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PubMed1979

Performance intensity functions in cochlear and eighth nerve disorders.

In this report, performance intensity functions for monosyllabic materials (PI-PB) were obtained on two groups of subject: 20 patients with eighth nerve pathology and 15 individuals with Meniere's disease. The subjects with retrocochlear involvement exhibited marked deterioration in word recognition as the speech intensity was increased beyond the level required for a maximum discrimination score. Such a dramatic breakdown, referred to as the "rollover effect," was not seen to occur among the subjects with cochlear disorders. Hence, the PI-PB function appears to be a valuable tool for use in the special test battery.

Adolescent↗
PubMed2002

[Idiopathic sudden deafness].

PURPOSE: Sudden idiopathic deafness is a sensorineural hearing loss with no recognized causes at the time of onset. The impairment site is usually localized in the cochlea, but some cases of retrocochlear lesions (e.g., cerebellopontine angle tumors, degenerative neural diseases, neuraxial ischemic lesions) can induce sensorineural deafness. The medical management of patients presenting with sudden deafness aims at detecting a causal mechanism, and at administering emergency therapeutic drugs. The diagnosis of idiopathic sudden deafness can be definitely made when no causes are found. Usually, the impairing mechanism involves the cochlea. The pathophysiology of this sensorineural alteration is still unknown. It is most likely that several mechanisms are associated together, their common point being an impairment to the feedback loop of the organ of Corti. CURRENT KNOWLEDGE AND KEY POINTS: It is very likely that reactivation of neurotropic viruses and/or cochlear ischemia are frequent etiologies. Whatever the cause, the treatment is to be administered urgently, and consists of a high-dose corticotherapy at the least. Other treatments have never really proven to be effective. It is secondarily checked that no retrocochlear pathological processes, such as a cerebellopontine angle tumor, is present, in particular in young people. FUTURE PROSPECTS AND PROJECTS: One of the current objectives is to determine when cochlear ischemia is involved, in a mini-invasive manner, such as with laser Doppler flowmetry, so that the treatment can be optimized. From a therapeutic point of view, early acoustic protection has been proven to be effective in cases of cochlear ischemia in small laboratory animals. Its efficacy in case of sudden deafness, non-exclusive of other causes than ischemia, is being assessed in a multicentric project.

Adrenal Cortex Hormones↗
PubMed1991

[Changes in early auditory evoked potentials in acoustic neuroma].

The auditory brain-stem responses (ABRs) of 38 patients with acoustic neuroma (AN) were recorded pre-operatively and evaluated with regard to pathological deviations from the normal. Several qualitative and quantitative features of the ABRs were compared statistically with the results obtained from a control group for age and sex, and considering patients with various cochlear pathologies (Meniere's disease, sudden deafness, presbycusis). Retrocochlear disorders were ruled out by computed tomography or magnetic resonance imaging. Statistical analysis yielded the following results. The central conduction time (latency difference between Jewett waves J1 and J5) is significantly prolonged in the AN group. A critical value of 4.3 ms separates the retrocochlear from the cochlear lesions, with a sensitivity and specificity of 90%. If conductive hearing loss can be excluded, and if the amount of cochlear hearing loss is taken into account, about the same separation effectively is achieved by evaluation of the sole latency of J5 and its stimulus level dependence. The interaural difference of the central conduction time can be a useful parameter for indicating the presence of acoustic tumours, unless both sides are affected. The same is true of the interaural differences for the J5 latency, but this quantity must be corrected for the effects of peripheral hearing loss. The critical interaural difference values are 0.3 ms for the conduction time (at any stimulus level) and 0.5 ms for the latency of J5 (for stimulus levels above 60 dB nHL). A weaker distinction is provided by the amplitude of J5 and the amplitude ratio of J5 and J1. These quantities are only slightly smaller in cases of AN. Only the combination of small J5 amplitudes with large latency values can be regarded as being characteristic of retrocochlear lesions. This can be highlighted in an amplitude-latency diagram. Further hints as to the site of the lesion can be obtained from a distorted waveform morphology and from an abnormally large distance between hearing threshold and response threshold. The combined evaluation of all these features optimizes the effectivity of ABRs as a powerful tool in the diagnosis of retrocochlear disorders, but some false-positive and false-negative results cannot be excluded entirely.

