Search

Competing Interests: Die folgenden Verbindungen bestanden, stehen aber nicht in Beziehung zu o.g. Manuskript:Otonomy Inc., San Diego, USA: Consultancy; AudioCure Pharma GmbH, Berlin, Germany: Head of Scientific Advisory board; MedEl, Austria and MedEl, Germany: Research grant, travel supports for lectures; Federal Ministry of Education and Science in Germany: Research grant for clinical trial; Berufsverband HNO-Ärzte Deutschland e.V.. Honorary for Lectures; Merck Serono, Darmstadt, Germany: Honorary for Lectures; Hoffmann-La Roche, Basel, Switzerland: Consultancy; Boehringer Ingelheim, Germany: Consultancy; Cochlear Ltd., Australia: travel support; Infectopharm, Germany: Honorary for Lectures

According to current knowledge, it must be assumed that temporary idiopathic hearing loss and its spontaneous remission are based on mechanical and/or pathological alterations in the inner ear. The causal mechanisms might be based on inter-individual variations. Induced by dose-dependent activators, temporary as well as permanent damage might occur. Sudden hearing loss may be initiated by an increase in the local nitric oxide (NO) concentration. Spontaneous remission, i.e. functional restoration, can be explained by a local decrease in the NO concentration. In this context, regulatory systems such as the gap-junction system, blood vessels or synapses might be affected. In addition, alterations in the hormone level of estrogen and mineralocorticoids, as well as cellular glutathione and vitamin levels, might lead to temporary alterations in the inner ear. Recent experimental findings indicate a role for the shuttle protein Survivin in the spontaneous remission of sudden hearing loss.

Tinnitus is one of the 20 most common reasons why patients aged 45-64 years consult a general practitioner in Germany. In the literature a correlation is claimed between disease patterns of the cervical spinal column and nuclei of cerebral nerves. In the case report presented here, a 30-year-old female patient with acute tinnitus after acute hearing loss was cured from her tinnitus after a single manual medical treatment at C0/C1, which supports the correlation claimed. If cervical spine diseases should therefore regularly be included in the differential diagnoses of acute tinnitus is a matter of further research., (2010 S. Karger AG, Basel.)

A perilymph fistula is a possible cause for sudden unilateral sensory deafness. In this retrospective study the data of 73 patients with unilateral sudden deafness were analyzed. All of them underwent an exploratory tympanotomy during which both windows were packed with soft tissue. Postoperatively all patients received rheological therapy with pentoxifyllin and steroids. The following possible prognostic indicators were analyzed: age, sex, tinnitus, vertigo, vomiting, spontaneous nystagmus, positive fistula test, time between onset of symptoms and therapy, intraoperative proof of a perilymph fistula, and signs of barotrauma in the patient's history. A significant postoperative recovery of the hearing loss (>20%) was found in 29 patients (39.7%) (group 1), and 44 patients (60.3%) showed only an increase of <20% (group 2). The statistical analyses showed the following significant difference: The symptoms vertigo (p=0.002) and spontaneous nystagmus (p=0.014) occurred more frequently in group 2 (patients with a poor hearing recovery) than in group 1. Patients with a barotrauma,however, had an overproportionally good outcome (50-100% hearing recovery). A perilymph fistula was seen intraoperatively equally often in both groups. In summary, the symptoms vertigo and spontaneous nystagmus are indicators of complex damage in cases of sudden deafness and are associated with a worse prognosis concerning hearing recovery. Exploratory tympanotomy in combination with drug treatment is a reasonable therapy as an ultima ratio in every case of unilateral sudden deafness.

Background: Acoustic neuroma is one of the classic causes of retrocochlear hearing disorders. Auditory brainstem response (ABR) testing with a sensitivity of about 90 % in the diagnosis of acoustic neuroma plays an important role as a screening method., Case Report: A patient suffering from an acute, unilateral sensorineural hearing loss with tinnitus recovered for a short period of time after being treated with low density lipoprotein (LDL-) apheresis improving hemorheology. Surprisingly, the reason for the sudden hearing loss was a tumor in the cerebellopontine angle with a maximum diameter of 2 cm, which was detected by magnetic resonance imaging (MRI). Auditory brainstem response testing (ABR), audiovestibular tests and other standard tests were negative concerning this diagnosis. There was no previous evidence for an acoustic neuroma in all investigations of cochlear disfunction except MRI., Conclusions: The acoustic neuroma caused a compression of the labyrinthine artery in the inner auditory canal resulting in an acute unilateral hearing loss with tinnitus. Low Density Lipoprotein-apheresis was able to achieve a short term improvement of blood supply to the inner ear. Thus the compression of the labyrinthine artery caused by an acoustic neuroma could be compensated for the duration of about one week. Within this time the effect of LDL-apheresis decreased more and more. After a sudden unilateral hearing loss an acoustic neuroma must be ruled out even if the retrocochlear testing by ABR primarily shows no prolonged interpeak latency.

