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PubMedDate not supplied

Otoacoustic emissions and brainstem auditory evoked potentials in children with neurological afflictions.

Findings are reported for evoked otoacoustic emissions (EOAEs) recorded from 22 children with neurological afflictions, whose brainstem auditory evoked potentials (BAEPs) were pathological on at least one side (41 ears explored). Our results confirmed that EOAEs are always present in children and infants having normal BAEPs. Absence of EOAE (n = 22) was almost always related to middle ear or cochlear damage with BAEPs indicating diagnoses, respectively, of transmission damage (n = 7) or endocochlear damage (n = 16). Conversely, for BAEP diagnoses of retrocochlear damage (n = 12), EOAEs were always present. EOAEs associated with BAEPs, therefore, appear to offer a well-adapted technique for precise etiological diagnosis of childhood hearing loss. When no wave is identifiable by BAEP recording, EOAE presence indicates retrocochlear damage.

Child↗
PubMedDate not supplied

Superficial siderosis of the central nervous system. A case report on examination by ECoG and DPOAE.

This is a case of superficial siderosis of the central nervous system (SSCN). The diagnosis of SSCN was based on the result of T2-weighted magnetic resonance imaging and on suggestive clinical manifestations. The pure-tone audiogram showed bilateral progressive sensorineural hearing loss with a poor speech discrimination score and Jerger type IV. The remarkable elevation of the detective threshold of cochlear microphonics on electrocochleography was found and distortion product otoacoustic emission (DPOAE) showed no response: These electrophysiologic examinations, including electrocochleography and DPOAE, revealed that the progressive sensorineural hearing loss in this case was caused by both retrocochlear and cochlear damages.

Central Nervous System Diseases↗
PubMed2006

Hearing disturbances in hypertrophic cardiomyopathy. Is the sensorineural disorder neurogenic or myogenic?

BACKGROUND: In a previous study, we demonstrated that a hidden hearing defect is present in about 50% of patients with hypertrophic cardiomyopathy (HCM). Such defects were found significantly less frequently in age and clinical stage-matched patients with dilated cardiomyopathy (DCM), and were practically absent in patients with valvular aortic stenosis, and in patients taking beta-receptor blockers for different reasons (such as hypertension, ischemic heart disease, etc.). The hearing disturbances were first examined by means of brain-stem evoked response audiometry (BAEP). This method permitted only a rough differentiation between the origins of cochlear (myogenic) and retrocochlear (neurogenic) hearing disturbances, and did not allow us to establish their myogenic or neurogenic nature with certainty. AIMS: Our present aim was to determine whether the hearing disturbances present in HCM and DCM patients are myogenic or neurogenic in origin. METHODS: The neurogenic function of the inner ear was examined by BAEP as before, and the myogenic function by the distortion product otoacoustic emission technique. RESULTS: Myogenic abnormalities were found in 39/69 ears (57%) and neurogenic abnormalities in 19/69 (28%) ears among the HCM cases, as compared with 14/39 (36%) and 8/39 (21%) ears respectively among the DCM cases (p<0.005). Healthy controls displayed the lowest incidence of both types of hearing abnormalities. CONCLUSION: Our results lead us to conclude that myogenic lesions are more frequent than neurogenic lesions in patients with HCM. Both myogenic and neurogenic lesions are more frequent in HCM patients than in DCM patients or healthy controls. It may be hypothesized that abnormal sarcomeric proteins present in the muscular structures of the inner ear in HCM are possibly responsible for the hearing disorders in these patients, and that this is not merely a neurological defect.

Adolescent↗
PubMed1999

Topodiagnostic assessment of occupational noise-induced hearing loss using distortion-product otoacoustic emissions compared to the short increment sensitivity index test.

Because the efforts needed to produce an "automatic audiogram" by means of distortion-product otoacoustic emissions (DPOAE) are still experimental, their capacity to produce objective topodiagnostic information must still be defined. In the present study results of the short increment sensitivity index testing (SISI) test in 97 patients with noise-induced hearing loss (NIHL) were compared with those of a DP-gram. Pure tone, speech and impedance audiometry, SISI tests and DPOAE measurements were performed on all patients. SISI was measured in both ears of 93 patients and unilaterally in 4 patients. SISI was also determined at two frequencies in 46 patients. Six measurements were rejected because of conductive hearing losses in adjacent frequencies. In all, 230 measurements could be compared. After classifying topodiagnostic results of the SISI test and DP-gram into cochlear, inconclusive and retrocochlear lesions, statistically significant correlations were found and differences were noted in only 18% of cases. Findings demonstrated the superiority of DPOAE to the SISI test in obtaining topodiagnostic information about inner ear disease.

