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PubMed1987

[The relation between melorheostosis and tumors].

Melorheostosis was diagnosed in a 13 year old female. Two years later an x-ray of the skull showed an calcified intracerebral mass. At the age of 18 years the patient died. The autopsy confirmed the diagnosis of melorheostosis and revealed a meningioma of falx cerebri in close proximity to predominantly intraventricular calcified tissue. A neurinoma of the left acoustic nerve was also found. In the literature melorheostosis has been anecdotally described in combination with nevi, hemangiomas, pelvic lipoma, lipoma of the spinal cord, chondrofibromas of the median nerve, tuberous sclerosis and neurofibromatosis. The nature of the relationship between melorheostosis and neoplasms has not been clarified. Those observations as well as the present report indicate an increased frequency of tumors (particularly of the nervous system) in patients with melorheostosis.

Adolescent↗

PubMed1987

[Anatomy of the central auditory pathway. Demonstration with horseradish peroxidase in the guinea pig].

The neurones of the afferent and efferent auditory pathways were labelled by axonally transported horseradish peroxidase. The first neurone of the ascending system is the acoustic nerve with the spiral ganglion. These fibres terminate mainly in the ventral cochlear nucleus (VCN). The neurones of the latter chiefly run to the contralateral superior olivary complex (SOC), whereas the neurones of the dorsal cochlear nucleus (DCN) terminate mainly in the central nucleus of the contralateral inferior colliculus (IC). The nerve cells of the superior olivary complex and nuclei of the lateral lemniscus (DLL, VLL) project to the IC. The IC neurones run mainly uncrossed to the medial geniculate body (MGB), i.e. the neurones of the central nucleus of the inferior colliculus terminate in the ventral nucleus of the medial geniculate body. This latter nucleus projects to the primary region A I of the auditory cortex, whereas the neurones in the pericentral medial geniculate body terminate in the associated auditory regions A II, EP and SF. The auditory areas of both hemispheres is well interconnected by commissural fibres. In addition to this ascending pathway there is a descending system. The parent cells correspond to pyramidal neurones in layer V in the auditory cortex. These neurones terminate in the medial geniculate body and in the pericentral nuclei of the inferior colliculus. From this, efferent neurones project to the DCN and SOC. The neurones of the olivocochlear bundles originate in the SOC; these bundles terminate predominantly on the surface of the outer hair cells in the organ of Corti.

Animals↗

PubMed1987

[Electrocochleographic detection of the summation potential in patients with inner ear deafness].

Pre-operative information about cochlear mechanical function is a pre-requisite for intra-cochlear implants. In more than 300 pre-operative examinations we demonstrated cochlear microphonics in many patients suffering from complete inner ear deafness; we interpret this phenomenon as evidence of mechanical function of the cochlea. A summation potential in patients with sensorineural deafness is more certain proof. We have now performed thorough electrocochleography on 43 deaf patients whose acoustic nerve was intact, i.e. whose promontory test was positive. After stimulation firstly with rarefaction tonebursts and later with condensation tonebursts and consecutive, off-line addition and subtraction we found small amplitude microphonics in 32 patients. Of these patients, 16 showed a negative potential that we interpreted as a summation potential. If these findings are verified by further investigations it will be possible to examine patients with inner ear deafness electrophysiologically. Furthermore this investigation is a very sensitive indicator of residual mechanical function of the cochlear basilar membrane.

Audiometry, Evoked Response↗

PubMed1986

[The promontory test and electrocochleography in deafness caused by mumps].

Contradictory histological findings in patients with deafness following mumps led us to conduct electrophysiological investigations. Promontory testing (PT) and measurement of cochlear microphonics (CM) enabled us to distinguish between neural and sensory deafness. On the basis of a careful history and serological tests in 19 cases of unilateral deafness we found that the hearing loss was probably caused by mumps. In all patients except one auditory sensations could be obtained by electric stimulation of the acoustic nerve whereas no CM were detectable even with strong stimuli of 100 dB tonepips. In view of the electrophysiological findings, doubt is cast on the neural genesis of deafness following mumps as assumed by Lehnhardt (1962).

Audiometry, Evoked Response↗

PubMed1986

[Evoked potentials. Indications and diagnostic significance].

