Behçet’s disease presenting with a hypopharyngeal ulcer

A 40-year-old man presented with a single, major ulcer in the hypopharynx. Histopathological examination revealed necrosis and extensive vasculitis. Since the patient had recurrent oral ulcers and genital ulcers in his history and a positive pathergy test, a diagnosis of Behçets disease was made, according to the 1992 international criteria. Behçets disease should be kept in mind in differential diagnoses of hypopharyngeal ulcerous lesions, and detailed case histories should be taken.     

Audio-vestibular disturbance in patients with Behçet's disease

Objective: The present study prospectively investigated the frequencies and characteristics of audio‐vestibular disturbance and the results of audiologic and vestibular function tests in patients with Behçet's disease. Study Design: A prospective study. Methods: Seventeen patients with Behçet's disease (mean age 41.2 [30–56] years, 5 males and 12 females), as well as age‐ and sex‐matched healthy controls, were included in this study. All subjects satisfied the diagnostic criteria of the International Study Group for Behçet's Disease. Information on the subjects was obtained through questionnaires. Tests, including those for hearing impairment, tinnitus, ear fullness, and dizziness, pure‐tone audiometry, and vestibular function, were performed. Results: Auditory symptoms were reported by 7 (41.2%) and dizziness by 14 (82.4%) of the subjects. Sensorineural hearing loss was present in four (23.5%) subjects. One was treated with a cochlear implantation because of bilateral sudden deafness. Spontaneous nystagmus was detected in two (11.8%) subjects. Abnormal findings were noted in 1 (5.9%) in saccadic movement, 5 (29.4%) in the bithermal caloric tests, and 10 (58.9%) in the rotation chair tests. In total, audiologic or vestibular disturbance were noted in 16 (94.1%) of the subjects. However, audio‐vestibular abnormalities were not noted in the healthy controls. Conclusion: Audio‐vestibular disturbance is not rare in Behçet's disease patients compared with healthy controls. We believe that audio‐vestibular assessment and management may be helpful for the diagnosis and treatment of patients with Behçet's disease.     

Infliximab for refractory oral ulcers

Recurrent oral ulcer (ROU) is a common condition that significantly impacts quality of life. It is often related to systemic diseases, such as Behçet's disease (BD), Crohn's disease, and ulcerative colitis. Treatment of ROU depends on its severity: from topical agents for mild cases to systemic agents, such as corticosteroids, azathioprine, or other immunosuppressants for severe cases. Recently, good results have been reported with infliximab in refractory ROU. However, the optimal dosage and treatment duration have not been determined and the cost and potential side effects should be considered. We report on four patients who received a single-dose infliximab for refractory ROU. Two patients had refractory ROU with no underlying disease; one of them had soft palate perforation accompanied by severe oral ulcers. The two other patients had ROU of BD without major organ involvement. All patients received a single infusion of infliximab and an additional infusion was given on demand in one patient. Infliximab showed a rapid, good response in three patients and was also effective in improving the acute inflammation in the perforation of the soft palate, which had been resistant to conventional therapies. These effects diminished over a few weeks, but the ROU were tolerable and it was not necessary to increase steroids or add another medicine for about 1 year. We suggest that a single infusion of infliximab can be considered for refractory ROU.     

Inner ear involvement in Behçet's disease

Objective

To assess cochlear involvement and hearing loss in patients with Behçet's disease (BD).

Method

Forty-two patients with BD and 24 sex and age matched healthy subjects were included in the study. pure-tone audiometry including high frequencies (250–16000 Hz) and DPOAE were performed to all participants. Results of the audiological evaluation were compared and correlation between the audiologic status and clinical manifestations of the BD were investigated.

Results

Bilateral sensorineural hearing loss was detected in 27 (64.3%) patients. Hearing thresholds were found to be higher in patients with BD at all of the frequencies except at 500 Hz when compared to control group (p < 0.05). The difference in the hearing levels tend to increase in high frequencies. Compared with control group, distortion products and SNR of the BD patients were lower in all of the tested frequencies (p < 0.05) which indicates weaker outer hair cell motility. There was no correlation between the clinical manifestations and the audiological parameters.