Acoustic Impedance Tests↗
PubMed1992

[Ototoxicity of deferoxamine].

Deferoxamine or desferrioxamine (DFO) is a chelating agent, largely used in patients with chronic renal failure, although it has many side effects, being ototoxicity one of them. In this paper we studied the eventually adverse otologic effects of DFO in 20 patients receiving haemodialysis. A complete audiological evaluation, including pure-tone audiometry, brainstem auditory evoked potentials and high-frequency audiometry, was performed. The results showed a sensorineural hearing loss of retrocochlear origin in 3/20 cases (15%). We can accept that ototoxic effects of DFO are minimal, but no inexistent. Because of these we considered highly recommendable an accurate control of hearing in patients with renal disease receiving DFO.

Adult↗
PubMed1981

"Onion bulb" formation associated with a solitary neoplasm of the eighth nerve sheath.

Audiometric patterns associated with retrocochlear disorders include abnormal adaptation, delay of auditory evoked potential latencies, and characteristic abnormalities of speech intelligibility functions. The anatomic substrate of these psychoacoustic and electrophysiologic abnormalities is unknown. This paper describes the incidental observation of "onion bulbs" in a solitary neoplasm involving the eighth nerve sheath and in the cochlear nerve lateral to the neoplasm. Such hypertrophic neuropathy in the eighth nerve has not been described previously. This occurrence raises the question of sequential demyelination and remyelination in a neoplasm-bearing nerve as a possible histopathologic correlate to retrocochlear dysfunction.

Cranial Nerve Neoplasms↗
PubMed1994

Osteoma of the internal auditory canal. Case report and literature review.

Osteomas of the internal auditory canal are rare lesions, with only 12 reported cases in the world literature. Symptoms are those of eighth nerve compression, and include unilateral hearing loss and vestibular weakness, thus mimicking symptoms of acoustic neuroma. We report a patient with an osteoma of the internal auditory canal, along with a review of the literature. We note age and sex characteristics from the literature, give evidence of localized trauma as a possible etiologic factor for this lesion, and discuss the pitfalls of relying exclusively on magnetic resonance imaging in the workup of suspected retrocochlear lesions.

Female↗
PubMed1981

[Criteria for the differential diagnosis of cochlear-retrocochlear disorders with brain stem audiometry (author's transl)].

Brain stem audiometry turned out to be a useful tool in the differential diagnosis of cochlear-retrocochlear damage. Reviewing the literature of recent years it becomes clear that various criteria are used to distinguish such lesions when interpreting the brain stem potentials. The most frequently used parameters are: 1. ipsilateral latency of potential V as compared to mean of normal population; 2. difference of latency of potential V of ipsi- and contralateral side; 3. ipsilateral latency difference potentials I -- V as compared to mean of normal population; 4. difference of latency difference potentials I -- V of ipsi- and contralateral side. Evaluating the registrations of brain stem potentials of 50 patients with uni- or bilateral cochlear lesions these parameters had a different degree of liability. Comparing latency differences of potentials I -- V of ipsi- and contralateral sides was the safest method to avoid a false positive diagnosis. It is discussed why this parameter should be the most suitable in the distinction of cochlear and retrocochlear lesions.

Acoustic Stimulation↗
PubMed1996

Effects of brain stem lesions on cochlear function: mechanism of hearing improvement after removal of a brain stem tumor.

EOAEs are well correlated with changes of hearing sensitivity during the clinical course of brain stem lesions, as shown in this case study. They may serve as sensitive indicators to evaluate the possible effects of a brain stem lesion on cochlear function and monitor the attempted preservation of hearing during CPA or brain stem surgeries. It is thereby suggested that EOAEs should be included in an audiologic test battery in a differential diagnosis of retrocochlear lesions.