Background: A sudden unilateral hearing loss in childhood is commonly regarded as an unusual event., Case-Report: In this paper, we report of a 10 year old girl suffering from a unilateral right-sided sudden hearing loss following an acute infection with Mycoplasma pneumoniae, which is also rarely observed. Maximum hearing loss was estimated around 30 dB (0.25-6 kHz), which resolved after infusions using plasma expanders. Both transitory and distortion product otoacoustic emissions remained absent, while latencies of acoustically evoked potentials were normal., Discussion: This suggests a damage of outer hair cell function as an explanation for the observed hearing loss. The coincidence of two rare events for children such as a spontaneous sudden unilateral hearing loss combined with an infection with Mycoplasma pneumoniae gives rise to the hypothesis of a causal relationship that is discussed according to the literature.

Background: Problems of frequency-specific objective assessment of hearing threshold by means of auditory brainstem response (ABR) have been discussed recently. While a number of workers have recommended methods of selective masking to improve the frequency specificity, others believe that frequency-specific potentials can also be obtained without masking. In this context, the effects of rise-decay time and high-pass masking on ABRs were investigated., Method: ABRs were recorded in normal-hearing subjects and patients with high and low frequency hearing loss by means of surface electrodes between the vertex and the ipsilateral mastoid. The frequency of the stimulus was 1 kHz, and the rise-decay time 1 ms (1-0-1) or 2 ms (2-0-2). High-pass filtered noise (cutoff frequency 1.5 kHz; filter slope 250 dB/octave) was employed for masking. Particular attention was paid to the problem of efficient masking., Results: In normal-hearing subjects under the influence of high-pass masking compared to non-masked ABRs, longer mean latencies and diminished means of the amplitudes of wave V were found, with differences in the near-threshold domain being less pronounced. Similar results were observed in patients with high frequency hearing loss. In patients with low frequency hearing loss, the influence of high-pass masking was especially marked distinctly near to threshold. Furthermore, latency and amplitude differences of wave V of the 1-0-1 and the 2-0-2 stimuli were determined from the ABRs obtained with and without high-pass masking. The differences between the latency differences of both stimuli in the suprathreshold range (70 dB nHL) only were statistically significant., Conclusions: The results are suggestive of an inadequate frequency specificity of unmasked stimuli in the suprathreshold range. Evaluation of the latencies revealed for both rise-decay times a similar frequency specificity near the threshold and a higher frequency specificity of the longer stimulus in the suprathreshold range.

Central to inner ear research are questions regarding the homeostasis of the high endolymphatic potassium concentration (approximately 150 mmol/l) and the high endocochlear potential (approximately +80 mV). Disturbances of the endocochlear potential can lead to the immediate loss of hearing which may be irreversible. The molecular mechanism leading to the generation of the endocochlear potential has not yet been discovered in spite of its clinical relevance. It is long known, however, that the stria vascularis is responsible for both the generation of the endocochlear potential as well as the secretion of potassium into endolymph. Recent investigations have clarified the mechanisms leading to the secretion of potassium and have led to the formulation of a now widely accepted model. This model explaining potassium secretion as well as the generation of the endocochlear potential is discussed in the present article.

This study examined retrospectively the spontaneous recovery of patients with an acute peripheral vestibular deficit in order to determine whether the caloric test response and with it vestibular function improves over time. The caloric bithermal was tested three times on 79 patients who were hospitalised with an acute deficit. The first test was recorded on emergency admission by observing nystagmus beats under the Frenzel glasses. Two to five days later a complete electronystagmus (ENG) examination was performed. A second ENG was performed, on average, 4 months later. 46% of the patients recovered a normal caloric canal paresis value (less than 32%). By comparing the canal paresis values in the first and second ENG an improvement exceeding 30% was demonstrated in 50% of the patients and there was no correlation between the extent of the canal paresis deficit and the amount of recovery. A simultaneous cochlear deficit had no influence on the recovery of vestibular function.

