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Background Sudden Sensorineural Hearing Loss (SSNHL) is a medical emergency requiring immediate attention as delayed treatment can lead to permanent and devastating consequences. Primary care physicians are likely the first to be presented with SSNHL and therefore have the crucial role of recognizing it and initiating timely and appropriate management. The aim of this study was to gain insight into the current knowledge and practice trends pertaining to the diagnosis and management of SSNHL among family physicians in Canada. Methods An 18-question survey targeting Canadian family physicians was marketed through two, physician-only discussion groups on the social media platform Facebook. Responses were collected between August 1st and December 22nd 2019 then aggregated and quantified. Results 52 family physicians submitted responses. 94.2% (n = 49) reported that in their practice, unilateral SSNHL warrants urgent referral to otolaryngology and 84.6% (n = 44) reported that unilateral sudden-onset hearing loss warrants urgent referral for audiological testing. 73.1% of participants (n = 38) reported that they would attempt to differentiate between conductive and sensorineural hearing loss if presented with unilateral, acute or sudden-onset hearing loss. 61.5% (n = 32) would rely on tuning fork tests to inform management decisions, as compared to 94.2% (n = 49) relying on case history and 88.5% (n = 46) on otoscopy. 76.9% (n = 40) would prescribe corticosteroids if presented with confirmed, unilateral SSNHL. Conclusion The majority of family physicians in the study would make appropriate referral and treatment decisions in the management of SSNHL, understanding it is a medical emergency. Tuning fork tests are under-utilized for informing management decisions compared to other means of differentiating conductive and sensorineural hearing loss. Further research is needed to understand why some family physicians do not prescribe corticosteroids for treatment of SSNHL, which may then identify any gaps in knowledge or inform improvements in clinical protocol. Graphical abstract

Objective To describe the effect of sinusitis on Weber test lateralization which normally lateralizes toward the ear of a conductive hearing loss and away from the ear of a sensorineural hearing loss; to investigate whether the presence of an extra-aural condition might affect Weber test results. Study design Descriptive study. Setting Tertiary referral center. Subjects and methods Consecutive adult patients with maxillary and/or frontal sinusitis were included (study group), as well as patients with normal sinus structure and function (control group) were enrolled between February and September 2019. Presence or absence of paranasal disease and middle ear aeration was confirmed by computerized tomography. Subjects with otologic condition were excluded. The physical examinations, tympanograms, and audiograms were unremarkable. The Weber test consisted of 512, 1024, and 2048 Hz tuning forks that were applied on the central incisors and frontal midline, and lateralization patterns were compared with the extent of paranasal pathology. Results There were 44 participants (M:F=26:18), age 19 to 63 years (average 51). The cohort included 39 patients with sinusitis, affecting the frontal and/or maxillary sinus(es), as well as 5 controls without evidence of paranasal disease. There was a match between the extent of paranasal disease and the results of all 3 Weber test frequencies in 35 patients (80%). Omission of the 2048 and 1024 Hz tuning forks from the analysis yielded a match in 40 (91%) and 43 (98%) patients, respectively. No Weber test lateralized to the nondiseased sinus in any subject. Weber test lateralization was observed in 11% of patients, after the sinonasal pathology was successfully addressed. Conclusion Weber test lateralization in the absence of aural pathology may be explained by asymmetry related to paranasal disease and may alert to its presence.

Recent guidelines encourage the use of Webet test (WT) as a part of the physical examination of a newly suspected sudden sensorineural hearing loss (SSNHL) patient. However, the most sensitive tuning-fork frequency has never been identified.To identify the most sensitive frequency for initial WT of patients with suspected SSNHL.Medical records of patients with confirmed SSNHL, who underwent formal audiometry in which the WT was carried out with different frequencies were analyzed.319 medical records were identified. The most sensitive WT frequency was 500 Hz, with a sensitivity of 94.49% (223/236. confidence interval 90.76-97.03). There was a non-significant difference between 1000 Hz EBO and 500 Hz EBO (The most sensitive WT frequency for SSNHL diagnosis is 500 Hz. However, the sensitivity of this frequency is 94.49%.512 or 1024 Hz should be used to better identified SSNHL. Even WT lateralization to the affected ear, does not preclude the diagnosis of SSNHL. Formal audiometry should be used in any case of medical history suspected for sudden hearing loss with normal otoscopy.

Objective: The aim of this paper was to compare the accuracy of a smartphone-based Weber test (SPWT) with the traditional tuning fork Weber test (TFWT) in identifying and differentiating between sensorineural hearing loss (SNHL) and conductive hearing loss (CHL). Study Design: We conducted a prospective, noncontrolled clinical study. Methods: Sixty patients referred to the emergency department due to unilateral hearing loss (HL) were enrolled. They were asked to press a single uncovered smartphone on their forehead and conduct a Weber test by means of the smartphone’s vibration application. The results were compared to the TFWT. Results: Twenty-six (43%) patients were diagnosed with a SNHL, and 34 (57%) with a CHL. The SPWT was in agreement with the TFWT (at a frequency of 512 Hz) in 55 (92%) patients. The sensitivity and specificity of the TFWT were 84.6 and 94.1%, respectively. The sensitivity and specificity of the SPWT were 76.9 and 97.1%, respectively. Conclusion: SPWT can serve as an auxiliary diagnostic tool in the absence of a 512-Hz tuning fork to assist in the identification of the type of HL and to potentially hasten the diagnosis and provision of treatment when indicated.

