Cases reported "Hearing Loss, Conductive"

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21/323. Inner ear malformations and hearing loss in linear nevus sebaceous syndrome.

    OBJECTIVE: Linear nevus sebaceous syndrome (LNSS) is a rare disorder comprising of nevus sebaceous, seizures, and mental retardation. While extensive literature describe the dermatologic, neurologic, as well as ophthalmologic manifestations of this LNSS, otologic problems have not been previously described. The objective of this report is to describe the otologic manifestation of a patient with LNSS. methods: A child with LNSS was referred to the Otologic clinic for evaluation of hearing loss. Pertinent findings on history, physical findings, audiometric testing, and imaging studies are discussed. RESULTS: Audiometric testing showed bilateral conductive hearing loss. Computerized tomography of the temporal bone demonstrated widened internal auditory canals and dysplastic lateral semicircular canals. CONCLUSION: LNSS can be associated with hearing impairment and inner ear malformations. The evaluation of a child with LNSS should include otologic and audiologic testing. ( info)

22/323. otitis externa sicca/fibrotising external otitis (FEO) as a complication of sjogren's syndrome.

    sjogren's syndrome (SS) is a condition characterized by sicca symptoms and by autoimmune features. We describe two SS patients with otitis externa fibroticans/sicca. One of these 2 patients developed a lesion of the tympanic membrane making it necessary to perform a tympantomy and meatoplasty. Our findings suggest firstly that the epithelial cell-mediated secretion of lamellar bodies and the production of the permeability barrier are defective in SS. Secondly, local moisturing and/or topical corticosteroid treatment in SS patients with sicca symptoms in the auditory canal could help to avoid reconstructive surgical treatment. ( info)

23/323. Post-nasal space oncocytoma: a different approach to a rare tumour.

    Oncocytomas are rare tumours that occur predominantly in the major salivary glands, particularly the parotid of older individuals. We present the exceptionally rare occurrence of an oncocytoma in the post-nasal space and its treatment for the first time via a Le Fort I osteotomy. The potential for local spread to the surrounding skull base makes it vital to achieve good oncological clearance. We found that this was possible using the Le Fort I technique and would recommend that this approach should be considered in future, when approaching such lesions. ( info)

24/323. 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. ( info)

25/323. mandibulofacial dysostosis, microcephaly and thorax deformities in two brothers: a new recessive syndrome?

    We report two brothers who presented with mandibulofacial dysostosis, growth retardation, microcephaly, thoracic deformities and conductive hearing loss along with asplenia in one case and aplasia of the gallbladder in the other. The pattern of malformations differs significantly from established syndromes with mandibulofacial dysostosis such as Nager syndrome or Genee-Wiedemann syndrome and also from cerebro-costo-mandibular syndrome. As chromosome analysis revealed normal male karyotypes, we consider this to be a distinct heritable syndrome that may be either autosomal recessive or X-chromosomal recessive. ( info)

26/323. Mature teratoma of the middle ear.

    OBJECTIVE: The authors report a case of mature teratoma of the middle ear in a 3-year-old girl with a 1-year history of otitis media. methods AND RESULTS: Radiologic investigation revealed a partially cystic lesion of the petrous portion of the right temporal bone. It produced opacification of the middle ear as well as destruction of septal air cells. The patient underwent a subtotal petrosectomy. Histologically, the tumor was composed of an intimate admixture of mature tissues representing all three germ layers, including brain, myelinated nerve trunks, skeletal muscle, bone, immature cartilage, seromucinous glands, and respiratory epithelium. Of note within the brain tissue was choroid plexus within an ependyma-lined rudimentary ventricle. Immunohistochemical studies were also performed. Twenty months after surgery, the patient was well, with complete recovery from symptoms. CONCLUSION: Teratomas of the middle ear are rare neoplasms. Only a few examples have been reported. As a rule, they are cured by resection and do not require adjuvant therapy. ( info)

27/323. Fitting low ratio compression to people with severe and profound hearing losses.

    OBJECTIVE: In this study, we aimed to determine the extent to which wide dynamic range compression and compression limiting could benefit severely and profoundly hearing-impaired adults. DESIGN: Subjects were fitted with multi-memory hearing aids incorporating frequency independent input-controlled compression with a 2:1 compression ratio and output-controlled compression limiting. The input compression threshold (CT) was varied to establish the lowest level that maintained audibility for conversational intensity speech without acoustic feedback oscillation. Where a low (40 to 57 dB SPL) CT was possible, this was compared with a moderate (65 to 74 dB SPL) level. The preferred input compression setting was subsequently compared with linear, compression-limited amplification in the same aids. In the three cases where 2:1 input compression could not be used, because of feedback or loudness insufficiency problems, compression limiting was compared with peak clipping. Field trials were conducted over a 2- or 3-mo period to establish preferences. RESULTS: Nine of the 16 subjects preferred the inclusion of the higher level input compression, and one preferred lower level input compression. Four subjects preferred linear, compression-limited amplification, one favored peak clipping, and one could not be satisfied with any of the options provided. CONCLUSIONS: 2:1 input compression was useful, and preferred by 10 of the subjects, but for nine subjects the preferred CT was relatively high. With a group 4-frequency average loss of 87 dB HTL, the results demonstrate that fast-acting, low compression ratio systems can be useful for losses traditionally regarded as the domain of linear amplification. ( info)

28/323. Artificial tympanic membrane and ossiculoplasty.

    We report an unusual case of a subjective hearing improvement, confirmed by audiometry, in a patient using a paper towel prosthesis. ( info)

29/323. The cerebro-costo-mandibular syndrome: 9-year follow-up of a case.

    Cerebro-costo-mandibular syndrome (CCMS) is a rare multiple congenital anomaly with a low survival rate. There are few reports of long-term survival in this condition. We describe the findings and management of a 9-year-old survivor of CCMS, outline the importance of early intervention and multidisciplinary team approach. The child presented in the neonatal period in respiratory distress with classical features of the syndrome. Aggressive initial respiratory management was later followed up with an integrated multidisciplinary team approach. He has been carefully followed up for nine years now, illustrating well, the course of the syndrome. ( info)

30/323. Goldenhar's syndrome: congenital hearing deficit of conductive or sensorineural origin? temporal bone histopathologic study.

    BACKGROUND: Oculoauriculovertebral dysplasia (OAVD) (Goldenhar's syndrome) is a congenital syndrome with ipsilateral deformity of the ear and face, epibulbar lipodermoids, coloboma, and vertebral anomalies. Goldenhar's anomaly has often been associated with a degree of congenital hearing deficits, almost always of a conductive origin, but a sensorineural component is also suspected in some cases, evident through malformations of the inner ear. patients AND methods: Both temporal bones of a 10-day-old deceased patient with oculoauriculovertebral dysplasia were examined by light microscopy. RESULTS: The ear deformities included deformity of the auricle, atresia of the external auditory canal, and malformation of the tympanic cavity and ossicles. Abnormalities of the stria vascularis and the semicircular canals were also demonstrated. Further inner ear deformities were not identified in this case. CONCLUSION: These histopathologic findings appear to confirm the conductive component of the congenital hearing deficit, but a sensorineural component could not be omitted. The ear alterations favor early developmental field defects. The causes of this condition are controversial. Recent results in genetic research pertaining to the MSX class genes permit better understanding of the variety, variability, and different degrees of severity of the anomalies described here. ( info)
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