Search

Objectives:: The purpose of this study was to investigate the correlation between grades of endolymphatic hydrops (ELH) and clinical characteristics and determine the detailed clinical characteristics of Ménière's disease (MD) patients with evidence of hydrops based on magnetic resonance imaging (MRI)., Methods:: One hundred ninety-eight MD patients (396 ears) with MRI evidence of hydrops were included. ELH grades were evaluated using the Nakashima grading standard. Correlations between the extent of ELH and clinical features were evaluated. Detailed clinical characteristics were analyzed to assess the clinical diagnostic criteria., Results:: Of 198 patients, ELH was observed in 100% of cases on the clinically affected side and 8.6% of cases on the asymptomatic side. In addition, 98.5% of ELH was classified as moderate or significant grade. Low-frequency hearing loss was significantly correlated with the extent of both vestibular and cochlear hydrops, whereas the vertigo attack frequency showed no significant correlation with ELH grades. The disease duration of MD with bilateral ELH was longer than that with unilateral ELH. The clinical characteristics were variant and did not completely fit the proposed diagnostic criteria., Conclusions:: MRI findings have relevance to the clinical severity, to a certain extent, but not vestibular symptoms. The proposed diagnostic criteria based on clinical characteristics may be partially effective; analysis of the detailed clinical characteristics of MD was meaningful. Diagnosis of MD based on both MRI and clinical symptoms could facilitate an early diagnosis.

Objectives: To describe a case of inadvertent hearing preservation following a classical translabyrinthine resection of a vestibular schwannoma of the internal auditory canal in an otherwise healthy patient., Methods: Herein, we describe the case of an otherwise healthy patient who underwent resection of an intracanalicular vestibular schwannoma via a translabyrinthine approach. Furthermore, as part of an ongoing study aimed at characterizing hearing changes due to intraoperative events, cochlear hair cell and nerve activity were monitored using electrocochleography throughout surgery. Unexpectedly, the patient maintained serviceable hearing following surgery. As a result, we are able to provide electrophysiologic evidence of cochlear hair cell activity at various stages of this surgery., Results: Hair cell responses across tested frequencies were detectable prior to and following completion of the translabyrinthine procedure. Neural integrity of the auditory division of cranial nerve VIII was maintained throughout. Lastly, postoperative audiometric testing supported the patient's subjective assertion of serviceable hearing in the surgical ear., Conclusion: Our results suggest that some degree of hair cell and neural integrity can be maintained throughout the course of the translabyrinthine approach, and if preservation of the auditory division of cranial nerve VIII is feasible, a functional amount of hearing preservation is attainable.

Objectives: This study was aimed to elucidate the diagnostic significance of the summating potential (SP)-action potential (AP) ratio and the AP latency difference between condensation and rarefaction clicks (AP con-rar difference) in Meniere's disease., Methods: The AP and SP were recorded transtympanically in 67 patients with definite Meniere's disease. The SP/AP ratio and the AP con-rar difference were assessed in terms of 1) their interrelationship, 2) their relationship to hearing level, and 3) the rate of occurrence of abnormal values according to the stages of Meniere's disease., Results: No correlation was found between the SP/AP ratio and the AP con-rar difference. Neither the SP/AP ratio in general nor the AP con-rar difference was correlated with the hearing level. However, enhanced values of the SP/AP ratio (0.35 or higher) were moderately correlated with the hearing level (r = 0.51), and their occurrence rate was 55.2%. An increased AP con-rar difference (0.13 ms or longer) was not correlated with the hearing level, and its occurrence rate was 50.2%; it appeared most frequently at stage 3 (p <0.05)., Conclusions: An enhanced SP/AP ratio might not always indicate the presence of endolymphatic hydrops associated with an increase in endolymphatic pressure. An increased AP con-rar difference might reflect the presence of a biased basilar membrane resulting from an increased endolymphatic pressure, and hence it is diagnostically essential to simultaneously evaluate the SP/AP ratio and the AP con-rar difference.