Adolescent↗
PubMed1996

[The analysis of abnormal auditory brainstem responses].

The abnormal auditory brainstem responses (ABR) of 56 cases who had different retrocochlear disorders were analysed. These disease included acoustic neuroma, glioma, cholesteatoma, astrocytoma, meningoma in cerebellopontile angle (CPA), chromaffin tumor of jugular foramen, intracranial metastatic tumor, demyelinating disease, multiple cranial nerve paralysis, hepatolenticular degeneration, other CPA tumors, expansion or stenosis of internal auditory meatus (IAM), and so on. The ABR features were varied in these diseases, even in same disease. In general, the wave latencies and interwave periods prolonged in the disorders which occupied place in CPA, the differentiation of wave was not clear and/or the wave was not well repeated in the disorders which spreaded all over the brain. The cause of abnormal ABRin which CT and/or MRI were normal was also analysed. The ABR detection is a sensitive indication in retrocochlear lesions.

Cerebellar Neoplasms↗
PubMed1988

Computed tomography and magnetic resonance imaging of the inner ear.

The majority of temporal bone radiographic studies are obtained either for middle ear and mastoid disease or in the evaluation of retrocochlear pathology. With recent technologic advances, diagnostic imaging of the inner ear has developed an increasing role in the evaluation and management of diseases that affect the cochlea, semicircular canals, and the vestibular and cochlear aqueducts. High-resolution computed tomography (CT) provides excellent detail of the osseous labyrinth, whereas magnetic resonance imaging (MRI) generates images derived from the membranous labyrinth and its associated neural elements. Optimal techniques for obtaining high quality CT and MRI images of the normal and diseased inner ear are presented. CT has proved useful in the evaluation of inner ear malformations, cochlear otosclerosis, labyrinthine fistulization from cholesteatoma, translabyrinthine fractures, otic capsule osteodystrophies, in the assessment of cochlear patency before cochlear implantation, and in the localization of prosthetic devices such as stapes wires and cochlear implants. While MRI produces discernible images of the soft tissue and fluid components of the inner ear, it has yet to demonstrate any unique advantages in the evaluation of inner ear disease. However, MRI produces excellent and highly useful images of the audiovestibular and facial nerves, cerebellopontine angle, and brain.

Cholesteatoma↗
PubMed1994

Spectral analysis of auditory brain stem responses in perceptive hearing loss.

The Fourier analysis of auditory brain stem responses in 45 patients aged 19 to 56 treated because of perceptive hearing loss in Cracow ORL Clinic was carried out. The group consisted of: 15 patients with sudden deafness, 15 patients with Menier's disease and 15 cases with retrocochlear hearing impairment. In cases with recruiting cochlear hearing loss considerable and significantly extension of frequency range in brain stem responses was observed. Moreover, responses induced by stimulus intensity causing recruitment are identical for both ears as far as their shape and power spectrum is concerned, despite wide variety of responses caused by stimulus of different intensity. It is assumable that Fourier analysis of auditory brain stem responses gives possibility of objective evaluation of subjectively determined recruitment syndrome.

Acoustic Stimulation↗
PubMed2002

Demyelination of vestibular nerve axons in unilateral Ménière's disease.

We conducted a study to determine whether vestibular nerves in patients with unilateral Ménière's disease whose symptoms are refractory to medical management exhibit neuropathologic changes. We also endeavored to determine whether retrocochlear abnormalities are primary or secondary factors in the disease process. To these ends, we obtained vestibular nerve segments from five patients during retrosigmoid (posterior fossa) neurectomy, immediately fixed them, and processed them for light and electron microscopy. We found that all five segments exhibited moderate to severe demyelination with axonal sparing. Moreover, we noted that reactive astrocytes produced an extensive proliferation of fibrous processes and that the microglia assumed a phagocytic role. We conclude that the possible etiologies of demyelination include viral and/or immune-mediated factors similar to those seen in other demyelinating diseases, such as multiple sclerosis and Guillain-Barré syndrome. Our findings suggest that some forms of Ménière's disease that are refractory to traditional medical management might be the result of retrocochlear pathology that affects the neuroglial portion of the vestibular nerve.

Adult↗
PubMed1998

Successful prevention of retrocochlear hearing loss in murine experimental allergic encephalomyelitis with T cell receptor Vbeta8-specific antibody.

