Total Deafness Research Articles

遲發性 内淋巴水腫

Delayed endolymphatic hydrops(DEH), a clinical entity which was described as a syndrome with the characteristic clinical findings such as rotatory vertigo or fluctuating hearing loss on the normal ear appearing a few years after unilateral severe sensori-neural hearing loss or total deafness. DEH can be differentiated from Meniere’s disease, and was first reported simutaneously by Nadol et al and Wolfson et al in 1975, and was specified by Schuknecht in 1978. Schuknecht, in 1985, classified DEH into 3 types ; 1) ipsilateral : rotatory vertigo only, 2) contralateral: rotatory vertigo with fluctuating hearing loss on the normal side, and 3) bilateral. In many cases the hearing loss was caused by juvenile unilateral total deafness of unknown causes, viral diseases such as mumps, other postinflammatory or posttraumatically. There is no tendency of rule in latency between hearing loss and vertigo or fluctuating hearing loss on opposite ear, and it is more than 10 years in many cases. Diagnosis is made from history, clinical findings, audiologic and equilibrium function test. Treatment is similiar to that of Meniere’s disease with conservative (vasodilators, autonomic nerve controlling drugs, steroid, antivertiginous drugs, low salt diet, etc) and surgical method (endolymphatic sac surgery or labyrinthine destruction) in case of no effect by conservative. But, in general, the result of treatment is poorer than in Meniere’s disease.

Effect of contralateral auditory stimuli on active cochlear micro-mechanical properties in human subjects

The present study investigates the possibility that contralateral auditory stimulation along medial efferent system pathways may alter active cochlear micromechanics and hence affect evoked oto-acoustic emissions in humans. A first experiment, involving 21 healthy subjects showed reduction of oto-acoustic emission amplitude under low intensity contralateral white noise (from 30 dB SPL, 10 dB SL, upwards). The effect is found for intensities below the acoustic reflex threshold (85.2 dB HL). A second experiment, involving 10 of the above 21 subjects, sought to rule out any technical artefact. Recording was again carried out, but after sealing of the contralateral ear with a silicon putty plug. No contralateral intensity effect on oto-acoustic emission amplitude was found for contralateral intensities below 65 dB SPL. In subjective perception terms (dB SL) an effect was found under sealing when the sound reached or passed above the 10 dB SL level. These two findings confirm the preceding experiment. The third experiment investigated the role of transcranial transmission of the contralateral auditory stimulus. 16 subjects having total unilateral deafness and one healthy ear were tested by the same procedure as above. No fall-off in oto-acoustic emission amplitude was found for contralateral stimuli equal to or less than 80 dB SPL. There is thus a contralateral auditory stimulus effect on active cochlear micromechanics. The most appropriate explanation involves the medial cochlear efferent system, excited at brainstem level via the afferent auditory pathways. Alteration of active cochlear micromechanics seems promising at a basic level, pointing, as it does, to an interactive cochlear functioning which can be investigated by simple, non-intrusive, objective techniques which can be used with human subjects. We have here a model for functional exploration of the medial olivocochlear efferent system.

ムンプスによる両側聾の一症例

A case of bilateral total deafness after mumps infection is repotred. A 6-year-old boy was referred to us because of sudden hearing impairment in the course of mumps parotitis. Physical examination showed no abnormalities except bilateral parotid swellings. Neurological examination revealed no signs of meningitis or disepuilibrium. The audiogram showed marked bilateral hearing loses at allfrequencies. A diagnosis of sensorineural deafness following mumps was made. High dose of steroid and vasodilators was administered for ten days. However, he did not recover.

聴神経腫よう術後，対側の急性感音難聴をきたした２症例

Two patients suffered from acute hearing loss of the contralateral side after acoustic tumor surgery were reported. The first patient was a 42-year-old-male. He had had a right progressive hearing loss over two years and CT scan revealed a mass of 20mm in diameter in the right cerebellopontine angle. The patient noticed the contralateral hearing loss on next morning after the total removal of tumor by translabyrinthine approach. However he had no complaint of vertigo or facial palsy. An audiogram of the contralateral side just after the onset showed flat type audiogram of sensorineural hearing loss with positive recruitment. With steroid therapy for two weeks, hearing and ABR findings improved and returned to nearly normal. The second case was a 47-year-old male. The patient had chronic renal failure treated by hemodialysis. His hearing loss in the right ear had gradually decreased over two years and an acoustic tumor of 2.5cm in diameter was demonstrated by CT scan. The tumor was removed by translabyrinthine approach. Nine day after the operation, he noted total deafness in contralateral ear and vertigo. He was given steroid hormone and his hearing improved up to 68 dB. Four cases have been reported in the literature. The mechanisms of acute sensorineural hearing loss observed in these cases were discussed. The cause remained unknown, however there were some hypothesis such as the compression to the opposite brainstem, peripheral nerve or feeding vessels by tumor or brain edema after operation, and local vasospasms might be also the cause of hearing impairment.(ABSTRACT TRUNCATED AT 250 WORDS)