Visual, auditory and somatosensory evoked potentials are useful in the detection and localization of demyelinating lesions, which mainly cause a slowing of conduction and therefore a latency increase in the evoked potential. Primarily amplitude reductions or loss of the response are seen in partial or complete impulse conduction blocks due to a tumor e.g. Acoustic nerve tumors can be detected by AEP, orbit tumors or processes of the chiasm by VEP and spinal cord tumors by SEP. The use of evoked potentials for the evaluation of ophthalmologic disorders, for audiometry in children and for monitoring head-injured patients with coma have become a valuable aid to the physician too.

Brain Diseases↗

PubMed1970

Hereditary nephropathy with hematuria (Alport's syndrome).

Among 82 members and four generations of a French-Canadian family, 14 cases of hereditary nephropathy (Alport's syndrome) were documented. Five additional members of the family had died, probably because of this same illness. Deafness occurred in five family members with nephropathy and in one without renal disease. Ten of 12 affected males died in uremia before they had reached the age of 40 years. One of seven affected females died following a pregnancy. In two surviving patients, special investigations failed to elicit intrinsic tubular defects such as amino-aciduria, renal tubular acidosis, hyperphosphaturia or renal glucosuria. Systemic illness such as abnormal aminoacids in serum, primary hyperoxaluria, diabetes mellitus and infections were also excluded. Immunological defects were not demonstrable and the staining of renal biopsy tissue with fluorescein-labelled anti-beta(1)c, anti-IgG and antifibrinogen was negative. Renal tissue material of early, advanced and terminal hereditary nephropathy showed both tubular and interstitial, vascular and glomerular lesions. Electronmicroscopy showed marked thickening of tubular and glomerular basement membranes, increase of mesangial tissue and fusion of foot processes but failed to demonstrate "immune deposits." It is postulated therefore that hereditary nephropathy results from an inborn error of metabolism where an as yet unidentified metabolite damages the renal tissue as well as the acoustic nerve, analogous perhaps to the action of certain drugs, e.g. nephro-ototoxic antibiotics.

Adolescent↗

PubMed1978

[Electrical stimulation of hearing in humans: a survey of the actual state of development of an implantable prosthesis for sensorineural deafness (author's transl)].

Recent clinical results show that electrical excitation of hearing sensations with implanted electrodes in the inner ear can be successful in suitable deaf persons. A critical literature review shows that some results have been described too vaguely and that there still exist numerous methodical and technical deficiencies. Even with multichannel stimulation, true tonotopical hearing could not be achieved. Periodicity hearing alone is not sufficient for the discrimination of one-syllable words. However, most deaf persons fitted with surgically implanted electrodes judged the new hearing sensations as being agreeable and quite useful. There seem to be no long term harmful effects of these implants upon the state of the acoustic nerve. However there are still a series of basic questions in auditory physiology pending which can only be solved through intensive collaboration of the clinical disciplines involved.

Animals↗

PubMed1984

[Cystic pontine glioma associated with von Recklinghausen's disease--report of a case].

Pontine gliomas have been considered to be out of indication for operative treatment. However, in case of a cystic type, evacuation of the cyst alone can possibly extend the survival time of the patient. Since the advent of high resolution CT the nature of the pontine tumor, whether cystic or solid, can be easily differentiated, and cystic ones subjected to operation will be increasing in number. We report a case of cystic pontine glioma associated with von Recklinghausen's disease. The patient showed a remarkable improvement in her neurological status after evacuation of the cyst. A 16-year-old girl was admitted to our clinic with complaints of tinnitus and hearing difficulty of the left ear, progressive gait disturbance and double vision. Neurological examination revealed a sensory disturbance on the left side of the face, left abducens palsy, left facial paresis, left deafness, left cerebellar ataxia, right hemiparesis and right hemisensory disturbance excluding the face. Signs and symptoms of increased intracranial pressure were absent. There were many cafe-au-lait spots and several subcutaneous nodules. CT scan demonstrated a cystic lesion with a mural nodule in the left cerebello-pontine angle. The patient underwent left suboccipital craniectomy, and a puncture of the cyst between the trigeminal and facial-acoustic nerves which were displaced dorsally yielded yellowish fluid. The content of the cyst was evacuated and its wall was widely opened. After the operation the patient showed a remarkable improvement in her neurological deficits only with left deafness remained unchanged. Histological examination showed anaplastic astrocytoma. She was discharged after irradiation of 5000 rads.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1982

[Hypoglossofacial anastomosis, results in 19 cases].

