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PubMed1981

Abnormalities of the stapedius reflex in patients with vertigo.

Analysis of the latency, amplitude, and rise characteristics of the stapedius reflex as demonstrated on a strip recorder revealed abnormalities in patients with vestibular neuronitis and other vertigo syndromes. The findings in vestibular neuronitis, acute labyrinthitis, perilymphatic fistulas, Meniere's disease, cerebrovascular insufficiency, and metabolic vertigo are described. Alterations in the stapedius reflex included increase in latency, decrease in amplitude, stepping of the rise, and absence of the reflex. This study suggests that absence of the reflex can be associated with vertigo as well as with acoustic neuromas and other retrocochlear lesions. The findings of abnormalities in vestibular neuronitis support a brain-stem localization as the site of lesion in this disease.

Cerebrovascular Disorders↗
PubMed1990

Audiological findings of prolonged Menière's disease.

As Ménière's disease progresses, the fluctuations becomes less marked and the hearing level gradually declines. In the more established and advanced stages of the disease, a permanent and progressive hearing loss develops. It has been reported, however, that hearing acuity in some patients with Ménière's disease significantly improved after having had the disorder for many years. Such observation suggests that the inner ear pathology in late stages of Ménière's disease may not be necessarily irreversible. The purpose of this investigation was to assess whether or not various audiological evaluations including EcochG may be of value in predicting the irreversibility of hearing deficit in patients suffering from advanced stages of Ménière's disease. The audiological studies included pure tone audiogram, SISI test, Bekesy audiometry, glycerol (or urea) test, speech discrimination test, and electrocochleography. A combination of the recruiting type of response in the EcochG and the narrowing of the tracing width of continuous sound in the Bekesy pattern seemed to be indicative for irreversible hair cell damages. Patients who had a low score in the speech discrimination test as well as an absence of ABR response were suspected of having retrocochlear lesions. Our investigations suggest that the irreversibility of hearing impairment in patients with later stages of Ménière's disease may be predicted to some extent in the use of various audiological evaluations including the EcochG.

Adult↗
PubMed1979

Critical bandwidth in patients with acoustic neuroma.

Eleven patients with verified acoustic neuroma had critical band estimation performed by the method of loudness summation using noise bands centered around 1 kHz. The normal loudness difference between broad band noise and narrow band noise was reduced at all levels except the highest. Judged as single individuals, 9 of the 11 patients had a normal critical band. The pooled data indicated a normal critical band, both in patients with hearing loss less than 50 dB HL and in patients with hearing loss greater than or equal to 50 dB HL. The results are similar to those obtained in patients with Ménière's disease (Bonding, 1978c) and thus CB-measurements cannot be utilized for differentiating between cochlear and retrocochlear lesions. However, at the highest test levels applied the loudness difference between broad band noise and narrow band noise appeared to have some correlation to the presence or absence of recruitment.

Adult↗
PubMed2005

Rare auditory-electophysiology finding in Wilson's disease.

Wilson's disease is a rare genetic disease involving the malabsorption of copper by the body. The most common characteristic sign is the presence of Kayser-Fleischner ring surrounding the cornea. Other systemic and motor signs have been documented as well as MRI changes within the brain and brainstem. This rare case illustrates the potential importance of audiometric assessment for patients with Wilson's disease who complain of hearing loss, tinnitus and intra-aural pressure. Unilateral findings were significant for retrocochlear neural transmission delays.

Adult↗
PubMed1980

The acoustic reflex latency test: clinical application.

Acoustic reflex latency is the time interval between onset of an intense auditory stimulus and onset of middle-ear muscle contraction. The hypothesis was proposed that a retrocochlear lesion involving the afferent (VIIIth nerve) portion of the reflex arc should result in a prolongation of the reflex latency. A clinical procedure, the Acoustic Reflex Latency Test (ARLT), its protocol, methods and equipment were developed in order to test this hypothesis clinically. Absolute latency values and interaural latency differences (ILD) were established on 4 populations which included normals, cochlear lesions due to Ménière's disease, cochlear lesions of variable etiology, and 11 cases of surgically confirmed acoustic tumor. Results reveal a dramatic prolongation of latency in the presence of retrocochlear lesions (all tumors). The test was found to be most effective when 1. both ipsilateral and contralateral measurements were made, 2. stimulus frequencies of 100 Hz and 2000 Hz were employed and 3. both Absolute Latency and ILD were considered. The ARLT, while utilizing an indirect method of measurement, is a valid, cost-effective, and simple clinical procedure, yielding relative latency values and requiring only minor modifications of standard impedance equipment. The ARLT appears to have a higher efficiency rate than BERA, while providing comparable diagnostic information at a considerable saving of time, cost and personnel.

