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PubMed2006

Conversion deafness presenting as sudden hearing loss.

Conversion deafness is a somatoform disorder characterized by hearing loss without an anatomic or pathophysiologic lesion. Clinically, discrepancies between behavior hearing thresholds and objective electrophysiologic examinations, such as impedance audiometry, otoacoustic emissions (OAE), and auditory brainstem response (ABR), will raise the suspicion of this disorder. It is judged to be due to psychological factors and that patients do not intentionally produce the symptom. Conversion deafness is sometimes reported in children but is extremely rare among adults. Two young adults with this disease are presented. These 2 patients were both under enormous stress from the national entrance examinations for universities. Pure tone audiometry showed bilateral hearing deterioration, but OAE and ABR were normal. The hearing of both patients recovered after treatment. The diagnosis, prognosis and treatment of this disorder are also discussed. It is important to discover the psychological stress in patients with conversion deafness. This report aims to increase awareness of this condition and avoid unnecessary steroid use in its treatment.

Adult↗
PubMed1989

[The status of psychogenic hearing disorder today].

The general symptoms of psychogenic hearing disorders are pointed out and exemplified by 16 own cases seen by the author in 1987 to 1988. Psychogenic hearing disorders imitate various constellations of findings and histories of genuine organic hearing disorders. The individual's experience and the contemporary consciousness of hearing disorders seem to enter into the individual manifestation. The 16 cases can clearly be divided into 3 groups of equal size: 1. The unilateral acute hearing loss, imitating either an acute traumatic damage or an idiopathic sudden hearing loss. This was seen almost exclusively in children. 2. The bilateral chronic hearing loss. This, too, was seen mainly in children and youths. 3. The acute bilateral recurring or progressing hearing disorder. This was seen predominantly in adults, mimicking dramatic courses ending up in bilateral profound deafness. In part of the cases the psychological background of the neurotic reaction could be explored quite easily. Knowledge of the various manifestations of psychogenic hearing disorders is important, because otherwise, as the reported cases demonstrate, misleading interpretation of findings entails costly diagnostic procedures (computerized tomography, arteriography) and expensive therapeutic measures (hospitalization, fitting of hearing aids).

Adolescent↗
PubMed1984

The results of cortical electric response audiometry in medico-legal investigations.

Cortical ERA was conducted in a consecutive series of 118 medicolegal cases (467 threshold determination in 209 ears). In organic cases, the distribution of subjective (manual pure tone audiometry)/objective (CERA) discrepancies was normal and centred on 0 dB, with only 3.2% of the discrepancies exceeding + 15 dB. Averaged over 3 or more test frequencies in one ear, only 4.4% of the discrepancies exceeded + 7.5 dB, as compared to 95.1% exceeding + 7.5 dB in those suspected of having a non-organic overlay. The pattern of non-organic overlay superimposed on noise-induced hearing loss is expected and demonstrated to produce a flattening of the dip in the audiogram. It is argued that such flattening is very rarely the end product of noise exposure.

Audiometry↗
PubMed1992

[Psychosomatic factors in idiopathic sudden deafness].

A clinical and psychological history of 27 patients suffering from sudden deafness was taken shortly after the onset of symptoms and 4 months later. Psychosocial factors seem to be important in the beginning of sudden deafness, but there was no specific conflict or personality pattern. Predictors of a good outcome are stable personal relations, emotionally balanced personality factors and a reduction of stress factors after the onset of symptoms. A tendency to addictive behaviour was of unfavourable prognostic significance.

Adaptation, Psychological↗
PubMed1983

[Significance of ERA for differentiating hearing disorders of different origins].

The reliability on audiometric findings in hearing disorders of different genesis has been considerably improved by the ERA. By no means, the ERA can replace the conventional hearing tests, rather it is an additional technique of examination. Apparently it does not reveal specific findings of psychogenic hearing disorders - unless the prolongation of the cortical response as repeatedly seen by us. In case of aggravation ERA renders us possible to detect the kind of hearing defects and the actual threshold - e.g. with respect to high frequency range by brain stem responses, for 500 and 1,000 Hz according to the cortical responses. The neural hearing impairment caused by tumours always exhibits extremely prolonged latencies even at high intensities, eventually concomitant with a response threshold elevated versus the subjective threshold. These reactions, above all, become apparent in the brain stem rather than in the cortex. In contrast to this the sensoric hearing disorders the ERA elucidates even objectively the recruitment: normal latencies at high intensities. The latency of the objective response, thus, corresponds to the sound stimulus is perceived loud, and it is long for stimuli perceived relatively low - even in spite of great intensity. Comprising the ERA the quote of audiometric failures in excluding an acoustic neuroma is reduced to a minimum.