Experimental allergic encephalomyelitis is an animal model of a T cell-mediated autoimmune disease -- for example, multiple sclerosis. We demonstrated that mice with experimental allergic encephalomyelitis developed retrocochlear hearing loss, and that the lesion of the auditory pathway might be related to T cell receptor Vbeta8-expressing T cells. To investigate whether anti-Vbeta8 antibody could prevent hearing loss, we carried out brain stem auditory evoked potential testing, histologic examinations, and flow cytometry in antibody-treated and control myelin basic protein-immunized B10.PL mice. The antibody was administered just before immunization of myelin basic protein. The disease incidence and severity were significantly reduced in the mice injected with the antibody. The results of brain stem auditory evoked potential testing, histologic examinations, and flow cytometry indicated that the depletion of Vbeta8-expressing T cells brings the prevention of hearing loss, as well as prevention of other neurologic deficits. The development of T cell receptor-specific antibody therapy might help treat retrocochlear hearing loss in multiple sclerosis.

Animals↗
PubMedDate not supplied

[Topographic diagnosis of hearing loss in patients with ischemic heart disease. Use of high-rate brain stem evoked response audiometry].

Hearing loss in patients with ischemic heart disease traditionally has been attributed to atheromatous cerebral vascular disease. In this study, the site of the auditory lesion was sought in patients with ischemic heart disease and perceptive hearing loss. Standard brainstem evoked response audiometry (BSER) with increased stimulation rate (ISR) were performed. Sixty-seven patients admitted for coronary artery disease were examined. Fifty-seven recordings were complete and evaluable. All the cases of hearing loss were endocochlear. In 4 patients. BSER and ISR detected subclinical retrocochlear lesions that were compatible with ischemic disease. The results suggest that selective early cochlear ischemia can occur in young persons with heart disease. The sensitivity of BSER with ISR for detecting ischemic brainstem lesions was confirmed.

Adult↗
PubMedDate not supplied

The stapedius reflex tests in retrocochlear hearing disorders.

The commonly used parameters of the stapedius reflex responses in the diagnosis of sensorineural hearing disorders are the reflex threshold level and the persistence of the reflex response on prolonged stimulation, i.e., the reflex decay test. With the object of establishing the sensitivity of these reflex parameters to identify tumours affecting the eighth nerve in the early stages, a series of 97 such cases were examined. The mean hearing loss of the affected ears was 32 dB; 28 of the cases still had normal thresholds of hearing; 95 of the ears demonstrated elevated reflex thresholds and/or pathologic reflex decay. To check the specificity of the test, a control series with sensorineural hearing loss and reflex threshold elevation or pathologic reflex decay was analysed with respect to clinical diagnoses. In every third case of reflex threshold elevation and in every second case of reflex decay, the medical diagnosis confirmed a disease known to be associated with retrocochlear dysfunction. It was also noted that none of the cases with brain stem lesion demonstrated reflex decay--an observation which supports the idea of the reflex decay phenomenon as specifically associated with eighth-nerve lesions.

Cranial Nerve Neoplasms↗
PubMed2002

Cerebellar arteriovenous malformation with facial paralysis, hearing loss, and tinnitus: a case report.

OBJECTIVE: To describe cerebellar arteriovenous malformation in a 21-year-old man with symptoms resembling those of ear disease and to discuss the relationship between the findings of neurotologic examination and magnetic resonance imaging. STUDY DESIGN: Case report. SETTING: Department of Otolaryngology, Head-and-Neck Surgery of the Kyoto University Hospital, which is a tertiary care center, in Kyoto, Japan. PATIENT: A 21-year-old man had cerebellar arteriovenous malformation and symptoms resembling those of ear disease: recurrent left facial paralysis, left retrocochlear hearing loss, and tinnitus. Auditory brainstem responses showed only waves I and II on his left side. Downbeat nystagmus was seen by anteflexion and retroflexion of his neck. He also experienced a slight sensory disturbance on the left side of his face and right lower extremity. Magnetic resonance imaging and vertebral angiography revealed a cerebellar arteriovenous malformation and a varix functioning as a drainer of the arteriovenous malformation surrounded by an edematous region probably induced by a small hemorrhage in the brainstem. INTERVENTION: Conservative treatment, including intravenous prednisolone, vitamin B12, and oral adenosine triphosphate was performed followed by total extirpation of the arteriovenous malformation. CONCLUSION: In examining patients with peripheral facial paralysis (sometimes recurrent with a short interval) and other symptoms resembling those of ear disease, especially those suggesting certain central disorders, it is important to take intracranial arteriovenous malformation into consideration because the condition may be sometimes life threatening if overlooked.