The skull vibration-induced nystagmus test (SVINT) is a global vestibular test stimulating otoliths and semicircular canals at a frequency of 100Hz, not modified by vestibular compensation, which may reveal vibration-induced nystagmus (VIN). Bone-conducted vibration applied to the mastoid processes and the vertex instantaneously induces predominantly low-velocity (∼10°/s) horizontal nystagmus, with rapid phases beating away from the affected side in patients with unilateral vestibular loss (UVL). VIN starts and stops immediately with stimulation, is continuous, reproducible, beats in the same direction irrespective of which mastoid process is stimulated, with no or little habituation. The SVINT acts like a vestibular Weber test. In peripheral UVL, the SVINT is a good marker of vestibular asymmetry and demonstrates pathological nystagmus beating towards the healthy side in 90% of cases of vestibular neuritis, 71% of cases of Meniere's diseases and 44 to 78% of vestibular schwannomas. In superior semicircular canal dehiscence, VIN usually beats towards the affected side due to facilitation of bone conduction related to the presence of a third window. Stimulation of the vertex is more effective than in UVL patients, with sensitivity extending to higher frequencies, up to 700Hz. Observation of vibration-induced nystagmus then reveals equally represented vertical, torsional, and horizontal components beating towards the affected ear, suggesting dominant, but not exclusive, stimulation of the dehiscent superior semicircular canal.

Purpose Distal finger trauma is one of the most frequent emergencies in children and has the potential for functional and cosmetic damage to the hand. The Atasoy flap (AF) is a vascularized, subcutaneous pedicle V-Y advancement flap used to cover a loss of distal finger substance. Our hypothesis was that the AF is a safe, reliable flap that results in few complications and gives satisfactory functional and cosmetic results in children. Methods We retrospectively assessed children with distal finger trauma and AF pulp reconstruction in our pediatric orthopedic department between 2008 and 2017. The lesion zone was classified, and we also evaluated necrosis, infection, the shape of the pulp, pulp sensitivity (Weber test), hyponychial scarring, and the presence of a hook nail deformity. Lastly, we compared patients who developed a hook nail with those who did not. Results Thirty children were included (mean age at trauma, 6.4 years [range, 1.3–15.7 years]). In 21 cases, the finger damage was located in Ishikawa subzone II. No cases of necrosis or infection were reported. Epicritical tactile sensitivity was good in 20 patients (67%). A hook nail deformity was observed in 15 children (50%) and hyponychial scarring in 22 patients (73%). The pulp had a normal shape in 13 children (43%). The hook nail group displayed more hyponychial scarring, greater nail dystrophy , and lower pulp sensitivity. Conclusions The AF yielded contrasting results. High reliability, good coverage, and minimal donor-site morbidity were compromised by suboptimal tip length/shape, nail appearance, and sensitivity. Type of study/level of evidence Therapeutic IV.

Objectives: To compare the diagnostic performance of the Hum Test against the Weber Test using pure tone audiometry (PTA) as the “gold standard” comparator. Methods: 29 participants with normal hearing of ages 18 to 35 without any history of hearing abnormalities or otologic conditions were enrolled. Subjects underwent three tests (Hum Test, Weber Test, and PTA) across two conditions: with an ear plug in one ear (side randomized) and without ear plugs. Results: When examining the ability of the Hum Test to detect simulated conductive hearing loss (CHL), the test had a sensitivity of 89.7% and specificity of 100% with high pitched humming and 93.1% and 100%, respectively, with low pitched humming. The Weber Test had a sensitivity and specificity of 96.6% and 100%, respectively. McNemar’s test demonstrated agreement between the Hum Test, performed with either high pitched ( P = .32) or low pitched ( P = .56) humming, and the Weber Test. Receiver operating characteristic (ROC) curves for the Hum Test (both high and low pitched) and Weber test were compared and demonstrated no statistically significant difference. Conclusion: The Hum Test is comparable to the Weber Test with regards to its sensitivity, specificity, and diagnostic accuracy in assessing new onset unilateral CHL in previously normal hearing subjects.

