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Cost-effective evaluation of asymmetric sensorineural hearing loss with focused magnetic resonance imaging.

The poor sensitivity of audiometric brain stem response for small vestibular schwannomas (acoustic neuromas) creates a dilemma for the physician evaluating a patient with signs and symptoms of retrocochlear disease. Magnetic resonance imaging is recognized as the gold standard for the evaluation of these problems, but if a complete examination of the internal auditory canals and head is done on every patient, the cost is high. Although less expensive, screening with audiometric brain stem response risks missing up to 33% of small tumors. Therefore we developed a focused magnetic resonance imaging sequence for evaluation of patients with asymmetric sensorineural hearing loss and/or nonpulsatile tinnitus. The protocol includes a T1-weighted sagittal localizer, pregadolinium and postgadolinium T1-weighted 3-mm contiguous axial slices through the internal auditory canal and the region of the cerebellopontine angle, and T2-weighted axial images through the entire brain. Total scanning time is about 12 minutes, and the estimated cost is $300 to $500. We retrospectively reviewed the imaging records of 485 screening examinations done during an 18-month period. Twenty-four patients had diagnoses definitely or probably producing the hearing loss for an overall positive rate of 5%. By eliminating the need for follow-up audiometric or electrophysiologic studies, we believe a focused magnetic resonance imaging-based diagnostic scheme is actually more cost-effective on a cost-perpatient basis.

Cost-Benefit Analysis↗

Neural networks applied to retrocochlear diagnosis.

Methodologies have been developed, based on insights from signal detection theory, to evaluate quantitatively the diagnostic performance of tests. Several studies have demonstrated that, in fact, performance of a test battery can be inferior to the best of the tests it includes. These studies have been quite persuasive in damping enthusiasm for the test battery approach. Because the results of all tests in a battery were weighted equally in these studies, it is not surprising that an individual test with good sensitivity and specificity is more effective diagnostically than a combination of tests with poorer sensitivity and specificity. The authors of many of these studies were well aware of the limitations of this approach. In the present study, neural networks were applied to evaluate audiological tests used to predict retrocochlear pathology by differentially weighting the results of the tests in the battery. This technique avoids some of the limitations of previous approaches. Of the audiological tests evaluated in the present analysis, the superiority of the auditory brainstem evoked response (ABR) in predicting retrocochlear disease was again demonstrated. However, the results also demonstrated that identification accuracy could be improved by combining the ABR with other tests (in this case contralateral acoustic reflex at 2000 Hz, ipsilateral acoustic reflex at 2000 Hz, tone decay, and word recognition score). Further, it was demonstrated that performance could be improved over that obtained using dichotomous test measures (i.e., positive or negative presence of pathology) by using raw test measures in conjunction with ABR.

Adult↗

Sensitivity of auditory brainstem response in acoustic neuroma screening.

Auditory brainstem response (ABR) is the reference screening technique for acoustic neuromas, but because of a few false negatives and the increasing performance of magnetic resonance imaging (MRI), its role as the standard method has been questioned. We assessed sensitivity of screening tests in 89 patients with surgically proven acoustic neuromas. Sensitivity of ABR was 92%; 94% for extracanalicular neuromas and 77% for intracanalicular neuromas. For stapedius reflex (SR), sensitivity was 84% and for caloric vestibular response (CVR) 86%. The combined sensitivity of ABR + SR was 97% and of ABR + RS + CVR 98%. For false negatives, the greatest diameter including the intracanalicular portion was always less than 18 mm, with a mean of 15 mm, and none of these tumours reached the brainstem. For patients with unilateral cochleo-vestibular deficit, we propose ABR and SR as first-line screening tests. These tests are repeated at 6 months and at 1 year in the case of normal results. MRI is ordered for patients whose auditory threshold is too low and for those whose ABR or SR results favour retrocochlear disease.

Caloric Tests↗

Clinical significance of otoacoustic emissions: a perspective.

The aim of this paper is to review the properties of otoacoustic emissions from a clinical point of view and to discuss the perspective interest of this test. In adults, the clinical significance of evoked otoacoustic emissions seems to be limited either in endocochlear hearing losses or for detecting retrocochlear diseases. In infants, evoked otoacoustic emissions seem to be a reliable, simple, non-invasive, and precise method for estimating auditory sensitivity for midfrequencies (1-4 kHz). Then, EOEs could be considered as an interesting way for screening auditory dysfunction in infants.