Conclusion

Even having hearing levels within normal limits in speech frequencies, increased hearing thresholds in high frequencies and decreased signal–noise ratios (SNR) in distortion product otoacoustic emission (DPOAE) indicate a cochlear involvement in patients with BD.     

Paroxysmal vertigo with nystagmus in children

IntroductionA pathological nystagmus is an objective sign that a patient feels vertigo. However, there have been few opportunities to observe and record pathological nystagmus during a paroxysmal vertigo attack. Furthermore, it can be difficult to obtain cooperation in pediatric patients. We present two cases of paroxysmal vertigo in children in whom we successfully recorded and analyzed their pathological nystagmus during a vertigo attack.MethodsOf a total sample of 4349 patients seen at our hospital for dizziness in the last decade, a retrospective analysis revealed that 68 were children (<15 years old; 1.6%). Of these 68 children, we successfully identified pathological nystagmus during paroxysmal vertigo in only two (2.9%).ResultsCase 1 was a 4-year-old girl. She felt vertigo the strongest when her left ear was down in the supine position. We observed and recorded her nystagmus during a vertigo attack with her mother's permission. Her positional nystagmus in the supine position was horizontal persistent apogeotropic nystagmus. Rightward nystagmus in the left-ear-down supine position was stronger than leftward nystagmus in the right-ear-down supine position. Therefore, the diagnosis was right lateral canal type of benign paroxysmal positional vertigo, of which the pathophysiology was cupulolithiasis. The other patient was an 11-year-old boy. He had a family history of migraines. His vertigo attacks occurred after onset of a severe migraine and lasted between 2 and 48 h. During an attack that we observed, he showed nystagmus, which was direction-fixed right torsional and rightward in darkness. His mother had noticed that his eyes moved abnormally and that his left eye did not shift to the left side when he looked leftward. He was old enough to clearly express his own symptoms. Other neurological examinations were normal. The diagnosis was vestibular migraine.ConclusionsWe analyzed a pathological nystagmus during paroxysmal vertigo in two children. We conclude that children can be diagnosed with a combination of careful history taking and accurate examinations of a pathological nystagmus.     

Recovery nystagmus in ménière's disease

Spontaneous nystagmus occurs during a Ménière's attack although the literature indicates that the direction can be variable. Previous observations made during the acute and recovery stages of a Ménière's attack suggested that the direction of the spontaneous nystagmus was consistent with the primary-secondary sequence of nystagmus that occurs with relatively prolonged stimulation of the normal vestibulo-oculomotor system. To evaluate this nystagmus pattern further, spontaneous nystagmus was monitored in eight patients using DC electronystagmography during an acute Ménière's attack. All showed an initial contralateral nystagmus during the acute phase of the attack with reversal to an ipsilateral (or recovery) nystagmus, as the acute symptoms subsided. Such a pattern of nystagmus occurring over a few hours is a helpful diagnostic aid, and when surgery is being considered, it provides objective evidence of the ear with active disease.     

Ménière's--an inner ear seizure.

Data of 27 signs, symptoms or findings were collected on 125 confirmed cases of Meniere's disease. Statistical association, plus the percentage prevalence of these findings were studied to perform a cluster analysis. These findings were used to obtain a statistical picture of the disease in terms of the recorded variable (signs, symptoms and findings). The major finding was the existence of a group of variables (a central core) of high percentage occurrence. They consisted of intermittent rotational vertigo, intermittent to constant nerve hearing impairment, tinnitus, pressure in the ears, positional vertigo, nausea, abnormal caloric nystagmus, headache and normal laterotorsion. The first four are the classic criteria of Meniere's disease, the remainder are additions. The average patient had 8 of the 9 conditions; 44% had all 9. A graphic multi-factor analysis of the signs, symptoms and findings suggests that these patients had a disease of a specific central origin. An alternative suggestion is that of several closely related diseases arising from adjacent central locations. No indication was found that influenza, hypertension, arteriosclerosis or diabetes were consistent precursors of Meniere's disease. Spontaneous, positional, gaze and optokinetic (tracking) nystagmus showed no demonstrable relationship to the Meniere's signs and symptoms. They showed the ordinary population prevalence except for spontaneous nystagmus which was slightly higher. The possibility of using these associations as a guide for correlation of the disease manifestations with neuroatomic tracts and areas is suggested.     

