Paediatric otogenic cerebral venous sinus thrombosis: a multidisciplinary approach.

BACKGROUND: Paediatric otogenic cerebral venous sinus thrombosis is a rare, heterogeneous and life-threatening condition, with possible otological, neurological and ophthalmological sequelae. Its course and outcomes can be widely variable. The publications available often consider individual aspects of paediatric otogenic cerebral venous sinus thrombosis management. The condition itself and the nature of the currently available guidance can lead to uncertainties when holistically managing patients with paediatric otogenic cerebral venous sinus thrombosis. OBJECTIVES: Clear recommendations for the comprehensive assessment and management of paediatric otogenic cerebral venous sinus thrombosis are presented, along with the literature review upon which they are based. Its clinical and radiological assessment are discussed. CONCLUSION: A multidisciplinary approach to assessment and management is recommended, inclusive of infectious diseases, ENT surgery, neurology, ophthalmology and haematology. On balance, anticoagulation is recommended for three months. Follow-up imaging is not recommended in the absence of clinical concern. Follow up by ENT surgery, neurology and ophthalmology departments is recommended.

A randomised controlled trial assessing the efficacy of co-phenylcaine nasal spray in flexible transnasal pharyngolaryngoscopy.

INTRODUCTION The aim of this study was to assess whether using co-phenylcaine nasal spray before flexible transnasal pharyngolaryngoscopy results in reduced pain and discomfort during the procedure. METHODS This was a randomised controlled trial. Eighty-four patients were randomised to receive either co-phenylcaine nasal spray or placebo nasal spray before performing transnasal flexible pharyngolaryngoscopy. Patient reported outcome measures included pain, discomfort, unpleasantness and willingness to repeat the procedure while clinician reported outcome measures comprised ease of examination and quality of view obtained during the procedure. RESULTS There was no statistically significant difference in scores for pain, discomfort, unpleasantness and willingness to repeat the procedure between the co-phenylcaine and placebo groups. On the other hand, ease of examination scores were significantly better for the co-phenylcaine group than for the placebo group. CONCLUSIONS The majority of patients do not find flexible pharyngolaryngoscopy unpleasant or painful with or without topical nasal anaesthesia. However, the spray does appear to help the examiner in completing a satisfactory assessment.

Smartphone speech-to-text applications for communication with profoundly deaf patients.

OBJECTIVE: Visual communication aids, such as handwriting or typing, are often used to communicate with deaf patients in the clinic. This study aimed to establish the feasibility of communicating through smartphone speech recognition software compared with writing or typing. METHOD: Thirty doctors and medical students were timed writing, typing and dictating a standard set of six sentences appropriate for a post-operative consultation, and the results were assessed for accuracy and legibility. RESULTS: The mean time for smartphone dictation (17.8 seconds, 95 per cent confidence interval = 17.0-18.7) was significantly faster than writing (59.2 seconds, 95 per cent confidence interval = 56.6-61.7) or typing (44 seconds, 95 per cent confidence interval = 41.0-47.1) (p < 0.001). Speech recognition was slightly less accurate, but accuracy increased with time spent dictating. CONCLUSION: Smartphone dictation is a feasible alternative to typing and handwriting. Slow speech may improve accuracy. Early clinical experience has been promising.

Ear symptoms in children with Fabry disease: data from the Fabry Outcome Survey.

BACKGROUND: Hearing loss and tinnitus are common symptoms in Fabry disease and increase in prevalence with age. This study aimed to provide an epidemiological description of hearing impairment and tinnitus in children with Fabry disease in the Fabry Outcome Survey (FOS), an international database to assess the natural history of Fabry disease and the efficacy of enzyme replacement therapy with agalsidase alpha. METHODS: Signs and symptoms questionnaires were completed for 543 children with Fabry disease. Pure-tone audiograms were obtained from 101 children (53 girls, 48 boys). RESULTS: On questioning, 33% of the children (n = 179) reported subjective hearing impairment. However, when assessed by age-appropriate audiometry, only 19 of 101 patients (19%) had a persistent hearing loss at least one frequency. Of these, 14 had a high-frequency hearing loss, 4 a pan-frequency hearing loss, and 1 a pattern typical of noise-induced loss. Of the 101 children with audiometry, 44 complained of tinnitus. Only 2 children reported sudden hearing loss, which was not verified by audiometry. Children with tinnitus had greater disease severity scores. CONCLUSIONS: Hearing loss is a well-known clinical manifestation in patients with Fabry disease. It was reported in significant numbers of children in the FOS signs and symptoms questionnaire, but confirmed in only 19% by formal audiometry. The subjective hearing impairment may have been due to middle-ear effusions in many cases. Tinnitus is a well-recognized symptom in Fabry disease and can present in childhood. The presence of tinnitus correlated with overall disease severity.

