Nystagmus intensity and direction in bow and lean test: an aid to diagnosis of lateral semicircular canal benign paroxysmal positional vertigo.

The objective was to evaluate nystagmus intensity and direction (NID) during bow and lean test (BLT) in subjects suffering from idiopathic lateral semicircular canal benign paroxysmal positional vertigo (LSC-BPPV), in order to differentiate between the geotropic and the apogeotropic form and to determine the affected ear before using classic diagnostic procedures. The BLT was performed in 32 subjects affected by LSC-BPPV. "Nystagmus intensity" evaluation allows distinguishing the geotropic variant from the apogeotropic one, while the "nystagmus direction" allows identification of the side. In particular, a more intense nystagmus in the bow position compared to the lean position indicates an ampullipetal flow caused by the presence of free-floating particles in the non-ampullary arm, and is suggestive of geotropic form. In this case, if the nystagmus in the bow position is left beating, the free-floating particles necessarily occupy the left LSC non-ampullary arm, while a right-beating nystagmus indicates the right LSC involvement. In contrast, a more intense nystagmus in the lean position compared to the bow position indicates an ampullifugal flow due to the presence of particles adherent to the cupula (cupulolithiasis) or free-floating in the ampullary arm (canalolithiasis), suggesting an apogeotropic form. In this situation, if the nystagmus in the lean position is left beating, the particles are in the left LSC ampullar arm or are coated on the left LSC cupula; vice versa, a right-beating nystagmus in the lean position is suggestive of the involvement of the right LSC. As a general rule, in both forms the direction of the more intense nystagmus points to the affected side. "NID-BLT" was effective in identifying the form and the side in 22/28 subjects (79% of the study population). The proper execution and interpretation of the "NID-BLT" helps to establish the form (geotropic versus apogeotropic) and side (right versus left) in most cases of LSC-BPPV. Unlike Choung's test, which requires knowing a priori if the form is geotropic or apogeotropic, our test enables fast and accurate diagnosis, or at least provides indispensable elements if the diagnosis of the affected side is doubtful, with the patient remaining in the sitting position.

Cervico-oculo-Acoustic syndrome in a male with consanguineous parents.

BACKGROUND: The cervico-oculo-acoustic syndrome comprises Klippel-Feil anomaly, sensorineural deafness and Duane's retraction syndrome. Polygenic, autosomal dominant, and X-linked inheritance have been hypothesized. The disorder has rarely been reported in males. CASE REPORT: A 42-year-old male, born of consanguineous parents, presented with Duane's syndrome, mixed hearing loss, C2-C3 fusion, neck stiffness, and right facial palsy. A variety of cardiac, neurological and urogenital anomalies occurred in his relatives. The electro-oculographic studies showed impaired abduction and adduction of the right eye and impaired abduction of the left eye. Vergence, vertical eye movements and peripheral vestibular responses were normal. Somatosensory evoked potentials showed absence of the N13 peak and brainstem auditory evoked potentials bilateral delay of the I-III interpeak latencies. CONCLUSIONS: Consanguinity of the patient's parents, not previously reported, suggests autosomal recessive inheritance, but autosomal dominant inheritance is indicated by the family history. The pattern of the oculomotor deficit is consistent with bilateral dysplasia of the abducens nuclei with preserved internuclear neurons in the right abducens nucleus. Neurophysiological investigations revealed lower brainstem and cervical cord involvement.

Maxillary ameloblastoma.

Maxillary ameloblastoma is a rare odontogenic neoplasm that is histologically benign and originates from epithelial cells present in bone tissue. If excised through conservative surgery, this tumour has a high relapse rate and is locally aggressive. The risk, in particularly extensive forms, that the ameloblastoma will invade extra-maxillary structures such as the orbit, the pterygomaxillary fossa, the infratemporal fossa and the base of the skull, means that surgical treatment is difficult if it is to be oncologically radical while respecting function and aesthetics. Thus, in these cases a complete and in-depth diagnostic work-up and careful planning of surgical treatment are needed: surgery entails an ablative phase with en-bloc resection of the neoformation to margins free of neoplastic infiltration, and a reconstruction phase that, within a short time-frame, will re-establish functionality and provide a good aesthetic result. Our experience in treating 2 cases of maxillary ameloblastoma is reported.

Changes in clinical and instrumental vestibular parameters following acute exposition to auditory stress.