Adolescent↗
PubMed1986

Psychometric functions for frequency discrimination from listeners with sensorineural hearing loss.

Psychometric functions for pulsed pure-tone frequency discrimination were obtained from hearing-impaired listeners at frequencies with normal hearing and at frequencies with mild or moderate hearing losses. The general form of psychometric functions at hearing-impaired frequencies was found to be the same as at normal-hearing frequencies, i.e.,d' was linear with the frequency difference between tones, in Hz. For all but one psychometric function, the addition of an intercept term to the fitting equation did not account for significantly more variance than did the slope term alone. Therefore, it was concluded that psychometric functions for frequency discrimination can be adequately described with only one parameter: the slope of the psychometric function. Deficits in discrimination at hearing-loss frequencies were manifested by more gradual slopes of psychometric functions. Procedures for normalizing psychometric functions are presented, which facilitate comparisons of normal and impaired frequency discrimination data across studies and frequencies. Comparisons of dlf's (difference limen for frequency) obtained with adaptive and fixed procedures show a bias toward larger dlf's with adaptive procedures, but only at higher frequencies. A discussion of equal-interval and equal-ratio adaptive stepping rules indicates that an equal-ratio rule may be preferable.

Auditory Threshold↗
PubMed1977

An approach to calculating gain requirements for severely deaf children as a function of hearing loss which is independent of frequency response.

In an attempt to provide optimum listening conditions for hearing aid wearers a number of gain formulae have recently appeared in the literature. Required gains have invariably been measured at 1 kHz and expressed as a function of hearing loss. Recent research has shown that for children suffering from severe and profound deafness the 1 kHz required gain is highly dependent upon the low frequency response characteristic of the hearing aid. The present study proposes that the use of a 0.25 kHz gain formula would be advantageous with such children as it appears to be independent of the frequency response characteristic of the hearing aid.

Child↗
PubMed1978

Relationship between uncomfortable loudness level and acoustic reflex threshold as a function of hearing loss.

While many studies examined the relationship between Acoustic Reflex Threshold (ART) and Uncomfortable Loudness Level (UCL) and the possibility of predicting one from the other, no study to the authors' knowledge has shown the relationship between these two measures with respect to hearing loss, and uses such a relationship to predict either measure from the other plus the hearing loss. To achieve these relationships and uses, 80 Ss were studied, 20 in each of 4 groups, with normal hearing and with mild, moderate, and severe noise-induced hearing loss. ARTs and UCLs were obtained at 0.5, 1, 2, and 4 kc/s. The results demonstrated that db sensation level (SL) of ART and UCL were highly correlated with pure-tone hearing threshold level (HTL). An equation was derived: UCL (in db SL) - ART (in db SL) = 6 - .05 HTL, which shows that the relationship between ART and UCL varies as a function of hearing loss. Data from other studies were compared and discussed.

Adult↗
PubMed1998

[The Vibrant Soundbridge System: a new kind of hearing aid for sensorineural hearing loss. 1: Function and initial clinical experiences].

INTRODUCTION: Patients suffering from moderate to severe cochlear hearing impairment can not be considered for cochlear implantation on account of their relatively good residual hearing. Conventional hearing aids, on the other hand, have considerable disadvantages which clearly limit the benefit for these patients, e.g. feedback, sound distortion, unfavorable conditions for frequency transfer, occlusion, and recurrent infections of the auditory canal. In addition, many patients complain about a poor speech intelligibility in noise. VIBRANT SOUNDBRIDGE HEARING PROSTHESIS: Implantable hearing aids offer a new approach for improved auditory rehabilitation. The Vibrant Soundbridge system is based on an electromagnetic system, which is linked directly to the intact ossicular chain. Due to the high sound quality and the high frequency characteristic this system is ideally suited for the above-mentioned patient group. The usual disadvantages of conventional hearing aids can be avoided. Externally visible is merely the audio processor, which is worn in the retroauricular area and covered by hair. This processor transfers data and power via magnetic attachment transcutaneously. PATIENT POOL: During a European multicenter clinical study, 19 patients were implanted at MHH since February 1997. No significant complications occurred. RESULTS: In all patients, postoperative unaided pure tone threshold was unaltered in comparison with the preoperative recordings. The use of the audio processor leads to a significant functional gain, particularly in the high frequencies. The patients report about undistorted hearing resulting in a better speech understanding even in situations with loud background noise. CONCLUSION: The preliminary results show a promising new approach to the use of hearing prostheses for patients suffering from moderate to severe sensorineural hearing loss.

