1/10. Histopathology of residual and recurrent conductive hearing loss after stapedectomy.HYPOTHESIS: Histopathologic examination of temporal bones from patients who had undergone stapedectomy may provide information concerning the causes of both residual and recurrent conductive hearing loss (CHL). BACKGROUND: Although closure of the air-bone gap to within 10 dB occurs in approximately 90% of primary stapedectomies, a residual CHL occurs in approximately 10% and recurrent CHL may occur in up to 35% of cases. Putative causes of failure of surgery as determined during revision include erosion of the incus, bony regrowth at the oval window, and displacement of the prosthesis. Most reports on the histopathologic findings of temporal bones from such patients have focused on complications of surgery, with little attempt to correlate postoperative air-bone gap with the observed histopathology. methods: A retrospective review of the author's collection of temporal bones ascertained 22 cases with postoperative CHL of 10 dB or greater (air-bone gap averaged at 500, 1,000, 2,000, 3,000, and 4,000 Hz, using postoperative air- and bone-conduction levels) after stapedectomy. These temporal bones were prepared by standard methodology for light microscopy. RESULTS: Of the 22 cases with postoperative CHL equal to or greater than 10 dB, there were 19 with residual CHL, 2 with recurrent CHL, and 1 with both residual and recurrent CHL. The most common histopathologic correlates of residual and recurrent hearing loss included resorptive osteitis of the incus (64%); obliteration of the round window by otosclerosis (23%); the prosthesis lying on a residual footplate fragment (23%); the prosthesis abutting the bony margin of the oval window (18%); adhesions in the middle ear (14%); and new bone formation in the oval window (14%). CONCLUSIONS: Histopathologic examination of temporal bones from patients who in life had undergone stapedectomy provides useful information concerning causes of both residual and recurrent CHL. These data provide a basis for improving both surgical technique and prosthesis design.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/10. Trembling chin--a report of this inheritable dominant character in a four-generation Canadian family.Trembling chin is an unusual, benign, dominant trait. We describe the condition in a four-generation Canadian family. Investigations, which included E.E.G., electromyograph and facial nerve conductance studies on affected and unaffected individuals, were normal. Premature hearing loss, present in all affected members of this family over age 50, strongly suggests an association of trembling chin with otosclerosis. The merits of early diagnosis and preventive treatment for otosclerosis in persons affected by trembling chin are discussed.- - - - - - - - - - ranking = 1158.777349283keywords = family (Clic here for more details about this article) |
3/10. Cochlear otosclerosis presenting in children: a case report.A case of cochlear otospongiosis in a 14-year-old is reviewed. The patient, who had a strong family history of otosclerosis corrected with stapes surgery, presented with a mild-to-moderate predominantly neurosensory hearing loss and absent stapes reflexes. A CT scan demonstrated changes characteristic of otospongiosis. The relevant literature was reviewed and fluorides as a possible therapy discussed.- - - - - - - - - - ranking = 193.12955821383keywords = family (Clic here for more details about this article) |
4/10. Chondrogenic potential of tragal perichondrium: a cause of hearing loss following stapedectomy.Tragal perichondrium is a widely used tissue seal in the oval window following stapes surgery. Autogenous and easily accessible, it is a suitable substance to cover the vestibule in total stapedectomy, and to seal around the prosthesis in small-fenestra stapedotomy. The incidence of complications from the use of perichondrium in this manner is exceedingly low. We report a case where tragal perichondrium in the oval window resulted in the proliferation of cartilage. The cartilage displaced the stapes prosthesis, resulting in a conductive loss. Although the chondrogenic potential of perichondrium is known, we are not aware of other reports implicating this as a cause of failure in stapes surgery. The pertinent clinical and experimental literature regarding chondrogenesis is reviewed. This information suggests that the formation of cartilage from perichondrium in the oval window might be influenced by mechanical trauma and tissue orientation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/10. Progressive sensorineural hearing loss and cochlear otosclerosis: a prospective study.The association of otosclerosis with reduced bone conduction is well known but no experimental or valid clinical relationship has been established to confirm this relationship. The purpose of this presentation is to demonstrate a clinical