1/65. Carabid beetle invasion of the ear in oman.Two cases of human ear invasion by a predaceous beetle, Crasydactylus punctatus Guerin (Coleoptera; Carabidae), are reported from the Sultanate of oman. The first case was that of a 35-year old woman who suffered a severe otologic injury caused by the biting and chewing of the external auditory canal and the tympanic membrane. The beetle then entered the middle ear and caused sensorineural hearing loss. The second case involved a 22-year-old male from whose ear a specimen of the same species was successfully removed after it was immobilized with 10% lidocaine spray. These cases are presented with discussion.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
2/65. noise-induced hearing loss.hearing loss caused by exposure to recreational and occupational noise results in devastating disability that is virtually 100 percent preventable. noise-induced hearing loss is the second most common form of sensorineural hearing deficit, after presbycusis (age-related hearing loss). Shearing forces caused by any sound have an impact on the stereocilia of the hair cells of the basilar membrane of the cochlea; when excessive, these forces can cause cell death. Avoiding noise exposure stops further progression of the damage. noise-induced hearing loss can be prevented by avoiding excessive noise and using hearing protection such as earplugs and earmuffs. patients who have been exposed to excessive noise should be screened. When hearing loss is suspected, a thorough history, physical examination and audiometry should be performed. If these examinations disclose evidence of hearing loss, referral for full audiologic evaluation is recommended.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
3/65. A de novo missense mutation in a critical domain of the X-linked DDP gene causes the typical deafness-dystonia-optic atrophy syndrome.We report the first de novo mutation in the DDP gene in a Dutch 11-year-old boy with deafness and dystonia. Previously reported mutations in the DDP gene have all been frameshifts/nonsense mutations or deletion of the entire gene as part of a larger deletion encompassing the BTK gene. The clinical presentation was uniformly characterised by sensorineural hearing loss, dystonia, mental deterioration, paranoid psychotic features, and optic atrophy, indicating progressive neurodegeneration. Our report illustrates that de novo mutations occur and that a missense mutation, C66W, may cause an equally severe clinical picture. The diagnosis of sensorineural hearing impairment associated with neurologic and visual disability in a male, therefore, should encourage the search for mutations in the DDP gene, even in sporadic cases. The association of deafness-dystonia syndrome with a missense mutation provides valuable information for in vitro investigations of the functional properties of the deafness-dystonia peptide which was recently shown to be the human homolog of a yeast protein, Tim8p, belonging to a family of small Tim proteins involved in intermembrane protein transport in mitochondria.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
4/65. Neural hearing loss in a child with poliomyelitis: a histopathological study.The temporal bones of a 26-month-old white female with a paralytic syndrome clinically and pathologically identical to poliomyelitis were examined. The aetiological agent was unknown although a non-poliomyelitis enterovirus infection seemed likely. There was a complete absence of the cochlear neurons and substantially reduced peripheral and central axons with loss of some inner hair cells but preservation of outer hair cells. Scarpa's ganglion, and the geniculate ganglion were partially atrophied. The saccule and utricle were mildly dilated and Reissner's membrane of the apical turn was bulging. In two previous audiological studies a 10--20 dB bilateral sensorineural hearing loss was found in poliomyelitis patients and a neuronal lesion was postulated which is now supported by our findings. This is a rare example of an almost pure neural hearing loss.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
5/65. cholesteatoma behind an intact tympanic membrane: histopathologic evidence for a tympanic membrane origin.BACKGROUND: Several theories have been proposed with respect to the origin and pathogenesis of cholesteatoma behind an intact tympanic membrane. CASE REPORT: The authors describe a case of cholesteatoma behind an intact tympanic membrane in a 71-year-old man with a history of tympanic membrane retraction fixed to the incus without evidence of a perforation. The membrane eventually became detached, and remnants of keratinizing squamous epithelium were found on the incus. DISCUSSION: Mechanisms such as metaplasia, ectopic epidermis rests, or ingrowth of meatal epidermis have been proposed to explain the pathogenesis of cholesteatoma behind an intact tympanic membrane. These findings, based on temporal bone histopathology, support the role of an acquired epidermal rest. CONCLUSIONS: This case report provides evidence that cholesteatoma behind an intact tympanic membrane can be established from a resolved retraction of the pars tensa of the tympanic membrane.- - - - - - - - - - ranking = 15keywords = membrane (Clic here for more details about this article) |