Changes in slow phase eye velocity and time constant of positional nystagmus at transform from cupulolithiasis to canalolithiasis

Changes in slow phase eye velocity (SPEV) and time constant (TC) of benign paroxysmal positional nystagmus in horizontal canal type were examined at transitional period from cupulolithiasis (apogeotropic nystagmus) into canalolithiasis (geotropic nystagmus) in two patients. SPEV and TC of positional nystagmus were tri-dimensionally analyzed. The first patient showed an apogeotropic nystagmus. Head rotation to the left in supine position induced a right-beating nystagmus with an initial SPEV of 15.3°/s and a TC of 133 s. The nystagmus then gradually declined with a TC of 31.3 s after reaching a maximum SPEV of 28.8°/s. After the nystagmus disappeared, he showed a geotropic nystagmus. The second patient showed a left-beating nystagmus with an initial SPEV of 2.5°/s and a TC of 141 s when his head was rotated to the right in supine position. The nystagmus then gradually declined with a TC of 8.05 s after reaching a maximum SPEV of 16.7°/s. After the nystagmus disappeared, he showed a geotropic nystagmus. The present findings suggested that in both patients, at the period of an increase of SPEV of the positional nystagmus with the shortening of its TC, cupulolithiasis transformed into canalolithiasis.     

Multiple positional nystagmus suggests multiple canal involvement in benign paroxysmal positional vertigo

Conclusion. Video-oculography demonstrates a higher occurrence of atypical positional nystagmus in patients with benign paroxysmal positional vertigo (BPPV). This includes anterior and horizontal canal variants and multiple positional nystagmus, suggesting combined lesions affecting several canals. Objective. To analyse the video-oculographic findings of positional tests in patients with BPPV. Material and methods. Seventy individuals with symptoms of BPPV and positional nystagmus were included in this study. The diagnosis was based on a history of brief episodes of vertigo and the presence of positional nystagmus as confirmed by video-oculographic examination during the Dix–Hallpike test, the McClure test or the head-hanging manoeuvre. Patients were treated by means of different particle repositioning manoeuvres according to the affected canal (Epley's manoeuvre for the posterior or anterior canals and Lempert's manoeuvre for the lateral canal) and the effectiveness was evaluated at 7 and 30 days. Results. Twenty-nine individuals (41.43%) presented an affected unilateral posterior canal. Fifteen patients (21.43%) presented a pure horizontal direction-changing positional nystagmus consistent with a diagnosis of horizontal canal BPPV. Twelve individuals (17.14%) presented a unilateral down-beating nystagmus, suggesting possible anterior canal BPPV. In addition, 14 patients (20%) showed multiple positional nystagmus during the examination corresponding to simultaneous multi-canal BPPV, 5 had bilateral posterior canal BPPV and 2 presented a positional down-beating nystagmus in both left and right Dix–Hallpike manoeuvres and the head-hanging manoeuvre, which is highly suggestive of anterior canal BPPV. However, seven individuals showed positional horizontal and vertical side-changing nystagmus that could not be explained by single-canal BPPV. These patients with multiple positional nystagmus showed changing patterns of positional nystagmus at follow-up.     

