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PubMed2000

Vestibular evoked myogenic potentials in patients with spinocerebellar degeneration.

To estimate vestibulo-collic reflexes in patients with spinocerebellar degeneration (SCD), vestibular evoked myogenic potentials (VEMPs) were recorded in 16 patients with SCD and in 9 normal subjects. The patients with SCD were classified into three groups: those with olivo-ponto-cerebellar ataxia (OPCA; n = 10); those with cortical cerebellar atrophy (CCA; n = 3); and those with Machado-Joseph disease (MJD; n = 3). While all of the patients with OPCA and CCA showed biphasic responses (p13-n23), 2 of the 3 patients with MJD showed abnormal VEMPs bilaterally. Three sides in these 2 patients showed an absence of VEMPs, and one side showed a remarkably delayed p13. These two patients showed little response in caloric tests. These results suggest that the vestibulo-collic reflex as well as the vestibulo-ocular reflex could be damaged in MJD patients but preserved in OPCA and CCA patients.

Adult↗
PubMed2002

Three-dimensional analysis of human locomotion in normal subjects and patients with vestibular deficiency.

The purpose of this study was to clarify the role of the vestibular system in human locomotion. The subjects were nine healthy controls, nine patients with unilateral vestibular deficiency (UVD) and nine patients with bilateral vestibular deficiency (BVD). The UVD subjects were Ménière's disease patients who were being treated with administration of gentamicin into the tympanic cavity. BVD subjects were hearing-impaired individuals who showed no response to the ice-water caloric test. A total of 13 markers were attached to the head, trunk (C7), hip and foot in order to measure translational and rotational motions with the aid of a video image processing system. All subjects were instructed to restrict their stride length to approximately 80 cm while walking on a treadmill and watching a visual target. However, walking speed varied depending on the ability of the subject to maintain body equilibrium. The results showed that walking speed and step frequency were significantly lower for the UVD and BVD groups than for the normal group. Analysis of head movements in the sagittal plane showed a counteracting motion between pitch rotations and vertical translation as previously reported. We also found head counteracting motions between yaw rotation and lateral translation in the horizontal plane. These mechanisms are thought to help stabilize the gaze during walking. When the head fixation point was calculated by projecting the naso-occipital axis line during walking, the head counteracting motion was found to assist the vestibulo-ocular reflex in stabilizing the gaze. In addition, normal subjects seemed to use head stabilization as a space strategy in order to minimize head yaw movement. In contrast, UVD and BVD subjects adopted head stabilization as a trunk strategy.

Case-Control Studies↗
PubMed2003

Nystagmus-sensation dissociation in spinocerebellar degeneration.

OBJECTIVE: To study nystagmus-sensation dissociation (NSD), i.e. caloric nystagmus without the sensation of vertigo, in patients with spinocerebellar degeneration (SCD). MATERIAL AND METHODS: The neuro-otological and neuro-radiological records of 179 patients clinically diagnosed as having SCD (91 males, 88 females; age range 20-89 years) and 48 patients diagnosed as having peripheral vestibular disorders (15 males, 33 females; age range 21-80 years) were reviewed. Subjects underwent caloric tests using cold water (20 degrees C) in a totally dark room. Immediately after each test session, subjects were asked if they had experienced spinning or moving sensations during the test. Maximum slow phase eye velocities (SPEVs) were measured using electronystagmography. RESULTS: Among patients with peripheral vestibular disorders, all patients with SPEVs > 15 degrees/s experienced spinning or moving sensations during testing. Among the 179 patients with SCD, 21 (39 sides) showed maximum SPEVs > 15 degrees/s without spinning or moving sensations. In particular, 8 patients (10 sides) had maximum SPEVs > 25 degrees/s without spinning or moving sensations. Common lesions in the cerebral cortex could not be detected using either MRI or single-photon emission CT. CONCLUSION: Patients with SCD may also have NSD, which may be attributable to subcortical lesions in the ascending pathways to the vestibular cortex.