Mondini dysplasia is a developmental disorder of the inner ear structures and it is a rare cause of recurrent bacterial meningitis in children. A 10-year-old boy presented with acute febrile encephalopathy and right ear pain. In the past, he had suffered from two distinct episodes of pyogenic meningitis. On examination, he had signs of meningeal irritation and right ear sensorineural deafness. Magnetic resonance imaging of the brain and computerized tomography of the temporal bone was suggestive of Mondini dysplasia in the right ear. Our case highlights the need for (a) screening of hearing loss at the bedside by Rinne and Weber test in case of recurrent bacterial meningitis (b) searching for an underlying inner ear malformation if there is a hearing loss. Keywords: Recurrent meningitis, Mondini dysplasia, Cerebrospinal fluid, Sensorineural hearing loss

Objectives Analyze reasons for unilateral conductive hearing loss (CHL) with unknown etiology in children. Introduction Unilateral conductive hearing loss (HL) without known etiology can be undiagnosed despite of hearing screening programs. It can be difficult to find the reason for HL and to make a treatment plan. Middle ear endoscopy gives hard-evidence diagnosis and basis for an individual treatment plan. Methods and material Prospective clinical follow-up study for a cohort of generally healthy elementary school age children with unilateral conductive HL with unknown etiology. The study population was 192 children, of which 46 had a HL of at least 25 dB with more than 10 dB conductive component. Mean age was 8.7 years. Preoperative tests included otomicroscopy, bone- and air-conduction audiogram, tympanometry, stapes reflex tests, Rinne and Weber test and Otoacoustic emissions. The children underwent endoscopy of the middle ear with an individual treatment plan and long-term follow-up. The aim was to explore etiology and to give a treatment plan for hearing loss. Follow-up included air- and bone conduction hearing tests annually or every other year. Mean follow-up was 5.2 years. Results A clear etiological finding was found in 36 (78%) ears, stapes anomaly (23) as the most common (64%) finding. Other findings were two cholesteatomas, 2 status after trauma, 5 middle ear anomalies, 5 incus fixations and one incus erosion. Air conduction hearing improved spontaneously during follow-up in 81% (17/21, 2 dropouts) of the stapes anomaly ears (mean 11,3 dB, range 4–32 dB), and none of these ears showed hearing deterioration. In the incus fixation group, one ear showed hearing deterioration. There were no major complications for exploration, and 5 minor postoperative infections. Conclusions The most common reason for pediatric unilateral conductive hearing loss was stapes anomaly/fixation. The HL does not deteriorate. Hearing loss in stapes anomalies shows a tendency for spontaneous recovery. Stapes surgery can be postponed or avoided.

Objective To establish optimum stimulus frequency and location of bone conducted vibration provoking a skull vibration induced nystagmus (SVIN) in superior semi-circular canal dehiscences. Methods SVIN 3D components in 40 patients with semi-circular canal dehiscence (27 unilateral and 13 bilateral) were compared with a group of 18 patients with severe unilateral vestibular loss and a control group of 11 volunteers. Results In unilateral semi-circular canal dehiscences, SVIN torsional and horizontal components observed on vertex location in 88% beat toward the lesion side in 95%, and can be obtained up to 800 Hz (around 500 Hz being optimal). SVIN slow-phase-velocity was significantly higher on vertex stimulation at 100 and 300 Hz (P = 0.04) than on mastoids. SVIN vertical component is more often upbeating than downbeating. A SVIN was significantly more often observed in unilateral than bilateral semi-circular-canal dehiscences (P = 0.009) and with a higher slow phase velocity (P = 0.008). In severe unilateral vestibular lesions the optimal frequency was 100 Hz and SVIN beat toward the intact side. The mastoid stimulation was significantly more efficient than vertex stimulation at 60 and 100 Hz (P Conclusion SVIN reveals instantaneously in unilateral semi-circular canal dehiscences a characteristic nystagmus beating, for the torsional and horizontal components, toward the lesion side and with a greater sensitivity toward high frequencies on vertex stimulation. SVIN three components analysis suggests a stimulation of both superior semi-circular canal and utricle. SVIN acts as a vestibular Weber test, assessing a vestibular asymmetrical function and is a useful indicator for unilateral semi-circular canal dehiscence.

When bone is exposed, fingertip defects may require a flap procedure. Many types are available, with little consensus about which one is best. But each one has its own disadvantages: esthetic (claw nail deformity), neurological (sensory disorders) or functional (stiffness). The purpose of this study was to evaluate a palmar bipedicular island flap (our modification of the Tranquilli-Leali flap) for fingertip reconstruction. We retrospectively analyzed the prospective data of 42 patients with a mean follow up of 16.4 months (6–30 months) operated in two hospital facilities. Thirty-three patients were men. Mean age was 39.5 years (12–83 years) and 24 (57.1%) were smokers. In 23 cases (54.8%), the trauma was work-related. A crush mechanism was identified in 29 patients (69%). According to Allen's classification of fingertip injuries, there were 26 cases of stage II, 14 cases of stage III and 2 cases of stage IV. All procedures were performed on an emergency basis, within 24 hours of the trauma. No claw nail deformity was observed. The mean discrimination in the Weber test was 3.8 mm (2–6 mm). The mean Total Active Motion (TAM) was 268.3 ° (255–275 °). The patient satisfaction was graded at 8.7 on a 10-point scale. Results were considered as excellent in 15 cases and good in 23 cases. The modified palmar bipedicular island flap makes the old Tranquilli–Leali flap – which has a poor reputation in the hand surgeon community—simpler and safer to use. This flap restores proper balance between skin, bone and nail in Allen stage II and III fingertip amputations.

