic neuroma due to the absence of oestrogen receptor expression in the tissue samples. Since the progesterone receptor is expressed in all acoustic neuroma samples, further studies are necessary to find out about the inhibitory effect of antiprogesterone treatment on acoustic neuroma growth, which may be important particularly in elderly people or high-risk patients. Although Ki-67 is expressed in the majority of acoustic neuromas, it is not an important marker in clinical practice due to a lack of any correlation with the clinical parameters.

Isshiki type one medialisation thyroplasty is an accepted treatment for a unilateral immobile vocal fold. It can also be performed simultaneously as a bilateral procedure in patients with severe bowing of the vocal folds (e.g. presbyphonia). The objectives of this study were to assess the incidence and timing of post-operative complications, and to evaluate whether patients undergoing this operation could, in future, be treated as day cases.

A retrospective analysis was undertaken of 57 consecutive patients who had undergone a type one thyroplasty (52 unilateral and five bilateral) at a tertiary referral centre between April 2003 and April 2006. Post-operative improvement in the voice (measured subjectively, perceptually and quantitatively) was considered to constitute a successful outcome. Any complications were documented.

Fifty-seven patients who had undergone laryngeal framework surgery were recruited from the study database. All of these patients had undergone either unilateral or bilateral type one medialisation thyroplasty but no arytenoid surgery. Thirty-seven were male (65 per cent) and 20 female (35 per cent), and there was left-sided predominance (74 per cent). All patients were discharged the morning following afternoon surgery (i.e. within 24 hours). Complications occurred in four patients (7 per cent). One patient, who was taking warfarin, developed a post-operative haematoma which resolved with conservative treatment. Two patients (both of whom had undergone revision thyroplasty) developed a wound infection three days post-operatively, which resolved with antibiotics. One patient returned with hoarseness five months post-operatively, after an initially successful result. This patient had previously received radiotherapy for early glottic carcinoma, and the Silastic® implant was eroding through the mucosa. This was subsequently removed under general anaesthesia. No patients developed complications leading to airway compromise.

The only complications in this series were in patients taking anticoagulation medication, undergoing revision surgery, or in whom the laryngeal tissue was atrophic or absent. Careful patient selection to exclude any of the above should reduce the risk of complications. The authors would therefore advocate type one thyroplasty for unilateral or bilateral vocal fold paralysis as a suitable procedure for day-case surgery within our department.

The gold standard test for laryngopharyngeal reflux is 24-hour pH monitoring, which determines the reflux area index with a pH threshold of less than four (i.e. the reflux area index four). However, refluxed pepsin is able to cause laryngeal injury at pH levels above five.

Prospective study.

In order to establish normative values for a reflux area index with a pH threshold of less than five (i.e. the reflux area index five), 29 healthy volunteers underwent pH monitoring. In 45 patients with suspected laryngopharyngeal reflux, reflux area index four and reflux area index five were determined by pH study.

In healthy volunteers, the reflux area index five was 72.6 (95th percentile). In 29 of 44 patients, laryngopharyngeal reflux was diagnosed due to a reflux area index four of greater than 6.3. However, the reflux area index five revealed laryngopharyngeal reflux in six more patients.

For exact analysis of pH monitoring results, two pH thresholds (less than four and less than five) must be considered. Further studies with a larger number of healthy volunteers are necessary in order to reveal normative values for the reflux area index five parameter.

This study is the first attempt to link quantified phenylthiocarbamide bitter taste recognition threshold with susceptibility to motion sickness.

The study was conducted on a sample of 291 teenage Rajput children (146 males and 145 females; age range 13–19 years) from the Sirmour district of Himachal Pradesh, India. Phenylthiocarbamide taste sensitivity was measured by administering a serial dilution of a freshly prepared phenylthiocarbamide solution, following the method of Harris and Kalmus. Motion sickness susceptibility was assessed retrospectively via interview.