Unlabelled: The etiology of sudden hearing loss is still not known. Among other pathomechanisms, vascular disorders have been proposed as an underlying cause. We investigated patients with sudden hearing loss for signs of microcirculation disorders., Patients: Thirty-one patients (15 males and 16 females, mean age 37.5 years) were admitted because of sudden hearing loss. In addition, all patients underwent thorough ophthalmologic examination., Results: Refraction, vision, intraocular pressure and excavation of the optic nerve were normal. In 81% of all cases abnormal conjunctival vascular ectasia were detected. The fundus of 53% of the patients was characterized by slight tortuosity of arterial retinal vessels. The first 11 patients received an ocular electrophysiological examination and showed normal ERG, P-VEP and dark adaptation. Standard perimetry (TAP 2000 ct) revealed no abnormalities in 29 patients, 2 glaucoma patients had small arcuate scotomas. However, 90% of all 29 scotoma-free patients were abnormal in the white-noise campimetry (TEC, Oculus). Testing for color vision (Roth 28 HUE, desat.) revealed abnormally high values (right 120, left 131)., Conclusion: Although patients with sudden hearing loss appeared healthy in the standard eye examination, the more sensitive tests of white-noise campimetry and color vision, together with the conjunctival vascular ectasia and the tortuosity of arterial retinal vessels, indicated a disturbed microcirculation.

Background: A new method of diagnosis of endolymphatic hydrops by recording low-tone masked evoked otoacoustic emissions (TEOAE) is presented., Methods: A short acoustic stimulus and a masker tone of 30 Hz are applied in an adjustable phase relation simultaneously to the same ear. In the normal hearing ear the masker shows little influence on the TEOAE at 0 degrees, whereas the suppression at phase 270 degrees (maximal rarefaction at the eardrum) is nearly complete. However, in cases of endolymphatic hydrops this masking effect is reduced or absent, indicating impaired mobility of the basilar membrane., Results: The masked TEOAE were recorded of patients with normal hearing, Menière's disease, and sudden hearing loss without vertigo. In Menière cases with supposed endolymphatic hydrops, the amplitude modulation of the emissions was found to be much less than in the other groups., Conclusions: Where TEOAE can be recorded, low-tone masking is a quick, objective, and noninvasive method for the diagnosis of endolymphatic hydrops.

Intracranial pressure is transmitted to the perilymph of the cochlea via the cochlear aquaeduct and via Reissner's membrane to the endolymph. The "Tympanic Membrane Displacement Analyser (TDA)" is a new device that may be a useful non-invasive method to show intracranial and intracochlear pressure changes indirectly measured by tympanic membrane displacement during stapedial reflex contraction. The TDA was utilised in 20 normal persons and in 29 patients with unilateral diseases of the inner ear. No significant differences in the tympanic membrane displacement were found between patients with sensorineural hearing loss, patients with Ménière's disease, and normal persons.

It was the scope of two previous papers to outline the basics and possible neurotological applications of the tympanic membrane displacement technique (TMD). The present paper is aimed at briefly reviewing five distinct cases where a sudden sensorineural hearing loss with/without tinnitus could be monitored by the TMD technique. These features include a sudden hearing loss upon lumbar puncture, the audiological manifestation of an internal hydrocephalus, stapes gusher and the round window rupture. All symptoms are based on a change in intracranial and intracochlear pressure which in turn leads to the manifestation of the previously unknown sensorineural hearing loss and/or tinnitus.

The influence of vestibular dysfunction on severity of initial hearing loss and success of therapy was evaluated in a retrospective study with 142 patients suffering from idiopathic sudden hearing loss. In 48% of these patients combined cochleovestibular disorders were found. In this group mean hearing loss was significantly higher (45 dB versus 32 dB) and hearing recovery after therapy (25% versus 38%) less pronounced. 83% of patients with high grades of vestibular disorders (Vestibular Index: > or = 9) showed a decrease of hearing function between 50 dB and 130 dB compared to 32% in the group with signs of low vestibular dysfunction. Additional vestibular lesion in patients with sudden deafness can be used as a criteria for prognosis. High grade vestibular lesion reduce the probability of complete hearing recovery.