Conservative management of vestibular schwannomas: third review of a 10-year prospective study.

Seventy-two patients with a unilateral vestibular schwannoma have been treated conservatively for a median of 121 months. They have been followed prospectively by serial clinical examination, MRI scans and audiometry. Twenty-five patients (35%, 95% CI: 24-47) failed conservative management and required active intervention during the study. No factors predictive of tumour growth or failure of conservative management could be identified. Seventy-five per cent of failures occurred in the first half of the 10-year study. The median growth rate for all tumours at 10 years was 1 mm/year (range -0.53-7.84). Cerebellopontine angle tumours grew faster (1.4 mm/year) than intracanalicular tumours (0 mm/year, P < 0.01); 92% had growth rates under 2 mm/year. Hearing deteriorated substantially even in tumours that did not grow, but did so faster in tumours that grew significantly (mean deterioration in pure tone average at 0.5, 1, 2 and 3 kHz was 36 dB; speech discrimination scores deteriorated by 40%). Patients who failed conservative management had clinical outcomes that were not different from those who underwent primary treatment without a period of conservative management.

Evidence-based review of aetiopathogenic theories of congenital and acquired cholesteatoma.

Cholesteatoma is a non-neoplastic, keratinising lesion which has two forms: congenital and acquired. Congenital cholesteatoma develops behind a normal, intact tympanic membrane, whilst acquired cholesteatoma is associated with a defect in the tympanic membrane. The pathological substrate of cholesteatoma is keratinising stratified squamous epithelium, but the origin of this epidermal tissue in the middle ear is controversial. Here, we review the most relevant and recent evidence for the principal aetiopathogenic theories of both forms of cholesteatoma, in the light of recent otopathological findings. Congenital cholesteatoma is most plausibly explained by the persistence of fetal epidermoid formation. Conclusive 'proof' awaits the unambiguous demonstration of the metamorphosis of an epidermoid nidus into a lesion in vivo. Acquired cholesteatoma may develop by various mechanisms: immigration, basal hyperplasia, retraction pocket and/or trauma (iatrogenic or non-iatrogenic). However, squamous metaplasia of the normal cuboidal epithelium of the middle ear is a highly unlikely explanation. Chronic inflammation seems to play a fundamental role in multiple aetiopathogenic mechanisms of acquired cholesteatoma. Therefore early treatment of inflammatory conditions might reduce their sequelae, perhaps by preventing the development of hyperplastic papillary protrusions. Continued otopathological, cellular and molecular research would enhance our limited understanding of cholesteatoma and may lead to new therapeutic strategies for this erosive disease, which often defies surgical treatment.

Influence of ethnicity on the frequency of nasal surgery.

OBJECTIVE: To determine whether surgery for nasal obstruction differs in frequency between ethnic groups. METHOD: Ethnicity data was collected from all patients attending otolaryngology clinic appointments and compared to census data. Odds ratios with confidence intervals were calculated for attendance at otolaryngology clinics, rhinology clinics, undergoing septoplasty, septorhinoplasty and/ or turbinate surgery for each ethnic group over a 3-year period. RESULTS: The ethnic groups of the 39493 outpatient attendees closely mirrored demographic data from the 2001 Census. Non-Chinese Asian ethnic groups were more likely to undergo septal surgery than the general (mainly white) population (odds ratio 1.44, 95% CI 1.25 to 1.66, p < 0.00001), whereas Black groups (odds ratio 0.31 [0.23 - 0.41], p < 0.00001) and Chinese (odds ratio 0.28 [0.11 - 0.70], p = 0.00311) were much less likely. Similar results were found for rhinoplasty and turbinate surgery. CONCLUSIONS: There is strong statistical evidence for large differences in the frequency of surgery for nasal obstruction between ethnic groups. Asian groups were more likely to undergo surgery, whereas Chinese and Blacks were less likely than the general population, which was predominantly white in this study. This may be due to anatomical variations, differences cultural views towards surgery, or inequalities in clinician's attitudes.

How we do it: an audit of Action on ENT baseline standards in otolaryngology departments in England, UK.