Despite advances in microsurgery, digital nerve repair remains a challenge due to the lack of reproducible procedures with satisfactory functional results. The aim of this study was to compare the sensory and functional results of direct microsurgical sutures protected by a Revolnerv(®) nerve regeneration conduit, with results of a series of direct sutures without a protective conduit in the literature. From November 2009 to April 2010, 35 patients were treated by direct epiperineural suture for digital nerve injury, protected by a Revolnerv(®) nerve regeneration conduit at the FESUM centre "SOS-mains Lesquin/CHRU de Lille". Sensory recovery was assessed by the static two-point discrimination Weber test (WS) and the Semmes-Weinstein (SW) test at postoperative months 1, 3, and 6. The final evaluation was performed after a minimum follow-up of 6 months. Statistical analysis of sensory results (WS and SW) was mainly performed with non-parametric tests (Wilcoxon, Mann and Whitney). P

Background: The aim of the study was to analyze the possible effects of eyeglasses and palatal prostheses on the results of the Weber tuning fork test.Methods: We enrolled 96 patients diagnosed with unilateral conductive-type hearing loss. All were aged between 18 and 65 years. Group 1 was composed of 48 patients with eyeglasses and group 2 included 48 patients with palatal prostheses. All patients underwent the weber tuning fork test with and without their eyeglasses and prostheses, and the results were compared. Weber tests were performed using 256 Hz and 512 Hz tuning forks. Results: No significant between-group difference was apparent (both p>0.05).Conclusions Neither eyeglasses nor palatal prostheses significantly affected the results of the Weber test.

International audience; The skull vibration-induced nystagmus test is a robust, nonintrusive and easy to perform test. This test acts as a vestibular Weber test and is performed as a bedside examination. It usually instantaneously reveals vibration-induced nystagmus (VIN) even in long standing or chronic compensated unilateral vestibular lesions. The test requires stimulation at 30, 60 or more efficiently at 100Hz. The vibrator is applied perpendicularly to the skin on a subject sitting up straight on the right and then the left mastoid (level with external acoustic meatus) and vertex. The VIN can be observed under videonystagmoscopy or Frenzel goggles. Either the direct tracing or the VIN slow phase velocity can be recorded on a 2D or 3D videonystagmograph. The patients should be relaxed and not treated by strong sedative medications. This rapid first-line test is not influenced by vestibular compensation and usefully complements other tests in the multifrequency evaluation of the vestibule. It acts as a global vestibular test by stimulating both canal and otolithic structures at 100Hz. It is useful in case of external acoustic meatus or middle ear disease as a substitute for the water caloric test and is preferable in elderly patients with vascular disease or arthritis of the neck to the head-shaking-test or head-impulse-test.

Introduction Hand wounds are a common cause of emergency department admission. Digital nerve lesions are found in 5% of palm wounds. Early diagnosis reduces the risk of morbidity, sequelae and litigation. Screening for digital nerve injury by the usual tests is difficult in an emergency context. We assessed the diagnostic value of the simple “compress test” to screen for pulp sensibility disorder and the factors that may influence the value of this examination, with a view to validating routine use. Material and method A retrospective study included 821 palm wounds treated between January 2014 and May 2016. There were 605 male and 216 patients; mean age, 42.8 years (range: 18–90 years). The dominant hand was involved in 307 cases (37.4%). Sensitivity, specificity, positive and negative predictive values, and positive and negative likelihood ratios of the compress test were calculated. Diagnostic value was also calculated according to age, gender, affected digit, dominant side and examiner's experience. Results Clinical deficit was found in 412 cases (50.2%). A digital nerve lesion was found intraoperatively in 277 cases (33.6%). Test sensitivity was 87.3%, specificity 68.6%, positive predictive value 58.5%, negative predictive value 91.4%, positive likelihood ratio 2.78 and negative likelihood ratio 0.18. The test was more effective for thumb wounds and for examination by a junior surgeon. There were no differences according to injured side, innervation territory or gender. Conclusion This clinical test is reliable, with very good negative predictive value and good sensitivity, allowing its use in routine clinical practice. Nevertheless, surgical exploration of deep palm injuries should remain the rule.

SummaryMit den Stimmgabelprüfungen nach Rinne und Weber können Sie Schallleitungsstörungen und Schallempfindungsstörungen unterscheiden.