Audiometry, Pure-Tone↗
PubMed1998

[Studies of the evaluation of cochlea function with distortion product otoacoustic emission].

Distortion product otoacoustic emission (DPOAE) is one of the acoustic responses derived from the inner ears and it has very sharp frequency specificities. The purpose of this study is to investigate the utility of DPOAE as a method for evaluating human cochlea functions. The whole subjects of this study were 414 ears with inner ear hearing loss, 278 ears with normal hearing, 113 ears of neonates, 62 ears of the patients who were administered cisplatin, and 15 ears with functional hearing loss. At first, 5 studies described below were performed to examine the characters and reliability of DPOAE as a clinical test: 1) The average DPOAE-gram of normal hearing ears. 2) The comparison between DPOAE levels and hearing levels. 3) The reproducibility of DPOAE on the different examinations in the same subjects. 4) The influences of respiratory noises on DPOAE levels. 5) The detection of fine cochlea damages using DPOAE which can not be detected by pure tone audiometry. Then the following 2 clinical studies were performed: 6) The utility of DPOAE in neonates and children. 7) The utility of DPOAE in functional deafness cases. From these studies, the following characters of DPOAE were confirmed. Since DPOAE levels have correlations with hearing levels at the same frequencies, normal hearing could be determined by the DPOAE level. DPOAE is useful for following chronological changes in hearing because of its reproducibility in a same subject at different times. The early and fine damages on the cochlea that could not be detected by conventional pure tone audiometry might be detected by DPOAE in some cases. Considering these results, it was concluded that DPOAE could be a new objective hearing test which would help the conventional examinations. Especially in infants who can't be examined with pure tone audiometry, DPOAE is thought to be a very useful examination.

Acoustic Stimulation↗
PubMed2006

Auditory cortical responses evoked by pure tones in healthy and sensorineural hearing loss subjects: functional MRI and magnetoencephalography.

BACKGROUND: Blood oxygen level dependent functional magnetic resonance imaging (fMRI) and magnetoencephalography are new techniques of brain functional imaging which can provide the information of excitation of neurons by measure the changes of hemodynamics and electrophysiological data of local brain tissue. The purpose of this study was to study functional brain areas evoked by pure tones in healthy and sensorineural hearing loss subjects with these techniques and to compare the differences between the two groups. METHODS: Thirty healthy and 30 sensorineural hearing loss subjects were included in this study. In fMRI, block-design paradigm was used. During the active epoch the participants listened to 1000 Hz, sound pressure level 140 dB pure tones at duration 500 ms, interstimulus interval 1000 ms, which presented continuously via a magnetic resonance-compatible audio system. None stimulus was executed in control epoch. In magnetoencephalography study, every subject received stimuli of 1000 Hz tone bursts delivered to the bilateral ear at duration 8 ms, interstimulus intervals 1000 ms. Sound pressure level in healthy subjects was 30 dB; in sensorineural hearing loss subjects was 20 dB above everyone's hearing threshold respectively. All subjects were examined with 306-channel whole-scalp neuromagnetometer. RESULTS: In fMRI, all subjects showed significant activations in bilateral Heschl's gyri, anterior pole of planum temporale, planum temporale, precentral gyri, postcentral gyri, supramarginal gyri, superior temporal gyri, inferior frontal gyri, occipital lobes and cerebellums. The healthy subjects had more intensive activation in bilateral Heschl's gyri, anterior pole of planum temporale, inferior frontal gyri, left superior temporal gyri and right planum temporale than the hearing loss subjects. But in precentral gyri, postcentral gyri and occipital lobes, the activation is more intensive in the hearing loss subjects. In magnetoencephalography study, both in the hearing loss and the healthy subjects, the most evident audio evoked fields activated by pure tone were N100m, which located precisely on the Heschl's gyrus. Compared with the hearing loss subjects, N100m of the healthy subjects was stronger and had longer latencies in right hemisphere. CONCLUSIONS: Under proper pure tone stimulus the activation of auditory cortex can be elicited both in the healthy and the sensorineural hearing loss subjects. Either at objective equivalent stimuli or at subjectively perceived equivalent stimuli, the auditory responses were more intensive in healthy subjects than hearing loss subjects. The tone stimuli were processed in a network in human brain and there was an intrinsic relation between the auditory and visual cortex. Blood oxygen level dependent fMRI and magnetoencephalography could reinforce each other.