Adult↗
PubMed2005

Lesion site in idiopathic bilateral vestibulopathy: a galvanic vestibular-evoked myogenic potential study.

CONCLUSION: The result suggests that patients with idiopathic bilateral vestibulopathy may have nerve lesions when the inferior nerve system is affected, while the inferior vestibular nerve system may be spared. OBJECTIVE: To clarify the lesion site in idiopathic bilateral vestibulopathy, an acquired bilateral vestibulopathy of unknown cause. MATERIAL AND METHODS: Two 75-year-old males diagnosed with idiopathic bilateral vestibulopathy were enrolled. Both showed absent or highly decreased responses on the caloric test on both sides. They underwent vestibular-evoked myogenic potential (VEMP) testing by means of acoustical and electrical stimulation. As acoustic stimulation, 95 dB nHL clicks and short tone bursts (500 Hz) were presented, while 3 mA (1 ms) short-duration galvanic stimuli were presented as electrical stimulation. Responses were recorded on the sternocleidomastoid muscles. RESULTS: Both patients showed unilateral absence of VEMPs with both acoustic and short-duration galvanic stimuli.

Acoustic Stimulation↗
PubMed1987

Peripheral labyrinthine causes of dizziness.

A patient's complaint of dizziness must be defined specifically. Many dizzy patients do not have labyrinthine or balance system disease. The patient with dysequilibrium, on the other hand, often has a balance system disease but not necessarily a labyrinthine disorder. The patient with vertigo most likely has labyrinthine disease. Vertigo is accompanied by nystagmus, which can be identified only when the examiner specifically looks for it. Although vertigo is the classic symptom of labyrinthine disease, not all labyrinthine diseases have associated vertigo. Careful history taking, physical examination, audiometry, caloric testing, electronystagmography, and radiographic studies will identify patients with labyrinthine disorders. Treatment depends on the diagnosis. Usually it is medical, but occasionally it may be surgical. Most patients have no residual problems; a few have permanent disability.

Acute Disease↗
PubMed2002

Phenotype of DFNA11: a nonsyndromic hearing loss caused by a myosin VIIA mutation.

OBJECTIVES/HYPOTHESIS: To characterize the audiovestibular phenotype of DFNA11, an autosomal dominant nonsyndromic hearing impairment caused by a mutation in the myosin VIIA gene (MYO7A), including whether DFNA11-affected subjects have retinal degeneration as is characteristic of Usher syndrome type 1B, caused by different MYO7A mutations. STUDY DESIGN: Retrospective study of audiovestibular and ophthalmological data in a Japanese family linked to DFNA11. METHODS: Otoscopic examination and pure-tone audiometry were performed in all participants in the family. Selected subjects underwent additional examinations including speech discrimination scoring, acoustic reflex measurements, Békésy audiometry, evoked and distortion-product otoacoustic emissions, auditory brainstem responses, and bithermal caloric testing; visual acuity, ocular tonometry, slit-lamp examination, ophthalmoscopy, and electroretinography; and computed tomography of the temporal bone. RESULTS: Most affected individuals had moderate cochlear hearing loss beginning in the second decade and progressing at all frequencies. Variable degrees of asymptomatic vestibular dysfunction were present. Computed tomography showed normal inner and middle ear structures. No evidence suggested retinitis pigmentosa. CONCLUSIONS: The phenotype of DFNA11 is postlingual, nonsyndromic sensorineural hearing loss with gradual progression. Showing moderate hearing loss with asymptomatic variable vestibular dysfunction and no retinal degeneration, the DFNA11 phenotype is mildest among phenotypes caused by MYO7A mutations.

Adaptor Proteins, Signal Transducing↗
PubMed2003

Familial auditory neuropathy.