Audiometry, Pure-Tone↗
PubMed1991

Juvenile onset diabetes mellitus, central diabetes insipidus and optic atrophy (Wolfram syndrome)--neurological findings and prognostic implications.

The authors report on one case of Wolfram syndrome, a rare condition, which is characterized by juvenile onset diabetes mellitus, diabetes insipidus, optic atrophy and sensorineural deafness. The findings of this 13-year follow-up show that this patient developed typical neurological complications of long-standing diabetes mellitus as in the common type 1 variant. Moreover, some peculiar signs occurred such as anosmia, ophthalmoplegia interna, and central nystagmus. Since Wolfram syndrome is probably part of a more generalized neurodegenerative disorder, long-term prognosis will depend both upon the severity of chronic diabetic complications and upon the rapidity, by which degeneration of cerebellar, pontine and brain stem structures appear. Prognosis of the cardinal clinical signs is such that optic atrophy, though usually quite rapid in the beginning, generally does not lead to complete blindness. Sensorineural hearing loss progresses very slowly so that deafness might be expected exceptionally only. The hearing deficit in classical diabetics, however, is of retrocochlear origin. Therefore, in Wolfram syndrome, a combined inner-ear and retrocochlear hearing loss may occur.

Adolescent↗
PubMedDate not supplied

[Diagnosis of sensorineural hearing loss by ipsilateral masking of brain stem auditory evoked potentials].

The Authors show their original technique for the differential diagnosis of cochlear and retrocochlear hearing loss which employs ipsilateral masking of the A.B.R. in order to define the difference in V wave latency between a standard technique (clicks at 21 pps without noise) and a sensitized technique (clicks at 21 pps with noise at S/N of +40). This difference in latency has been named Sensitizing Latency Difference (SLD). Sixty normal subjects, 85 patients with cochlear hearing loss and 6 with retrocochlear hearing loss, were studied. The obtained data showed a SLD value: in normal hearing of: a) 0.31 msec. (+/- 0.14 SD) from 18 to 40 years old; b) 0.36 msec. (+/- 0.26 SD) from 41 to 60 years old; c) 0.48 msec. (+/- 0.21 SD) over 60 years old; in patients with cochlear hearing loss 0.12 msec. (+/- 0.14 SD) in 2 subjects affected by retrocochlear hearing loss a value superior to 1.5 msec. in the other 4 cases the V waves disappeared during recording of the sensitized test. A differential value of 1 msec. between cochlear and retrocochlear hearing loss was established by the Authors who with this value correctly identified all the patients with retrocochlear hearing loss and 97.8% of the subjects with cochlear hearing loss. An incorrect diagnosis of the hearing loss origin (false positives) was made in 2.2% of the patients with cochlear hearing loss. In conclusion, the Authors feel that the SLD evaluation, in consideration of its large clinical utility and of the high reliability of obtained data, is a particularly useful test in making a differential diagnosis between cochlear and retrocochlear hearing loss.

Adolescent↗
PubMedDate not supplied

[Electrocochleography and topographic diagnosis].

We present a group of 50 patients with retrocochlear pathology, divided on 41 (82 per 100) non tumoral and 9 (18 per 100) tumoral: 8 cerebello-pontine angle meningiomas and 1 intracranial cholesteatoma. The retrocochlear diagnosis of the pathology is assessed by the results of the audiovestibular explorations, specially the electrophysiology (early auditory evoked response, electrocochleography). The neuroradiologic explorations (TAC, MNR) define the tumoral or non tumoral nature of the pathology. We review some technical aspects of the common electrocochleographic practice, as actually is realised by the Bordeaux group; also, the different parameters of a retrocochlear pathologic response. In this group, the electrocochleography was necessary on 64 per 100 of the cases to obtain an electrophysiologic retrocochlear diagnostic.

Acoustic Impedance Tests↗
PubMed1997

[Controversial aspects of magnetic resonance imaging in sudden onset cochleovestibular diseases].