Sudden deafness constitutes a diagnostic challenge. Classically, 2 causes, viral and vascular, are considered in the origin of idiopathic sudden hearing loss. More recently added to the list of possibilities are rupture of the membranous labyrinth and immune-mediated sensorineural hearing loss. The latter can be either primary and localized to the inner ear or, in perhaps fewer than one third of cases, secondary to generalized systemic autoimmune disease. The purpose of the present review is to define immune-mediated sudden sensorineural hearing loss as a distinctive entity, on the basis of clinical, immunologic, and pathological findings, and suggest a profile of the typical patient.

The test-retest reliability of the asymmetry of the Unterberger stepping test was obtained over a 3-mo. period. This stepping test is a test examining the labyrinthine deviation when testing the lower extremities. Also, self-report items were validated against individual measures of the stepping test. The investigations show high reliability of the asymmetry of the stepping test. The data indicate further that a questionnaire is a valid method for the assessment of the asymmetry of the stepping test.

The influence of handedness on a measure of the vestibulospinal response was investigated with 25 right-handed and 25 left-handed subjects to examine the asymmetry of the stepping test. Right-handed subjects showed dominance of the right side indicated by direction of movement, but left-handed subjects tended much less to the left side so a functional asymmetry is for left-handers somewhat less clearly expressed.

Relationships between middle ear pressure and non-infection-related cochleovestibular dysfunction have been suggested by several authors. According to some data, vertiginous attacks can be prevented by the insertion of a ventilation tube in patients suffering from Meniere's syndrome. The aim of our study was to investigate if the incidence of eustachian tube malfunction and pathologic middle ear pressure is frequent, and if routine implantation of ventilation tubes is reasonable in ears with dysfunctions of the labyrinth, including clinical Meniere's syndrome. So, we determined in our pressure chamber all active and passive parameters of eustachian tube function in 40 patients suffering from Meniere's syndrome, sudden sensory hearing impairment (SSHI), or vestibular neuronitis. Our results disclosed no nonrandom incidence of impaired tubal function among our patients compared to healthy control subjects. Pressure equalization was sufficient in most patients suffering from clinical Meniere's syndrome, and only one patient with vestibular neuronitis presented with a patulous tube. Our results show that impairment of vestibular or cochlear function is not regularly accompanied by eustachian tube dysfunction. Furthermore, no patient reported symptoms while pressure variation was performed. We conclude that variation of middle ear pressure does not usually play a role in the genesis of Meniere's syndrome, vestibular neuronitis, or SSHI. Thus, from our data, we cannot recommend routine implantation of tympanic ventilation tubes in patients suffering from Meniere's syndrome, vestibular neuronitis, or sudden hearing loss.

In order to determine the characteristics and incidence of hearing loss in Behçet's disease, 72 consecutive cases and 72 sex- and age-matched normal subjects were submitted to this study. Detailed audiologic tests were performed in all cases. Twenty patients (27%) showed some degree of hearing loss; but in only 7 patients (9%) was the average of the frequencies between 500 to 4,000 Hz more than 25 dB hearing level, and the cochlear function of 43 patients (59%) was within the 25-dB range in all frequencies. The averaged pure tone audiograms of the two groups showed a statistically significant hearing loss in the Behçet's group. No relationship could be found between hearing loss and other system involvements. There was no correlation between hearing loss and duration of the disease, but the mean age of the Behçet's patients with hearing loss was found to be significantly higher than the mean age of the patients without hearing loss.

A 44-year-old man with a documented 12-year history of progressive sensorineural hearing loss developed a generalized tonic-clonic seizure followed by a visual field deficit and apraxia. Six months later he developed a peripheral neuropathy and muscle fatigue followed by a slowly progressive aphasia and cortical blindness as well as increased seizure activity. A computed tomography scan showed bilateral basal ganglion calcification. The serum lactate level was elevated at 3.4 mEq/dL. A muscle biopsy enabled the diagnosis of mitochondrial myopathy. This disorder is presented as an unusual cause of progressive sensorineural hearing loss in adults.

