1/49. Familial progressive vestibulocochlear dysfunction caused by a COCH mutation (DFNA9).OBJECTIVE: To describe the decline of vestibulocochlear function in a man with vestibulocochlear dysfunction caused by a Pro51Ser mutation within the COCH gene on chromosome 14q12-13 (DFNA9). methods: A follow-up of more than 15 years was performed in a single case. Clinical investigations were supplemented by oculomotor, vestibular, and auditory tests. RESULTS: A 50-year-old man had had progressive sensorineural hearing loss and dysequilibrium for 15 years; he had been asymptomatic at the age of 35 years. He suffered from instability in the dark, head movement-dependent oscillopsia, paroxysmal positional vertigo, and vertigo with and without nausea. Hearing impairment started unilaterally, predominantly in the high frequencies. He also reported tinnitus. Disease progressed to severe bilateral high-frequency hearing impairment and vestibular areflexia. Fluctuation of vestibulocochlear function was documented and mentioned by the patient. CONCLUSIONS: Our patient proved to suffer from an autosomal dominant vestibulocochlear disorder caused by a COCH gene mutation. The remarkable medical history has some features in common with meniere disease; however, there are also different clinical and neurophysiological features. In the family, phenotypic variability is present.- - - - - - - - - - ranking = 1keywords = hearing loss, sensorineural hearing loss, sensorineural, sensorineural hearing, hearing (Clic here for more details about this article) |
2/49. neurosyphilis as a cause of facial and vestibulocochlear nerve dysfunction: MR imaging features.The prevalence of syphilis increased for several decades before the mid-1990s in the united states, particularly in the southern states. We report a case of neurosyphilis causing bilateral facial and vestibulocochlear nerve dysfunction in which the diagnosis was not initially suspected based on the patient's demographics and history. The MR imaging features helped to make the diagnosis in this case and to exclude other possible causes of multiple cranial nerve dysfunction in this patient. hearing loss associated with neurosyphilis is one of the few treatable forms of progressive hearing loss, and it is essential that a diagnosis of neurosyphilis be made expeditiously.- - - - - - - - - - ranking = 0.38098370476586keywords = hearing loss, hearing (Clic here for more details about this article) |
3/49. Vestibular function in auditory neuropathy.Auditory neuropathy is characterized by mild-to-moderate pure-tone hearing loss, poor speech discrimination out of proportion with this loss, absent or abnormal auditory brainstem responses and normal outer hair cell function as measured by otoacoustic emissions and cochlear microphonics. We followed three patients in our clinic whom we classified as auditory neuropathy patients. These patients also complained of balance disorders and we report our auditory and vestibular system analyses of these patients. The data presented herein include results of audiometric tests (serial pure-tone audiometry and speech discrimination tests), otoacoustic emissions, auditory-evoked brainstem responses and vestibular function tests (clinical tests of balance, electronystagmography, damped rotation tests and vestibular-evoked myogenic potentials). In all patients, pure-tone audiometry revealed mild-to-moderate sensorineural hearing loss, markedly poor speech discrimination scores and absent auditory-evoked brainstem responses, all in the presence of normal otoacoustic emissions. Balance tests (caloric tests and damped rotation test) were abnormal. saccades, smooth pursuit eye movements and optokinetic nystagmus were normal in all patients. Neurological and motor system evaluations were normal in all patients. These three auditory neuropathy patients manifest a disorder of cochlear nerve function in the presence of normal outer hair cell activity. They additionally manifest a disorder of the vestibular nerve and its end organs. We conclude that, in patients with isolated auditory neuropathy, the vestibular branch of the VIIIth cranial nerve and its innervated structures may also be affected. We suggest the use of the term "cochlear neuropathy" to characterize those patients with involvement of only the auditory branch of the VIIIth cranial nerve and its innervation.- - - - - - - - - - ranking = 1.2517800400452keywords = hearing loss, sensorineural hearing loss, sensorineural, sensorineural hearing, hearing (Clic here for more details about this article) |
4/49. An isolated and sporadic auditory neuropathy (auditory nerve disease): report of five patients.Five patients of various ages with difficulty in speech discrimination were evaluated. All showed evidence of abnormal auditory brainstem responses (ABRs) beginning with the VIIIth cranial nerve. Broad summating potentials were evoked on their electrocochleograms (EcochGs) and they all exhibited almost normal cochlear outer hair cell function by otoacoustic emissions (OAEs) recordings. Behavioural audiometric testing revealed a mild to moderate elevation of pure-tone threshold in all patients. The shape of their pure-tone losses varied, being predominantly low-frequency in four patients (rising slope pattern) and flat across all frequencies in one patient. speech intelligibility scores of all patients were poor and out of proportion to what would have been expected if threshold elevation of pure-tone was of cochlear origin (i.e. markedly poor scores on the speech audiogram with good scores on the auditory comprehension test). patients were neurologically normal when the hearing impairment was first manifested. We suggest that this type of hearing impairment is due to an isolated and sporadic disorder of auditory nerve function. It occurs in isolation and does not seem to be part of a generalized neuropathological process.- - - - - - - - - - ranking = 0.25840732944139keywords = hearing (Clic here for more details about this article) |
