Neural Deafness Research Articles

Radiologic aspects in differential diagnosis of neural and sensory deafness.

The use of radiologic techniques as part of the physical examination of patients with sensory and neural deafness is reviewed. Polytomes, line drawings, and a clinical discussion of the following conditions are presented: osseous displasia, congenital deformities of the internal auditory canal and/or the labyrinth, purulent labyrinthitis, temporal bone fractures and tumors of the temporal bone and cerebellopontine angle. It is felt that current diagnostic radiologic techniques should be utilized to their fullest extent in the diagnosis and management of temporal bone problems.

Family with Charcot-Marie-Tooth disease showing unusual biochemical-clinical and genetic features.

A family with Charcot-Marie-Tooth disease is described. An irregular autosomal dominant transmission is observed. Some unusual clinical symptoms were present, such as trophic ulcers and neural deafness. In one generation of sibships with affected and unaffected individuals, some younger sibs had raised serum alkaline phosphatase activities. A connection between the neurology cal disorder and these abnormal values may be assumed.

A metabolic myopathy associated with chronic lactic acidemia, growth failure, and nerve deafness

Two sisters with signs of growth failure, severe muscle weakness, and moderate neural deafness had identical abnormalities on muscle biopsy. Light microscopy revealed areas of “granular necrosis” which proved to be large and numerous mitochondria. Biochemical assessment has revealed large amounts of alanine in the urine, as well as hyperalaninemia and reduced clearance from the blood of an oral alanine load. Pyruvate concentration in the blood was found to be elevated, and each girl had marked lactic acidemia. This led to a fatal episode of lactic acidosis in the younger girl.

Clinicopathology of Congenital Deafness

There were enomous number of reports concerning congenital deafness, but unfortunately the exact pathologic findings of the deafness were not still fully understood.It is a purpose of this paper to present new pathologic findings in a case of deafmute, and the findings were compared with the known inner ear lesions of congenital deafness and other sensory neural deafness. These findings were, for instance, dilated cochlear duct, calcified thrombi in striate vessels, abnormal tectorial membrane, conspicuous spiral ganglion atrophy, and these might be important in clarifying true pathology of congenital deafness and in classifying it.

Usher’s syndrome

Usher’s syndrome is characterized by retinitis pigmentosa, sensori neural deafness and vestibulo cerebellar ataxia and mental disorder. Three cases of this syndrome all belonging to the same family has been presented. The available literature briefly reviewed. It was felt that Usher’s syndrome and Siebenmann and Bing’s disease, also characterized by retinitis pigmentosa and sensori neural deafness but without any nedrological manifestations, are two varients of the same condition in view of the closely related clinical picture. Deafness in Usher’s syndrome is probably a local aural manifestation of a congenital weakness of central nervous system due to an identical etiological condition responsible for retinitis pigmentosa.

耳鼻咽喉科疾患に対する星状神経節プロツクの効果

84 patients (100 ears) with sudden deafness, other neural deafness, tinnitus, or unidentified discomforts in the head were treated with the stellate ganglion block.The treatment was effective in 80% of the sudden deafness cases. This result is in conformity with the general belief that the stellate ganglion block has a remarkable effect on sudden deafness. Even with the other neural deafness cases, for which no treatment has been believed available, we could get the efficacy rate of 12.2%. As for the tinnitus cases the rate was 69.5%. Generally we can expect a good result when remission is observed after the first or second treatment.Our study shows that the stellate ganglion block is a very effective method that should be tried in the case of nerve disturbances in the field of otolaryngology. It is true that this treatment method is sometimes accompanied by side effects such as shocks, palpitation with cold perspiration, etc., even if we take enough care. They are, however, of a very slight kind, and we need not worry about them when an oxigen inhaler and other emergency measures are prepared. Therefore, it is highly desirable for every otolaryngologist to become skillful with this technique.The working mechanism of the stellate ganglion block cannot be explained by the improvement of blood circulation alone. Clarification of the mechanism is a subject for the future studies.

Optic atrophy, neural deafness, and distal neurogenic amyotrophy; report of a family with two affected siblings.

BILATERAL optic atrophy^1-9and neurogenic deafness^8-10are rarely associated with Charcot-Marie-Tooth disease. However, familial occurrence of the triad of primary optic atrophy, acoustic involvement, and polyneuropathy was not clearly described until 1967, when Rosenberg and Chutorian⁹described three men in two generations of the same family with this triad of deficits. They deduced that this disorder belongs in the spectrum of spinocerebellar degenerations, the closest member being Charcot-Marie-Tooth disease. We recently found another family in which a 25-year-old man and his 15-year-old sister, have chronic progressive optic atrophy, neurogenic hearing deficit, and neurogenic distal amyotrophy. This paper describes the clinical features in these two siblings. <h3>Report of Cases</h3> CASE1.—A 25-year-old man was admitted to the Neurological Institute, Kyushu University Hospital (KUH), in November 1968. Pregnancy, delivery, and neonatal period were uncomplicated. He walked at age 12 months. Mental and physical development seemed normal until