Two experiments were carried out to investigate how the correspondence between sentence accentuation and distribution of information is used in human word processing. A forced-choice task with target words embedded in sentences was employed for this purpose. Target words provided either 'given' or 'new' information, and were either accented or unaccented. The subjects had to choose between two words that differed in the last consonant by one phonetic feature (e.g., mouth/mouse). The first experiment involved both normal-hearing and hearing-impaired listeners. A fixed amount of noise was used to reduce the quality of speech for normal-hearing listeners, in order to enable a comparison between the two listener groups. The results of the first experiment showed different processing patterns for normal-hearing and hearing-impaired listeners. The hearing-impaired listeners were more accurate with words that were properly accented for their information value, whereas the normal-hearing subjects were more accurate with accented than unaccented words regardless of their information value. A new group of normal-hearing subjects was tested in a second experiment with speech of more severely reduced quality. The results indicated that, under these circumstances, the normal-hearing listeners changed their strategy and also showed an interaction between information value and accent. It seems that, as speech becomes less intelligible, listeners depend increasingly on linguistic expectations stemming from the correlation between information value and accentuation.

There is a scant literature regarding vestibular evaluation of children with complaints of dizziness or vertigo. Considerable time and effort are expended on the problem and prevention of hearing loss in children, yet we often ignore concurrent or subsequent vestibular disorders. This neglect could be due to several factors, perhaps the most common being the fact that vertiginous crises in childhood are often attributed to problems of behavior or incoordination. In this article, we offer an approach to the dizzy child based on presenting symptoms. We discuss features of the history, examination, and laboratory evaluation key to determining the cause of dizziness. Finally, we discuss management, which varies according to the diagnosis.

A newly developed technique (endolymphatic sac ballooning surgery [ESBS]), based on revisional sac surgery observations, was conducted on 135 patients with intractable Meniere's disease. The surgery involves the insertion and fan-folding of a thin Silastic sheet in the endolymphatic sac (ES) lumen to increase its volume. The results of this sac ballooning surgery proved more effective for treatment of incapacitating Meniere's disease symptom complex than did primary ES surgery (ESS) with either the nonballooning sac techniques we employed previously or ESBS revision operations in which primary surgery used nonexpansion techniques. It appears that ESBS, with its preservation of ES integrity and expansion of its lumen, may indeed prevent surgically induced damage and be more efficacious in the drainage of hydropic endolymph. The efficacy of ESS remains controversial because of the failure to understand its pathophysiologic basis. Nevertheless, we are confident that if the ES is definitively delineated, as evidenced by turbid or clear fluid or bubbles when entered, and enlargement of its lumen with maintenance of the sac's integrity is verified during surgery, ESS is undoubtedly efficacious treatment for intractable Meniere's disease. Furthermore, we maintain that equitable judgment may only be rendered when reports evaluating the efficacy of ESS include definitive identification, delineation, and drainage of the sac.

The efficacy of adenoidectomy in the surgical treatment of children with otitis media with effusion (OME) persisting after adequate medical therapy has been established in three independent randomized clinical trials. Although each of these studies used a different experimental design, all showed significant reductions in morbidity from OME after adenoidectomy as compared to the control groups. Subsequent application of these findings in formulating clinical guidelines for the use of adenoidectomy has yet to be realized, and recommendations to parents for or against the procedure appear to vary more with the surgeon's philosophy than with the condition of the child. This discussion examines the effectiveness and cost of adenoidectomy for the treatment of children with chronic OME and addresses the question of whether adenoidectomy should be used as a primary or a secondary surgical therapy. The argument is made to use adenoidectomy as a primary therapy in selected cases on the basis of patient age, type of OME, and patient preference, and to base the decision not on the size of the adenoid, but on its known pathophysiology.