An internet-based audit was conducted to determine how well English otolaryngology departments apply Action on ENT baseline clinical and administrative standards. A total of 91% (97 of 107) departments responded. Only 8% of 97 departments met all 23 standards but the majority complied with most standards. Microsuction and outpatient endoscopy were almost universally available (99% and 97% respectively) and 98% monitored in-patient and day surgery activity. Compliance was poor (<60%) for three standards: common waiting lists for common conditions (51%), facilities to elicit patient feedback (56%) and the inclusion of a treatment plan in the notes (46%). More than one in four departments lacked dedicated facilities to treat children or a lead clinician for paediatric audiology, despite the Children Acts of 1989 and 2004. It is hoped that this audit will help sub-optimal units to correct their deficiencies.

Hearing loss in Fabry disease: data from the Fabry Outcome Survey.

Hearing loss is a common symptom in Fabry disease, but neither its natural course nor its aetiology has been defined precisely. The aim of this study was to provide a detailed epidemiological description of hearing impairment in patients in the Fabry Outcome Survey (FOS), which is the largest available database of Fabry patients. Questionnaires were completed by 566 Fabry patients, of whom 316 reported ear-related symptoms. Pure-tone audiograms from 86 patients, performed before starting enzyme replacement therapy, were analysed and compared with age- and sex-specific normal values (International Organization for Standardization, ISO 7029). When compared to an age-matched population (ISO 7029), 74% of patients had a threshold elevated above the 95th centile in at least one tested frequency. All frequencies were affected to a similar degree. However, only 14 patients (16%) were clinically affected by hearing impairment according to the age-independent World Health Organization (WHO) classification (mean threshold at 0.5, 1 and 2 kHz worse than 25 dB). Hearing loss was sensorineural in 63 patients (73%) of whom 7 patients (8%) had also a conductive component. One patient had a purely conductive hearing loss. Episodes of sudden hearing loss seemed to occur more frequently than in the general population. Men were affected earlier and more severely than women. Hearing in Fabry disease is significantly worse than in an age-matched general population but leads to clinically relevant hearing impairment in only 16% of cases. It resembles accelerated presbycusis with an additional Fabry-specific strial-type hearing loss.

Agalsidase alpha and hearing in Fabry disease: data from the Fabry Outcome Survey.

Fabry disease is an X-linked lysosomal storage disorder characterized by multi-organ dysfunction, including hearing loss - mainly sensorineural. The recent introduction of enzyme replacement therapy (ERT) has resulted in improvements in renal and cardiac function, pain and quality of life. One study has also suggested small improvements in high-frequency hearing. In this paper, we study the effect of ERT on hearing in patients in the Europe-wide database - the Fabry Outcome Survey (FOS). Twenty-six patients in FOS had pure-tone audiometry performed up to 6 months before starting ERT with agalsidase alpha and after a median of 12 months of treatment. We assessed changes in hearing thresholds, expressed as deviations from the 50th centile of the normal population (International Organization for Standardization ISO 7029) to correct for age-related non-specific hearing deterioration. Hearing did not change significantly in ears with normal hearing (less than 10 dB deviation from the 50th centile of ISO 7029) or those with severe hearing loss (more than 40 dB deviation from the 50th centile of ISO 7029) at baseline. In ears with a mild or moderate hearing loss at baseline, hearing thresholds, expressed as deviations from the normal 50th centile, improved significantly by 4-7 dB at most frequencies (P < 0.05). Agalsidase alpha stabilizes, and possibly improves, hearing in Fabry patients who have not already progressed to severe hearing loss. Further follow-up of these patients will determine the longer-term effects of ERT.

Adenoid size assessment: a comparison of palpation, nasendoscopy and mirror examination.

OBJECTIVES: To compare the digital, mirror and nasendoscopic assessment of adenoid size and posterior choanal obstruction in patients undergoing adenoidectomy. DESIGN: Prospective, blinded study. SETTING: Otorhinolaryngology department at a London teaching hospital. PARTICIPANTS: Twenty-eight consecutive patients undergoing adenoidectomy in conjunction with tonsillectomy or myringotomy under general anaesthesia, aged 17 months to 16 years. MAIN OUTCOME MEASURES: Adenoid size and postnasal space obstruction as assessed by digital examination, nasendoscopy and trans-oral mirror visualization. These examination methods were each compared with each other. RESULTS: Nasendoscopy and mirror examination correlated well (Spearman's R(S) = 0.71, P < 0.0001) but Passing and Bablock regression analysis demonstrated that mirror examination consistently underestimated the degree of choanal obstruction in comparison with nasendoscopy. There was no significant correlation between nasendoscopy and palpation (R(S) = 0.26, P = 0.17) and only a moderate correlation between mirror examination and palpation (R(S) = 0.46, P = 0.014). CONCLUSION: If nasendoscopy is considered the gold standard, then palpation is a poor measure of adenoid hypertrophy and mirror examination consistently underestimates choanal occlusion.