5/49. Enhancement of the eighth cranial nerve and labyrinth on MR imaging in sudden sensorineural hearing loss associated with human herpesvirus 1 infection: case report.The case of a 61-year-old woman who presented with herpes labialis, subclinical meningitis, and sudden onset of bilateral sensorineural hearing loss is presented. Contrast-enhanced MR imaging showed marked bilateral enhancement of the intracanalicular portion of the eighth cranial nerve, right cochlea, and left vestibule. polymerase chain reaction was positive for human herpesvirus 1 obtained from the cerebral spinal fluid, which suggested the diagnosis of viral neuritis.- - - - - - - - - - ranking = 4.3539816763965keywords = hearing loss, sensorineural hearing loss, sensorineural, sensorineural hearing, hearing (Clic here for more details about this article) |
6/49. Hearing habilitation with auditory brainstem implantation in two children with cochlear nerve aplasia.patients with aplasia and hypoplasia of the cochlear nerve have no chance of having their hearing restored by stimulating the periphery of the auditory system using the traditional cochlear implant. A possible approach to auditory rehabilitation may be direct electrical stimulation of the cochlear nuclei with an auditory brainstem implant (ABI). Recently, two children, aged 4 and 3 years, respectively, with bilateral severe cochlear malformations and cochlear nerve aplasia received an ABI. The present paper reports the technique and the preliminary results of this experience. The classic retrosigmoid approach was used. The correct position of the electrodes was estimated with the aid of EABRs and neural response telemetry (NRT). No postoperative complications were observed. High-resolution CT scans with a bone algorithm reconstruction technique were taken postoperatively to evaluate electrode placement before discharge. The ABI was activated 30 days after implantation in both patients. To date 16 and 13 electrodes, respectively, have been activated in the two children. Three months after activation the first patient had achieved good environmental sound awareness, good speech detection and some speech discrimination. The second child, 1 month after activation, had achieved good environmental sound awareness and moderate speech detection. To the best of our knowledge this is the first report of patients with hypoplasia of the cochlea and aplasia of the cochlear nerve, aged below 5 years and treated with an ABI.- - - - - - - - - - ranking = 0.12920366472069keywords = hearing (Clic here for more details about this article) |
7/49. Malignant melanoma presenting as sudden onset of complete hearing loss.The authors describe a rare case of rapidly progressive bilateral hearing loss associated with carcinomatous meningitis secondary to a primary malignant melanoma of the lower limb 6 years previously. Carcinomatous meningitis is a relatively rare diagnosis, with the involvement of the eighth cranial nerve being present in only a small proportion of patients. diagnosis relies on clinical suspicion (when multiple cranial nerve lesions occur), magnetic resonance imaging, and cerebrospinal fluid cytology.- - - - - - - - - - ranking = 1.9049185238293keywords = hearing loss, hearing (Clic here for more details about this article) |
8/49. Auditory neuropathy in children.Auditory neuropathy is a sensorineural disorder characterized by absent or abnormal auditory brainstem evoked potentials and normal cochlear outer hair cell function. A variety of processes is thought to be involved in its pathophysiology and their influence on hearing may be different. We present here the diagnostic sequence and management of two new cases of auditory neuropathy in breastfeeding children.- - - - - - - - - - ranking = 0.27362656824904keywords = sensorineural, hearing (Clic here for more details about this article) |
9/49. A case of multiple schwannomas of the trigeminal nerves, acoustic nerves, lower cranial nerves, brachial plexuses and spinal canal: schwannomatosis or neurofibromatosis?In most cases, while schwannoma is sporadically manifested as a single benign neoplasm, the presence of multiple schwannomas in one patient is usually indicative of neurofibromatosis 2. However, several recent reports have suggested that schwannomatosis itself may also be a distinct clinical entity. This study examines an extremely rare case of probable schwannomatosis associated with intracranial, intraspinal and peripheral involvements. A 63-year-old woman presented with a seven-year history of palpable lumps on both sides of the supraclavicular area and hearing impairment in both ears. On physical examination, no skin manifestations were evident. Facial sensory change, deafness in the left ear and decreased gag reflex were revealed by neurological examination. magnetic resonance imaging revealed multiple lesions of the trigeminal nerves, acoustic nerves, lower cranial nerves, spinal accessory nerve, brachial plexuses, and spinal nerves. Pathological examination of tumors from the bilateral brachial plexuses, the spinal nerve in the T8 spinal position and the neck mass revealed benign schwannomas. Following is this patient case report of multiple schwannomas presenting with no skin manifestations of neurofibromatosis.- - - - - - - - - - ranking = 0.12920366472069keywords = hearing (Clic here for more details about this article) |
10/49. Intraosseous dural arteriovenous fistula of the skull base associated with hearing loss. Case report.The most common clinical presentations of dural arteriovenous fistulas (DAVFs) are bruit, headache, increased intracranial pressure, and intracranial hemorrhage. In particular locations, such as the cavernous sinus or middle cranial fossa, cranial nerve involvement due to dural arterial steal or venous occlusion may develop. A case in which a DAVF is associated with hearing loss, however, has not previously been reported. The authors report a case in which an intraosseous DAVF and associated hearing loss probably resulted from cochlear nerve or vascular compression caused by the draining vein or nidus of the DAVF.- - - - - - - - - - ranking = 2.2859022285952keywords = hearing loss, hearing (Clic here for more details about this article) |
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