Hypoglossofacial anastomoses was performed in 19 patients, in the majority of cases for tumors located in the pontocerebellar angle. Results were a total success in 16 cases, one patient has not been seen again, and operation had been performed too recently to assess results in two cases. Anastomosis was conducted during initial surgery in 12 cases, and at a later stage in the other 7. In one patient, anastomosis 3 years after facial paralysis following excision of an acoustic nerve neuroma resulted in satisfactory re-innervation of the face after 13 months. Results were not significantly different in 6 patients with an intramastoid anastomosis and in 13 where it was performed in the parotid region. In 11 cases, a classical anastomosis was performed, involving suture of the XIIth and VIIth nerve trunks (type I anastomosis). A more complex procedure in the remaining 8 cases involved separate anastomosis of the XIIth and facial nerve branches, with, in some patients, implantation of sternothyroid muscle fragments, supplied by the descending branch of XII, into the orbicularis muscle. Results were good using these modifications, but valid comparison with those following classical methods cannot be made at the present time. Technical features of the procedure employed are described. Hypoglossofacial anastomosis is currently the only method giving satisfactory constant results when end-to-end suturing of the facial nerve or a superficial cervival plexus graft is obligatory.

Cerebellar Neoplasms↗

PubMed1982

[Development of the structure and stability of Mauthner's neurons in Xenopus laevis tadpoles on the implantation of additional auditory vesicles].

The ultrastructure and functional changes of the Mauthner cells were studied in the X. laevis tadpoles under the conditions of the experimentally doubled source of their afferentation, an additional pair of ear vesicles. Additional afferent fibers grow from the receptors to the brain and form additional synapses on the soma of the Mauthner cells, indistinguishable by their morphology from the terminals of their own acoustic nerves. The altered interneuronal interactions lead, on one hand, to the accelerated maturation of the Mauthner cells and, on the other one, to the vacuolization of a part of the cytoplasm. The superafferentation of the Mauthner cells is characterized by a long-term marked decrease in the length of sections of the active zones of axosomatic synapses. It is suggested that this structural rearrangement provides for a marked increase in the stability of the Mauthner cells with respect to extreme natural stimulation which is noted in the tadpoles with the double vestibular system.

Animals↗

PubMed1980

[The auditory evoked brain stem potential andits value for clinical diagnosis].

Auditory evoked brain stem potentials (seven waves within the first 10 milliseconds after the stimulus) obtained from the intercalated nuclei within the auditory pathway by averaging are suitable for a functional diagnosis of the brain stem. Disorders affecting the auditory pathway (direct or indirect tumour pressure or infiltrates, demyelinisation processes, traumatic brain stem injuries, ischemia) from the acoustic nerve through the cochlear nuclei, the superior olivary nuclei, the nuclei of the lateral lemnisci, the inferior collicular nuclei, to the medial geniculate bodies can be identified and used for the topical diagnosis.

Adolescent↗

PubMed1980

Inner ear disease characterized by rolling in C3H mice.

A neurologic disease characterized by rolling developed in C3H mice. Pseudomonas aeruginosa was isolated from the middle and internal ears or meninges of affected mice. The principal pathologic finding was aggressive, primary, purulent otitis media, with extension to the inner ear, acoustic nerve, and meninges. Stresses that may have contributed to induction of the disease were not delineated; however, acidification of the drinking water resulted in near elimination of the disease.

Animals↗

PubMed1995

[Analysis of antigenity of membranous labyrinth proteins of guinea pigs].

An electrophoretic dot immunobinding assay and a modified western blotting method were established. The components and antigenity of the membranous labyrinth proteins of guinea pigs were analysed. There are the same epitopes between some components of the antigen, between the antigen and extractives of acoustic nerve, cochlear nuclei, kidney and Klebsiella pneumoniae. The antigen distributes in stria vascularis, spiral ligament, spiral ganglion, the organ of Corti and Reissner's membrane etc.

Animals↗

PubMed1994

[Changes of auditory brainstem response and auditory cortex response after exposure to intensive noise].

