External auditory canal paraganglioma: an atypical presentation.

OBJECTIVE: We present the first published description of a painful paraganglioma of the external auditory canal. Atypical histopathology made the diagnosis difficult. We discuss the potential pitfalls of clinical diagnosis and treatment of such a case. CLINICAL PRESENTATION: A 49-year-old woman presented with left-sided otalgia, hearing loss and tinnitus. Physical examination revealed a firm swelling arising from the superior portion of the left external auditory canal. A clinical diagnosis of otitis externa was made. INTERVENTION: There was minimal response to medical treatment. The swelling was aspirated, leading to brisk bleeding. A tumour was suspected from the computed tomography scan, and confirmed by a biopsy. The patient underwent excision of the paraganglioma. The histopathology was atypical, making diagnosis difficult. CONCLUSION: Such unusual masses of the external ear should always be borne in mind, especially when dealing with atypical presentations of commonly encountered diseases. Clinicians should have a low threshold for early intervention with imaging and biopsy.

Does an encouraging letter encourage attendance at psychiatric out-patient clinics? The Leeds PROMPTS randomized study.

BACKGROUND: The aim was to reduce non-attendance for first-time consultations at psychiatric out-patient clinics. METHOD: The study was a pragmatic randomized controlled trial; the setting was seven inner-city UK out-patient clinics in Leeds. The participants were 764 subjects of working age with an appointment to attend a psychiatric out-patient clinic for the first time. The intervention was an 'orientation statement' letter delivered 24-48 h before the first appointment compared with standard care. The primary outcome measure was attendance at the first appointment; secondary outcomes included hospitalization, transfer of care, continuing attendance, discharge, presentation at accident and emergency and death by 1 year. RESULTS: Follow-up was for 763 out of 764 subjects (>99%) for primary and for 755 out of 764 subjects (98.8%) of secondary outcome data. The orientation statement significantly reduced the numbers of people failing to attend [79 out of 388 v. 101 out of 376 subjects, relative risk 0.76, 95% confidence interval (CI) 0.59-0.98, number needed to treat 16, 95% CI 10-187]. CONCLUSIONS: Prompting people to go to psychiatric out-patient clinics for the first time encourages them to attend. Pragmatic trials within a busy working environment are possible and informative.

Pharmacological treatments for psychosis-related polydipsia.

BACKGROUND: Polydipsia is the intake of more than three litres of fluids per day. Primary polydipsia occurs when excessive drinking cannot be explained by an identified medical condition, and is not secondary to polyuria. The prevalence of this problem in psychiatric inpatients has been estimated at between 6 and 17%. It can hinder standard care and be a highly disabling, even life-threatening condition. OBJECTIVES: To review the effect of pharmacological interventions for the treatment of psychosis-related polydipsia. SEARCH STRATEGY: We searched the Cochrane Schizophrenia Group's Register (January 2002 and February 2005) which is compiled by up-to-date methodical searches of BIOSIS, The Cochrane Library, CINAHL, Dissertation abstracts, EMBASE, LILACS, MEDLINE, PSYNDEX, PsycINFO, RUSSMED and Sociofile and is supplemented with hand searching of relevant journals and numerous conference proceedings. References of all identified studies were also searched for further trials. SELECTION CRITERIA: We included all randomised controlled trials involving people with a psychotic illness and secondary polydipsia, which evaluated drug treatments, and measured clinically meaningful outcomes. DATA COLLECTION AND ANALYSIS: Working independently, we inspected citations, ordered papers, and then re-inspected and quality assessed the studies and extracted data. For homogeneous dichotomous data, we calculated the relative risk (RR), 95% confidence interval (CI), and, where appropriate, the number needed to treat (NNT) and the number needed to harm (NNH), on an intention-to-treat basis. We assumed that people who left the study early or who were lost to follow-up had no improvement. We calculated weighted mean differences (WMD) for continuous data. We excluded data if loss to follow-up was greater than 50%. MAIN RESULTS: We identified two small trials (Alexander 1991 and Nishikawa 1996) which fulfilled the inclusion criteria, (total n=17, duration 3-6 weeks). Few data were reported and, because of inappropriate use of crossover methodology, we could not include all of the data in this review. For the few chronically ill people in these trials, neither the 'active' tetracycline bacteriostatic agent, oral demeclocycline, nor the opiate antagonist naloxone, nor placebo, gave any suggestion of serious adverse effects for a period of up to six weeks. The studies did not report any useful data on measures of polydipsia, physical symptoms secondary to increased fluid intake, mental state, general functioning or economic outcomes. AUTHORS' CONCLUSIONS: The trials offer little useful data to the clinician hoping to treat psychosis-related polydipsia with drugs, except that further evaluative studies need to be conducted in this area. Treatment of any sort for psychosis related polydipsia might only be informative within a well designed, conducted and reported randomised study. The two pioneering studies suggest that larger trials, though difficult, would not be impossible with adequate support and co-ordination.