Besides Tullio's phenomenon, resulting from anatomic changes in the labyrinth, a hypersensitivity to acoustic stimuli of the saccular structures appears to be the underlying cause of the vestibular responses detected in some patients. In order to evaluate the incidence of vestibular symptoms triggered by acute exposure to auditory stress (disco music), 40 subjects aged between 18 and 26 years, with no audiological and vestibular disorders, were submitted to otoneurologic tests. Subjects were exposed to disco music [intensity 128 dB (C)], for 3 hours. Tests have been carried out before and immediately after exposure. Canalar and macular functions have been evaluated using vestibular investigation techniques and vestibular evoked myogenic potentials. When compared to baseline data, post-exposure test results did not reveal any canalar damage. Pre- and post-exposure recordings of the vestibular-oculomotor reflex threshold have shown no significant changes. Conversely, post-stimulus recordings have shown a significant increase in the amplitude of the vestibular evoked myogenic potential response, thus indicating a possible irritative involvement of the macular receptor. This result suggests a direct action upon the receptor by acoustic stimulation which could, therefore, be the underlying cause of vestibular symptoms reported by patients following exposure to sufficiently intense acoustic stimuli. Prior to this study. a questionnaire concerning the relationship between habitual disco visiting and audio-vestibular symptoms has been completed by 310 students at the University of Catanzaro. This survey revealed a significant incidence of vestibular symptoms due to acoustic stress (Tullio's phenomenon) which led us to hypothesise that balance disorders due to auditory stress are much more frequent than commonly held, particularly since, in many cases, diagnoses is unknown or not easy due to the difficult procedures by which these conditions are diagnosed.

Giant cervico-mediastinal lipoma. A clinical case.

The lipoma is a circumscribed mesenchymal tumour originating from adipose tissue. The lesion is usually small and asymptomatic, and is most frequently located in the neck region. The case of a 77-year-old woman with chronic extrasystolic arrhythmia caused by a non-specified ischemic cardiopathy is reported. The woman presented a swelling at the front of her neck, observed for the first time about 6 months previously. This swelling progressively increased in size, provoking dysphagia, dysphonia, persistent cough, dyspnea, light jugular turgor and palpitations. Chest X-rays showed and opaque area at the front of the neck, which extended beyond the jugular incisure by about 2 cm. NMR of the neck showed a gross lipomatous formation at the front, mainly of the left, continuing in the front mediastinal region; the trachea was dislocated to the right and compressed at the back; the vasculo-nervous fasciculus, especially on the left, was compressed and enveloped by the adipose formation. The Holter test confirmed the presence of ventricular and supra-ventricular extrasystoles. Surgery was carried out under local anaesthesia because the displacement of the laryngo-tracheal axes precluded intubation. Histological analysis of the 9 x 4 x 2.2 cm mass confirmed the diagnosis of lipoma. After removal of the mass all the symptoms, which had been provoked by compression, as well as the cardiac arrhythmias disappeared. The prompt disappearance of the latter was particularly surprising. The possibility of the external compression of the nervous structures of the neck should be taken into consideration in cases of ventricular arrhythmia of unknown origin, and systematic study of the region carried out.

Middle ear involvement in children with chronic rheumatoid juvenile arthritis.

In a selected sample of patients affected by juvenile rheumatoid arthritis (JRA) little is known about middle ear involvement, even though many synovial joints are affected. Multifrequency tympanometry was used to measure admittance, conductance, susceptance and phase angle at different probe frequencies and resonant frequencies. In all, 35 children with JRA and a control group (30 children) were studied. Findings showed that mean resonant frequency values in all children with JRA were greater than in the control children. The multifrequency tympanometry parameters measured in acute JRA subjects are not different from parameters of remission JRA subjects except for a change in the phase angle. The changes found are due to involvement of the incudomalleolar and incudostapedial joints.

Type 2 autoimmune hepatitis and hepatitis C viraemia.

We present 10 Italian patients with type 2b autoimmune hepatitis (anti-LKMI positivity) and HCV infection. 6 patients had IgG concentrations above the upper limit of normal and all had histological features of chronic autoimmune hepatitis or chronic persistent hepatitis or cirrhosis. ANA and SMA were positive in 2 patients, pANCA in 3 patients. Anti-GOR were negative in all patients, 6 of them were HLA B8 DR3 and 2 HLA B8 DR4. Antibodies to HCV (tested by ELISA 2nd and 3rd generation) were positive in all patients and in 9 subjects were detected HCV RNA. The two patients with positivity for ANA and SMA were treated successfully with corticosteroids, but they relapsed after the drug withdrawal; the others received interferon, that had to be suspended in 2 patients because inducing an autoimmune thyroiditis. Although, at present, it is still not known if HCV is a really trigger factor in developing autoimmunity or if the two diseases are coincidental, the authors suggest that it is important for clinicians to use appropriate treatment strategies on the basis of the predominant illness.