Auditory brainstem response and auditory cortex response were recorded repeatedly in 35 guinea pigs after exposure to intensive white noise (125 dB, 150 min.) for 62 d. the amplitude of evoked potential of acoustic nerve was decreased by 29% (P < 0.05), of the cochlear nuclei by 28% (P < 0.05). However, the amplitude of response of superior olives nuclei was increased by 21% (P < 0.05), of the inferior colliculi by 37% (P < 0.05), of the cortical evoked response by 131% (P < 0.001). The results indicate that the amplitudes of auditory evoked potential showed a centripital augmentation after exposure to intensive noise. The centripital augmentation was observed not only during the period of the temporary threshold shift (TTS) but also during that of the permanent threshold shift (PTS).

Animals↗

PubMed1975

[Acoustic neurinoma and Recklinghausen's disease].

The present paper contains a brief survey of the literature on neurofibromatosis. A case of Recklinghausen's disease with multiple neurofibromas of the acoustic nerve, which affected the hypothalamo-hypophyseal region and thus caused adiposo-genital dystrophy, is described in detail.

Adolescent↗

PubMedDate not supplied

[Progressive nuclear external ophthalmoplegia].

66-year-old female patient presents palpebral bilateral ptosis and external ophthalmoplegia with a progressive evolution. The clinical aspects are completed with the affectation of the sensitive and motor trigemen, with the masseter atrophy and bilateral trophic keratitis. The affectation of the facial and acoustic nerves was associated with bilateral perception surditate. The hereditary transmission was dominant, with incomplete penetration. The neurogenic pathogeny was sustained on the basis of the association of neurological multilesions with III, IV, V, VI, VII, VIII nerves, with progressive extension.

Aged↗

PubMed1993

[Birth of an illness, exemplified by Menière disease].

Martin Luther's history and a case report by Grapengiesser in 1801 demonstrate that there were typical clinical manifestations of Menière's disease long before Menière's description. Luther suspected Satanic inflictions as cause of his disease; later on clinical pictures of this kind were interpreted as cerebral apoplectiform congestions, or as symptoms of a psychosis. It is Menière's merit to have shown that lesions of the inner ear can cause deafness, tinnitus and vertigo without involving the middle ear, the acoustic nerve or the brain. The biography of Prosper Menière (1799-1862) is briefly outlined. He made his pioneering observation between 1834 and 1838 when he had to attend to a young lady who after catching a cold presented with vertigo and deafness on both ears, and died 5 days later. He reported on this case in an annotation to his translation of W. Kramer's textbook on otology in 1848, but did not mention the vertigo. It was only in 1861 that he described the complete syndrome he had discovered, referring in detail to the case and the post mortem findings of 25 years ago to prove his hypothesis that lesions in the inner ear can cause such symptoms. Following his presentation a controversy prevailed for many decades about the question whether there is an idiopathic Menière's disease or not. This is highlighted by quotations from the literature. The first audiogram-like picture of the hearing loss involving all frequencies was presented by Gradenigo in 1892.(ABSTRACT TRUNCATED AT 250 WORDS)

Europe↗

PubMed1995

[The human genome--chromosome 22].

The 22nd chromosome is known mainly due to chromosome (Philadelphia) which is its derivative-a typical cytogenetic sign of chronic myeloid leukaemia (CML). The molecular genetic finding in these patients is the fused gene which developed by combination of the 3' part of the oncogene ABL from chromosome 9 and 5' part of the gene which developed by combination of the 3' part of the oncogene ABL from chromosome 9 and 5' part of the BCR "gene". The product of the gene retains the original kinase activity (ABL) which is even higher. Detection of BCR/ABL is an important diagnostic aid whic makes it possible to investigate residual diseases in patients after intensive treatment and transplantation of bone marrow and early detection of possible relapses. Among locuses of the 22nd chromosome the author mentions also the locus of the second one of the light immunoglobulin chains-lambda, incl. some of its "related" genes, the group of crystalline locuses (CRYB), the locus of the beta-chain of the GM-CSF receptor, the myoglobin locus (MB) and finally locus NF2 of central neurofibromatosis-bilateral neurinoma of the acoustic nerve.

Chromosome Mapping↗