Adult↗
PubMed1995

[Audiological test results of the autoimmune sensorineural hearing loss].

Audiological test results of 71 cases (130 ears) of clinically diagnosed autoimmune sensorineural hearing loss were analysed. Their audiograms can be divided into 4 types: low-frequency loss (55 ears), flat-type (26 ears), bell-type (17 ears) and high-frequency loss (32 ears). The test results of TEOAE, ABR, and -SP/AP amplitude ratio showed that the site of lesions in most of the low-frequency loss were retrocochlear, and most of the other three types were cochlear. Since about 1/3 of cases were chiefly involved retrocochlearly, the term "Autoimmune inner ear diseases" should not be used to substitute "Autoimmune sensorineural hearing loss".

Adolescent↗
PubMed1989

[Auditory electrophysiology and retrolabyrinthine non-tumorous diseases]].

The authors present 55 cases of non-tumoral retrocochlear impairment, studied over the period between 1984 and 1988 in the ENT University Clinic of Bordeaux. Deafness was the most frequent symptom leading patients to come for consultation (89%). The stapedial reflex was pathological in 70% of the cases. No conclusion could be drawn from electro-occulography. Only the early auditory evoked responses made it possible to reach the diagnosis of retrocochlear impairment in 35% of the cases. Electrocochleography was required in 65% of the cases to establish the topographic diagnosis. The neuro-radiological examination proved to be negative in 48 cases, and positive in 7 cases (6 atrophic symptoms, 1 left vertebral dolichoartery). Among the other etiologies, we noted 14 cases of auditory trauma, 12 vascular pathologies, 9 neurological pathologies, 7 cases of Ménière's disease, and 7 sudden onsets of deafness. The incidence of vasculo-nervous conflict reported by other authors appears very low in this series (with only 1 case).

Audiometry, Evoked Response↗
PubMed2005

[Auditory neuropathy--new disease].

Auditory neuropathy, a disorder of neural synchrony, is a retrocochlear hearing disorder identified by absence of auditory brainstem responses (ABR) and presence of evoked acoustic otoemissions (EOA). Registration of emissions shows normal function of external cilliary cells. Patients with auditory neuropathy have difficulties to understand speech especially in presence of beckground noise. Normal conversation requires the support of orofacial reading. The authors presents electrophysiological and behavioral tests useful in diagnostic process. An option in treatment of auditory neuropathy is cochlear implantation. Such treatment is indicated when conventional hearing aids doe not help to improve speech understanding.

Cochlear Implantation↗
PubMed2004

Auditory brainstem response versus magnetic resonance imaging for the evaluation of asymmetric sensorineural hearing loss.