Temporal Bone Pathology in Intracochlear Schwannoma with Profound Hearing Loss

Intracochlear schwannoma was found in the temporal bone of a 85-year-old man in whom audiometric study, 26 days before death, had shown total deafness in the left ear. The tumor occupied the entire lumen of the cochlea in the basal turn involved Rosenthal's canal, but it occupied only the scala tympani in the second turn. Intralabyrinthine schwannomas are difficult to diagnose by clinical examination. They were discovered accidentally during destructive labyrinthectomy for presumed Ménière's disease or discovered incidentally by postmortem temporal bone pathology. Although intralabyrinthine schwannomas are a rare occurrence and cannot usually be diagnosed without surgery or postmortem histopathology, it is important to suspect the possibility of their existence in differential diagnosis of atypical Ménière's disease or unilateral idiopathic progressive deafness. Long-term follow-up is obviously necessary to exclude the tumor.

Electrophonic Hearing and Cochlear Implants.

It has been difficult to explain the good speech understanding obtained by some cochlear implant patients fitted with a single-channel electrode and analog transmissions of the speech signal (Vienna/3M implant). It has also been difficult to explain the variation in results reported by different groups using the same implant. One hypothesis asserts that the above differences can be explained by the observation that electric stimulation with an implanted electrode might result in two different auditory sensations, the first resulting from the stimulation of the remaining hair cells (electrophonic component) and the second from the electric stimulation of the auditory nerve (electro-neural component). The two sensations are very different. As a result of different definitions of total deafness (functional or threshold definition), patients with remaining hair cells are operated on by some groups, but not by other groups. Some published results from different studies are discussed with reference to the above hypothesis and the possible consequences for the selection of the patients, the use of extra-or intracochlear electrodes, and the selection of the speech coding strategy are discussed.

Development of speech-like vocalizations in a child with congenital absence of cochleas: The case of total deafness

ABSTRACTIn order to assess the effect of total deafness on vocalization development, tape recorded utterances of a 3-year-old child who was born without cochleas were examined. In the beginning of the study, the subject's speech consisted almost exclusively of small numbers of sounds characteristic of early infancy. Across the study, the subject participated in extensive vocal stimulation activities. He also initially received intermittent exposure to tactile speech information via a 16-channel vocoder and, subsequently, a 2-channel tactile aid. Following introduction of the 2-channel aid, the subject made rapid improvement in the quality of his vocalizations, which consisted increasingly of speech-like utterances, including well-formed or canonical syllables. These results suggest that, although hearing impairment slows the onset of canonical babbling, even total deafness does not preclude its eventual appearance.

Survival of spiral ganglion cells in profound sensorineural hearing loss: implications for cochlear implantation.

Ninety-three temporal bones from 66 patients who were profoundly deaf during life were reconstructed by analysis of serial light microscopic sections. The correlations of total and segmental spiral ganglion cell counts with age, duration of hearing loss and profound deafness, and cause of hearing loss were evaluated. Bivariate analysis demonstrated that total spiral ganglion cell count tended to be lower in older than in younger deaf individuals and lower with longer duration of hearing loss and total deafness. However, multiple regression analysis demonstrated that the cause of hearing loss was the single most significant determinant of total spiral ganglion cell count. Patients with deafness due to aminoglycoside toxicity or sudden idiopathic deafness had the highest residual spiral ganglion cell count and patients with deafness due to presumptive postnatal viral labyrinthitis, bacterial labyrinthitis, and congenital or genetic causes had the lowest numbers of residual spiral ganglion cells.