This study analyzes permanent paresthetic disorders regarding the inferior alveolar nerve (IAN) after mandibular ramus sagittal osteotomy procedures. Fifty patients (ie, 100 nerves) who underwent mandibular bilateral sagittal split osteotomy between 2003 and 2007 were evaluated to detect sensorial disorders of the IAN. The evaluation was performed for each patient at least 1 year after surgical intervention. The sagittal osteotomy of the mandible ramus was performed according to Epker-Hunsuk technique. The method of fixing through titanium plates and monocortical screws and the displacement width of the osteotomized stumps were also considered. The evaluation of the IAN functionality was performed both subjectively, by means of a questionnaire, and clinically, by using 4 types of tests: light-touch sensation, pinprick sensation, Weber test, and Dellon test.The clinical test analysis revealed that no nervous lesion was detected in 52% of the tested sites, whereas 24% reported significant nervous lesions. In the subjective evaluations, 74% of the patients described the discomfort related to the neurologic alteration as "absent to mild" or "mild to moderate," 10% as "moderate to serious," and 4% as "serious."We observe that the percentage of significant nervous lesions is relatively low and that it matches the mean described in literature. The central nervous system capacity to hide or compensate for functional deficits due to peripheral nervous lesions was confirmed by the comparison between the results of the clinical tests and the patients' subjective evaluations.

Should the palmar digital artery be repaired in the pedicular section of the finger? This repair is discussed in the case of a unilateral pedicular lesion on a vascularised finger, and often neglected in current surgical practice. The problem is knowing the role of a permeable artery in sensory recovery and cold intolerance. The author presents a retrospective study on palmar digital nerve reinnervation after section with or without palmar digital artery section. Artery permeability was studied by doppler ultrasonography. Forty nerves in 35 patients were studied after at least 3 years had passed. Twenty-five nerves were associated with a permeable artery, 15 with a non permeable artery. The functional result was evaluated with the Weber test (S2PD) and by the presence or absence of cold intolerance. This work demonstrates the statistically significant role of the palmar digital artery in sensitive recovery quality and cold intolerance appearance. Its permeability ensures a better sensitive result with an 8.84 mm S2PD versus 13.47 mm with a non permeable artery. The benefit of the systematic repair of palmar digital artery is thus demonstrated for the first time. This repair is all the more justified if local conditions are unfavorable, in particular in contuse wounds with associated lesions (tendinous or bone).

Case presentation. A 51-year-old right-handed woman was admitted to the hospital because of two syncopal episodes. Both events had similar features with sudden onset of loss of consciousness. There were no preceding symptoms. They lasted only few seconds and were not accompanied by any abnormal movements, incontinence, or tongue biting. There was no confusion following the events. A feeling of vertigo, which gradually dissipated over the following 2 days, was the only residual symptom following both episodes. The patient also had a history of hypertension. She was on no medications. She consumed alcohol occasionally but denied tobacco or drug abuse. Her family history was positive only for heart disease in her mother. ### Question for consideration: 1. What is the differential diagnosis of syncope in this case? GO TO SECTION 2 Syncope is a sudden and brief loss of consciousness, associated with a loss of postural tone, with spontaneous recovery. It results from transiently decreased or interrupted cerebral blood flow. Frequently, the etiology remains unknown. Among diagnosed cases, neurocardiogenic syncope (including vasovagal attack, situational syncope, and carotid sinus syncope) is the most frequent cause. It stems from reflex-mediated changes in the vascular tone or heart rate. Other causes include cardiac (organic heart disease or arrhythmia) or neurologic diseases (concussion or seizure), orthostatic hypotension, medications, or psychiatric disorders.1,2 In this patient, based on the history, both reflex-mediated and primary cardiac causes were contemplated. On admission, the patient reported 3 years of right hearing defect without tinnitus or ear pain. She denied dysphagia or hoarseness of her voice. She was otherwise asymptomatic. On neurologic examination, cranial nerves I–VII were intact. There were multiple lower cranial neuropathies (VIII–XII) on the right side. There was sig-nificant right ear hearing loss. The tuning fork tests were equivocal: the Weber test lateralized to the affected ear (suggesting a conductive hearing loss in …

Objective: To present a case of Type 1 glomus tympanicum, its clinical presentations, surgical management and outcome. Methods: Study Design: Case Report Setting: Tertiary Government Hospital Patient: One Results: A 44-year-old woman with pulsatile tinnitus, vertigo, headache, ear fullness and decreased hearing on the right had a pulsatile reddish mass behind the tympanic membrane and Brown sign. Weber test lateralized to the right with mild conductive hearing loss on pure tone audiometry. Contrast CT scan demonstrated a 5x6 mm well-defined enhancing mass in the meso- and hypotympanum. Internal auditory canal MRI showed an avidly enhancing 5x3x4 mm nodule within the right middle ear adjacent to the cochlear promontory and anterior to the lateral semicircular canal. Impression was glomus tympanicum, type 1. The mass was excised via transcanal approach with post-operative resolution of tinnitus, headache, vertigo and improvement of hearing. Final histopathology was consistent with glomus tumor. Conclusion: Glomus tympanicum tumors are rare, benign middle ear paragangliomas that arise from Jacobson’s nerve, are slow-growing and locally destructive. CT scan and MRI may detect involvement of other structures. Surgical resection is the primary treatment modality. Type 1 glomus tympanicum tumors are small and limited to the promontory, and a less-invasive transcanal approach may be employed. Keywords: glomus, tympanicum, paraganglioma, transcanal approach

