Cases reported "Hearing Loss, Conductive"

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11/323. osteogenesis imperfecta of the stapes: an histological study.

    Two cases of Van der Hoeve's syndrome are presented with histological findings consistent with the diagnosis of osteogenesis imperfecta. These findings support the view that otosclerosis can be clearly differentiated histologically from osteogenesis imperfecta. Pre-operative impedance studies are recommended on each patient with osteogenesis imperfecta as a useful diagnostic adjunct to determine whether the conductive component in the hearing loss is secondary to stapedial crural deformity and/or footplate fixation. ( info)

12/323. malleus ankylosis: a clinical, audiometric, histologic, and surgical study of 123 cases.

    OBJECTIVE: malleus ankylosis is a cause of conductive hearing loss that can be difficult to assess, particularly in association with otosclerotic stapes fixation. The aim of this study is to seek the clinical and audiometrical features unique to this pathology. STUDY DESIGN: The study design was a review of malleus ankylosis cases and an analysis of functional results after ossiculoplasty. SETTING: The study was performed at Jean Causse Clinic in france. patients: The authors reviewed a series of 123 ears in 112 patients who underwent surgery for incudo-mallear ankylosis from January 1991 to September 1997. Intervention: The surgical technique depends on the type of pathology encountered. In case of associated stapedial fixation, a stapedotomy with vein graft interposition and reconstruction with a total prosthesis will be performed during a same step. MAIN OUTCOME MEASURES: Clinical evaluation, preoperative and postoperative audiometrical evaluation, operative findings, histologic examination, and postoperative functional results. RESULTS: In our series, a postoperative air-bone gap smaller than 10 dB was obtained in 77% of cases. These results confirm the possibility of managing both pathologies in a single surgical step. CONCLUSION: Incudo-mallear ankylosis remains an unusual pathology but should be systematically assessed during surgery and preferably after separation of the incudo-stapedial joint. A preoperative diagnosis is difficult to ascertain; some audiometrical features allow a suspicion. In this series, it is shown that otosclerosis can be responsible for ankylosis as seems to be confirmed by the two cases presented in this study. ( info)

13/323. Neurotologic manifestations and treatment of multiple spontaneous tegmental defects.

    OBJECTIVE: To describe the causes, histopathologic features, manifestations, and treatment of symptomatic multiple spontaneous tegmental defects. STUDY DESIGN: Retrospective review of three clinical cases and one temporal bone histopathology report. CLINICAL FEATURES: Varied, including spontaneous cerebrospinal fluid otorhinorrhea, conductive hearing loss, chronic headaches, pneumocephalus, extradural abscess, and meningitis. A notable common feature was multiple (8-15) tegmental defects, 1 to 6 millimeters in diameter. Three of the four cases also included associated dural defects and small meningoencephaloceles or arachnoid granulations. Imaging studies generally underestimated the number of defects. INTERVENTION AND OUTCOMES: Successful middle cranial fossa repair with temporalis fascia was accomplished in the three clinical cases. Extension of exposure anteriorly and medially was necessary. Closure of the defects with a bone graft or equivalent synthetic material was not always possible, given the anatomic and pathologic features. Our data suggest that there are both congenital and acquired causes of the tegmental dehiscences. CONCLUSIONS: Multiple tegmen defects constitute a special entity. Successful repair requires a middle fossa craniotomy with extended exposure. ( info)

14/323. Spondyloepiphyseal dysplasia congenita associated with conductive hearing loss.

    Spondyloepiphyseal dysplasia is a disorder characterized by abnormalities of growth. Previous studies of this disorder have identified a significant incidence of associated hearing loss. hearing loss has been reported to occur in 25 to 30% of affected patients. To date, all reports of associated hearing loss have indicated the presence of a sensorineural component. In this article, we report the case of a child who was diagnosed with spondyloepiphyseal dysplasia congenita and who was found to have a significant conductive hearing loss with a Carhart's notch, indicating the likely presence of stapes footplate fixation. We also review the diagnosis of this condition and the literature associated with hearing loss as it occurs with this disorder. ( info)

15/323. A family with autosomal dominant inherited dysmorphic small auricles, lip pits, and congenital conductive hearing impairment.