Adult↗

Analog and digital filtering of ABR: ipsi- and contralateral derivations.

OBJECTIVE: In audioneurological evaluations, peak latency is an important parameter regarding the determination of possible wave delays. Digital filtering entails suppression of less informative low-frequency components without phase distortion, thus accentuating the peak readings. Ipsi- and contralateral recordings have improved the reliability as regards the identification of ABR waves. This applies specifically to the wave IV-V complex. The purpose of this study has been to compare 1) analog and digitally filtered waveforms and 2) ipsi- and contralateral derivations. DESIGN: Two-channel ABR data were collected from 120 unselected subjects referred for assessment of possible retrocochlear diseases. The analog filter bandwidth was 30 to 3000 Hz. Each response was subsequently digitally filtered with a bandwidth of 300 to 2500 Hz, and a comparison of wave identification between the analog and digitally filtered responses was performed. Wave identification was also compared between the digitally filtered ipsi- and contralateral responses, and the differences of the wave latencies between the two derivations were calculated. RESULTS AND CONCLUSIONS: Digital filtering improves ABR wave identification. For the digitally filtered waveforms, a better wave identification is found ipsilaterally for waves I to III, whereas the opposite is found for wave IV. Wave V identification is identical in both derivations. Furthermore, significant ipsi- and contralateral latency difference were found for all waves except for wave IV. This must be taken into consideration if ipsi- and contralateral derivations are to be summed.

Cochlea↗

Management of vestibular schwannomas (acoustic neuromas): the value of neurophysiology for evaluation and prediction of auditory function in 420 cases.

OBJECTIVE: From 1978 to 1993, 1000 vestibular schwannomas were operated on at the Department of Neurosurgery at Nordstadt Hospital. The goal was to improve the chances of hearing preservation by recording auditory brain stem responses (ABRs). ABRs can be used for preoperative classification of cochlear nerve impairment and for prediction of the chances of hearing preservation. PATIENTS AND METHODS: In addition to the previously described audiometric testing, the patients underwent perioperative and intraoperative bilateral ABR recording at 100-dB condensation and rarefaction click stimulation. The classification system of five types of ABRs, as presented before, is based on the presence and on the latencies of Waves I, III, and V, with a special emphasis on Wave III's representing the activity of the first brain stem nuclei within the auditory pathway. According to an analysis of 420 preoperative ABRs, in case of a preoperative Type 1 or 2, the rate of hearing preservation is 80%. DISCUSSION: In the case of good clinical and audiometric hearing, a severely deteriorated ABR is mostly an indicator of severe nerve compression and adhesion by the tumor. In view of subsequently reported experiences with intraoperative ABR monitoring, the value of the presented system emphasizing the importance of Wave III is stressed and discussed with other views in the literature. The criteria presented here are not designed for recognition of retrocochlear disease but aim for evaluation of the state of the auditory nerve and its perspective. CONCLUSION: By the presented classification of ABR Type B1 through B5, preoperative prediction of the likelihood of hearing preservation is improved.

Adult↗

A practical approach to the patient with vertigo: an outline of diagnosis and management for the nonspecialist.

A careful history is the most important part of a medical examination for vertigo, especially to establish whether it is acute, chronic or recurrent. If the patient spontaneously volunteers ear complaints, such as fullness, tinnitus, or hearing loss, the cause is probably otologic and deserves immediate referral. If no otologic complaints are volunteered, the whole body becomes a source of investigation. All nonvital drugs should be withheld while the vertigo study is progressing. A complete physical examination and blood profile should be carried out. Roentgenograms of the skull and chest should be obtained. Accurate diagnosis and proper management are possible only with prolonged follow-up. Unnecessary expense, inconvenience, and discomfort for the patient can be avoided by a screening audiogram to identify unilateral hearing loss. All unilateral hearing loss should be investigated thoroughly in order to diagnose serious retrocochlear disease early.

Acute Disease↗

Analysis of the efficiency of retrocochlear screening.

The auditory brainstem response (ABR), as well as associated audiologic and radiographic studies of 175 patients suspected of having cerebellopontine angle tumors were reviewed. The majority of patients presented with asymmetric hearing loss. Eight acoustic neuromas were identified. All eight had abnormal ABRs. The false-positive rate was 22%. Rollover and acoustic reflex testing played a minimal role in the decision as to whether or not an ABR was ordered and, as a result, are not important in the search for retrocochlear disease. The cost per tumor diagnosed was roughly $7600. A rational approach to tumor screening based on the efficiency and cost of the screening tests, the frequency of abnormal ABRs, and the cost per tumor diagnosed is presented.