OBJECTIVES/HYPOTHESIS: Auditory neuropathy is a sensorineural hearing disorder characterized by absent or abnormal auditory brainstem responses and normal cochlear outer hair cell function as measured by otoacoustic emission recordings. Many risk factors are thought to be involved in its etiology and pathophysiology. Four Chinese pedigrees with familial auditory neuropathy were presented to demonstrate involvement of genetic factors in the etiology of auditory neuropathy. STUDY DESIGN: Probands of the above-mentioned pedigrees, who had been diagnosed with auditory neuropathy, were evaluated and followed in the Department of Otolaryngology-Head and Neck Surgery, China People Liberation Army General Hospital (Beijing, China). Their family members were studied, and the pedigree maps established. METHODS: History of illness, physical examination, pure-tone audiometry, acoustic reflex, auditory brainstem responses, and transient evoked and distortion-product otoacoustic emissions were obtained from members of these families. Some subjects received vestibular caloric testing, computed tomography scan of the temporal bone, and electrocardiography to exclude other possible neuropathic disorders. RESULTS: In most affected patients, hearing loss of various degrees and speech discrimination difficulties started at 10 to 16 years of age. Their audiological evaluation showed absence of acoustic reflex and auditory brainstem responses. As expected in auditory neuropathy, these patients exhibited near-normal cochlear outer hair cell function as shown in distortion product otoacoustic emission recordings. Pure-tone audiometry revealed hearing loss ranging from mild to profound in these patients. Different inheritance patterns were observed in the four families. In Pedigree I, 7 male patients were identified among 43 family members, exhibiting an X-linked recessive pattern. Affected brothers were found in Pedigrees II and III, whereas in pedigree IV, two sisters were affected. All the patients were otherwise normal without evidence of peripheral neuropathy at the time of writing. CONCLUSION: Patients with characteristics of nonsyndromic hereditary auditory neuropathy were identified in one large and three smaller Chinese families. Pedigree analysis suggested an X-linked, recessive hereditary pattern in one pedigree and autosomal recessive inheritances in the other three pedigrees. The phenotypes in the study were typical of auditory neuropathy; they were transmitted in different inheritance patterns, indicating clinical and genetic heterogeneity of this disorder. The observed inheritance and clinical audiological findings are different from those previously described for nonsyndromic low-frequency sensorineural hearing loss. This information should facilitate future molecular linkage analyses and positional cloning for the relative genes contributing to auditory neuropathy.

Adolescent↗
PubMed2004

Intratympanic gentamicin therapy for Ménière's disease: a meta-analysis.

OBJECTIVE: This study compared the effectiveness of five different techniques of intratympanic gentamicin administration for Ménière's disease. DATA SOURCES: A MEDLINE search of the English language literature from 1978 to 2002 was performed using the key words "intratympanic," "gentamicin," "therapy," "Ménière's," and "disease." STUDY SELECTION: Inclusion criteria to select articles for meta-analysis were clear description of gentamicin delivery technique, clearly reported vertigo control results, and report of hearing loss posttreatment. Seven studies (n = 218) describing the multiple daily dosing technique (delivery three times per day for >or=4 d), two studies (n = 84) describing the weekly dosing technique (weekly injections for four total doses), eight studies (n = 253) of the low-dose technique (one to two injections with retreatment for recurrent vertigo), four studies (n =156) of continuous microcatheter delivery, and six studies (n =269) of the titration technique (daily or weekly doses until onset of vestibular symptoms, change in vertigo, or hearing loss) were entered into the model. DATA EXTRACTION: Vertigo control results were stratified into complete, substantial, or poor control. Hearing results were separated by profound, partial, or no hearing loss. Individuals undergoing caloric testing were separated by degree of vestibular ablation (complete versus partial) and analyzed for vertigo control (n = 301) and hearing loss (n = 333) after treatment. DATA SYNTHESIS: Comparisons between the rates of complete vertigo control, effective vertigo control (complete plus substantial control), overall hearing loss (partial plus profound), and profound hearing loss by delivery method were based on a parametric empirical Bayes analysis using binomial generalized linear models and backward variable selection (joining). Relative risk for vertigo control and hearing loss by partial or complete ablation was examined study by study using residual maximum likelihood to carry out a parametric empirical Bayes analysis. CONCLUSION: The titration method of gentamicin delivery demonstrated significantly better complete (81.7%, p = 0.001) and effective (96.3%, p < 0.05) vertigo control compared with other methods. The low-dose method of delivery demonstrated significantly worse complete vertigo control (66.7%, p < 0.001) and trends toward worse effective vertigo control (86.8%, p = 0.05) compared with other methods. The weekly method of delivery trends toward less overall hearing loss (13.1%, p = 0.08), and the multiple daily method demonstrated significantly more overall hearing loss (34.7%, p < 0.01) compared with other groups. No significant difference in profound hearing loss was found between groups. Degree of vestibular ablation after gentamicin therapy is not significantly correlated with the resulting vertigo control or hearing loss status.