About 40 per cent of the subjects had experienced motion sickness in the past. The mean and standard deviation of phenylthiocarbamide taste thresholds in non-tasters and tasters were 0.83 ± 0.87 and 7.98 ± 1.86, respectively. A bimodal distribution test (D/S) index of 5.24 confirmed bimodality of phenylthiocarbamide taste threshold distribution. The Mann–Whitney U test rejected the null hypothesis of μ1 = μ2 and thus confirmed the existence of differences in the distributions of phenylthiocarbamide taste threshold between individuals susceptible and not susceptible to motion sickness. Individuals susceptible to motion sickness had lower mean and median taste thresholds, indicating higher phenylthiocarbamide taste sensitivity, compared with non-susceptible individuals. The frequency of non-tasters was about 10 per cent in both motion sickness susceptible and non-susceptible individuals. The simple division of phenylthiocarbamide tasting ability into tasters and non-tasters was a less sensitive criterion with which to measure the association of this ability with motion sickness susceptibility. However, further differentiation of tasters into weak threshold, medium threshold and super threshold (‘supersensitive’) tasters clearly revealed a highly significantly increased risk of motion sickness in super threshold tasters (i.e. threshold solution number ≥12). The ratio of motion sickness susceptible individuals to non-susceptible individuals was 1:1.7 for non-tasters (threshold solution numbers zero to three) and weak and medium tasters (threshold solution numbers four to 11), but the trend was reversed for super threshold tasters (threshold solution numbers 12 and 13), in whom the ratio was 2:1.

Individuals exhibiting greater phenylthiocarbamide taste acuity (i.e. supersensitive tasters) had a higher susceptibility to motion sickness than did non-, weak and medium phenylthiocarbamide tasters, as measured in terms of their taste thresholds (i.e. threshold solution numbers zero to 11).

We report what we believe to be a unique aetiology of the superior semicircular canal dehiscence syndrome, a recently described condition in which vestibular imbalance and/or hearing loss results from the loss of continuity of the bone overlying the superior semicircular canals.

A 58-year-old woman presented with autophony in the right ear and momentary imbalance when shouting (Tullio phenomenon). Temporal bone computed tomography revealed a defect of the right superior semicircular canal caused by an enlarged superior petrosal sinus receiving drainage from a large cerebellar developmental venous anomaly.

We review superior semicircular canal dehiscence syndrome and its management, and we discuss common aetiologies, contrasting these with the unusual aetiology presented here. We conclude that superior semicircular canal dehiscence syndrome may present with a solely developmental aetiology, despite presenting late in life.

Phenotypical Down syndrome includes pharyngeal and maxillary hypoplasia and, frequently, constricted maxillary arch with nasal obstruction.

This clinical trial assessed the effects of rapid maxillary expansion on ENT disorders in 24 children with Down syndrome randomly allocated to receive either rapid maxillary expansion or not. Each group received ENT and speech therapy assessments before expansion and after the device had been removed.

In the rapid maxillary expansion group, the yearly ENT infection rate was reduced when assessed after device removal (p < 0.01). The parents of rapid maxillary expansion children reported a reduction in respiratory obstruction symptoms. Audiological assessment revealed improvements in the rapid maxillary expansion group (p < 0.01). Cephalometry showed increased maxillary width in the rapid maxillary expansion group.

Rapid maxillary expansion resulted in a reduction in hearing loss, yearly rate of ENT infections and parentally assessed symptoms of upper airway obstruction, compared with no treatment. These findings are probably related to expanded oronasal space, due to rapid maxillary expansion.

The origin of vestibular schwannoma has always been a matter of debate. The aim of our study was to identify the nerve origin of this tumour.

Prospective case review. This study was conducted at Gruppo Otologico, a private referral centre for neurotology and skull base surgery.

A total of 200 cases of vestibular schwannoma were included in the study. All the tumours were removed surgically utilising the translabyrinthine approach. The origin of the tumour was sought at the fundus of the internal auditory canal.