Sudden deafness and vertigo are a challenge for the otolaryngologist. With its high fluid content, the membranous labyrinth is best evaluated through Magnetic Resonance Imaging (MRI). Recently gadolinium-enhanced MRI has opened new perspectives in the imaging of the pathological labyrinth, able to directly detect labyrinthine lesions. To date the main role of MRI has been to rule out the presence of retrocochlear involvement in patients with sudden deafness and/or vertigo. However, in the past few years, several authors, reporting on gadolinium-enhanced MRI, have described better labyrinthine imaging, revealing direct labyrinth or nerve bundle involvement in all such patients. On the contrary, other studies have not revealed any enhancement of post-contrast labyrinthine images upon MRI but have shown an increase in signal intensity upon T2-weighted images. In this light, the purpose of the present study has been a) to evaluate the prevalence of labyrinthine abnormalities found upon MRI in consecutive patients with sudden deafness and vertigo and b) to assess the correlation between the severity of clinical and audiological findings and the MRI abnormalities encountered. Twelve consecutive patients with sudden hearing loss and/or vertigo were included in the study (age range 9-59 years; 6 males and 6 females). All subjects underwent complete otoneurological examination. MRI was performed with a 0.5 T superconducting magnet system allowing 2 mm-thick sections through the temporal bone. Within 29 days of onset of the complaints all patients were studied both before and after administration of the contrast medium (gadolinium-DTPA, 0.01 mmol/l, i.v.). Two of the 12 subjects had sudden deafness, 5 sudden deafness and vertigo while 5 had vertigo alone. Gadolinium enhancement and/or a high signal intensity upon T2-weighted images of the pathological labyrinth and nerve bundle was observed in 4 patients. The patients were divided into groups by etiology. MRI abnormalities were only found in the group where the etiology was viral. There was no correlation between the severity of clinical findings and the presence of MRI abnormalities nor between MRI and ABR findings. Analysis of such controversial data is discussed, stressing the importance of gadolinium-enhanced MRI of the labyrinth as a new diagnostic tool in sudden deafness and vertigo.

Acute Disease↗
PubMed2003

Hearing deterioration in patients with a non-growing vestibular schwannoma.

OBJECTIVE: The aim of this study was to document and analyse the course of several audiometric parameters in 49 patients with a non-growing unilateral vestibular schwannoma (VS). MATERIAL AND METHODS: Patients received conservative management and absence of tumour growth was ascertained by means of serial magnetic imaging studies. Pure-tone audiometry and speech audiometry were performed at yearly intervals. RESULTS: Pure-tone audiometry revealed a significant increase in thresholds at all frequencies, except for 8.0 kHz. The maximum yearly threshold increase was 2.4 dB hearing level at 1.0 and 2.0 kHz. Speech audiometry revealed a significant decrease in maximum discrimination over the course of time. No significant changes were observed in the following parameters: the intensity level at which maximum discrimination was achieved; the roll-over index; the speech reception threshold; and the slope of the curve in the speech audiogram. No change was observed in the relation between pure-tone audiometry thresholds and speech audiometry scores. Apparently, the deterioration of pure-tone perception and speech discrimination ran parallel courses. CONCLUSIONS: The results of this study indicate that hearing loss is a predominant symptom in patients with a non-growing VS, as is also known in patients with a growing lesion. Moreover, it seems unlikely that the hearing loss in VS patients is merely the result of mechanical influences on retrocochlear neural or vascular structures.

Adult↗
PubMedDate not supplied

SISI test and adaptation. III. Subjects with perceptive hearing defects.

The effect of the adaptation of the ear on the SISI test was studied in 60 completely and 18 incompletely recuriting patients and in 5 patients with a verified acoustic neurinoma at 500 and 2000 Hz. The subjective suprathreshold adaptation to a 3 min tone of 20 dB (SL) ranged from 10 dB to over 20 dB, depending on the frequency and type of defect. The pre-adaptation mean SISI values in the recruiting groups ranged from 38 to 51% and the post-adaptation means from 38 to 49%. In retrocochlear lesions only one SISI value was questionable before and after adaptation, the others being 0%. The dispersion of the SISI scores was considerable, a fact that made statistical treatment difficult. Adaptation did not significantly affect the SISI value. It could be ascertained that high SISI values did not reliably distinguish between the hearing defect types, but if the SISI was low the possibility of a retrocochlear lesion should always be borne in mind.

Acoustic Stimulation↗