Changes in slow phase eye velocity and time constant of positional nystagmus at transform from cupulolithiasis to canalolithiasis

Changes in slow phase eye velocity (SPEV) and time constant (TC) of benign paroxysmal positional nystagmus in horizontal canal type were examined at transitional period from cupulolithiasis (apogeotropic nystagmus) into canalolithiasis (geotropic nystagmus) in two patients. SPEV and TC of positional nystagmus were tri-dimensionally analyzed. The first patient showed an apogeotropic nystagmus. Head rotation to the left in supine position induced a right-beating nystagmus with an initial SPEVof 15.3 degrees/s and a TC of 133 s. The nystagmus then gradually declined with a TC of 31.3 s after reaching a maximum SPEV of 28.8 degrees/s. After the nystagmus disappeared, he showed a geotropic nystagmus. The second patient showed a left-beating nystagmus with an initial SPEV of 2.5 degrees/s and a TC of 141 s when his head was rotated to the right in supine position. The nystagmus then gradually declined with a TC of 8.05 s after reaching a maximum SPEV of 16.7 degrees/s. After the nystagmus disappeared, he showed a geotropic nystagmus. The present findings suggested that in both patients, at the period of an increase of SPEV of the positional nystagmus with the shortening of its TC, cupulolithiasis transformed into canalolithiasis.     

Olfactory function and nasal manifestations of Behçet's disease

ObjectiveTo investigate the effect of Behçet's disease on olfactory function, the nasal mucosa, and nasal symptoms.MethodsA total of 30 patients with Behçet's disease and 30 healthy individuals volunteered to enroll in the study. Any condition leading to olfactory dysfunction was a criterion for exclusion. Nasal endoscopy was carried out individually, and nasal symptoms were assessed. An olfactory function assessment test defined by the Connecticut Chemosensory Clinical Research Center (CCCRC) was carried out. n-Butanol odor threshold and odor identification tests were performed, and the CCCRC test score (0: worst score; 7: best score) was calculated. The relationship between nasal findings, nasal symptoms, and olfactory function was evaluated.ResultsThe mean age of each group was the late 1930s, and demographic differences were not significant. n-Butanol threshold test scores were 5.57 ± 1.0 and 6.47 ± 0.7 out of 7 for subjects with Behçet's disease and the healthy control group, respectively. Identification test scores were 4.93 ± 1.3 and 6.15 ± 0.8 out of 7 and the mean CCCRC scores were 5.25 ± 1.0 and 6.31 ± 0.6, respectively, with significant differences (p < 0.001). Some nasal symptoms were significantly more common in patients with nasal findings, but no correlation was found between nasal findings and olfactory function.ConclusionAlthough Behçet's disease has been shown to involve the nasal cavity, its effects on olfactory function are unknown. The significant difference in CCCRC scores suggests, for the first time, that olfactory dysfunction is associated with Behçet's disease. Nasal symptoms are associated with the presence of nasal lesions, but there is no correlation between nasal findings and olfactory function.     

Periodic alternating nystagmus during caloric stimulation

Periodic alternating nystagmus (PAN) is a form of horizontal jerk nystagmus characterized by periodic reversals in direction. We report a case who exhibited transient PAN induced by caloric stimulation. The patient was a 75-year-old male. He had experienced floating sensation in January 2010. Eight months later, he was referred to our university hospital. Gaze nystagmus and positional tests revealed no nystagmus. Only weak right-beating horizontal nystagmus was observed during left Dix–Hallpike maneuver. Electronystagmography showed normal saccadic and smooth pursuit eye movements. The optokinetic nystagmus pattern test was also bilaterally normal. However, during the caloric stimulation to the right ear, at 166 s from the start of irrigation, the direction of nystagmus alternated from leftward to rightward, and thereafter this reversal of direction repeated 15 times. Magnetic resonance imaging showed no significant lesion except for chronic ischemia in the brain. The patient probably had some kind of latent lesion of impaired velocity storage and exhibited transient PAN induced by caloric stimulation. Caloric stimulation is useful and simple examination to disclose latent eye movement disorders of which velocity storage mechanism is impaired.     