Acoustic Impedance Tests↗
PubMed1995

Neurologic features of chronic Minamata disease (organic mercury poisoning) certified at autopsy.

To better understand the neurologic events related to chronic Minamata disease (organic mercury poisoning), we studied data from 77 patients with Minamata disease as certified at autopsies performed from 1976 to 1994 (mean age: 72.3 years). Major neurologic findings included: sensory impairment in 80.5% of the patients which was limited to the extremities in 42.9%. Impairment of lower extremity coordination was present in 35.8% of the patients, constriction of the visual fields in 28.8%, and retrocochlear hearing loss in 15.3%. There was no correlation between the degree of cerebellar incoordination and the methylmercury concentration in the cerebellum. Compared with the classic type of Minamata disease, the incidence of major neurologic findings was markedly decreased. In light of these findings, supplemental examinations including brain computed tomography (CT), magnetic resonance imaging (MRI), short latency somatosensory evoked potential (SSEP), or tremogram may be necessary to clinically diagnose Minamata disease, especially in atypical or mild cases.

Adult↗
PubMed1976

Clinical study of chronic arachnoiditis in the posterior fossa.

A series of 12 cases of chronic arachnoiditis in the posterior fossa is reported. The diagnosis in six patients was established at suboccipital craniectomy whch brought about marked improvement in symptoms. The other six were diagnosed as having arachnoiditis in the posterior fossa from the facts that improvement in clinical symptoms was achieved by lumbar air insufflation, which has often been said to be effective in arachnoiditis, and no abnormal findings were revealed by pneumoencephalo-roulette tomography. By investigating the clinical features of these cases, we came to the following conclusions: many cases of this disease have had head trauma or chronic inflammatory disease in the past; the symptoms are apt to occur abruptly, and display repeated remission and aggravation; symptoms originating in the cranial nerves are diversified, and are often bilaterial; peripheral vestibular disturbances and retrocochlear hearing losses are observed on neuro-otological examination. In the caloric test especially the patients show canal paresis (CP) on the more impaired side, but none of them appeared to be nonresponsive to the test; lumbar air insufflation is often an effective treatment for this disease; pneumoencephalo-roulette tomography is very useful for diagnosing pathological changes in the posterior fossa.

Adult↗
PubMed1979

Brain stem evoked response audiometry in a clinical practice.

Brain stem evoked response audiometry (BERA) is an effective method of evaluating the auditory pathway from the peripheral endorgan through the brain stem. The authors have found this test to be a valuable adjunct to the neurotologic evaluation and have applied the study to over 500 patients (639 ears) in a 22 month time span. BERA has an excellent success rate (98%) in determining the presence of cerebellopontine angle lesions and is helpful in establishing whether a hearing loss is of cochlear or retrocochlear type. The authors have found BERA of use in the diagnosis of multiple sclerosis as well as other neurologic diseases affecting the brain stem. It is a valid method of dealing with suspected malingerers and those who cannot respond to standard audiometric techniques. BERA has been helpful in determining the threshold of hearing in these patients. The authors conclude that BERA is a clinical study of practical significance and that more otolaryngologists should consider adding it to their diagnostic protocol.

Adolescent↗
PubMed1990

Scanning electron microscopy and immunoglobulins of the endolymphatic sac in normal human subjects and sensorineural deafness. With special reference to Menière's disease.