6/65. temporal bone histopathologic and genetic studies in Mohr-Tranebjaerg syndrome (DFN-1).OBJECTIVE: To describe the temporal bone histopathologic and genetic abnormalities in a case of Mohr-Tranebjaerg syndrome. BACKGROUND: Mohr-Tranebjaezrg syndrome (DFN-1) is an X-linked, recessive, syndromic hearing loss, characterized by postlingual sensorineural hearing loss with onset in childhood, followed in adult life by progressive dystonia, spasticity, dysphagia, and optic atrophy. The syndrome is caused by mutations in the DDP (deafness/dystonia peptide) gene, which are thought to result in mitochondrial dysfunction with subsequent neurodegeneration. The temporal bone pathologic changes in this syndrome have not been reported. methods: hearing loss developed in the patient at age 4, blindness at age 48, and dystonia at age 57. Genetic studies on peripheral blood showed a l51delT mutation in his DDP gene. He died at age 66. The right temporal bone was subjected to light microscopy and polymerase chain reaction-based analysis of the DDP gene sequence. RESULTS: There was near complete loss of spiral ganglion cells with loss of nearly all peripheral and central processes. Only 1,765 spiral ganglion cells remained (8.5% of mean normal for age). The organ of corti (including hair cells), stria vascularis, and spiral ligament were preserved. There was also a severe loss of Scarpa's ganglion cells with preservation of vestibular hair cells. The population of geniculate and trigeminal ganglion cells appeared normal. sequence analysis from temporal bone dna showed the 15ldelT DDP gene mutation. CONCLUSION: Sensorineural hearing loss in Mohr-Tranebjaerg syndrome is the result of a postnatal, progressive, severe auditory neuropathy.- - - - - - - - - - ranking = 1.3260939877129keywords = ligament (Clic here for more details about this article) |
7/65. Otopathology in a case of type I Waardenburg's syndrome.We report a case of type I Waardenburg's syndrome that provides insight into the etiopathogenesis of sensorineural hearing loss (SNHL) in this syndrome. The subject, a 76-year-old woman with type I Waardenburg's syndrome (dystopia canthorum, heterochromia irides, and white hair), had congenital low-frequency SNHL in her right ear only, which had remained relatively stable throughout her life. blood leukocyte dna studies revealed a PAX-3 mutation with a 1 base pair C-to-A substitution in exon 5 at base 602. light microscopic studies of the right cochlea showed intact neurosensory structures in only the lower basal turn, with the remainder of the cochlea showing absence of melanocytes, absence of stria vascularis, missing hair cells, dysmorphogenesis of the tectorial membrane, and lack of peripheral processes of the spiral ganglion cells. There was pathological alteration of the vestibular dark cells with marked reduction of melanocytes associated with these dark cells. The left inner ear was normal, with a full complement of neurosensory structures, including melanocytes. Because the PAX-3 gene is involved in neural crest development and melanocytes migrate from the neural crest to the ear, the findings in this case are consistent with the hypothesis that defective melanocyte migration or defective melanocyte function results in defective development of the stria vascularis (and perhaps other structures of the ear), leading to SNHL.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
8/65. Sensorineural hearing loss in linear IgA disease.A 23-year-old female with progressive sensorineural hearing loss developed widespread blistering eruptions while under evaluation for the hearing loss. The lesions showed findings characteristic of linear IgA disease (LAD) on histochemical studies. Linear IgA disease is a dapsone responsive autoimmune subepidermal blistering disease characterised by linear IgA deposits in the basement membrane zone. The complete spectrum of this systemic disease is still not clear and this is the first report of association between LAD and sensorineural deafness.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
9/65. Ototoxicity induced by gentamicin and furosemide.OBJECTIVE: To present a case of ototoxicity induced by furosemide and once-daily gentamicin therapy. CASE SUMMARY: A 60-year-old white woman presented to the hospital with community-acquired pneumonia and urinary tract infection. The antibiotic regimen included gentamicin and, after 5 doses, the patient reported profound bilateral hearing loss. A Pure Tone Audiogram suggested moderate to moderately severe sensorineural hearing loss bilaterally. The only risk factors present included her age, elevated temperature, and the use of furosemide. DISCUSSION: Several risk factors may predispose a patient to developing aminoglycoside ototoxicity: the 1555 chromosomal mutation, preexisting disorders of hearing and balance, hypovolemia, bacteremia, liver and renal dysfunction, and the simultaneous administration of other ototoxic medications. The cumulative dose and duration of aminoglycoside therapy are more important than serum concentrations. Administration of an aminoglycoside followed by furosemide may increase the risk of ototoxicity. The aminoglycoside interacts with the cell membranes in the inner ear, increasing their permeability. This theoretically allows the loop diuretic to penetrate into the cells in higher concentrations, causing more severe damage. CONCLUSIONS: Auditory toxicity occurred after only 5 days of gentamicin therapy and 1 dose of furosemide. An aminoglycoside followed by furosemide may increase the risk for ototoxicity. Clinicians need to be aware of the synergistic potential of ototoxic medications.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
10/65. Vascular occlusion in the endolymphatic sac in Meniere's disease.In 2 patients with severe Meniere's disease (MD), there was histologic evidence of occlusion of the vein of the vestibular aqueduct (VVA). This finding coincided with total or partial occlusion of numerous small vessels around the endolymphatic sac (ES), flattening of epithelium, extensive perisaccular fibrosis, and signs of new bone formation. Ultrastructural analysis of the occluding material showed foci with dense connective tissue, calcification, lipid deposits, and layers of basement membrane, sometimes concentrically arranged. The exact nature of the occluding material was unknown. In another 2 MD patients, the VVA was not visualized, and the ES vessels showed no signs of occlusion. Seven controls with acoustic schwannoma or meningioma had normal vasculature. The presence of vascular impairment in the ES in MD patients indicated that altered hemodynamics may contribute to the pathogenesis of endolymphatic hydrops and MD.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
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