[Endoscopic technique utilized into the selective vestibular neurotomy by retrosigmoid approach].

OBJECTIVE: To investigate the micro-wound technique and the signification of reducing brain tissue oppression and complication at the surgery with utilizing endoscopic technique during the operation of selective vestibular neurotomy (SVNT) by retrosigmoid approach. METHODS: Twelve patients were preceded the SVNT by retrosigmoid approach (as routine operation group, ROG), and nine patients were utilized endoscope for review and neurotomy during the process of the SVNT by retrosigmoid approach (as endoscopic operation group, EOG). All of them were examined by auditory level, vestibular function, facial nerve function and observed of clinical situation (including complication and equilibrium function and so on) before and after operation. RESULTS: Two patients appeared cerebral edema and demanded the treatment of dehydration and depressing intracranial pressure in ROG after operation. In each group, the average auditory threshold in 2 patients rose more than 15 dB post-operation. The interval time of average equilibrium function compensation is (29.00 +/- 9.60) d in ROG and (28.56 +/- 7.91) d in EOG after surgery. Peripheral facial paralysis didn't happen to any patient in two groups. After follow-up two years post-operation, the vertigo recurred in 2 patients (4 times and 1 times, respectively) in ROG and 1 patient (2 times) in EOG. CONCLUSIONS: The SVNT by retrosigmoid approach is widespread adopted presently as expedience and safe surgery method for Ménière's disease, and could effectively eliminate vertigo and simultaneously conserve the hearing function and the integration of facial nerve. This surgery process became micro-wound, more facility and safe following the endoscopic technique utilization, which could reduce the brain tissues depression and trauma and could not cause any of complications. Therefore, the endoscopic operation of the SVNT by retrosigmoid approach is worth to clinically popularize as the therapy method of vertigo for the patients of Ménière's disease, especially who associate with contralateral sensorineural hearing loss.

[The endoscopic technique utilized in removal process of acoustic neuroma by retrosigmoid approach].

OBJECTIVE: Research into the technique and signification of reducing remnant tumor with utilizing endoscopic technique during removal of acoustic neuroma. METHOD: 15 patients proceeded the removal of acoustic tumor through retrosigmoid approach (as routine retrosigmoid-approach operation group, RRSO-G), 11 patients utilized endoscope for inspecting and eliminating remain of tumor during the proceed of the removal of acoustic neuroma through retrosigmoid approach (as retrosigmoid-approach operation combined using endoscope, RSOCE-G), all of them were examined of auditory level, vestibular function, facial nerve function, and MRI before and three months after operation. RESULT: The remain tumor were found in 3 cases of RRSO-G and no any one in RSOCE-G after operation. The rates of auditory and vestibular dysfunction, and facial paralysis have no difference between RRSO-G and RSOCE-G. CONCLUSION: It is one of expedient and safe surgery methods that the acoustic neuroma are removed through the retrosigmoid approach, and some degrees of hearing function are reserved at same time. If the endoscope is utilized during this operation, the rate of tumor remaining and neuroma recrudescing could be reduced evidently. The acoustic neuroma removed through the retrosigmoid approach combining with utilizing endoscope is a worthy method for using widely in otoneurosurgery.

Migraine and Ménière's disease: is there a link?