The pathophysiology of the endolymphatic sac (ES) in Meniere's disease was studied by scanning electron microscopy and staining of immunoglobulins in the intradural portion of the ES. The peroxidase-antiperoxidase method by means of paraffin sections was used for staining of immunoglobulins. First, subjects without hearing impairment and malformation of the temporal bone, ranging from a 7-month-old fetus to an 80-year-old adult, were investigated. All subjects, including fetuses, showed well-arranged epithelial cells by scanning electron microscopy. The epithelial cells in the proximal portion of the intradural ES were oval, showing a tendency of transitional change to be flat as they drew near the distal portion of the ES. The epithelial cells consisted mostly of light cells, but sporadic dark cells were seen. Regarding the immunoglobulins. IgG was slightly positive in the epithelial and subepithelial layers. All 15 patients with Meniere's disease showed various types of degeneration of the epithelial cells though to varying degrees. However, these findings were also seen in cases of cochlear deafness. On the other hand, the ES of acoustic tumors, with retrocochlear or neural deafness revealed a normal finding, as found in healthy subjects. Inner ear deafness experimentally produced in animals by Kanamycin sulfate (KM) injection showed degeneration of the epithelial cells of the ES similar to that found in human cochlear deafness. IgG of the ES in Meniere's disease showed moderately evident deposits compared to normal subjects. However, this was also found not only in inner ear deafness other than Meniere's disease, but also in animal deafness experimentally produced by KM injection. It is very interesting to note that moderate endolymphatic hydrops was found in animals one year after Preyer's reflex had disappeared. It is postulated that endolymphatic hydrops develop because of impairment of endolymphatic fluid resorption at the rugose portion and stenosis of the lumen in the same portion, due to degeneration of the epithelial cells. From the above results, it is argued that degenerated epithelial cells and immunoglobulins of the ES in Meniere's disease may arise from the sequelae of cochlear deafness. It is also hypothesized that endolymphatic hydrops--at least in the terminal stage of Meniere's disease--may be consistent with the same pathophysiological conditions as in animal experiments.

Adult↗
PubMed1992

Labyrinthine enhancement on gadolinium-enhanced magnetic resonance imaging in sudden deafness and vertigo: correlation with audiologic and electronystagmographic studies.

Sudden deafness with or without vertigo presents a difficult diagnostic problem. This article describes 12 patients with enhancement of the cochlea and/or vestibule on gadolinium-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (MRI), correlating the enhancement with the auditory and vestibular function. All patients were studied with T2-weighted axial images taken through the whole brain, enhanced 3-mm axial T1-weighted images taken through the temporal bone, and enhanced T1-weighted sagittal images taken through the whole brain. Cochlear enhancement on the side of hearing loss was found in all the patients. The vestibular enhancement correlated with both subjective vestibular symptoms and objective measures of vestibular function on electronystagmography. In 2 patients, the resolution of symptoms 4 to 6 months later correlated with resolution of the enhancement on MRI. No labyrinthine enhancement was seen in a series of 30 control patients studied with the same MRI protocol. Labyrinthine enhancement in patients with auditory and vestibular symptoms is a new finding and is indicative of labyrinthine disease. While abnormalities on electronystagmograms and audiograms are nonspecific and only indicate a sensorineural problem, enhanced MRI may separate patients with retrocochlear lesions, such as acoustic neuromas, from those in whom the abnormal process is in the labyrinth or the brain.

Adult↗
PubMed2000

Audiometric characterization of a family with digenic autosomal, dominant, progressive sensorineural hearing loss.

In this study, a non-syndromic progressive bilateral high frequency hearing loss is described in a family with 141 identified members. Recent genetic analyses indicated a digenic inheritance with linkage to the gene loci DFNA2 and DFNA12. The affected family members who shared haplotypes at both loci (type I) showed an early postlingual onset and a more rapid rate of progress compared with those with one either of the two disease associated haplotypes (type II). The audiometric pattern was cochlear without a vestibular involvement. Auditory brainstem response audiometry and magnetic resonance imaging indicated normal retrocochlear features. The otoacoustic emissions were affected for both type I and type II, whereas the acoustic stapedius reflex thresholds were normal in most cases. It is concluded that both types had an outer hair cell/micro-mechanical abnormality, but that the DFNA 2 type might have an additional dysfunction at the level of the inner hair cells. It is furthermore pointed out that the application of refined audiometric techniques as well as a further development of new techniques is needed in order to characterize the phenotypes of the rapidly expanding number of genetically defined inner ear abnormalities.

Adult↗
PubMed1988

Controversy in menière's disease: results of microvascular decompression of the eighth nerve.

We have identified a group of patients with classic symptoms of Meniere's disease, all of whom had abnormalities of brain stem auditory evoked potentials and middle ear acoustic reflex response that strongly indicated auditory nerve and brain stem involvement. Ten patients with disabling symptoms and retrocochlear audiologic signs were selected to undergo microvascular decompression of the eighth nerve at its exit from the brain stem (root exit zone [REZ]). In all 10 patients, the eighth nerve was found to be significantly compressed by one or several vessels near its REZ. One patient died 4 months after the operation from an unrelated cause but had been free of symptoms of Meniere's disease until her death; the remaining nine patients have all been free from vertigo for 8 to 48 months after the operation. None of the patients suffered any complications as a result of the surgical procedure.

Adult↗