Audiometry↗

Auditory brain stem responses in the diagnosis of cerebellopontine angle tumours.

There is a constant search for more reliable methods of diagnosing cerebellopontine angle tumours at an early stage. The auditory brain stem responses promise to be of use as such a method. In two patients with extracanalicular neurinomas we found a definitely abnormal brain stem response even though conventional tests produced a cochlear type of test pattern. In a third patient with a meningeoma we obtained a similar type of response. Here the conventional tests clearly pointed to the presence of retrocochlear disease. Characteristic findings are a broadening of the whole nerve action potential and a delay in the appearance of the Jewettv-FFP7 complex.

Adult↗

Auditory brainstem responses in ears with hearing loss: case studies.

Auditory brain stem evoked responses (ABRs) were recorded from six selected adult patients with various audiometric configuration, histories, and diagnoses. Although none of the patients were diagnosed medically as having retrocochlear disease, ABR latencies and waveform morphology were considered abnormal in some cases. By contrast, other patients with similar hearing loss configuration yielded normal ABRs. These observations emphasize the potential difficulty encountered in the interpretation of ABRs when hearing loss exists. Further, because ABRs and all other special auditory tests are not diagnostic extreme caution should be exercised prior to any definitive interpretation and subsequent diagnosis.

Adult↗

Correcting for factors affecting ABR wave V latency.

The latency of auditory brainstem response (ABR) wave V has been shown to be affected by several subject and technical factors. Previous studies have often concluded with a recommendation to account or correct for one or more of these factors when latency is used as a means of detecting retrocochlear dysfunction. This study investigated the effect of subject age, sex, hearing loss and stimulus intensity on ABR wave V latency in a group of 189 clinical subjects in whom retrocochlear disease was effectively excluded. Regression analysis showed that all four factors exerted a significant influence on latency and that hearing loss and intensity were most effectively represented when combined to form a sensation level variable, together with a measure of audiogram slope.

Acoustic Stimulation↗

How significant is sensorineural hearing loss in primary Sjögren's syndrome? An individually matched case-control study.

OBJECTIVE: We evaluated whether sensorineural loss and vestibular abnormalities are common in patients with primary Sjögren's syndrome (pSS) and whether such abnormalities are clinically significant. METHODS: In an individually matched case-control design, 48 patients with pSS underwent complete audiovestibular evaluation along with 48 age and sex matched individuals without otologic problems. Differences of > 20 dB between patient and control ears at any frequency tested were considered to be significant. RESULTS: Significant differences in hearing loss were seen at 4,000 Hz (6 vs 0 ears; p = 0.03) and at 8,000 Hz (9 vs 0 ears; p = 0.003). Small differences in hearing acuity were also observed in the lower frequencies, but the absolute mean difference was < 3 dB. A decrease of at least 60 dB in hearing acuity at any frequency up to 4,000 Hz was seen only in 3 elderly pSS patients. Abnormal brainstem auditory evoked responses were recorded in 7 patients and 5 controls, but no patient had retrocochlear lesions identified on magnetic resonance imaging. Four patients in each group had abnormalities on electronystagmography. CONCLUSION: pSS is associated with sensorineural hearing loss affecting preferentially the high frequencies, but clinically significant defects are not common. There is no evidence of retrocochlear disease or increased vestibular involvement in pSS.

Aged↗

[Diagnostic reliability of ipsilateral evoked impedance changes of the tympanic membrane (author's tranl)].