Functional loss of the horizontal doll's eye reflex following unilateral vestibular lesions

The doll's eye reflex represents the vestibulo-ocular reflex (VOR) elicited by high-acceleration head rotation. After complete unilateral vestibular lesions, the ipsilateral, horizontal doll's eye reflex is replaced by a series of “catch-up” saccades. These cause permanent symptoms of blurred vision and dizziness during ipsilateral turns. We compared normal controls and patients with complete surgical lesions or canal paresis of up to 9 years duration via electronystagmography (ENG) to determine the usefulness of the doll's eye test as a diagnostic test for complete vestibular lesions. This test was found to be more sensitive in diagnosis of such lesions than head-shaking nystagmus, rotatory directional preponderance, and spontaneous nystagmus. It is also useful to document VOR function in patients in whom caloric irrigation is contraindicated.     

The membrane rupture theory of meniere's disease — is it valid?

The Membrane Rupture Theory states that the acute attack of Meniere's disease occurs when endolymph, with its high potassium ion concentration, escapes into the perilymph and surrounds the first-order neuron. Initially, this causes partial depolarization of the nerve and an increased resting discharge, followed by complete depolarization. Clinically, one would expect an initial irritative nystagmus (toward the affected side) followed by a paralytic nystagmus (toward the unaffected side). To confirm this, the guinea pig perilymphatic space was perfused with an artificial endolymph solution. The results confirmed an irritative/paralytic sequence of nystagmus that differs from the pattern of spontaneous nystagmus observed with ENG monitoring during the acute attack of Meniere's disease. Early in the attack, one observes a paralytic nystagmus followed by a secondary nystagmus beating toward the affected side (similar to an irritative nystagmus) as the patient recovers. This raises questions about the validity of the Membrane Rupture Theory.     

Periodic alternating nystagmus in Meniere's disease: the peripheral type?

Periodic alternating nystagmus (PAN) is a spontaneous horizontal nystagmus that reverses direction periodically. PAN has been reported in acquired and congenital forms. The lesion site of the acquired form of PAN has been attributed to the caudal brainstem or cerebellum. We report an extremely rare case (a 59-year-old woman) with bilateral Meniere's disease, who presented PAN. PAN in this patient was suppressed in the light. This patient had smooth pursuit and normal optokinetic nystagmus, although patients with PAN usually show saccadic pursuit and impaired optokinetic nystagmus. These findings were different features from those of the central or congenital form of PAN. It should be kept in mind that peripheral vestibular disorders such as Meniere's disease could produce PAN.     

Benign paroxysmal positional vertigo and canalith repositioning: clinical correlations

OBJECTIVE: To correlate patient response and recurrence rate after canalith repositioning to clinical presentation and cause of BPPV. STUDY DESIGN: This was a retrospective chart review. SETTING: This study was performed at a tertiary referral center. PATIENTS: The study included patients with a diagnosis of benign paroxysmal positional vertigo based on history and the presence of a positive response to the Dix-Hallpike maneuver (affected ear down) who were treated at the University of Arkansas for Medical Sciences between January 1993 and February 1997 using canalith repositioning. There were 52 patients (13 men, 39 women) with an average age of 63 years. INTERVENTION: Canalith repositioning was performed without the use of vibration, with the maneuver repeated up to three times as necessary at the initial session until vertigo and nystagmus were abolished. MAIN OUTCOME MEASURE: Initial response, defined as complete response (elimination of nystagmus and symptoms), improved response (elimination of nystagmus but with some residual symptoms), or no response (continued nystagmus and symptoms), as well as recurrence rate. RESULTS: Initial complete response was seen in 66% of patients, and 33% showed improved response. This difference was believed to be caused by the pathophysiology. Recurrence rates appeared to be correlated to cause, with higher rates reflecting ongoing inner ear injury, such as with Meniere disease. CONCLUSIONS: Canalith repositioning was effective in 99% of patients based on elimination of nystagmus. Partial responders probably experienced resolution naturally over time because of a difference in pathophysiology. Higher recurrence rates can be expected when the cause is thought to involve an ongoing process.     