Tetra-ataxiametric posturography is based on the measurement and computerized elaboration of electronic signals emitted by four footplates, one for each heel and toe, respectively. These are sensitive to vertical pressure produced by a subject standing straight but in various positions (feet parallel, in tandem, eyes closed, on pads, etc.). The method yields additional parameters not obtained by the traditional monoplate stabilometers, namely, weight-distribution patterns and correlations among six combinations of paired outputs from the two heels, two toes, heel/toe of each foot, and the two diagonals (tetra-ataxiametric synchronisations). Comparing age-matched learning disabled, mentally retarded, autistic, and hearing impaired (with and without labyrinthine hypofunction) with normal children, significant and clinically meaningful differences were detected between the tetra-ataxiametric measures of stability, interaction between Fourier Spectral Power Ranges of body sway, weight distributions, and synchronisations of toe parts. The same parameters correlated significantly with cognitive school readiness in normal populations. While the stability and spectral quotients show significant developmental changes, weight distribution and toe synchronisations are stable from 5 years onwards. The method is suitable for young subjects and attractive to children who may ordinarily be reluctant to cooperate, such as the autistic ones. The equipment is portable and tests can be conveniently carried out in a child's familiar educational setting.

Most of the previous literature concerning otologic problems in compressed gas environments has emphasized middle ear barotrauma. With recent increases in commercial, military, and sport diving to deeper depths, inner ear disturbances during these exposures have been noted more frequently. Studies of inner ear physiology and pathology during diving indicate that the causes and treatment of these problems differ depending upon the phase and type of diving. Humans exposed to simulated depths of up to 305 meters without barotrauma or decompression sickness develop transient, conductive hearing losses with no audiometric evidence of cochlear dysfunction. Transient vertigo and nystagmus during diving have been noted with caloric stimulation, resulting from the unequal entry of cold water into the external auditory canals, and with asymmetric middle ear pressure equilibration during ascent and descent (alternobaric vertigo). Equilibrium disturbances noted with nitrogen narcosis, oxygen toxicity, hypercarbia, or hypoxia appear primarily related to the effects of these conditions upon the central nervous system and not to specific vestibular end-organ dysfunction. Compression of humans in helium-oxygen at depths greater than 152.4 meters results in transient symptoms of tremor, dizziness, and nausea plus decrements in postural equilibrium and psychomotor performance, the high pressure nervous syndrome. Vestibular function studies during these conditions indicate that these problems are due to central dysfunction and not to vestibular end-organ dysfunction. Persistent inner ear injuries have been noted during several phases of diving: 1) Such injuries during compression (inner ear barotrauma) have been related to round window ruptures occurring with straining, or a Valsalva's maneuver during inadequate middle ear pressure equilibration. Divers who develop cochlear and/or vestibular symptoms during shallow diving in which decompression sickness is unlikely or during compression in deeper diving, should be placed on bed rest with head elevation and avoidance of maneuvers which result in increased cerebrospinal fluid and intralabyrinthine pressure. With no improvement in symptoms after 48 hours, exploratory tympanotomy and repair of a possible labyrinthine window fistula should be considered. Recompression therapy is contraindicated in these cases...

A group of dyslexic students was examined by a central auditory test (CAT) battery including competing sentences, binaural fusion, filtered speech, and compressed speech. Auditory evoked brainstem responses (BSER) were measured in conjunction with the CAT data. The auditory tests indicate a high degree of failure in those areas requiring sophisticated integration, coordination, and identification of the modified speech stimuli. The BSER did not identify a significant abnormality. The cortical and subcortical areas of integration are proposed as the multicentric sites of impairment in the hearing function found in dyslexic students.