A 35-year-old woman presented for evaluation of slowly progressive right-sided hearing loss. For the past 6 months, she noted when lying on her left side an inability to hear the television or her baby crying. She also noted right-sided aural fullness and occasional right-sided headache. She had no facial weakness, tinnitus, vertigo, otorrhea, or otalgia; and no history of prior ear surgery, ear infections, noise exposure, or ear or head trauma. She currently takes no medications. Otoscopic examination results were normal. A 512-Hz tuning fork examination showed a left-sided lateralization when the vibrating tuning fork was placed on the center of her forehead (Weber test) and bilateral air greater than bone conduction with the vibrating tuning fork placed in front of her ear canal vs her mastoid tip (Rinne test). Facial motor and sensory function were intact and symmetric. The rest of the head and neck examination was unremarkable. An audiogram was ordered (Figure).

Problem statement: Ramsay Hunt syndrome is characterized by peripheral facial palsy and eruptions in external ear reportedly due to the reactivation of latent varicela zoster virus in the sensory ganglia of facial nerve. Our purpose is to describe a case of this syndrome accompanied by the Neurology Service of Nova Iguacu General Hospital. Approach: A 60-year old female patient sought the emergency room due to a complaint because she was not able to close her left eye, followed by a drift of the labial fold to the right as well as a sensation of &#34burning eyes&#34 for the last three days. She also referred vertigo and bilateral hypoacusis, more intensely felt on the left side. Results: Physical examination showed a left facial palsy with a vesicular eruption in the left external auditory canal, ear lobe and neck on that side. Her taste sensation was decreased on the anterior 2/3 of the tongue, a negative rinne test, a positive weber test indicating a neurossensorial hearing loss, ataxia in walking, a Romberg sign and an abnormal fukuda pace test, however the CT scan was normal. Conclusion: The diagnosis is basically clinical, in turn, treatment is controversial. In addition to clinical findings, the diagnosis is confirmed by the presence of viral DNA in the involved tissue and vesicular exudate, as assessed by polymerase chain reaction. Ramsay Hunt syndrome involves severe dysfunction, with poorer facial nerve prognosis than in Bell’s palsy. Some studies suggest that treatment with prednisone and acyclovir may improve outcome, although a prospective randomised treatment trial remains to be undertaken.

International audience; The skull vibration-induced nystagmus test (SVINT) acts as a vestibular Weber test and reveals a vibration-induced nystagmus (VIN), elicited mainly on the vertex location, with a horizontal or torsional component beating more often toward the side of the lesion in superior canal dehiscence (SCD) than in otosclerosis (OS). In SCD, the VIN vertical component is most often up-beating. These results suggest more a global vestibular contribution than the sole stimulation of the superior semicircular canal. Objectives: This study aimed to evaluate the possible occurrence of nystagmus during SVINT in unilateral conductive hearing loss related to SCD or OS. Methods: The slow-phase velocities (SPVs) of the VIN horizontal, torsional, and vertical components were recorded in patients with a unilateral otologic lesion (17 SCD, 38 OS) and 12 control subjects. Vibratory stimulations (60 Hz, 100 Hz) were applied on the vertex and on each mastoid. Results: In SCD, VIN was observed in 82% of patients with a primarily torsional, horizontal, and vertical (up-beating) component in 40%, 30%, and 30%, respectively. Horizontal and torsional components beat toward the side of the lesion more often than in OS. Higher SPVs were observed after vertex stimulation. In OS, VIN was sparse with low amplitude and was not systematically lateralized to a specific side.

Objectives/Hypothesis The objective of this study were to present an assessment of a new theory to explain lateralization during the Weber test using an animal model. This theory is based on the discovery that a major pathway in bone conduction stimulation to the inner ear is through the skull contents (probably the cerebrospinal fluid [CSF]). The placement of a bone vibrator or tuning fork on the skull excites the inner ear by the classic osseous pathway and by the suggested CSF pathway. We assume that there is a phase difference between the stimulation mediated by the ossicular chain (inertial and occlusion mechanisms) and the one mediated by the CSF. The presence of a conductive pathology will decrease the magnitude of the sound energy mediated by the ossicular chain. Thus, the out-of-phase signal arriving through the bony pathways will be decreased, hence increasing the resultant sound intensity stimulating the cochlea. Study Design Prospective animal study. Methods The experiment was performed on 10 fat sand rats, which had undergone unilateral cochleostomy and a small craniotomy. The auditory nerve brainstem response (ABR) thresholds were measured to air-conducted stimulation, to stimulation with the bone vibrator applied to the skull, and to stimulation with the bone vibrator applied directly to the brain through the craniotomy. The ossicular chain of the second ear was then fixed to the middle ear walls with cyanoacrylate glue to induce a conductive hearing loss. The ABR thresholds to the same three stimuli were then measured again. Results After ossicular chain fixation, the ABR threshold to air-conducted stimulation increased, to bone vibrator stimulation on the bone decreased (hearing improvement), and to bone vibrator stimulation directly on the brain remained unchanged. Conclusions This experiment confirms the proposed theory. During clinical bone conduction stimulation, there is a phase difference between sound energy reaching the inner ear through the middle ear ossicles and from the CSF. A middle ear conductive pathology removes one of these components, thus increasing the effective sound intensity in the affected ear. On the other hand, when the bone vibrator is applied on the brain, the inner ear is stimulated only through the CSF, so ossicular chain fixation does not change the ABR threshold. Moreover, this study proves that lateralization during the Weber phenomenon is the result, at least in part, of an intensity difference between sound energy reaching the two cochleae.