The ipsilateral registration of impedance change is necessary in case of unilateral cochlear or retrocochlear disease with middle ear impairment of the other ear and, therefore, impossibility of contralateral evocation of the reflex. If contralateral evocation fails, moreover, it will be useful in order to clarify, whether the evocation did not succeed or the registration at the contralateral ear; thus enabling a differentiation between lesions of the reflexe's afferent and efferent way. In ipsilateral impedance-change-measurements it should be distinguished between an increase of the acoustic impedance (as searched for--turn to the right at the balancemeter) and a decrease of it (turn to the left at the balancemeter). The "turns to the left" at ipsilateral evocation occur without latency and only at SPL 100 dB or more. They can be observed in patients suffering from otosclerosis, complete deafness and indissected temporal bones as well. This acoustic-mechanical effect rises at high sound pressures from the non-linearity of the sound-field, which produces an extra pressure unbalancing the pressure before and behind the tympanic membrane. This effect may interfere with the real reflex-response, but only at more than 100 dB SPL; however, it must be distinguished from the proper response in order not to find the reflex falsely positive. In three clinical examples (angioblastoma of the right cerebellum hemisphere, multiple sclerosis, cerebello-pontine angle lesion) the absence of ipsilateral impedance reflexes (isolated or additional) is demonstrated.

Acoustic Impedance Tests↗

Significance of false-positive auditory brainstem response: a clinical study.

Auditory brainstem response (ABR) is a good screening test for cerebellopontine angle (CPA) tumors and other retrocochlear diseases. The pathologic correlates of many cases with positive ABR remain unknown. A two-staged clinical study was performed to help us understand the significance of a false-positive ABR. In the first stage, data from 66 patients with abnormal ABR were reviewed. Nineteen patients had identifiable causes for the ABR abnormality, whereas 47 patients had no lesions identified. In the second phase of the study 21 of the 47 patients were re-evaluated with a minimum follow-up period of 1 year. A detailed interval history was obtained, and the work-up, including imaging studies, was repeated in an attempt to reach a diagnosis. One patient was found to have a CPA tumor, but for nine patients a definite final diagnosis could not be determined. The implications of abnormal ABR and normal initial imaging study are discussed, and a plan for the work-up of these patients is suggested.

Adult↗

Asymmetric hearing loss: toward cost-effective diagnosis.

OBJECTIVE: Unilateral audiovestibular symptoms are commonly seen in clinical practice, and the treating physician frequently considers retrocochlear disease in the differential diagnosis. These considerations often precipitate a lengthy and expensive battery of tests. This study was conducted to evaluate the diagnostic yield of the various tests available in the investigation of these symptoms and to recommend changes to practice patterns, if appropriate. METHODS: We examined a cohort of 310 patients with unilateral audiovestibular symptoms who were referred to our radiology department for investigation. All patients had complete auditory assessments, and 144 underwent auditory evoked potential testing (ABR). There were 258 contrast-enhanced CT (CECT) scans and 86 gadolinium-enhanced MRI (Gd-MRI) scans performed. RESULTS: A total of 12 cerebellopontine angle (CPA) tumours were diagnosed. CONCLUSIONS: MRI is the diagnostic tool of choice in patients with asymmetric sensory neural hearing loss (ASHL), but it is expensive and not always easily available. Analysis of diagnostic accuracy of CECT and ABR, both used as screening tests, demonstrate their limitations and reveals that CECT is no more accurate than ABR, although it is three times more costly. Based on these data, we have changed our diagnostic approach to patients with unilateral audiovestibular symptoms. Patients are either evaluated with MRI or are screened initially with ABR based on clinical suspicion. CECT is no longer routinely included in the evaluation of patients with ASHL. Finally, newer emerging MRI techniques promise faster and less expensive examinations with increased diagnostic accuracy.

Algorithms↗

Hearing disorders in sickle cell disease: cochlear and retrocochlear findings.

The auditory system was assessed in 34 adult subjects with sickle cell disease using standard audiometric test techniques. The study results indicate that patients with sickle cell disease are at risk for retro-cochlear as well as cochlear dysfunction of the auditory system. However, there is no consistent audiometric pattern that is pathognomonic of sickle cell disease. The test results indicate that auditory status should be carefully monitored in all adult patients with sickle cell disease.

Acoustic Impedance Tests↗

Diagnosis of retrocochlear and central vestibular disease by magnetic resonance imaging.

Magnetic resonance imaging has become the study of choice for the diagnosis of retrocochlear and central vestibular disease. Three groups of lesions, each characterized by a specific site of origin, have been recognized: 1) petrous apex lesions such as congenital cholesteatomas and cholesterol granulomas involving the eighth cranial nerve within the internal auditory canal; 2) cerebellopontine angle tumors, mainly acoustic neuromas and meningiomas; and 3) CNS disease involving the brain stem and the central auditory and vestibular pathways such as tumors, multiple sclerosis, infarcts, and hemorrhage.

Auditory Diseases, Central↗