A Case of 25 Year Old Dwarf with Classic Cockayne Syndrome

A 25 year old, short stature man of non-consanguineous parents, attended with acute respiratory tract infectionalong with progressive difficulties in walking, hearing and vision. He had the complaints of growth retardation, poorfeeding, listless attitude and delayed milestones of development since one year of age. At presentation, he was noncooperative, IQ below 50; height and weight were below 5th percentile. He had progeria with enophthalmos, cataract,corneal opacity, miotic pupils, tremor, ataxia, in-coordination of movement, diminished tendon reflexes, unsteadygait, bilateral sensory neural deafness and hepatomegaly. Lateral skull X-ray showed cortical calcification. MRI ofbrain revealed bilateral dentate nucleus and basal ganglia calcification, generalized cerebral and cerebellaratrophy and ventricular dilatation. Typical clinical and imaging findings clinched the diagnosis of classic CockayneSyndrome; which is a rare, autosomal recessive, DNA repair deficient, multisystem disorder. It has no cure and theprognosis is poor.Keywords: Classic Cockayne syndrome; Dwarfism; Basal ganglia calcification; Cerebral atrophyDOI: 10.3329/jom.v11i2.5470J MEDICINE 2010; 11 : 186-188

Sensorineural deafness.

A NUMBER of articles dealing with familial nerve deafness have added to the previously published reports of this irreversible and often progressive affliction. Mengel et al¹state that only nine of 54 types of hereditary nerve deafness have no associated abnormalities. In the sex linked are congenital, severe, early onset, high tone deafness; in the dominant are midfrequency, progressive and unilateral deafness. Finally a recessive congenital severe deafness is recognized. They propose another recessive type (recessive, early onset, neural deafness) on the basis of their study of a Mennonite family with 16 deaf members. History, sonograms, and auditory tests suggest some hearing in early childhood that deteriorates by late childhood. Proctor and Proctor²present a useful classification of dominant and recessive nerve deafness associated with a wide variety of hereditary disease. They point out that the pathological changes are usually confined to the organ of Corti and stria

Recessive early-onset neural deafness.

In three generations of the S. family there were 16 members with severe deafness all of whom were examined. By history all members were born with at least some hearing, with progressive severe loss by later childhood. Sonographic and speech analysis gave further evidence of at least some hearing in early childhood. Audiologic tests on 3 members of the family suggested a cochlear location of the defect. Other tests done on 10 affected members including physical, neurological, and vestibular examinations showed no abnormalities. The deafness appears to be transmitted as an autosomal recessive. We suggest that this entity be called “Recessive early-onset neural deafness”.

The Effect of Trauma in Causing Cochlear Losses After Stapedectomy

Trauma undoubtedly may play an important role in causing sensori neural deafness after stapedectomy. In stapedectomy, trauma may be considered indirect (stimulation of the intact ossicles) or direct (disturbance of the vestibular contents). In evaluating indirect trauma the ossicular chain was stimulated in trained cats resulting in acoustic trauma lesions of the basal turn. Direct trauma was evaluated by making sac-cular lesions through the oval window in the squirrel monkey and cat. This resulted in normal cochlear findings. These findings indicate that acoustic trauma more likely results from a massive volume displacement of perilymph beneath an intact footplate. Although trauma sometimes explains severe cochlear deafness immediately following stapedectomy it does not explain the delayed deafness which may follow an initial good result.Stapedektomie spielt zweifellos eine wichtige Rolle in der Verursachung von Nervenschwerhorigkeit. Trauma bei Stapedektomie kann auf zwei Arten erfolgen. Auf indirektem ...

SENSORY NEURAL DEAFNESS.

SENSORY NEURAL DEAFNESS.

Experiences with Nutrase in the Treatment of Neural Deafness

Drug therapies for neural deafness have long been studied in various manners, with no satisfactory outcomes yet. A rigorous fact about the above treatment is that the restoration of hearing functions is beyond hope after the nerve cells have undergone irreversible changes. Adequate treatment, therefore, has to be started and completed while the pathologic changes are still in the functional phase and are reversible.Nutrase with a structural formula of a free-form vitamin B1 which is phosphorylated has recently been administered daily for 21 consecutive days to subjects picked up at random from among the inpatients and outpatients of our Department with neural deafness and also from among the Streptomycin-deaf inmates of the National Oshima Seishoen Leprosarium. Evaluation of the drug effect mainly in terms of improvement in hearing acuteness of bone conduction, found improved hearing acuteness in 27% of the former group of the drug-recipients and in 33% of the latter.We have found Nutrase to be an effective drug for the treatment of neural deafness.

SENSORY NEURAL DEAFNESS IN PELLAGRA.