BACKGROUND: A possible link between Ménière's disease (MD) and migraine was originally suggested by Prosper Ménière. Subsequent studies of the prevalence of migraine in MD produced conflicting results. OBJECTIVE: To determine the lifetime prevalence of migraine in patients with MD compared to sex- and age-matched controls. METHODS: The authors studied 78 patients (40 women, 38 men; age range 29 to 81 years) with idiopathic unilateral or bilateral MD according to the criteria of the American Academy of Otolaryngology. Diagnosis of migraine with and without aura was made via telephone interviews according to the criteria of the International Headache Society. Additional information was obtained concerning the concurrence of vertigo and migrainous symptoms during Ménière attacks. The authors interviewed sex- and age-matched orthopedic patients (n = 78) as controls. RESULTS: The lifetime prevalence of migraine with and without aura was higher in the MD group (56%) compared to controls (25%; p < 0.001). Forty-five percent of the patients with MD always experienced at least one migrainous symptom (migrainous headache, photophobia, aura symptoms) with Ménière attacks. CONCLUSIONS: The lifetime prevalence of migraine is increased in patients with MD when strict diagnostic criteria for both conditions are applied. The frequent occurrence of migrainous symptoms during Ménière attacks suggests a pathophysiologic link between the two diseases. Alternatively, because migraine itself is a frequent cause of audio-vestibular symptoms, current diagnostic criteria may not differentiate between MD and migrainous vertigo.

Pharmacological treatments for psychosis-related polydipsia.

BACKGROUND: Polydipsia is the intake of more than three litres of fluids per day. Primary polydipsia occurs when excessive drinking cannot be explained by an identified medical condition, and is not secondary to polyuria. The prevalence of this problem in psychiatric inpatients has been estimated at between 6 and 17%. It can hinder standard care and be a highly disabling, even life-threatening condition. OBJECTIVES: To review the effect of pharmacological interventions for the treatment of psychosis-related polydipsia. SEARCH STRATEGY: The reviewers searched the Cochrane Schizophrenia Group's Register (January 2002) which is compiled by up-to-date methodical searches of BIOSIS, The Cochrane Library, CINAHL, Dissertation abstracts, EMBASE, LILACS, MEDLINE, PSYNDEX, PsycINFO, RUSSMED and Sociofile and is supplemented with hand searching of relevant journals and numerous conference proceedings. References of all identified studies were also searched for further trials. SELECTION CRITERIA: All randomised controlled trials involving people with a psychotic illness and secondary polydipsia, which evaluated drug treatments, and measured clinically meaningful outcomes. DATA COLLECTION AND ANALYSIS: Reviewers, working independently, inspected citations, ordered papers, and then re-inspected and quality assessed the studies. They also worked independently to extract data. For homogeneous dichotomous data, the relative risk (RR), 95% confidence interval (CI), and, where appropriate, the number needed to treat (NNT) and the number needed to harm (NNH), were calculated on an intention-to-treat basis. Reviewers assumed that people who left the study early or were lost to follow-up had no improvement. Weighted mean differences (WMD) were calculated for continuous data. Data was excluded if loss to follow-up was greater than 50%. MAIN RESULTS: The reviewers identified two trials which fulfilled the inclusion criteria, (total n=17, duration 3-6 weeks). Few data were reported and, because of inappropriate use of crossover methodology, it could not all be used in this review. For the few chronically ill people in these trials, neither the 'active' tetracycline bacteriostatic agent, oral demeclocycline, nor the opiate antagonist naloxone, nor placebo, gave any suggestion of serious adverse effects for a period of up to six weeks. The two small studies did not report any useful data on measures of polydipsia, physical symptoms secondary to increased fluid intake, mental state, general functioning or economic outcomes. REVIEWER'S CONCLUSIONS: The trials offer little to the clinician hoping to treat psychosis-related polydipsia with drugs, except that further evaluative studies need to be conducted in this area. Treatment of any sort for psychosis related polydipsia might only be informative within a well designed, conducted and reported randomised study. The two pioneering studies suggest that larger trials, though difficult, would not be impossible with adequate support and co-ordination.