難聴を伴った大動脈炎症候群１１症例の検討

Aortitis syndrome is known to be an autoimmune disease, which has many symptoms of occulusive thromboaortopathy. It has recently been reported that the patients with systemic autoimmune diseases may have sensorineural hearing loss and that steroid therapy should be effective not only for the original disorder but also for the associated hearing disturbance.Eleven patients (3 males and 8 females) with aortitis syndrome and sensorineural hearing loss have been treated in Kitasato University Hospital during the 16 years since the opening of the hospital.The onset of hearing impairment was at 32 to 48 years of age, 10 years older than the mean age of onset of the aortitis syndrome reported in the literatures.Various types of hearing impairment were noted : slight hearing loss, total deafness of sudden onset and fluctuating type. Tinnitus was associated with hearing loss in many cases.Several patients also had vertigo and dysequilibrium, and in some of them the diagnosis was peripheral vestibular hearing loss, although in the cases of aortitis syndrome with hearing loss reported in the literature neither vertigo nor dizziness was mentioned.The usual treatment for acute stage sudden deafness was not effective in most of our patients. However, one female patient treated with long-term steroid therapy for systemic arteritis showed marked improvement of both her hearing and her general condition.We cannot conclude that the origin of sensorineural hearing loss is arteritis in the inner ear in all cases of aortitis syndrome with hearing loss. However, our cases of aortitis syndrome with steroid-dependant hearing loss indicate that the possibility remains of hearing loss due to arteritis of the labyrinthine artery in aortitis syndrome.

Non-invasive electrical stimulation of the ear canal as a communication aid in acquired total deafness.

Some profoundly deafened patients, who cannot be helped by sound amplification, claim to perceive auditory sensations when alternating currents up to 100 Hz are passed through electrodes applied to the skin of the external ear canal. A portable speech processor has been developed which supplies this current. The signal is a balanced square wave, the frequency of which is proportional to the first formant frequency. The amplitude is proportional to the intensity of voiced sounds. In order to fit the narrow frequency and dynamic range of the electrical stimulus, the speech processor produces a downward frequency transposition and strong limitation of the dynamic range. The device has been tested for (1) discrimination of environmental sounds; (2) question/statement discrimination; (3) identification of vowels and consonants in vowel/consonant/vowel context; (4) lip-reading with and without the prosthesis.

Jugulotympanic paraganglioma (Glomus jugulare tumour) presenting as a parotid neoplasm

A case of glomus jugulare tumour which presented as a parotid swelling, is reported. The patient also had facial palsy, total deafness and a polypoid lesion in the auditory canal. She was treated by surgery and radiotherapy. In this paper we review the literature and discuss the diagnostic problems and significance of long-standing facial palsy.

Response Latency Measurements to Visual Cognitive Tasks by Normal Hearing and Deaf Subjects

Visual cognitive differences between hearing (N = 16) and deaf (N = 32) high-school and middle-school students were studied. Visual tasks were presented on a microcomputer and response latencies were collected. Significant differences were noted between the deaf and normal groups but not between total communication deaf and oral deaf students. These differences support the hypothesis that deaf students prefer a visual cognitive strategy. Implications for educating the deaf are discussed.

Prognostication of Speech Performance in Patients With Multichannel Cochlear Implants

James L. Parkin, MD, and collaborators at the University of Utah, Salt Lake City, reported on the usefulness of preimplant anamnestic and test data to predict the performance of the Symbion multichannel cochlear implant. The study presented at the fall meeting of the American Academy of Otolaryngology–Head and Neck Surgery, Chicago, consisted of 20 patients. The patients had a wide range of performance scores on postimplant audiometric tests, environmental sound recognition ranged from 7% to 100% and open-set CID (Central Institute for the Deaf) sentences ranged from 0% to 100%. The investigators found that good lip readers, and those who had worn a hearing aid in the past, performed better with the cochlear implant, probably because these factors selected patients with higher motivation. Patients who had a duration of total deafness for less than five years performed best, and males performed slightly better than females. They found no correlation between

Transtemporal planned partial resection of bilateral acoustic neurinomas.

In an attempt to overcome the complete auditory deprivation of young patients with bilateral acoustic nerve schwannomas (Morbus v. Recklinghausen) a planned partial resection of the tumour was carried out in 11 hearing ears. The cerebello-pontine angle was exposed by the enlarged middle fossa approach (Wigand et al., HNO 1985). A strip of tumour was left in contiguity to the facial and cochlear nerves. Hearing was initially preserved in all cases. Four of them, however, developed total deafness after 3-39 months. Recurrent tumour growth was checked by CT scans. During the observation time one resting tumour became invisible, five remained unchanged. Two grew larger. Three cases dropped out of the follow-up. With regard to the high value of preserved auditory function during the first decades of life the planned partial resection may be considered preferable for cases of bilateral acoustic neuromas.