A total of 200 consecutive cases was included in the study. The origin of the tumour was limited to one nerve at the fundus in 152 cases (76 per cent). Out of these cases, the tumour originated from the inferior vestibular nerve in 139 cases (91.4 per cent), from the superior vestibular nerve in nine cases (6 per cent), from the cochlear nerve in two cases (1.3 per cent) and from the facial nerve in two cases (1.3 per cent).

The vast majority of vestibular schwannomas originate from the inferior vestibular nerve; the incidence of involvement of this nerve increases as the tumour size increases. An origin of vestibular schwannoma from the inferior vestibular nerve can be considered as one of the explanatory factors for the poor functional outcome of the extended middle cranial fossa approach, and probably accounts also for the better hearing preservation rate reported in some series for the retrosigmoid approach.

To document the results of endoscopic orbital decompression performed in a group of patients with thyroid eye disease in order to improve cosmesis.

A retrospective case review was performed of all patients who had undergone endoscopic orbital decompression for cosmesis in our departments during the period January 1996 to September 2002. A postal questionnaire was sent to all patients who had undergone the operation, in order to assess patient satisfaction.

Twenty orbits of 15 patients underwent endoscopic decompression. For all orbits, the reduction in proptosis was 3.7±2.2 mm (mean±standard deviation; t = 6.5, p = 0.001). For 16 of the endoscopic decompressions (89 per cent), patients reported that they were satisfied with the appearance of their eye post-operatively, and all patients reported a reduction in eye prominence post-operatively.

Endoscopic decompression is an effective procedure for improving cosmesis in patients with inactive thyroid eye disease.

To analyse current trends in our population with respect to the presentation, diagnosis and management of tubercular and chronic pyogenic osteomyelitis of the cranio-facial bones.

Retrospective study.

Tertiary healthcare centre.

The study population comprised 14 patients with tubercular and chronic pyogenic osteomyelitis who were managed in the otorhinolaryngology department between May 2002 and December 2005.

Odontogenic infections, sinus infections and aural infections were the most commonly identified aetiological factors. Most of the patients presented with swelling, pain and discharging sinus. The diagnosis was established on the basis of clinical evaluation, radiological investigations and histopathological analysis, with six cases diagnosed with tubercular osteomyelitis and eight cases with chronic pyogenic osteomyelitis. All the patients were initially commenced on oral antibiotics, which were continued for two weeks in all cases with chronic pyogenic osteomyelitis. All the patients with pyogenic osteomyelitis underwent surgical management, with one patient requiring repeated surgical interventions. All the patients with tubercular osteomyelitis received anti-tubercular chemotherapy, with complete cure.

Osteomyelitis of the cranio-facial bones is an uncommon entity which requires a high index of clinical suspicion along with radiological and histopathological investigations in order to establish the diagnosis. Tubercular osteomyelitis is clinically and radiologically indistinguishable from pyogenic osteomyelitis, and the two conditions can be differentiated only on the basis of histopathological evaluation of involved tissue.

Retrospective.

Among 963 cases undergoing revision mastoid surgery, 13 patients suffered brain herniation. These cases were identified and analysed.

All 13 patients' initial diagnosis was chronic suppurative otitis media with cholesteatoma, and all had undergone previous mastoid surgery resulting in a defect in the tegmen and weakening of the dura mater. The revision procedures performed included 10 (76.9 per cent) modified radical mastoidectomies without ossicular chain reconstruction and one (7.6 per cent) modified radical mastoidectomy with ossicular chain reconstruction; two (15.3 per cent) patients required a blind sac closure. Brain herniation and/or cerebrospinal fluid leak were repaired by a transmastoid ± minicraniotomy procedure.

Injury to the tegmen and dura should be avoided during surgery for chronic middle-ear disease. Cerebrospinal fluid leaks, if encountered, should be managed in the same surgical session. The transmastoid approach is helpful in repairing defects smaller than 1 cm in diameter, whereas the combined transmastoid-minicraniotomy approach provides good access when closing defects larger than 1 cm in diameter and also enables auto-calvarial grafting.