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Report on Dr. Lombard's Original Research on the Voice Reflex Test for Malingering:Contribution à la Seméiologie de la Surdité, Un Noveau Signe pour en Devoiler la Simulation

A report is given in translation concerning the initial experimentation by Dr. Lombard (1910) with his voice reflex test for nonorganic deafness. The technique is described as useful in the differential diagnosis of certain cases of unilateral and bilateral sensory neural deafness. Various sources of the masking stimulus are enumerated and testing methods are outlined. The Lombard voice reflex test is projected as a potentially valuable contribution to the field of forensic medicine.

Effect of Alinamin F on Deafness

Alinamin F was given, per os and intravenously, to neural deafness and satisfactory results were obtained. The effect was more pronounced in relatively early cases but it was also effective in a case of streptomycin deafness of 3 years standing.Alinamin F was considerd effective in several cases, in which other agents were not used at the same time.There was no difference in effect between the oral and the intravenous administration.No noteworthy side effects were observed.

Treatment of neural deafness with prednisone

1. (1) Nineteen patients with neural deafness due to congenital syphilis and twenty patients with neural deafness of other origin have been treated with prednisone in gradually tapering dosage. Dosage was in general regulated according to the results of standard pure-tone air and bone-conduction audiometry, speech hearing threshold, and discrimination score, measured before, and at short intervals following the commencement of therapy. 2. (2) The neural deafness of congenital syphilis occurs late, often not until adult life. It is usually an isolated neurological finding without abnormalities in the cerebrospinal fluid. Its development and progression are not prevented by treponemicidal therapy, even with penicillin. It is almost always bilateral and usually of gradual onset, although there may be either sudden onset or sudden dramatic worsening. Episodes of vertigo and continuous or intermittent tinnitus are common. Hearing impairment is often fluctuant but, in the absence of effective treatment, inexorably progressive at a highly variable rate. The major difficulty is in discriminating speech. 3. (3) Objective improvement, often marked, occurred during the first month of prednisone therapy in ten of the nineteen congenital syphilitics, and was subsequently maintained. In three therapy was deliberately stopped, with no retrogression over periods of 40 months, 1 month, and 2 months respectively. In the nine without definite improvement progression may in some instances have been prevented. These results show that the neural deafness of congenital syphilis is not usually a purely degenerative process. 4. (4) In long-standing neural deafness of undetermined etiology prednisone therapy was in general unsuccessful. 5. (5) In sudden non-syphilitic deafness of neural type which is often unilateral and occurs with or without an apparent concomitant upper respiratory infection, prednisone therapy sometimes results in dramatic improvement and may be urgently indicated.

Sensory neural deafness, 1959-1960.

Our understanding of sensory neural deafness in man has been increased through various experimental, histopathological, and clinical studies. New information on the structure and functioning of the normal cochlea and its central pathways is obtained through a wide variety of experimental methods. Iurato¹has obtained elegant photographs with the electron microscope of the hair cells and supporting cells of the organ of Corti of the rat. He points out some of the structural differences between the inner and outer hair cells that probably have functional significance. For example, almost all of the nerve endings on the outer hair cells are richly granulated, while in the inner hair cells some endings were also of the poorly granulated variety. The size of the hairs on the inner hair cells is twice that of the outer hair cells. In the outer hair cells the intermediate zone is far larger, and there

聽覚による方向識別に関する研究(其の三)

The source of the sound came from a loud speaker connected to a beat frequency oscillator. The examinees were all patients with hearing defect or deafness. The localisation of the subjective median for the source of the sound in the horizontal plane of their ears were examined and the results were as follows;1. The monaural localization was unable to be made.2. They were able to locate the source between 515 d. b. or more from the threshold (of Intensity) by their poorer ear.3. In ears with equal hearing, the source of the sound was localized accurately in the median. But when the hearing of one ear was better, the source of the sound localized to that one.4. The position of his apparent median plane in the horizontal plane was shown as the angular displacement. The longer the period of difficult hearing was, the smaller the angular displacement was. (general type of angular displacement)5. When the threshold was 50-60 d. b. or more (of sensation level), the angular displacement was very great and did not become smaller as the period difficult hearing grew.6. In patients with neural deafness the range of subjective median was as small as normal hearing, and in a patient with conductive deafness it was very large.7. After catheterization in the so-called tubotympanic catarrh the range of subjective median become very small, especially during the early stage. This was due to the improvement of conduction mechanism.

Tests for determining hearing impairment and disability; analysis of pure tone and speech tests for clinical and compensation purposes.

Recent attempts to revert solely to speech testing for determining the percentage of deafness in persons for compensation purposes and to get away from pure tone testing entirely impel me to set forth the facts, as I see them, concerning this matter. For a number of reasons it is difficult to estimate the over-all loss of hearing capacity by the use of speech tests alone. For one thing, each of the three major categories of deafness must be handled differently. These categories, in the order of simplicity of understanding, are (a) impedance deafness, (b) neural deafness, and (c) cortical deafness.1Any one, or two, or all three categories may be, and frequently are, encountered in a single person. Speech tests fail to differentiate these types of deafness as well as pure tone tests do. The threshold air and bone conduction audiograms are essential for diagnosis, and as yet they