1/30. A case of laryngeal neurinoma with neurofibromatosis 2.We present a case of a laryngeal neurinoma in a patient with neurofibromatosis 2. A 39-year-old man presented to our hospital with multiple complaints including progressive bilateral hearing loss, dizziness, dyspnea, dysphagia, and a 9-year history of right lower leg weakness. magnetic resonance imaging demonstrated multiple lesions including bilateral cerebellopontine angle tumors, a foremen magnum tumor, multiple tumors of the spinal cord, a laryngeal tumor, and several retrocervical tumors. Fiberoptic laryngoscopy revealed a large submucosal supraglottic tumor. The laryngeal tumor was visualized through microlaryngoscopy and excised with a KTP laser directed through a quartz fiber.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/30. Persistent bilateral hearing loss after shunt placement for hydrocephalus. Case report.Transient hearing decrease following loss of cerebrospinal fluid (CSF) has been reported in patients undergoing lumbar puncture, spinal anesthesia, myelography, and/or different neurosurgical interventions. The authors present the first well-documented case of a patient with persistent bilateral low-frequency sensorineural hearing loss after shunt placement for hydrocephalus and discuss the possible pathophysiological mechanisms including the role of the cochlear aqueduct. These findings challenge the opinion that hearing decreases after loss of CSF are always transient. The authors provide a suggestion for treatment.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
3/30. Inner ear abnormalities in Kabuki make-up syndrome: report of three cases.Three patients, a female and two males, 28, 15, and 14 years of age, with Kabuki make-up syndrome (KMS) were studied for middle and inner ear abnormalities by using CT scanning of the petrous bones. All three patients had bilateral dysplasia of the inner ear, i.e., hypodysplasia of the cochlea, vestibule, and semicircular canals (so-called Mondini dysplasia), whereas their middle ears had no abnormalities. audiometry demonstrated a sharp decrease in hearing of the high tone range, bilateral in one and unilateral in another, while the third patient was noncooperative. In view of these findings, it would be advisable to study each individual with KMS and hearing impairment for possible inner ear abnormalities.- - - - - - - - - - ranking = 48.857368177523keywords = canal (Clic here for more details about this article) |
4/30. Factitious hearing loss and otorrhea in an adolescent boy.Asialo, or beta(2), transferrin occurs in cerebrospinal fluid and a few other body fluids, e.g., perilymph and aqueous humor. It is used clinically as a marker protein to detect CSF otorrhea and rhinorrhea. Asialo-transferrin is separated from sialylated serum transferrin glycoforms by electrophoresis and detected by Western blotting. Potential pitfalls in interpreting Western blots occur when other transferrin isoforms approximate the migratory behavior of asialo-transferrin. In the present report an adolescent boy was seen by an otolaryngologist for otorrhea and acute hearing loss. The otorrhea fluid, subsequently identified as saliva, contained a transferrin isoform with electrophoretic mobility similar to that of asialo-transferrin.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
5/30. Hearing impairment in 18q deletion syndrome.The 18q-syndrome is associated with hearing impairment in 50-80 per cent of cases. The hearing loss may be sensorineural or conductive. A high proportion of cases are associated with narrow or stenosed external auditory canals. This may be a useful clinical pointer to the syndrome. Two cases with impaired hearing are presented in this paper including one case with complex external ear and middle ear malformations. The clinical and audiological features in each case are described.- - - - - - - - - - ranking = 48.857368177523keywords = canal (Clic here for more details about this article) |
6/30. Improvement of chronic hearing loss after shunt revision. A case report.BACKGROUND: hearing loss after intracranial and spinal procedures involving cerebrospinal fluid loss is rarely reported in the literature. We report a patient who suffered from delayed hearing loss after cerebrospinal fluid shunting that improved after revising the shunt to a higher-pressure valve. CASE DESCRIPTION: A 32-year-old woman presented with bilateral hearing loss 4 years after ventriculoperitoneal shunting for communicating hydrocephalus. Her otologic work-up revealed sensorineural hearing loss. In an attempt to improve her hearing, 6 years after the hearing loss began (10 years after the shunt was placed), she underwent a shunt revision in which her valve was changed to a higher-pressure device. After the procedure, she had a significant improvement in her speech discrimination and a mild improvement in her pure tone recognition. These changes were documented with serial audiograms. CONCLUSION: hearing loss after cerebrospinal shunting procedures is not always limited to the immediate postoperative period. It may be a late complication of cerebrospinal fluid diversion. Chronic hearing loss after ventriculoperitoneal shunting may be treatable by changing the valve to a higher-pressure device. The etiology of hearing loss from intracranial hypotension is briefly discussed.- - - - - - - - - - ranking = 5keywords = spinal (Clic here for more details about this article) |