Primary malignant melanoma of the cerebellopontine angle.

BACKGROUND: Malignant tumors of the cerebellopontine angle are very rare, accounting for less than 1% of lesions at this site. These may be primary or secondary tumors of the temporal bone, central nervous system (CNS), or leptomeninges. Malignant melanoma is uncommon, accounting for 1.5% of all types of malignant tumors. Metastatic melanoma is a frequent cause of CNS metastasis, often with leptomeningeal spread. Primary leptomeningeal melanoma is, however, rare and even more so at the cerebellopontine angle. The prognosis for CNS malignant melanoma is generally very poor. PATIENT: The authors describe the case of a 29-year-old woman with unilateral hearing loss and facial paresis. Magnetic resonance imaging (MRI) demonstrated a mass that was thought to be an acoustic neuroma but was seen to involve the cochlea as well as the internal auditory meatus and cerebellopontine angle. The lesion was subsequently excised completely by a trans-labyrinthine approach, with facial nerve preservation, and was shown on histologic examination to be a malignant melanoma. Further comprehensive investigation did not reveal a primary extracranial site or any sign of CNS spread. The clinical features of this case, including the radiologic and histologic findings, are described, and literature concerning management is reviewed.

Tilted perception of the subjective 'upright' in unilateral loss of vestibular function.

OBJECTIVE: To demonstrate that unilateral vestibular loss (UVL) may cause an erroneous perception of the subjective (bodily) vertical. STUDY DESIGN: Comparison of patients with UVL with age-matched controls. SETTING: The study was performed in a clinical neurophysiology laboratory. PATIENTS: Seven patients with unilateral, mixed acute, and chronic losses of vestibular function (six surgical), two patients with bilaterally absent vestibular function, one patient with a plugged posterior canal, and twenty-two healthy individuals. INTERVENTIONS: Subjects were seated, whole body restrained, in a flight simulator. The simulator executed intermittent stepwise tilts, in roll, up to 28 degrees that subjects had to correct to maintain an "earth upright" attitude using the joystick. Subjects performed in both "calm" conditions and also when the simulator was oscillating in roll at 1 Hz, 4 degrees peak displacement to simulate "turbulence." The purpose of the turbulence was to destabilize (or mask) somatosensory cues to uprightness. MAIN OUTCOME MEASURES: Accuracy of corrections of subjective attitude to earth upright after tilts. RESULTS: All subjects accepted the initial attitude of the simulator as "upright." In response to imposed tilts, normal subjects (n = 22) corrected their attitude to a mean 0.4 degrees SD 1.5 degrees (tilted slightly rightward) in calm and 0.7 degrees SD 1.3 degrees in turbulence. On average, corrections were normometric. All patients with UVL responded to imposed tilts by corrections that left them 'flying' tilted slightly to the side of their lesion, mean 3.2 degrees SD 2.5 degrees when calm, 6.4 degrees SD 2.7 degrees in turbulence (p<0.01). Their corrections were hypometric in response to imposed tilts to the lesioned side (i.e., undershooting true upright) and hypermetric in response to tilts to the intact side. CONCLUSIONS: Unilateral vestibular loss causes a "vestibular perception" of an erroneous tilt of the body that is probably caused by an imbalance of otolith signals and apparently never fully compensates. The tilt is enhanced when rapid perturbations of posture make somatosensory cues difficult to interpret. An erroneous perception of upright may contribute to vestibular ataxia, which is provoked when motion context involves rapid change.

The magnetless Clarion cochlear implant in a patient with neurofibromatosis 2.

We present our experience using the Clarion magnetless multichannel cochlear implant with a woman profoundly deafened following bilateral acoustic neuromata as a consequence of neurofibromatosis 2 (NF2). The right neuroma had been previously removed without an attempt at neural preservation. On the left, however, a posterior fossa approach had been taken with the aim of preserving hearing. Although the left cochlear nerve appeared to be undamaged at the end of the operation, no hearing thresholds could be elicited on post-operative audiometry, because of damage either to the cochlear nerve or to the blood supply to the cochlea. Round window electrical stimulation subsequently produced a perception of sound, confirming that the cochlear nerve was capable of functioning and that a cochlear implant would be effective. Because she would need regular magnetic resonance imaging (MRI) to monitor existing and future NF2 lesions, it was decided to use a magnetless Clarion implant, which has been shown to be MRI compatible. We report our experience of using the device in this case and discuss some of the issues related to the provision of cochlear implants to patients with NF2.