Anti-Bacterial Agents↗
PubMed2003

Auditory and vestibular findings in Fabry disease: a study of hemizygous males and heterozygous females.

AIM: This study aimed to evaluate audiological and vestibular involvement in Fabry disease and the early effects of enzyme replacement therapy with human alpha-galactosidase A. METHODS: Fourteen patients (10 males, 4 females) aged 14-57 years were studied. Each patient underwent a clinical (history of otological and vestibular aspects, otoscopy) and instrumental (pure tone and speech audiometry, impedance, auditory brainstem response and oto-acoustic emission recordings, vestibular caloric tests, electronystagmography during acceleratory stimulation, dynamic posturography) evaluation before starting enzyme replacement therapy. RESULTS: Fifty per cent of patients complained of hearing symptoms (hearing loss, tinnitus, ear fullness). Subjective hearing loss was present in six cases and in three cases it was the first reported symptom of Fabry disease. In six of the seven cases the onset and/or progression of hearing symptoms were sudden. Vertigo or dizziness was reported by four patients and in two cases was associated with hearing symptoms. Audiological evaluation showed sensorineural hearing loss in eight patients (5 males, 3 females). Hearing loss was unilateral in six cases and bilateral in the remaining two cases. The hearing loss (HL) ranged from 30 to 80 dB HL (mean, 43 dB HL) and the lesion was always cochlear. Vestibular examination showed abnormalities in four patients (bilateral weak/abolished response in three cases, side prevalence in one case), which were not related to either the audiological results or the history of vertigo/dizziness. CONCLUSION: Involvement of the inner ear is common in men and women with Fabry disease. We found a high incidence of cochlear hearing loss, which was typically unilateral and showed onset and/or progression by sudden episodes. Vascular or hydropic mechanisms could be hypothesized to explain audiological findings. Vestibular involvement had a lower incidence and showed a different pattern, thus suggesting that several pathophysiological mechanisms could play a role in determining inner ear damage in Fabry disease. Our preliminary results show that enzyme replacement therapy may stabilize hearing function; however, further follow-up is required.

Adolescent↗
PubMed1991

CRF triggers the CNS release of TRH in stress-induced changes in gastric emptying.

Stress elicited by exposure to cold induces an increase of gastric emptying (GE) and intestinal transit of a caloric meal in mice and the release of corticotropin-releasing factor (CRF) and thyrotropin-releasing hormone (TRH) in the central nervous system (CNS). The present study proposed 1) to compare in mice the central effects of TRH, CRF, and cold-exposure stress on GE and intestinal transit of a caloric test meal consisting of 0.5 ml of reconstituted milk marked with 51Cr-labeled sodium chromate, and 2) using CRF and TRH antisera to determine whether TRH and CRF act in cascade or independently. The intracerebroventricular (icv) injection of TRH (0.5 microgram/kg) and CRF (1 microgram/kg), as well as cold stress, significantly increased GE, whereas 10-fold higher doses injected intraperitoneally were ineffective. The effect of cold stress on GE was abolished by prior icv injection of both CRF and TRH antisera. The effect of TRH was not blocked by CRF antiserum, but TRH antiserum suppressed the increase in GE induced by CRF. Moreover, both CRF and TRH antisera abolished changes in the rate of GE induced by exogenous CRF and TRH injection, respectively, therefore demonstrating the specific efficiency of immunoneutralization. CRF and cold stress both induced an increase in the rate of intestinal transit, while TRH had no effect. Antibodies to CRF prevented the intestinal stimulatory effect induced by CRF and cold stress. In contrast, antibodies to TRH were unable to antagonize either CRF or cold-stress induced increase in the rate of intestinal transit.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1977

Benign paroxysmal positional vertigo and rotatory-induced nystagmus.