Keratosis obturans and external ear canal cholesteatoma: how and why we should distinguish between these conditions.

Keratosis obturans and external ear canal cholesteatomas have been considered as separate entities for the last 20 years, after being regarded as variations of the same disease for at least 87 years. While both disorders are distinct, they do have some overlapping characteristics which may make it difficult to reach a definite diagnosis. This review explores the diagnostic dilemmas which may arise, and discusses the classification, aetiology, pathogenesis and management of these conditions. We concur that external ear canal cholesteatoma and keratosis obturans are different conditions and conclude that the presence of osteonecrosis and focal overlying epithelial loss are the most reliable features favouring the diagnosis of external ear canal cholesteatoma over keratosis obturans. Furthermore, whilst keratosis obturans can be managed successfully by regular aural toilet, external ear canal cholesteatoma may require surgical intervention depending on the extent of the disease.

Preoperative localization of parathyroid adenomas: ultrasonography, sestamibi scintigraphy, or both?

Minimal access techniques are increasingly used to remove parathyroid adenomas. Such surgery depends on accurate preoperative localization but the selection of imaging modality remains controversial. We have reviewed the accuracy of ultrasonography, sestamibi scintigraphy and their combination in 48 cases of primary hyperparathyroidism. Ultrasound had a sensitivity of 64.3% (95% confidence interval 44.1-81.4) and positive predictive value (PPV) of 100% (81.5-100) for correct lateralization. Sestamibi had a sensitivity of 83.3% (69.8-92.5) and PPV of 87.1% (73.7-95.1). The simple combination of ultrasound with sestamibi had a sensitivity of 82.1% (63.1-93.9) and a PPV of 92.0% (74.0-99.0): little different from sestamibi alone. However, if the sestamibi result was disregarded in favour of ultrasonography in discordant cases, the sensitivity reached 96.4% (81.7-99.9) and the PPV was 100% (87.2-100). These results were not dependant on a learning curve or the size of adenoma.

Intranasal schwannoma in a young woman.

A rare case of a schwannoma arising from the middle meatus in a 24-year-old woman is reported. The patient presented with a 12-month history of right-sided nasal obstruction, rhinorrhoea, anosmia, headache and recurrent minor nosebleeds. Examination of the right nasal cavity revealed a polypoidal mass. Computerised tomography showed the mass completely occluding the nasal passage with evidence of a secondary maxillary sinusitis. She underwent a complete intranasal excision of the polypoidal mass which was arising from the right middle meatus. The histological features of the lesion were consistent with a schwannoma including diffuse immunoreactivity for S-100 protein. This case illustrates the need to consider schwannoma, amongst many other lesions, in the differential diagnosis of a unilateral nasal mass and reinforces the established principle of sending all material removed from the nose for histological examination.

A novel method for replacement of a blocked fine bore nasogastric tube.

Nasogastric intubation is often difficult in patients with upper aero-digestive tract tumours. We present a new method for replacing obstructed fine-bore nasogastric tubes. This method eliminates the need for specialized equipment and skills, minimizes the risk of tracheal intubation and oesophageal trauma, and is better tolerated by patients.

Otological trauma resulting from the Soho Nail Bomb in London, April 1999.

We report the otological effects of the April 1999 Soho Nail Bomb on 17 patients. Twenty-one (62%) tympanic membranes were perforated (pars tensa only); 78% closed spontaneously within 6 months. The mean size of the perforation in the tympanic membrane nearer to the blast was significantly larger than the opposite side [33% +/- 8.3 (mean +/- SD) and 13% +/- 4.1 respectively; P = 0.02]. All patients reported hearing losses that were mixed conductive and sensorineural but mainly high-frequency sensorineural (4, 6 and 8 kHz, pure tone average 42.3 dB +/- 20.5). The sensorineural hearing loss correlated inversely with the distance from the explosion but not with the size of perforation. There was no significant difference in the hearing loss between the ear facing the blast and the opposite ear. Fifteen patients (88%) had temporary tinnitus. No patient complained of any vestibular symptoms. The otological effects of a nail bomb in an enclosed space have not been previously reported. Furthermore, an inverse correlation between hearing loss and distance from the explosion and a significant difference in perforation size facing the blast, compared with the opposite side, are also presented for the first time. The high spontaneous closure rate of perforations and minimal ongoing disability from sensorineural losses favour conservative management in most cases.