The individual effects of an influenza B viral infection, aspirin, and an arginine-deficient diet on the inner ear were assessed in the ferret model for Reye's syndrome using both functional and morphological parameters. Auditory brainstem evoked responses recorded from inoculated ferrets revealed threshold elevations and increased latencies during the first 72 hours, but approximated those of control animals by 96 hours. Although there was a mild distention of Reissner's membrane, no pronounced structural alterations in sensory or supporting cells were observed in cochleas from inoculated ferrets. The administration of aspirin appeared to alter neither the functional nor the structural integrity of the cochlea. The presentation of an arginine-deficient diet, creating a hyperammonemic condition, led to both altered auditory evoked responses and vacuolization of cochlear tissues after treated animals had undergone seizures and coma. These data demonstrated that both influenza B and the arginine-deficient diet individually affected the hearing of treated animals. The individual agents did not alter the cochlea as severely as when they were presented in combination. These results suggest that hearing impairment in patients with Reye's syndrome may be a result of potentiation of certain metabolic-altering agents.

Vestibulo-ocular reflex (VOR) gain and the ability of gaze fixation were investigated in patients with unilateral loss of labyrinthine function during the time course of compensation (four days to 6 months). A marked directional difference in the VOR gain in the acute stage (0.51 to 0.73 at four days) decreased proportionately to the logarithm of time on rotations to the affected side and to the intact side by an increase and a slight decrease of the gain, respectively. Gaze disturbance, which occurred only on rotations to the affected side, recovered in parallel with the decrease of directional difference in the VOR gain; it was still found at 1 Hz at 6 months. Recovery of gaze disturbance in unilateral lesions seems to be accomplished not only by the change in the VOR gain, but also by intensified amplification of this gain to make up for a low VOR gain.

Responses of 135 patients subjected to Sinusoidal Harmonic Acceleration Labyrinthine Test are reported. These patients are categorized into four clinical impression disease groups: 1) classic Menière's disease; 2) labyrinthine ischemia; 3) neurological diseases; 4) no labyrinthine or neurological disease. A case report in each category is presented. We find Sinusoidal Harmonic Acceleration Labyrinthine Test to be a useful tool in evaluation of the dizzy patient.

Fistulization at various parts of the membranous labyrinth has been currently in use as one of the treatments for the symptoms of Meniere's disease. The present report concerns our experience with membranous fistulae in experimental animal ears with and without manifestation of endolymphatic hydrops. The materials presented are mostly new; however, our previous data are also incorporated in order to bring this issue into better perspective. The first part describes the nonproduction aspect of endolymphatic hydrops in the rat, chinchilla, and squirrel monkey after obliteration of the endolymphatic duct in relation to the occurrence of spontaneous fistulae. The second part describes surgical fistulization of the vestibular membranous labyrinth and cochlear duct in order to prevent or control development of endolymphatic hydrops in the guinea pig, the species in which hydrops can be produced consistently. These results are tabulated and analyzed in terms of the pathological consequences that can be expected in performing fistulization of the membranous labyrinths in animals as well as in humans.

Vestibular function testing employing caloric stimulation has been of limited value in the evaluation of patients with Menière's disease, with its cardinal shortcoming being poor repeatability. This modality has not been useful in determining appropriate treatment for these individuals. We have used low frequency harmonic acceleration to study patients with vestibular pathology and have noted a high level of repeatability of responses to this type of stimulation. This study was designed to determine if the clinical status of patients with Menière's disease could be correlated with their responses to harmonic acceleration. Thirty-six patients with typical symptoms of Menière's disease were evaluated and divided into four groups based on severity of symptoms. Analysis of variance of the phase shifts in asymmetry (or preponderance) showed a significant difference between the groups. Responses to this type of stimulation correlated with the clinical status of the patients to a significant degree. These data indicate that harmonic acceleration can be of value to the otologist in the evaluation of patients with Menière's disease.

Histologic examination of the temporal bone of a patient with Wegener's granulomatosis showed involvement of the vessels of the endolymphatic sac (ELS) but no involvement of the vasculature of the rest of the inner ear. Autoimmune sensorineural hearing loss in this patient may have been due to interference of antigens and antibodies with the filtering capacity of the ELS, leading to imbalance of inner ear fluids. Histologic findings reemphasize that the cochlea and ELS have different blood supplies.