Acoustic Stimulation↗
PubMed1984

Congenital and postnatally acquired cytomegalovirus infections: long-term follow-up.

To determine long-term outcome of children with inapparent congenital cytomegalovirus infection, an assessment of congenitally infected children observed since birth was undertaken. Children with early postnatal acquisition of CMV infection were also evaluated. Cognitive, behavioral, neurologic, audiometric, and speech and language evaluations were performed in 48 patients, including 17 congenitally infected children, 10 children with postnatal infection, and 21 uninfected control subjects. Mean IQ of the three groups of children did not differ significantly. Behavioral, neurologic, speech and language examinations similarly failed to distinguish differences among the three groups. Audiologic abnormalities were present in four congenitally infected children, including one child with a severe unilateral sensorineural loss; in none of the children was hearing loss functionally significant. No hearing abnormalities were detected in postnatally infected children. Although inapparent CMV infection can result in audiologic sequelae, the continued lack of cognitive, behavioral, and neurologic sequelae in these school-age children reemphasizes the need to focus attention on prevention of primary maternal CMV infection to avoid the potentially devastating effects of intrauterine CMV infection.

Audiometry↗
PubMedDate not supplied

Functional recovery in the avian ear after hair cell regeneration.

Trauma to the inner ear in birds, due to acoustic overstimulation or ototoxic aminoglycosides, can lead to hair cell loss which is followed by regeneration of new hair cells. These processes are paralleled by hearing loss followed by significant functional recovery. After acoustic trauma, functional recovery is rapid and nearly complete. The early and major part of functional recovery after sound trauma occurs before regenerated hair cells become functional. Even very intense sound trauma causes loss of only a proportion of the hair cell population, mainly so-called short hair cells residing on the abneural mobile part of the avian basilar membrane. Uncoupling of the tectorial membrane from the hair cells during sound overexposure may serve as a protection mechanism. The rapid functional recovery after sound trauma appears not to be associated with regeneration of the lost hair cells, but with repair processes involving the surviving hair cells. Small residual functional deficits after recovery are most likely associated with the missing upper fibrous layer of the tectorial membrane which fails to regenerate after sound trauma. After aminoglycoside trauma, functional recovery is slower and parallels the structural regeneration more closely. Aminoglycosides cause damage to both types of hair cells, starting at the basal (high frequency) part of the basilar papilla. However, functional hearing loss and recovery also occur at lower frequencies, associated with areas of the papilla where hair cells survive. Functional recovery in these low frequency areas is complete, whereas functional recovery in high frequency areas with complete hair cell loss is incomplete, despite regeneration of the hair cells. Permanent residual functional deficits remain. This indicates that in low frequency regions functional recovery after aminoglycosides involves repair of nonlethal injury to hair cells and/or hair cell-neural synapses. In the high frequency regions functional recovery involves regenerated hair cells. The permanent functional deficits after the regeneration process in these areas are most likely associated with functional deficits in the regenerated hair cells or shortcomings in the synaptic reconnections of nerve fibers with the regenerated hair cells. In conclusion, the avian inner ear appears to be much more resistant to trauma than the mammalian ear and possesses a considerable capacity for functional recovery based on repair processes along with its capacity to regenerate hair cells. The functional recovery in areas with regenerated hair cells is considerable but incomplete.

Aminoglycosides↗
PubMed1998

Cochlear origin of hearing loss in MELAS syndrome.

There have been few studies investigating the mechanism and nature of the hearing loss that occurs in the mitochondrial disorders. We studied 18 patients with the MELAS A3243G point mutation from four different kindreds. Pure tone audiometry, speech discrimination testing, acoustic reflexes, tympanometry, and brain stem auditory evoked responses were performed to localize the site of pathology in the auditory pathways. In 12 patients, we performed electrocochleography and otoacoustic emissions to assess cochlear involvement. Neuroimaging and promontory nerve stimulation were performed to exclude retrocochlear pathology. Audiological testing confirmed sensorineural hearing loss in 14 of the 18 patients studied; hearing loss was usually gradual in onset, was symmetrical, and initially affected the higher frequencies. In some patients, there were features that distinguished the hearing loss from presbyacusis, including a young age at onset, asymmetrical involvement, stepwise progression, and partial recovery. We treated one patient who had profound bilateral hearing loss with cochlear implantation; this restored good functional hearing. Hearing loss in MELAS syndrome appears to be due to dysfunction of the cochlea, probably resulting from metabolic failure of the stria vascularis and outer hair cells. Cochlear implantation is a therapeutic option worth considering in those patients who become deaf.

Adolescent↗