West Nile virus (WNV), is an emerging virus [1]. Most human infections are subclinical or manifest as a mild febrile illness, while fewer than 1% develop a neuroinvasive disease (WNND), including meningitis, encephalitis, and/or an acute flaccid paralysis (AFP) [2–4]. We describe a case of sensorineural hearing loss (SNHL) secondary to WNV infection. A 55 year-old previously healthy and normoacousic man, was admitted (in September 2010) for bilateral hearing loss and leg weakness. He complained, about 2 weeks before, of malaise, fever, generalized fatigue, and diffuse myalgias, initially attributed to a flu-like syndrome. After a few days he noted a mild weakness in both of his legs evolving over a week into a flaccid paraparesis. At the same time, he also developed a sudden bilateral hearing loss. He was afebrile, with stable vital signs. Routine and immunological laboratory data were within the normal range. Neurologic examination was remarkable for a flaccid areflexic paraparesis, weakness being more pronounced in the right leg and proximally (3/5 strength in the right leg, 4/5 strength in the left leg), with flexor plantar responses. No cranial nerve involvement, vestibular, cerebellar, brainstem or meningeal signs were noted. MRI of the brain and whole spine was normal without meningeal, parenchymal or ventral nerve root enhancement. Audiological tests, including the Weber and Rinne tuning fork tests, pure tone and speech audiometry, tympanic admittance, auditory evoked potentials (ABRs) and a screening test for acoustic emissions (OAEs), demonstrated a bilateral, down-sloping SNHL—mild on the left side, severe on the right—with normal acoustic admittance, and impaired OAEs, which were absent in the right ear. Weber test was lateralized to the left side, and the Rinne test was not significant. ABR with click at maximum output level (125 dB SPL) was not evocable by stimulating the right ear and normal for the left ear after latencies correction for the hearing threshold elevation. On the whole, the data were compatible with a bilateral asymmetric cochlear damage. Electrodiagnostic studies showed normal sensory and motor nerve conductions, and moderate decrease of compound motor action potential amplitudes. Late responses were normal. Concentric needle electromyography showed active denervation with a neurogenic recruitment pattern in all four limbs, with a clear predominance in legs. The findings fulfilled the diagnostic criteria for WNV AFP [4, 5]. Neurophysiological examination of cranial nerves and brainstem reflexes studies gave normal results. Since the symptoms began during a work-related stay in Romania where a WNV infection outbreak was occurring [6, 7], we considered the above infection in the differential diagnosis. Serum IgM and low-avidity IgG WNV-specific antibodies were detected (titre:1:1,600; 1:51,200, respectively). The presence of IgMand low avidity IgG WNV-specific I. Casetta (&) E. Cesnik Section of Neurology, Department of Medical and Neurological Sciences of Communication and Behaviour, University of Ferrara, Corso della Giovecca 203, 44121 Ferrara, Italy e-mail: cti@unife.it