The study of 25 cases of 'benign paroxysmal position vertigo' showed us that irrigation of the ear opposed to the injured side inhibited this nystagmus. In these patients, it is difficult, not to say impossible, to provoke rotatory nystagmus, either by the caloric test (Brünings position II) or by the rotatory test in frontal plane. The significance of these observation is discussed and ascribed to a central compensation system.

Eye Movements↗
PubMed2002

Audiological assessment in Ramsay Hunt syndrome.

Ramsay Hunt syndrome is known to cause symptoms and signs of vestibulocochlear dysfunction, including sensorineural hearing loss. The present study investigates the audiological features of a group of 15 patients with this syndrome. A complete otolaryngological, neurologic, and audiological workup was performed in every patient, including auditory brain stem response measurements and recording of transiently evoked otoacoustic emissions. In most patients, some degree of hearing loss was evident, and abnormal latencies and interpeak latencies of the auditory brain stem response, or even absence of the waves, were observed. Transiently evoked otoacoustic emissions were present in only 6 cases, and caloric tests showed unilateral weakness in the majority of the patients. In all of the performed tests, abnormalities were present only on the affected side. The audiological data suggested cochlear or retrocochlear involvement or involvement at more than one site along the auditory pathway.

Adolescent↗
PubMedDate not supplied

Familial dysautonomia (Riley-Day syndrome). Temporal bone findings and otolaryngological manifestations.

Familial dysautonomia, or Riley-Day syndrome, is inherited in an autosomal recessive fashion and occurs almost exclusively in Jewish families. This disorder is characterized by a smooth tongue devoid of fungiform papillae and of taste buds, and is clinically associated with poor taste discrimination. An unsteady gait and dizziness on change in position are also common presenting symptoms. This study reports the histopathological findings of eight temporal bones from four patients with documented familial dysautonomia. For the control series, 13 normal temporal bones were also studied. The most striking finding in the dysautonomic patients was an extreme paucity of geniculate ganglion cells (P less than 0.001). A statistically significant reduction in the number of neurons was also found both in the superior and in the inferior divisions of the vestibular nerve (P less than 0.001). The paucity of the geniculate ganglion cells correlates well with the impairment of the taste in dysautonomic individuals, since the afferent fibers leaving taste buds of the anterior two-thirds of the tongue run via the chorda tympani and have their cell bodies in the geniculate ganglion. Furthermore, the reduction in the number of Scarpa's ganglion cells observed in the dysautonomic patients studied here could account for a poor response to caloric test, positional vertigo and an unsteady gait in this condition.

Adolescent↗
PubMedDate not supplied

Implications of Ewald's second law for diagnosis of unilateral labyrinthine paralysis.

The feasibility of replacement of the caloric test with sinusoidal rotatory stimulations of differing frequencies was investigated in ten normal subjects and seven patients with unilateral labyrinthine paralysis. Testing was performed at frequencies ranging from 0.0125 to 0.2 Hz at 60 degrees/sec and at 0.05 Hz at peak velocities of 30 degrees/sec to 180 degrees/sec. Gain, phase, and asymmetry of the nystagmic responses were measured in these groups. Results indicated that the rotatory test was not reliable enough to be used as the sole measure for identifying patients with unilateral labyrinthine lesions.

Analog-Digital Conversion↗
PubMed1984

Comparison of symptoms and laboratory findings in unilateral vestibular dysfunction.