    DESIGN: We examined 3 generations in a family for congenital conductive hearing impairment, dysmorphic small auricles, and lip pits. SETTING: Department of Otorhinolaryngology, University Hospital Nijmegen, Nijmegen, the netherlands. RESULTS: Seven members of the family had bilateral dysmorphic auricles. Three subjects had either a pit or dimple in the lip. Two subjects had congenital conductive hearing impairment. CONCLUSION: Using gene linkage, we confirmed that these autosomal dominant inherited branchial anomalies present a new separate branchial arch syndrome. ( info)

16/323. Congenital absence of the long process of the incus.

    OBJECTIVE/HYPOTHESIS: To review the case reports of three generations of one family, suffering from bilateral conductive hearing loss due to congenital absence of the long process of the incus. STUDY DESIGN: review of the literature regarding hereditary congenital absence of the long process of the incus to determine its mode of inheritance such as autosomal dominant or X-linked. Also document the types and results of surgical repairs performed by previous investigators of this defect and compare them with the present study. methods: Three female patients of direct lineage. Mother, daughter, and granddaughter, who were personally seen, operated on, and followed up over a period of 26 years. RESULTS: All three female patients presented with similar symptoms and findings of hearing impairment since birth, with no history of ear infections, and with normal appearing tympanic membranes. However, they had severe conductive hearing loss with normal bone conduction and discrimination. Operative findings were identical in all three patients, with the incus ending in a blind stub, and with absence of the capitulum of the stapes. The type of ossicular reconstruction varied with the changing of times and advances in otologic surgery, as well as the prosthetic materials used. All three patients received improvement in their hearing, and this improvement has been maintained over the 26-year span of the study. CONCLUSIONS: hearing loss due to congenital absence of the incus is uncommon but not rare. Interestingly, no other instance of inherited cases were reported in the literature. In this family the defect appears to be an inherited trait either due to an autosomal dominant mutation or an X-linked dominant inheritance. This abnormality is amendable to surgery, and any well-executed technique usually gives good hearing results. ( info)

17/323. Conductive hearing loss and multiple pre- and supra-auricular skin defects: a variant example of the Branchio-Oculo-Facial syndrome.

    Conductive hearing loss and multiple pre- and supra-auricular skin defects: a variant example of the Branchio-Oculo-Facial syndrome: We describe a 3-year-old girl with bilateral severe conductive hearing loss and multiple bilateral supra- and preauricular defects with unusual overlying thin skin. The severe conductive hearing loss is due to abnormal configuration of the ossicular chain. Multiple pre- and supra-auricular skin defects have been described in the Branchio-Oculo-Facial syndrome, and we propose that the findings in our present patient may be a variant manifestation of this syndrome. ( info)

18/323. Glial lesion of the infratemporal fossa presenting as a soft tissue middle ear mass - rudimentary encephalocele or neural crest remnant?

    We report about ectopic glial tissue of the skull base and the parapharyngeal space presenting as a soft tissue mass in the middle ear. An 11-year-old boy presented with bilateral conductive hearing loss since early childhood. The history included previously removed lesions consistent with ectopic neuroglial tissue of the tongue and the parapharyngeal space soon after birth, as well as surgery for cleft palate. High resolution computed tomography of the petrous bone and magnetic resonance imaging were useful in identifying the skull base defect and in characterizing the lesion's relation to the brain. There was no clinical, radiological or surgical evidence of any associated dural defect. The lesion was removed via a modified infratemporal approach. histology revealed neuroglial tissue with calcifications without any signs of mesodermal or entodermal origin. On the basis of this case the pathogenesis and diagnosis of ectopic brain tissue and its relation to the more commonly encountered meningoencephalic herniations are reviewed. Furthermore therapeutical implications are discussed. ( info)

19/323. Audit of stapedectomy in the north west of england for 1996 and an analysis of the criteria used to describe success.

    A questionnaire was used to collect information related to stapedectomy from all the NHS Trusts in the North West region. The data collected included the identity and rank of the surgeon, the age and sex of the patients, the side of the operation, the audiometric values of the air conduction audiogram preoperatively and postoperatively, and an account of any complications. The results were analysed and compared to national and international standards. The criteria for analysis of success in surgery for conductive hearing loss were reviewed. ( info)

20/323. Aberrant internal carotid artery in the middle-ear space.

    The incidence of an aberrant internal carotid artery in the middle ear is approximately one per cent and most patients are asymptomatic. We present two patients with an aberrant internal carotid artery who presented with pulsatile tinnitus and an intra-tympanic mass. Here we discuss the clinical presentation, relevant radiographic investigations and further management of these patients. ( info)
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