relationship between otosclerosis and sensorineural hearing loss. Experimental proof will await the accumulation and study of temporal bones of those individuals who, in life, exhibited the clinical relationship to be developed in this dissertation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/10. Combination of fibromuscular hyperplasia, renal aplasia, hypoplasia or dysplasia and otosclerosis occurring in the same individual or the same family.We have found 3 reports of fibromuscular hyperplasia (FMH) of the renal arteries with hypertension occurring in 2 siblings as well as a few instances of renal agenesis and unilateral renal aplasia occurring in the same family. In this paper we report on FMH of the renal arteries in 2 women, one giving birth to a child with renal agenesis, the other to a child with a focally dysplastic hypoplasia of the kidneys. A third family is reported, heavily loaded with hypertension and otosclerosis, in which 2 siblings with FMH and otosclerosis were found. Another 4 patients without known heredity for hypertension, but with FMH combined with renal or urinary tract anomalies, are also reported. The findings are discussed, particularly in relation to the findings in a large material of chronic non-obstructive pyelonephritis, where in those with well maintained renal function, hypertension below the age of 40 was found predominantly in females with a positive family history of hypertension and signs highly suggestive of infected hypoplasia or dysplasia of the kidney.- - - - - - - - - - ranking = 1351.9069074968keywords = family (Clic here for more details about this article) |
7/10. Histologically proven cochlear otosclerosis with pure sensorineural hearing loss.This paper discusses cochlear otosclerosis without stapedial fixation as a cause of sensorineural hearing loss. Because many cases of cochlear otosclerosis may remain undiagnosed, we believe that patients with a progressive sensorineural hearing loss and a family history of cochlear otosclerosis should be suspected of having cochlear otosclerosis. A positive stapedial reflex on-off effect or polytomographic findings help make the diagnosis. We prescribe sodium fluoride, calcium, and vitamin d for these patients in an effort to prevent progression of the loss.- - - - - - - - - - ranking = 193.12955821383keywords = family (Clic here for more details about this article) |
8/10. Plasma-cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes: the poems syndrome. Distinctive radiographic abnormalities.Three patients with an unusual multisystemic syndrome characterized by polyneuropathy, organomegaly *especially hepatosplenomegaly), endocrine dysfunction, M-protein, and skin abnormalities (poems syndrome) are discussed. Characteristic radiographic features include single or multiple osteosclerotic lesions and peculiar variety of bony proliferation. The relationship of the disorder to multiple myeloma and plasmacytoma and the pathogenesis of the skeletal abnormalities remain obscure.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/10. Buschke-Ollendorff syndrome, otosclerosis, and congenital spinal stenosis.We report a family with the Buschke-Ollendorff syndrome and several unusual features, including one individual with congenital spinal stenosis and another with hearing loss, probably due to otosclerosis. Other reported abnormalities associated with this syndrome, including otosclerosis, are reviewed.- - - - - - - - - - ranking = 193.12955821383keywords = family (Clic here for more details about this article) |
10/10. Camurati-Engelmann disease presenting as 'juvenile otosclerosis'.Camurati-Engelmann disease (CED, MIM # 131300) was diagnosed in a 27-year-old woman, who had pain in her lower legs. There was no history of hearing loss (or CED) in her family. She had been suffering from progressive hearing impairment since childhood. Serial audiograms demonstrated progressive mixed hearing loss from the age of 11 years onwards, for which she underwent successful stapes surgery bilaterally at the age of 13-18 years. Clinical ('juvenile') otosclerosis was found with stapes footplate fixation. Unfortunately, the sensorineural hearing loss component proved to be progressive bilaterally after surgery. At the age of 30 years, she experienced more rapid deterioration of her hearing loss in the right ear and episodes of vertigo. Vestibular examinations demonstrated progressive vestibular impairment 'otospongiotic' lesions with encroachment on the cochlear and vestibular labyrinth were found on high resolution CT scans at the age of 40 years; the internal auditory canal was normal bilaterally. CED may have formed a chance combination with juvenile otosclerosis in our patient, or otosclerosis may be part of the CED phenotype.- - - - - - - - - - ranking = 193.12955821383keywords = family (Clic here for more details about this article) |
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