Existing investigations of cervical hearing disorders have been carried out predominantly in patients in whom those with vertebrobasilar insufficiencies (VBI) could not be distinguished from patients suffering from functional deficit of the upper cervical spine. Since two different syndromes exist, no statement can be made about cervical hearing disorders. That deafness can occur in VBI is uncontested. In contrast, there remains a dispute whether a "vertebragenic hearing disorder" exists. This latter disorder is believed accompanied by tinnitus, a feeling of ear pressure, otalgia and deafness as symptoms of a functional deficit of the upper cervical spine. In reviewing the medical findings of 259 patients with well-defined functional deficits of the upper cervical spine and symptoms of cervical vertigo, subjective hearing disorders occurred in 15%. Audiometric threshold shifts of 5-25 dB, most often in lower frequencies, were observed in 40%. Additionally, results of click-evoked otoacoustic emissions (OAE) were negative in spite of approximately normal hearing. Findings in 62 patients suffering from vertebragenic hearing disorders are reported before and after chiropractic management. Results indicate that these hearing disorders are reversible, as demonstrated by audiometry and OAE. The therapy of choice is chiropractic manipulation of the upper cervical spine. The commoness of vertebragenic hearing disorders emphasizes their clinical and forensic importance.

23 patients with unilateral hearing loss and presence of TEOAE on both ears were examined with regard to the association between contralateral acoustic stimulation and evoked otoacoustic emissions. Contralateral white noise of 40 dB HL, 50 dB HL and 60 dB HL decreases the amplitude of TEOAE both at the ear with and without hearing loss. In comparison with a group of subjects with normal hearing, the decrease in the amplitude of TEOAE was less in the group of patients with hearing loss. Two paradoxical cases were found with an increase in the TEOAE amplitude in the ear with hearing loss during contralateral stimulation. It is assumed that impairment of the efferent synapses on the outer hair cells or of the decussated efferent nerve fibres at the floor of the fourth ventricle contributes to the pathogenesis of hearing loss. Impairment of the efferent system at brainstem level can explain the lower decrease in the amplitude both in the diseased ear and the normal ear. The behaviour of the normal ear during stimulation of the diseased ear can also be caused by disturbance of the afferent system.

In 50 patients auditory threshold and brain stem evoked potential studies were carried out before and after myelography. Due to the analysis of amplitudes and latencies of auditory brain stem measurements, significant functional disorders of the hearing organ and the auditory pathway could be demonstrated. In most of the patients these functional disorders were found to be subclinical, whereas 12 patients showed alterations extending from a subjectively slight hearing loss to an audiometrically objectified acute hearing loss depending on its intensity in each case. The reasons of these functional disorders could not be clarified. An open cochlear aqueduct through which perilymph enters the subarachnoidal space leading to a secondary endolymphatic hydrops can be considered as the cause in cases where manifest symptoms develop. The changes in brain stem audiometry can be additionally explained by changes in osmolality of the inner ear fluids which may lead to the development of an endolymphatic hydrops.

Cochlear and vestibular effects of variation of middle ear pressure have been discussed for more than 50 years. In 1975, Densert reported on temporary improvement of hearing threshold in patients suffering from Ménière's disease after reduction of air pressure in a pressure chamber. One year later Ingelstedt observed reduction of spontaneous nystagmus under this condition. The transmission of relative overpressure from the middle ear to the labyrinth with consecutive decongestion of the hydropic endolymphatic system to the saccus was thought to be the cause of this phenomenon. Meanwhile a direct effect of labyrinthine pressure on the activity of the inner ear and labyrinth in healthy subjects and animals has been described. In order to gain further information on the phenomenon observed by Densert and Ingelstedt we performed investigations on patients suffering from several cochleovestibular diseases. Chamber pressure was altered and simultaneous electronystagmographic recording was performed. As a striking result, we observed a reduction of the pathological spontaneous nystagmus in a majority of patients with vestibular damage during and after the reduction of chamber pressure. Many of these subjects showed a partial restitution of nystagmus if the chamber pressure was increased again. This observation was made in patients with Méni ere's disease, vestibular neuritis and sudden hearing loss with vestibular involvement. Hence, we conclude that this phenomenon is not specific for Ménière's disease. A transmission of middle ear pressure to the labyrinth may be an explanation of our observations.