Hearing improvement in patients with Fabry disease treated with agalsidase alfa.

AIM: To describe the nature and prevalence of hearing loss in Fabry disease, and its response to enzyme replacement therapy (ERT) with agalsidase alfa. METHODS: Fifteen male patients with Fabry disease were enrolled in a randomized, double-blind study and received placebo (n = 8) or ERT (n = 7) with agalsidase alfa for 6 months. This was followed by an open-label extension of 36 months thus far. Alongside this trial, an additional eight men and two women have so far received open-label ERT for between 6 and 30 months. Pure-tone audiometry, impedance audiometry and otoacoustic emission testing were performed at 0 (baseline), 6, 18, 30 and 42 months. RESULTS: Nine patients (36%) had bilateral and ten (40%) had unilateral high-frequency sensorineural hearing loss (SNHL). Three (12%) had unilateral middle ear effusions with conductive losses persisting beyond 6 months. Only five patients (20%) had normal hearing. The high-frequency SNHL deteriorated over the first 6 months in both placebo and active treatment groups by a median 6.3 dB (p < 0.0001, Wilcoxon matched-pairs). This hearing loss subsequently improved above baseline by 1.5 dB at 18 months (p = 0.07), by 5.0 dB at 30 months (p = 0.006) and by 4.0 dB at 42 months (p = 0.01). CONCLUSION: Significant hearing loss, usually high-frequency SNHL, is a common manifestation of Fabry disease in adults. Alpha-galactosidase A replacement therapy with agalsidase alfa appears to reverse the hearing deterioration in these patients. This improvement, however, is gradual, suggesting the need for long-term ERT.

Hearing loss in Fabry disease: the effect of agalsidase alfa replacement therapy.

The aim of this study was to describe the nature and prevalence of hearing loss in Fabry disease (McKusick 301500), a rare X-linked lysosomal storage disorder, and its response to enzyme replacement therapy with agalsidase alfa. Fifteen hemizygous male Fabry patients (aged 25-49 years) were randomized to receive placebo or enzyme replacement therapy for 6 months; all have received open-label enzyme replacement therapy for an additional 24 months thus far. Pure-tone audiometry, impedance audiometry and otoacoustic emission testing were performed at 0 (baseline), 6, 18 and 30 months. Four patients (27%) had bilateral and 7 (47%) had unilateral high-frequency sensorineural hearing loss (SNHL). Two (13%) had unilateral middle ear effusions with conductive losses persisting beyond 6 months. Only 3 (20%) had normal hearing. High-frequency SNHL deteriorated over the first 6 months in both placebo and active treatment groups by a median 4.3 dB ( p =0.002, Wilcoxon matched pairs). This hearing loss subsequently improved above baseline by 2.1 dB at 18 months ( p =0.02) and by 4.9 dB at 30 months ( p =0.004). In conclusion, significant hearing loss, usually high-frequency SNHL, is a common manifestation of Fabry disease in adults. alpha-Galactosidase A replacement therapy with agalsidase alfa appears to reverse the hearing deterioration in these patients. This improvement is gradual, however, suggesting the need for long-term enzyme replacement therapy.

Re-evaluation of normative electronystagmography data in healthy ageing.

Previous work by our group questions the validity of existing electronystagmography (ENG) reference ranges in the elderly. We aim to establish valid reference ranges for ENG in people over 65 on the Nicolet Nystar Plus system. Ninety-six healthy asymptomatic subjects over 65 underwent ENG, including spontaneous and positional nystagmus, saccades, smooth pursuit, optokinetic nystagmus and bithermal calorics; 95% reference ranges with confidence intervals were calculated. The newly determined reference ranges were far wider than those provided by the ENG equipment manufacturer for eight out of 11 parameters (all P < 0.001). Vestibular function is known to deteriorate and become more variable with age. The failure to reflect this change in currently used reference ranges may have contributed to the high rates of vestibular disease reported in some series of dizzy elderly patients. Clinical interpretation of ENG depends on valid reference ranges.