Persistent Direction-Fixed Nystagmus Following Canalith Repositioning Maneuver for Horizontal Canal BPPV: A Case of Canalith Jam

The authors report a 64-year-old man who developed persistent direction fixed nystagmus after a canalith repositioning maneuver for horizontal canal benign paroxysmal positional vertigo (HC-BPPV). The patient was initially diagnosed with right HC-BPPV given that the Dix-Hallpike test showed geotropic horizontal nystagmus that was more pronounced on the right side, although the roll test did not show any positional nystagmus. The patient was treated with a canalith repositioning maneuver (Lempert maneuver). The next day, the patient experienced a different character of dizziness, and left-beating spontaneous nystagmus regardless of head position was observed. After a forced prolonged left decubitus and frequent head shaking, his symptoms and nystagmus resolved. This condition, referred to as canalith jam, can be a complication after the repositioning maneuver in patients with BPPV. Atypical positional tests suggest that abnormal canal anatomy could be the underlying cause of canalith jam.     

Vestibular disorders in childhood and adolescents

The aim and objective of the study is to analyze the data of 145 vertiginous patients aged up to 18 years, which were examined at the Otoneurological Department of the Semmelweis University during the last 5 years. The study design for the analysis: the group of children (66 patients, aged up to 14 years) and group of adolescents (79 patients, ages between 14 and 18 years). Children and adolescents, who sent by otolaryngologists or pediatric neurologists, were examined. The detailed case history and routine physical examinations were followed by cochlear function tests. The vestibular test contained statokinetic test, spontaneous nystagmus with electronystagmographical (ENG) registration, positional and positioning nystagmus with Frenzel’s glasses, smooth pursuit eye movement test and caloric test with computer-based ENG. Most of the patients experienced true rotatory vertigo. The ratio of the dizziness (vertigo without direction) was 38% in the children’s and 44% in the adolescent group; 21 children and 31 adolescents had headache. Nausea and vomiting were frequent in both the groups: normal hearing was measured in 62% of children and in 82% of adolescents. The vestibular system was normal in 36% of the children’s group, and in 39% of the adolescents. In conclusion, it is important to realize that 64% of the children and 61% of the adolescents do have certain vestibular abnormalities. The most frequent cause of dizziness (24%) in the adolescent group is the extravestibular disorder-like panic disease and the anxiety disorder. At younger age, the most frequent disease that can cause vestibular symptoms is the migraine.     

An unusual complication of cochlear implant: benign paroxysmal positional vertigo

Three days after the initial fitting of the cochlear device a 40-year-old woman complained of severe rotational vertigo following head movements associated with neurovegetative symptoms. Otoneurological evaluation revealed a horizontal paroxysmal positional nystagmus beating towards the lowermost ear, induced by rolling the patient's head from supine both to the right or to the left lateral position suggesting the diagnosis of benign paroxysmal positional vertigo of the left horizontal semicircular canal. The nystagmus characteristics were the same whether the cochlear device was switched on or off. The hypothesis of an otolith dislodging due to the electrical stimulation during the initial fitting is discussed.     

Evaluation of cochlear involvement by distortion product otoacoustic emission in Behçet's disease

Objective

The aim of this study was to investigate cochlear involvement in patients with Behçet's disease.

Materials and methods

Twenty-six Behçet's disease patients (52 ears) and 24 sex and age-matched healthy control subjects (48 ears) were included in the study. Pure-tone audiometry at frequencies 250, 500, 1000, 2000, 4000, 6000 Hz, immittance measures including tympanometry and acoustic reflex testing and DPOAE (distortion product otoacoustic emission) testing were performed in the patients and controls.

Results

A sensorineural hearing loss was found in eight patients (30.7%) as it was bilateral in five and unilateral in three patients. Although no typical audiometric configuration was found, one patient had a flat type audiogram, and the others had a high frequency hearing loss. The DPOAE responses of the patients and controls were significantly different in all frequencies (p < 0.05).

Conclusion

These results indicate that cochlea is affected by damage of outer hair cells in Behçet's disease.