The suitability of transiently evoked otoacoustic emissions (TEOAE) for the observation of changes in inner ear function was examined in 28 normal hearing subjects and 25 patients with sudden deafness. The measurements were performed with the ILO88 system using a nonlinear sequence of click stimuli. The TEOAEs of both ears of each control subject were measured in 7 sessions at 4 different stimulus levels. The evaluation involved visual inspection of the time-dependent records and an analysis of response amplitudes and cross correlational coefficients. Minor changes in curve appearance and variations of the response level in the order of +/- 4 dB could be detected which were attributed to the noise floor and variations in probe application. Within the patient group, at least three TEOAE measurements were performed during the course of rheologic therapy. Pure-tone audiograms were recorded prior to each TEOAE session for comparison. The most significant result from the data analysis is that response amplitudes increase significantly in almost all cases in which pure-tone audiometry reveals normalization of hearing threshold. This indicates a recovery of outer hair cells. Furthermore, comparison of TEOAE records obtained in different sessions shows an initial growth of correlation coefficients followed by a final saturation. These findings indicate that the combination of amplitude evaluation and correlation analysis is suitable for observing changes in intensity and waveform of cochlear emissions.

The function of the Eustachian tube was assessed in 50 patients suffering from sudden hearing loss, using the pressure chamber impedance method. We investigated the existence of a relationship between sudden hearing loss and the occurrence of a patulous tube. We could not observe more patulous tubes in patients with sudden hearing loss compared to a control group with healthy ears. We registered the parameters "tissue resistance pressure" and "tubal opening pressure", both characterising the passive qualities of the Eustachian tube. There was no indication of a facilitated passive tubal opening nor a tendency to a patulous tube among our patients. Statistical evaluation of these parameters showed no significantly different tubal function on comparing the right and the left ear, as well as the ear affected by sudden hearing loss and the healthy ear. We conclude that there is no relationship between sudden hearing loss and patulous tube.

In the course of a pilot study, the effects of fibrinolysis treatment with rt-PA (Actilyse, Thomae, Biberach; 100 mg) on the recovery of hearing and hemorheologic parameters were investigated in 12 patients suffering from sudden hearing loss. The authors' approach was based on two considerations, (1) the possibility that, in cases of sudden hearing loss, the oxygen supply to the cochlea is totally or partially blocked by a microthrombus which could be dissolved by rt-PA and (2) the fact that lysis markedly improves the flow characteristics of the blood (as has been demonstrated in studies with streptokinase and urokinase). This human-plasminogen activator, produced by genetic engineering, is superior (also in terms of possible side-effects) to other lysin enzyme preparations (streptokinase, urokinase) because of its highly selective effects on thrombi (low activation of plasma plasminogen) and its short half-life (approx. 4 min). In nine of 12 patients, the treatment brought about a marked improvement in hearing or its complete restitution (three of the patients has initially been deaf in the affected ear). In the three nonresponders there was serologic evidence of an inner-ear infection (neuroborreliosis, cytomegaly, influenza B). The most interesting rheologic effect was the reduction of plasma viscosity (average decrease about 18%), since such a reduction cannot be attained with other, comparable rheologic measures (isovolemic or hypervolemic hemodilution). In spite of the convincing hemorheologic effects, fibrinolysis cannot yet be recommended as treatment for sudden hearing loss. Further studies have still to be done on spontaneous healing, the effects of hemorheologic measures on the blood circulation of the inner ear, and on the oxygen requirements of the ear affected by hearing loss.

Three patients with complete sudden unilateral hearing loss suffered from delayed vertigo and presented a strong geotropic positional nystagmus during the evolution of their disease. Significant direction-fixed spontaneous nystagmus was absent, although two of the three patients had markedly decreased caloric responses on the diseased side. These findings are considered as a special form of peripheral cochleovestibular disorder restricted mainly to the cochlea and the otolith organs.

At some patients suffering from Menières disease or sudden hearing loss the brainstem potentials and the late auditory potential N1 were registered. It could be shown that an exact differential diagnosis between these both illnesses was not possible by means of these acoustically evoked potentials. Furthermore it was not possible by means of these potentials to predict an improvement of hearing at patients with sudden hearing loss. Finally the potentials showed an increasing of their latencies which was greater at patients with sudden hearing loss than at patients with Menières disease.