Surgical Treatment of Vertigo

Surgical therapy for disabling vertigo usually comes in at a late stage in the course of treatment for this patient group. The surgical procedures can be divided into two categories: non-destructive and destructive surgery. The former constitutes primarily procedures directed against the endolymphatic sac such as endolymphatic sac shunt procedures or decompression procedures. Peri-endolymphatic shunt procedures have also been proposed by some surgeons. None of these latter procedures have, however, gained widespread clinical acceptance. Destructive surgery is aimed against the end-organ itself or the vestibular nerve. Labyrinthectomy entails ablation of the vestibular end organs. This procedure is advocated in patients with disabling vertigo and a severe hearing loss or total deafness in the affected ear. Vestibular neurectomy can be performed at different levels. The middle fossa approach allows visualization of the vestibular nerve inside the internal auditory canal, whereas the retrolabyrinthine approach reveals the course of the nerves in the posterior cranial fossa. Vestibular neurectomy offers good results in the hands of a trained otoneurosurgeon.

高空間分解能ＣＴによる内耳奇形の計量診断

High resolution CT scanning of the temporal bone in our clinic has provided a more detailed radiological classification of inner ear anomalies than before. The statistical analysis of inner ear malformations based on the theory of quantification II has produced discriminant equations for the measurable diagnosis of hearing impairment and development of the inner ear. This analysis may make it possible to diagnose total and partial deafness on ipsi- and contralateral sides.

Early results with two types of multiple electrode intracochlear implants in postlingually totally deaf persons.

Early results with two types of multiple electrode intracochlear implants in postlingually totally deaf persons.

Vertigo in a district NHS hospital.

Thirteen general ENT clinics are held for a population of 600,000 people. The present study comprises an individual experience in managing patients with dizziness seen in three general ENT clinics. Eighty-six patients with a complaint of dizziness were seen over a period of 10 years from 1974 to 1984. Postural vertigo in elderly patients was the commonest cause of vertigo (50 per cent) in the present study. Most of these patients suffered from momentary dizzy spells with excessive neck movements. Symptomatic relief was achieved with reassurance and advice to avoid precipitating neck movements in most of the cases. Menière's disease was the second commonest cause of dizziness, which was seen in 15.1 per cent of the patients. Most of the cases were managed successfully with firm reassurance and a short course of treatment with either Betahistine or Cinnarizine. Only two patients showed a very moderate deterioration in hearing and only one patient needed saccus decompression surgery for uncontrolled vertiginous spells. Three patients with congenital unilateral total deafness showed a characteristic picture of secondary hydrops. One needed chemical destruction of the labyrinth to control the dizziness. Two cases (2.4 per cent) had posterior fossa lesions, one with an acoustic neuroma and the other with a large subarachnoid cyst. Both these cases were successfully managed by surgery.

Reflection-impulsivity in total communication and oral deaf and hearing children: a developmental study.

The purpose of the study was to examine the relationship of the cognitive style of reflection-impulsivity to communication mode (oral or total communication) and age in deaf and hearing boys. The Porteus Maze Test, Matching Familiar Figures Test, and a behavioral observation in the classroom were administered to 71 deaf and hearing children, at two age levels: 6 to 10 years and 11 to 15 years. Results indicate that the deaf groups were more impulsive than the hearing children on some of the measures. No significant differences were found between the oral and total communication groups. Significant differences between age groups were found for both the deaf and hearing subjects, with the older group exhibiting less impulsivity than the younger group.

Indications for Multi‐ or Single‐Channel Cochlear Implant for Rehabilitation of Total Deafness

Only totally deaf patients who do not experience improvement when using classical hearing aids are candidates for a cochlear implant. Preoperative assessment of potential benefits of a cochlear implant may improve selection of patients with pre- or postlingual total deafness, so that the most appropriate system can be implanted. The psychological status of potential candidates for implantation is particularly important because the results of postoperative re-education depends on motivation of the patient. The Round Window electrical stimulation test supplies us with important and measurable data (electrical threshold level, tone decay test). The duration of deafness, the degree of the deafness, and the cochlear tomodensitometry do not affect the decision to implant a cochlear device, (i.e., since the socialization level of the patient is a considerable factor). Preoperative assessment of the patient is very useful. As a result of our experience in 115 implanted cases suffering from pre- and postlingual deafness, we are now able to predict the good or bad results in our patients. Therefore, in patients who have many adverse factors for recovery, it seems to us more reasonable and cheaper to implant a single-channel system instead of a multichannel system, which must be reserved for patients who should do well. In very young children, the most appropriate system seems to be extra cochlear single-channel implant.