One hundred and nine patients with primary antibody deficiencies were selected in order to determine the frequency of ENT complications.

Demographic information and ENT medical histories were collected for each patient. Duration of study for each patient was divided into two periods of before diagnosis and after diagnosis and the initiation of treatment.

Eighty-two of 109 patients (75.2 per cent) experienced ENT infections during the course of the disease (63: otitis media, 75: sinusitis and nine: mastoiditis). At the time of diagnosis, 52 (47.7 per cent) out of 109 patients presented with an ENT symptom. The frequencies of episodes were 27 for sinusitis and 25 for otitis media (one complicated with mastoiditis). After immunoglobulin replacement therapy the incidence of otitis media was reduced from 1.75 before treatment to 0.39 after treatment per patient per year (p = 0.008). The incidence of sinusitis also significantly decreased from 2.38 to 0.78 (p value = 0.011).

ENT infections are common medical problems in primary antibody deficiency patients. Persistent and recurrent ENT infections should be suspected as originating from a possible underlying immunodeficiency.

A wide range of well validated instruments is now available to assess voice quality and voice-related quality of life, but comparative studies of the responsiveness to change of these measures are lacking. The aim of this study was to assess the responsiveness to change of a range of different measures, following voice therapy and surgery.

Longitudinal, cohort comparison study.

Two UK voice clinics.

One hundred and forty-four patients referred for treatment of benign voice disorders, 90 undergoing voice therapy and 54 undergoing laryngeal microsurgery.

Three measures of self-reported voice quality (the vocal performance questionnaire, the voice handicap index and the voice symptom scale), plus the short form 36 (SF 36) general health status measure and the hospital anxiety and depression score. Perceptual, observer-rated analysis of voice quality was performed using the grade–roughness–breathiness–asthenia–strain scale. We compared the effect sizes (i.e. responsiveness to change) of the principal subscales of all measures before and after voice therapy or phonosurgery.

All three self-reported voice measures had large effect sizes following either voice therapy or surgery. Outcomes were similar in both treatment groups. The effect sizes for the observer-rated grade–roughness–breathiness–asthenia–strain scale scores were smaller, although still moderate. The roughness subscale in particular showed little change after therapy or surgery. Only small effects were observed in general health and mood measures.

The results suggest that the use of a voice-specific questionnaire is essential for assessing the effectiveness of voice interventions. All three self-reported measures tested were capable of detecting change, and scores were highly correlated. On the basis of this evaluation of different measures' sensitivities to change, there is no strong evidence to favour either the vocal performance questionnaire, the voice handicap index or the voice symptom scale.

The aim of this study was to determine the correlation between tuberculous meningitis and tuberculous otomastoiditis.

Meningeal involvement sites were investigated by magnetic resonance imaging in 32 patients (21 males, 11 females) who had previously been diagnosed with tuberculous meningitis. Clinical and laboratory findings and responses to anti-tuberculous treatment were evaluated, and the presence of concomitant tuberculous otomastoiditis was also investigated.

The meningeal involvement site was unilateral (in the sylvian fissure and the perimesencephalic cistern) in 28 patients (87.5 per cent), and bilateral and widespread in four patients (12.5 per cent). Tuberculous otomastoiditis was found in 11 of the patients with tuberculous meningitis (34.3 per cent). Otomastoiditis was on the same side as the meningeal involvement in nine of these 11 patients. Bilateral otomastoiditis with meningeal involvement was observed in two patients.

Tuberculous meningitis is frequently accompanied by otomastoiditis, although the exact causal relationship between the two conditions is unclear. Since meningitis is a serious clinical condition, concomitant otomastoiditis generally remains unrecognised. Tuberculosis should be considered in the differential diagnosis of patients with otitis or otomastoiditis who do not respond to antibiotic therapy.