OBJECTIVES/HYPOTHESIS: Auditory brainstem response (ABR) testing and magnetic resonance imaging (MRI) are compared for the evaluation of patients with asymmetric sensorineural hearing loss (SNHL). MRI with gadolinium administration is the current gold standard for identifying retrocochlear lesions causing asymmetric SNHL. The study seeks to determine the sensitivity and specificity of ABR in screening for possible retrocochlear pathology. Most important among SNHL etiologies are neoplastic lesions such as vestibular schwannomas, cerebellopontine angle (CPA) tumors, as well as multiple sclerosis, stroke, or other rare nonneoplastic causes. The study results will allow the author to recommend a screening algorithm for patients with asymmetric SNHL. STUDY DESIGN: The study is a multi-institutional, institutional review board approved, prospective, nonrandomized comparison of ABR and MRI for the evaluation of patients with asymmetric SNHL. METHODS: Three hundred twelve patients (between the ages of 18 and 87) with asymmetric SNHL completed the study. Asymmetric SNHL was defined as 15 dB or greater asymmetry in two or more frequencies or 15% or more asymmetry in speech discrimination scores (SDS). These patients prospectively underwent both ABR and MRI. The ABR and MRI were interpreted independently in a blinded fashion. In addition to the ABR and MRI results, a variety of clinical and demographic data were collected. RESULTS: Thirty-one (9.94%) patients of the study population of 312 were found on MRI to have lesions causing their SNHL. Of the 31 patients with causative lesions on MRI there were 24 vestibular schwannomas, 2 glomus jugulare tumors, 2 ectatic basilar arteries with brainstem compression, 1 petrous apex cholesterol granuloma, 1 case of possible demyelinating disease, and 1 parietal lobe mass. Twenty-two of the 31 patients had abnormal ABRs, whereas 9 patients (7 with small vestibular schwannomas) had normal ABRs. This gives an overall false-negative rate for ABR of 29%. The false-positive rate was found to be 76.84%. Sensitivity of ABR as a screening test was 71%, and specificity was 74%. CONCLUSIONS: Ten percent of patients with asymmetric SNHL (by this study's criteria) are likely to have causative lesions found on MRI. Although the recently reported annual incidence of vestibular schwannoma in the general population is 0.00124%, for patients with asymmetric SNHL in this study, the incidence was 7.7% (nearly 4 orders of magnitude higher). ABR has been demonstrated to have low sensitivity and specificity in the evaluation of these patients and cannot be relied on as a screening test for patients with asymmetric SNHL. Keeping the use of MRI conditional on the results of ABR will annually result in missed or delayed diagnosis of causative lesions in 29 patients per 1,000 screened. The author recommends abandoning ABR as a screening test for asymmetric SNHL and adoption of a focused MRI protocol as the screening test of choice (within certain guidelines).

Adolescent↗
PubMed2004

The mechanism of hearing loss in Paget's disease of bone.

OBJECTIVES/HYPOTHESIS: The mechanism of hearing loss (HL) in Paget's disease of bone was investigated. The present study was a systematic, prospective, controlled set of clinical investigations to test the hypothesis that there is a general underlying mechanism of HL in Paget's disease of bone and to gain additional insights into the auditory and otologic dynamics of this disease. Specific questions were 1) whether the mechanism is cochlear or retrocochlear and 2) whether the bone mineral density of the cochlear capsule is related to hearing levels. STUDY DESIGN: Several double-blinded, cross-sectional, prospective, correlational studies were conducted in a population of elderly human subjects with skull involvement with Paget's disease versus a control population of elderly subjects free of Paget's disease. Demographic and clinical data were recorded. Longitudinal observations were made in subjects under treatment. METHODS: Subjects were recruited from a Paget's disease clinic. Pure-tone auditory thresholds, word recognition, and auditory brainstem responses (ABRs) were recorded. The dimensions of the internal auditory canals were measured using computed tomographic (CT) images and digital image analysis. The precision, accuracy, and temporal stability of methods to measure the bone mineral density of the cochlear capsule and an adjacent area of nonotic capsule bone were validated and applied. Correlations were sought between hearing levels and cochlear capsule bone mineral density. RESULTS: ABRs were recorded in 64 ears with radiographically confirmed Paget's disease involving the skull. Responses were absent in eight ears, all of which had elevated high pure-tone thresholds. ABRs were interpreted as normal in 56 ears; none were abnormal. The mid-length diameter and minimum diameter of the internal auditory canal of 68 temporal bones from subjects with Paget's disease were found to have no statistically significant relationship to hearing thresholds. The Pearson product-moment correlation coefficients (age- and sex-adjusted) in the group with Paget's disease involving the temporal bone were -0.63 for left ears and -0.73 for right ears for high-frequency air conduction pure-tone thresholds (mean of 1, 2, and 4 kHz) versus cochlear capsule density. Correlation coefficients (age- and sex-adjusted) between cochlear capsule density and air-bone gap (mean at 0.5 and 1 kHz) for the affected group were -0.67 for left ears and -0.63 for right ears. All correlations between hearing thresholds and cochlear capsule density in pagetic subjects were significant at P <.001. The regressions were consistent throughout the ranges of hearing level. There were no significant correlations between cochlear capsule mean density and hearing level in the volunteer subjects. CONCLUSIONS: The evidence supports the existence of a general, underlying, cochlear mechanism of pagetic HL that is closely related to loss of bone mineral density in the cochlear capsule. This mechanism accounts well for both the high-frequency sensorineural HL and the air-bone gap. Early identification, radiographic diagnosis of temporal bone involvement, and vigorous treatment with third-generation bisphosponates are important to limit the development and progression of pagetic HL.

Adult↗