Neurenteric cyst of the cerebellopontine angle: case report.

OBJECTIVE AND IMPORTANCE: We report a case of a neurenteric cyst of the cerebellopontine angle and review the five previously reported cases. The pathology and classification of these cysts are discussed. CLINICAL PRESENTATION: The patient presented with a 1-month history of nausea, vomiting, vertigo, and sudden hearing loss. INTERVENTION: The cyst was decompressed by a retrosigmoid approach. After recurrence of symptoms at 2 months, further decompression was required. CONCLUSION: The patient achieved a good outcome after the second operation, with cessation of her vomiting and vertigo, although she had residual hearing loss. Four of the five previously reported patients experienced satisfactory outcomes after surgery. The definitive diagnosis of these rare lesions is made using immunocytochemical techniques.

Horizontal otolith-ocular responses in humans after unilateral vestibular deafferentation.

We studied horizontal eye movements evoked by lateral whole body translation in nine patients who underwent vestibular nerve section. Preoperatively, all had preserved caloric function on both sides. Testing was performed before, 1 week and 6-10 weeks after surgery. Patients were seated upright in an electrically powered car running on a linear track. The car executed acceleration steps of 0.24 g, randomly to the left and right in the dark. The normal response consisted of a bidirectionally symmetrical nystagmus with compensatory slow phases. Response asymmetry of the slow-phase velocity of the desaccaded and averaged eye position signal was less than 13% in normals (n = 21). Before surgery, patients' responses were mostly symmetrical. Postoperatively, responses were diminished or absent with head acceleration towards the operated ear in all patients, causing a marked asymmetry which averaged 56% after correction for spontaneous nystagmus. On follow-up, responses regained symmetry. Thus, early after vestibular nerve section, a single utricle produces a normal LVOR only with ipsilateral head translation. Therefore, afferents for the LVOR seem to originate from the mid-lateral area of the macula, where hair cells are stimulated in their on-direction during ipsilateral head translation. Compensation may depend on recovery of the off-directional responses from lateral hair cells of the remaining utricle.

Medical management of Menière's disease.

The management of Meniere's disease often creates a formidable clinical challenge. Drugs may be helpful in treating the acute attacks of vertigo, and in more long-term management. These remedies are largely symptomatic and there are few properly controlled studies of their efficacy. A critical overview of the scientific and clinical basis for these various pharmacological agents is discussed.

The role of vestibular nerve section in Menière's disease.

The role of vestibular nerve section (VNS) surgery in the management of Menière's disease is considered by prospective analysis of a large series of 531 patients treated by the author over a period of 11 years. Twenty-seven percent were referred by their primary care physicians, while the rest were secondary (45.6%) or tertiary (27.3%) referrals. Overall, 62 (11.7%) Menière's patients underwent 63 VNS procedures. This surgery was undertaken more frequently in the secondary and tertiary referral patients (14.2%) than in the primary referrals (5.5%). The retrolabyrinthine technique was the preferred approach in almost 90% of ears. The results and complications of these and other surgical options are discussed in an attempt to define the present role of VNS in intractable Meniere's disease. Vertigo was abolished after VNS in 93% of cases. Compensation was significantly impaired in 12.9%. All these patients had contralateral Menière's disease or marked labyrinthine hypofunction. Although highly effective and associated with few postoperative complications, VNS is generally reserved for sac failures, though may be appropriate as a primary procedure in severe unilateral cases.

Evaluating 3D semicircular canal function by perception of rotation.