Two neuro-ophthalmological syndromes due to paramedian pontine infarction have previously been described. The ‘‘one and a half’’ syndrome was first reported and named by Miller Fisher [1], and consists of horizontal gaze palsy combined with an ipsilateral inter-nuclear ophthalmoplegia (INO) resulting in loss of all horizontal eye movements except abduction of the contralateral eye. The causative lesion is usually within the ipsilateral pontine tegmentum and is due to involvement of the abducens nucleus plus the medial longitudinal fasciculus (MLF), or alternatively to involvement of the MLF plus the paramedian pontine reticular formation (PPRF). These structures lie in close proximity to the nucleus and intra-axial fasiculus of the facial nerve. Consequently, ipsilateral fascicular lower motor neurone facial nerve palsy may accompany the ‘‘one-and-a-half’’ syndrome. This situation was named the ‘‘eight and a half syndrome’’ by Eggenberger [2], who reported 3 cases of this syndrome that had occured secondary to ischaemic stroke. In continuation of this concept, we here describe the ‘‘sixteen and a half syndrome’’, which is the ‘‘one and a half’’ syndrome with an additional 7th and 8th cranial nerve (vestibulo-cochlear nerve) palsy. A 78-year-old right-handed man presented with sudden onset of incomplete right upper limb paresis, horizontal diplopia and ataxia. This was shortly followed by complete right lower limb paresis. There was associated nausea and vomiting. Examination revealed a left-sided lower motor neurone facial nerve palsy and dysarthria with loss of conjugate gaze to the left and loss of adduction of the left eye. Abduction of the right eye was accompanied by horizontal nystagmus. Convergence was spared. The patient also complained of new-onset left-sided hearing loss. The patient and his family were certain that his hearing before this event had been normal. Magnetic resonance imaging (MRI) with diffusion-weighted sequences revealed an area of restricted diffusion consistent with an acute infarct. The lesion extended beyond and lateral to the paramedian pontine territory supplied by the anteromedial group of pontine perforating arteries, and dorsally to the fourth ventricle and the region of the facial colliculus (Fig. 1a, b). Pure tone audiometry showed no detectable hearing in the left ear. Brainstem auditory evoked potential testing showed no reproducible wave I, wave III or wave V after left ear stimulation. Waves I, III and V from right ear stimulation were of low amplitude with normal absolute and interwave latencies. Eight months later, the Weber test lateralized to the right ear, and the Rinne test revealed absent air-conducted and bone-conducted sound perception in the left ear. Our patient had an ‘‘eight and a half’’ syndrome plus additional ipsilateral hearing loss. We name this clinical entity the ‘sixteen and a half syndrome’. Hearing loss has not previously been described in association with either ‘‘one and a half’’ or ‘‘eight and a half’’ syndrome. A clinical series of 20 cases of ‘‘one and a half’’ syndrome noted G. Cummins R. Dunne K. O’Rourke (&) T. Lynch Dublin Neurological Institute, Mater Misericordiae University Hospital, 57 Eccles St., Dublin 7, Ireland e-mail: killian.orourke@gmail.com

The Rinne and the Weber tests were carried out using a 256-Hz tuning fork on 687 6- to 15-year-old school children, the majority of whom were normally hearing subjects. The Rinne test was positive in 97% of ears with normal hearing or sensorineural hearing loss and negative in 72% of ears with conductive hearing loss. The Rinne test changed from positive to negative at 13 dB when performed using the normal loudness comparison method. In midline Weber results, the mean value of the air conduction difference at 0.25 kHz between the right and left ear was 1.4 dB. The lateralization in the Weber test occurred at a difference of 2.5 dB between the right and left ear. The Weber test was in midline in 96-98% of ears with bilateral normal hearing or sensorineural hearing loss. It lateralized to the poorer ear in 63% of ears with unilateral conductive hearing loss. The values of the Rinne and Weber tests were not good in the diagnosis of mild conductive hearing loss when the air-bone gap was 5-35 dB.

Objective. To compare the use of the Weber tuning fork test and ‘Scratch test’ in postoperative patients following tympanomastoid surgery. Method. Prospective cohort study with control group. Southampton University Hospitals NHS Trust. Secondary care. 19 patients undergoing routine tympanomastoid surgery for middle ear disease and 18 patients undergoing translabyrinthine surgery for lateral skull base lesions. Main Outcome Measures. Localisation of Weber tuning fork. Positive or negative Scratch test: clinician applies a gentle scratch using index finger to the bandage over the operated ear. Postoperative pure tone audiometry. Results. In the context of diagnosing a hearing or non-hearing ear, the Weber test had a specificity of 78% and sensitivity of 100%. The scratch test had a specificity of 100% and sensitivity of 100%. The Weber was 93% specific and 93% sensitive for diagnosing a unilateral conductive hearing loss. Conclusions. As a means of diagnosing an acute postoperative dead ear the Scratch test is an accurate alternative to the Weber test. References 1 Miltenburg D.M. (1994) The validity of tuning fork tests in diagnosing hearing loss. J Otolaryngol. 23, 254–259 2 Behn A., Laszlo C.A., Black D., et al. (2005) Which is mightier, the tuning fork or the bone oscillator? J Otolarygol. 34, 135–139

A 52-YEAR-OLD MAN WITH HUMAN IMMUNOdeficency virus (HIV), AIDS, chronic hepatitis B, and stage IV Burkitt lymphoma presented to the emergency department with a 2-week history of nausea, vomiting, diarrhea, and lethargy as well as a 2-day history of falls at home. He reported no vertigo, tinnitus, otalgia, or otorrhea; however, he did report a 2-month history of left-sided aural fullness and subjective hearing loss. On physical examination, the left tragus, antitragus, and conchal bowl (Figure1) exhibited nontender purple nodules and plaques extending into the cartilaginous external auditory canal. The external auditory canal was patent but narrowed. The tympanic membrane was intact and there was no otorrhea. There was no trismus or tenderness of the temporomandibular joint. A Weber test demonstrated lateralization to the right, and the results of a Rinne test were positive bilaterally. Relevant laboratory findings included a viral load of 152 000 copies/mL and a CD4 cell count of 8/μL. A skin punch biopsy specimen was obtained for histopathologic evaluation (Figure 2 and Figure 3). What is your diagnosis?