7/30. The potential risk of carotid injury in cochlear implant surgery.BACKGROUND: The advent of cochlear implantation has revolutionized the options afforded to the deaf population. With the increase in the prevalence of this procedure have come larger experiences in the associated technical challenges and complications. RESULTS: We present the evaluation and management of a patient with an unusual complication of improper placement of the implant electrode into the carotid canal and its management. We discuss the anatomy of the carotid artery and its proximity to the cochlea to emphasize the potential risk to this large vessel. CONCLUSIONS: Damage to the carotid canal and the carotid artery is a potential risk of cochlear implant surgery. When available, we recommend intraoperative electrical testing of the cochlear implant be performed. If there is doubt as to the placement of the electrode, a radiograph should be obtained before the patient is taken out of the operating room to avoid this complication.- - - - - - - - - - ranking = 97.714736355046keywords = canal (Clic here for more details about this article) |
8/30. Longitudinal analysis of hearing loss in a case of hemosiderosis of the central nervous system.OBJECTIVE: To describe cochleovestibular aspects of superficial hemosiderosis of the central nervous system. BACKGROUND: Superficial hemosiderosis of the central nervous system is a rare disease in which cochleovestibular impairment, cerebellar ataxia, and myelopathy are the most frequent signs. Chronic recurrent subarachnoidal hemorrhage with bleeding into the cerebrospinal fluid is the cause of deposition of hemosiderin in leptomeningeal and subpial tissue, cranial nerves, and spinal cord. Removing the cause of bleeding can prevent irreversible damage to these structures. Because this is the only effective treatment, an early diagnosis is crucial. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENT: A 72-year-old woman with superficial hemosiderosis of the central nervous system that developed when she was age 39. methods: Neurologic and imaging diagnostic examinations and longitudinal evaluation of cochleovestibular features were performed. neurosurgery was not performed. RESULTS: Progressive bilateral sensorineural hearing loss and severe vestibular hyporeflexia developed within 15 years, which can be attributed to lesions in the cochleovestibular system. Additional pathology of the central nervous system developed later. CONCLUSION: The patient demonstrated cochlear and vestibular findings that are typical of this pathologic condition. It is the first documented case with extensive serial audiometry used to precisely outline the degree of hearing deterioration during the course of the disease.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
9/30. Vestibular-evoked myogenic potentials in three patients with large vestibular aqueduct.An enlarged vestibular aqueduct (LVA) is a common congenital inner ear anomaly responsible for some unusual vestibular and audiological symptoms. Most of the cases show bilateral early onset and progressive hearing loss in children. The gross appearance on CT scan of the inner ear is generally normal. However, precise measurements of the inner ear components reveal abnormal dimensions, which may account for the accompanying auditory and vestibular dysfunction. Despite extensive studies on hearing and the vestibular apparatus, saccular function is not studied. To our knowledge this is the first report of saccular malfunction in three patients with LVA by means of vestibular evoked myogenic potentials. Conventional audiograms revealed bilateral severe sensorineural hearing loss in two patients and mixed type hearing loss in one patient. Two of the patients complained about vertigo and dizziness but vestibular assessments of the patients showed normal results. The diagnosis had been made by high-resolution CT scans and MR images of the skull that showed LVA in the absence of other anomalies. The VEMP threshold measured from the ear with LVA in two patients with unilateral enlargement of the vestibular aqueduct was 75-80 dB nHL whereas the threshold from normal ears was 95 dB nHL. The third patient with mixed type hearing loss and bilateral LVA had VEMP responses despite a big air-bone gap in the low frequency range. The VEMP in this patient was greater in amplitude and lower in threshold in the operated ear (the patient had a tympanoplasty which did not improve her hearing). These findings and results of other patients with Tullio phenomenon and superior semicircular canal dehiscence, who also showed lower VEMP threshold, confirmed the theory of a 'third window' that allows volume and pressure displacements, and thus larger deflection of the vestibular sensors, which would cause the vestibular organ to be more responsive to sound and pressure changes.- - - - - - - - - - ranking = 48.857368177523keywords = canal (Clic here for more details about this article) |
10/30. Audiovestibular evolution in a patient with multiple sclerosis.multiple sclerosis is characterized by the presence of multiple plaques within the central nervous system, manifesting as remission and exacerbation of neurologic dysfunction over variable time courses. We present the case of a 20-year-old woman. Before treatment, her auditory brain stem response (ABR) test revealed bilateral prolongation. A caloric test showed canal paresis of the right ear and a normal response on the left. A vestibular evoked myogenic potential (VEMP) test displayed an absent response in the right ear and a delayed response in the left. A magnetic resonance imaging (MRI) scan demonstrated multiple diffuse high signal lesions in the hemispheres, brain stem, and cerebellum. Six months after treatment, the demyelinating plaques were shown to have resolved spontaneously on MRI. Recovery of caloric responses was anticipated. Bilateral prolongation of ABRs remained, but the VEMP test disclosed a normal response in the right ear and a delayed response in the left. Accordingly, in addition to MRI, caloric tests and ABR and VEMP tests are useful in monitoring the evolution of audiovestibular function in patients with multiple sclerosis.- - - - - - - - - - ranking = 48.857368177523keywords = canal (Clic here for more details about this article) |
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