Rupture of the round window has been mostly described as an occasional lesion with sudden deafness and vertigo after diving, flying and physical stress, usually according to the patient's history. It also seems to be the cause of sudden progressive hearing loss of so-called idiopathic origin as shown by the findings in 6 out of 9 patients with perilymphatic fistulas. Therefore, transtympanic endoscopic evaluation of the round window is performed in the author's clinic as a routine measure in patients with sudden complete deafness or severe progressive loss of hearing even without any pointer to a possible trauma.

Theories concerning the influence of function disturbances of the cervical spine on the development of sudden hearing loss should be re-examined. Forty-eight patients with unilateral sudden hearing loss and 94 controls were examined. X-rays of the cervical spine were made in lateral projection in normal position, and in maximum flexion and extension. The posture and movement of the vertebrae were recorded using a computer digitalization program. Overall mobility of the cervical spine was reduced in the group with sudden hearing loss: reduced movement was found in segments C2/C3, C5/C6, and C6/C7. Segments P0/P1 and C1/C2 were conspicuous by very high standard deviations in the group with sudden hearing loss. The results indicate that sudden hearing loss and function disorders of the cervical spine are related.

For more than 15 years, rupture of the round window membrane has been discussed as a possible cause of sudden sensineural hearing loss. With regard to the pathophysiological basis for such a rupture, an abnormality in the width of the aquaeductus cochlearis as well as anatomical variations of the round window membrane itself are presumed as causative factors. The authors report on six cases concerning this incidence. The indication for tympanoscopy and the technique for the closure of the round window fistula are reported. In 5 of the 6 patients treated in this manner, surgery the resulted in a significant and lasting hearing gain.

Report on 77 patients with sudden deafness, of whom 59 (77%) showed elevated erythrocyte sedimentation rates (ESR). Beside the ESR the patient-collective was subdivided into age, sex, audiogramm-findings and conception of therapy. A discussion follows and conception of therapy. A discussion follows about the possible reasons of the ESR-elevations as virus diseases or the pharmacogenic influence of the ESR by low molecular dextrans.

Acute auditory damage is produced in 127 guinea pigs. In one group (n = 68), sound damage is effected (140 dB SPL white noise for 5 min.). In a second group (n = 59), the cochlea is damaged with a cryoprobe (-196 degrees C, 1 min.). Both methods produce a middle-grade hearing impairment. With the aid of indwelling cathodes, the input-output curves are determined before and after the damage as well as up to the 16th postoperative day. Statistical comparison of the results shows that the reaction of the cochlea to the same irritation can be very different. This must be taken into consideration especially in evaluating the effect of pharmaca.

The clinical features of pontine deafness are demonstrated in six patients with typical findings in pure-tone audiogram and speech audiometry, in stapedial reflex recordings, in brainstem-evoked potentials and cortical evoked potentials. The results of positron emission tomography (PET), magnetic response tomography (MRT), electrocochleography and the promontory test are presented. Results of cerebral spinal fluid and antibody titres in the serum are given. Pontine deafness is characterised by an interruption of the central auditory pathway cranial to the olive. This deafness should be differentiated from other forms of sensorineural hearing loss. The pathogenesis of pontine deafness is still unknown. However we should take it into consideration in the diagnosis of sudden unilateral deafness. No medical treatment method is known to date.

112 cases of sudden unilateral deafness are reported 62% of the patients were treated by means of improving microcirculation, in 38% the treatment was based on drugs with different points of attack. The results of the both groups were compared. There was no significant difference between the audiometric results after therapy. In 84% of the patients the treatment started within 14 days after the sudden hearing loss. The early treatment within 3 days after the occurrence showed no better results than the beginning of treatment later than 3 days.

A case of sudden deafness is described. Examination showed a mixed deafness on the left side, suspected to be due to fixation of the stapes and a simultaneous perilymph fistula. These lesions were proved by an exploratory tympanotomy. The interesting aspects of the case are emphasized: firstly recruitment caused by a perilymph fistula, present for 5 months without causing complete deafness; secondly improvement of hearing after surgical treatment of both pathological conditions; and finally the complete absence of vestibular signs.