Although vertigo is the expected symptom of unilateral vestibular disease, exceptions have been observed. To determine the relative frequency of this observation, a retrospective study of patients seen over a 1-year period was done. Of the 126 patients found to have unilateral labyrinthine impairment with bithermal caloric testing, only 58 (46%) characterized their symptoms as vertigo. The ramifications of these findings are discussed, and a question is raised as to the propriety of reserving vestibular testing for only those patients describing vertigo.

Adolescent↗
PubMed1989

Transmastoid labyrinthectomy: reliable surgical management of vertigo.

Transmastoid labyrinthectomy has continued to be an important part of the surgical armamentarium for patients with vertigo and nonserviceable hearing loss. Continuing experience substantiates our earlier impression that the vestibular system usually accommodates rapidly to complete unilateral surgical ablation, regardless of age or degree of residual vestibular activity in the ear (as measured by preoperative bithermal caloric testing). Although the symptom of vertigo is reliably treated by transmastoid labyrinthectomy, a patient questionnaire has demonstrated a significant incidence of mild to moderate persisting postoperative dysequilibrium. Although this dysequilibrium is usually not debilitating, this questionnaire has demonstrated its existence more precisely than a retrospective review of the patients' clinical records. This study reviews 110 patients who underwent labyrinthectomy between 1978 and 1985. We remain impressed at the efficacy of the transmastoid labyrinthectomy in relieving the symptom of vertigo.

Adult↗
PubMed1995

Sensorineural hearing loss with delayed onset of vertigo.

Sensorineural hearing loss with delayed onset of vertigo is a syndrome in which episodic vertigo arises in a person who has preexisting unilateral severe-to-profound sensorineural hearing loss. This syndrome has an ipsilateral form in which the vertigo arises from the poorer hearing ear and a contralateral form in which the aural symptoms arise from the better hearing ear. The existence of this syndrome has only been noted within the past two decades. This report details our clinical experience with 17 persons with the ipsilateral form of this disorder. The onset of the vertigo after the occurrence of the hearing loss was quite variable. It ranged from 1 to 60 years after the development of the hearing loss. The hearing loss occurred for several reasons. Most patients had hearing loss due to an unknown cause. The development of the vertigo and the timing of the onset of the vertigo were not related to the cause of the hearing loss. Bithermal caloric testing identified the offending labyrinth in most patients who underwent ablative vestibular surgery. Ablative vestibular surgery was performed in 13 of the 17 persons in this study because of disabling symptoms. In all 13 cases, the episodic vertigo was eliminated. Surgical treatment for sensorineural hearing loss with delayed-onset vertigo, as with all surgery for vertigo, should be based on the severity of the afflicted person's symptoms.

Adult↗
PubMed2004

Assessment of child neurology outpatients with headache, dizziness, and fainting.

Neurologic symptoms such as headache, vertigo, dizziness, and fainting can create a diagnostic problem in pediatric neurology practice because they are also the most common presenting symptoms of psychiatric disorders. Children, especially adolescents, who are often admitted with such autonomic symptoms, are frequently misdiagnosed. In this study, we aimed to investigate the psychiatric morbidity and comorbidity rate in children and adolescents presenting with neurologic symptoms such as headache, vertigo, and syncope. We investigated 31 children who presented with these symptoms. All children were evaluated for their medical history and had a physical and neurologic examination. We attempted to rule out a possible organic etiology. All patients received a complete laboratory examination (blood count, electroencephalography), pediatric cardiology and otorhinolaryngology consultations, and a caloric test. All patients were assessed according to Diagnostic and Statistical Manual of Mental Disorders-IV (DSM-IV) criteria. The majority of the patients (93.5%) received a psychiatric diagnosis according to the DSM-IV criteria. Most of these patients were adolescents and female. Psychosocial stressors such as academic problems, familial dysfunction, parental psychopathology, and child sexual abuse were associated with somatic symptoms. The results of this study demonstrated the importance of differential diagnosis and psychiatric comorbidity in a pediatric neurologic outpatient population. Treatment should be directed at biopsychosocial integrity, and a multidisciplinary treatment approach should be applied.

Adolescent↗