OBJECTIVE: We aimed to develop a clinical test of 3D canal dysfunction by perceptual matching of motion stimuli as an alternative to vestibular ocular reflex assessment. STUDY DESIGN: The study was a comparison with age-matched controls. SETTING: The study was performed in a clinical neurophysiology laboratory. PATIENTS: Ten patients with acute unilateral vestibular nerve section and 9 patients in the chronic stage of recovery, 2 acute-stage and 2 chronic-stage patients with posterior canal plugging, and 35 healthy individuals were studied. INTERVENTIONS: Seated on a motorized rotating chair in darkness, subjects were exposed to discrete, raised cosine velocity (60 degrees/s peak) rotations, for random displacements < or = 180 degrees rightward and leftward. They responded by rotating themselves back to the starting position with a joystick control. Horizontal canals were tested with head upright, ipsilateral versus contralateral vertical canal pairs were tested with the head down, face horizontal and co-planar anterior-posterior canal pairs were tested with the head down and turned approximately 45 degrees to the left or right. MAIN OUTCOME MEASURES: These were accuracy and symmetry of responses. RESULTS: Normal responses were approximately accurate returns to start. Nine patients with acute nerve section were hypometric (undershooting start) when displaced toward the lesion but normometric to the intact side. Eight chronic-stage nerve section patients with chronic dysfunction were hypometric to the lesion for vertical canal stimuli, but one third showed normal responses for horizontal canal testing. Patients with posterior canal plugging were hypometric specifically toward the plugged canal. CONCLUSIONS: The method reliably identifies acute and chronic dysfunction of vertical canals and acute dysfunction of horizontal canals. Dysfunction of a single canal can be specified.

Effect of olivocochlear bundle section on evoked otoacoustic emissions recorded using maximum length sequences.

Presenting clicks according to maximum length sequences (MLS) enables transient evoked otoacoustic emissions (TEOAE) to be recorded at very high stimulation rates. As the click rate is increased from 40 clicks/s up to a maximum rate of 5000 clicks/s there is a reduction in TEOAE amplitude that reaches an approximate asymptote at 1500 clicks/s. One hypothesis put forward to explain this MLS 'rate effect' is that ipsilateral efferent activity is involved. To test this hypothesis TEOAEs were recorded from both ears of five patients who had undergone a unilateral vestibular nerve section--a surgical procedure which also entails sectioning the olivocochlear bundle. TEOAEs were recorded conventionally at 40 clicks/s and using MLS stimulation at 5000 clicks/s. Increasing the rate from 40 to 5000 clicks/s was found to reduce the amplitude of the TEOAEs by equivalent amounts in ears ipsilateral and contralateral to a vestibular nerve section as well as in the ears of normal-hearing adults. Since an ear ipsilateral to a vestibular nerve section should have no efferent innervation the hypothesis that efferent activity is the major mechanism involved in the MLS rate effect is rejected. Instead, the possibility that intracochlear processes are the underlying mechanism will now be investigated.

Central nervous system complications of acute tonsillitis.

Acute tonsillitis is a common infection of early childhood which may even run a self-limiting course without antibiotic therapy in some cases. Complications are encountered infrequently and rarely assume a life-threatening propensity. Central nervous system involvement has not to our knowledge been described in association with tonsillitis without local abscess formation. We describe three cases in which acute tonsillitis/peritonsillitis was complicated by major central neurological sequelae. The neurological complications encountered in young previously healthy adults were: facial palsy and hemiplegia; superior sagittal sinus thrombosis with communicating hydrocephalus and papilloedema; Guillain-Barré syndrome and facial palsy. The pathogenesis and management is discussed. All patients made satisfactory recoveries, though with minor residual neurological disabilities.

CT screening for temporal bone abnormalities in idiopathic bilateral sensorineural hearing loss.

Bilateral sensorineural hearing loss can be caused by a variety of temporal bone abnormalities including primary cochlear otosclerosis, local and systemic bony diseases and some metabolic conditions. These may be identified using computerized tomography (CT), with attenuation recordings taken across the cochlear capsule (CT densitometry). Eighty patients with bilateral sensorineural hearing loss were screened over a period of six and a half years using this technique, and only three cases (3.8 per cent) of treatable disease were detected. Positive yields may be increased by screening selected cases with other clinical or biochemical stigmata of temporal bone disease.