Cases reported "Hearing Loss, Conductive"

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1/40. The leaking labyrinthine lesion resulting from direct force through the auditory canal: report of five cases.

    The leaking labyrinthine lesion is treated by conservative methods or surgical procedures. With respect to the stapes, the surgical treatment is controversial. Five cases of middle ear injuries accompanying oval window rupture are reported herein. In each case, direct force through the auditory canal damaged not only the ossicular chain but also the oval window. Initial symptoms were sudden hearing loss with significant conductive disturbance and various degrees of unsteadiness. Spontaneous horizontal nystagmus directed toward the uninvolved ear was observed in each case. Tympanic cavities were promptly explored under general anesthesia and oval window injuries were confirmed. In each case, the damaged stapes was temporally removed from the oval window. Perilymphatic leakage was recognized in each case. Two patients had subluxation of the stapes with a paucity leakage. Three had complete luxation of the stapes with a relatively huge oval window fistula. Disrupted oval windows were repaired with temporalis muscle fascial grafts that were inserted under the middle ear mucosae surrounding the oval windows. The stapes were replaced in the repaired oval windows, and the ossicular chains were reconstructed without artificial grafts. Vestibular dysfunctions disappeared within 7 days, and satisfactory audiologic results were obtained in each case.
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ranking = 1
keywords = mucosa
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2/40. 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.
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ranking = 18.81138453218
keywords = membrane
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3/40. 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.
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ranking = 18.81138453218
keywords = membrane
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4/40. 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.
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ranking = 75.245538128718
keywords = membrane
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5/40. The natural history of congenital cholesteatoma.

    OBJECTIVES: To describe the natural history of congenital cholesteatoma (CC) and to determine whether such a description provides clues about the origins and end points of these lesions. DESIGN: A retrospective qualitative analysis of intraoperative illustrations of 34 consecutive patients with 35 CCs (1 bilateral). SETTING: Two tertiary care children's hospitals. patients: Thirty-four children with CC, mean age, 5.6 years (range, 2-13 years). RESULTS: Congenital cholesteatoma originates generally, but not universally, in the anterior superior quadrant. The progression of growth is toward the posterior superior quadrant and attic and then into the mastoid. Contact with the ossicular chain generally results in loss of ossicular continuity and in conductive hearing loss. CONCLUSIONS: Congenital cholesteatoma appears to have a predictable trajectory of growth, starting as a small pearl in the middle ear, eventually growing to involve the ossicles and mastoid, and causing varying degrees of destruction and functional impairment. The clinical picture of a young child with otorrhea, conductive hearing loss, tympanic membrane perforation in a nontraditional location, and a mastoid filled with cholesteatoma may represent the end point in the natural history of CC, despite the fact that this type of lesion is outside the accepted definition of CC.
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ranking = 18.81138453218
keywords = membrane
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6/40. Lateralization of the tympanic membrane as a complication of canal wall down tympanoplasty: a report of four cases.

    OBJECTIVE: To describe the pathophysiology and treatment of the lateralized tympanic membrane that occurs after canal wall down tympanoplasty. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral hospital. patients: Four patients in whom lateralization of the tympanic membrane developed as a complication of canal wall down tympanoplasty. RESULTS: The patients had undergone middle ear surgery 20 to 34 years before their first visit to the authors. A Bondy operation with soft-wall reconstruction of the ear canal had been performed in three patients and a modified radical mastoidectomy in one patient. They all had severe conductive hearing loss. Common findings were anterior canal sulcus blunting, good tubal function, normal middle ear mucosa, and mobile stapes. At revision surgery, the lateralized tympanic membrane was removed, and the temporalis fascia was grafted medial to the malleus manubrium. The exposed bony surface in the ear canal was covered with a split-thickness skin graft, and the ear canal and the mastoid cavity were tightly packed to secure the graft. All the patients regained good hearing after the revision. Although deterioration of the anterior tympanic ring was presumed to be the primary cause of the graft lateralization, the lack of a posterior bony ear canal might have facilitated this condition. CONCLUSION: Lateralization of the tympanic membrane can occur even in an ear with a radicalized mastoid cavity, especially when the anterior tympanic ring is torn and the posterior ear canal is reconstructed with soft tissue.
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ranking = 151.49107625744
keywords = membrane, mucosa
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7/40. Supralabyrinthine approach to petrosal cholesteatoma.

    Petrosal cholesteatomas are rare lesions, which may be congenital or acquired in nature. We report an exceptional case occurring in a seven-year old girl who presented with a unilateral conductive hearing loss, despite normal tympanic membrane appearance. early diagnosis was facilitated by computed tomography (CT) scanning. Although this case satisfied the criteria for congenital cholesteatoma, it is likely that the petrous apex was secondarily involved. Complete cholesteatoma removal was accomplished using a transtemporal supralabyrinthine approach, which allowed for hearing preservation, while avoiding the morbidity associated with a craniotomy. The present case constitutes the youngest case of petrosal cholesteatoma reported. We suggest that a lower threshold for the use of CT scanning in unilateral conductive hearing loss may allow for the earlier detection of more cases of petrosal cholesteatomas, as well as facilitating their removal using more limited approaches associated with less morbidity.
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ranking = 18.81138453218
keywords = membrane
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8/40. Overlay versus underlay tympanoplasty. comparative study of 122 cases.

    INTRODUCTION: In tympanoplasty, the most common two techniques for positioning the graft relative to the remnant of both the tympanic membrane and of the annulus, are the "overlay" and the "underlay" techniques. Each technique has advantages and disadvantages. methods: One hundred and twenty-two cases over the age of 8 years who had undergone a tympanoplasty for tympanic membrane perforation secondary to chronic otitis media were included. All patients had a minimum 3-month postoperative otoscopic and audiometric follow-up. RESULTS: of 122 cases, 115 tympanoplasties (94%) were anatomically successful. At frequencies of 0.5, 1, 2, and 4 kHz, the mean air-bone gap improved significantly from 21.7 dB preoperatively to 8.4 dB postoperatively giving a mean gain of 13.3 dB. CONCLUSION: In our series the underlay or overlay positioning of the graft does not significantly influence the rate of postoperative perforations or complications with the exception of epithelial pearls, which occur significantly more frequently following the overlay technique for perforations that require fibro-epidermal cleaving across a large area.
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ranking = 37.622769064359
keywords = membrane
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9/40. A case of congenital bilateral stapes agenesis.

    Congenital absence of the stapes and the oval window is an anomaly reported in only sporadic cases. We present a 17-year-old male patient with congenital bilateral conductive hearing loss. The external auditory canal and the tympanic membrane appeared normal on both sides. An exploratory tympanotomy in the right ear revealed dehiscence of the the horizontal segment of the facial nerve, which was displaced inferiorly, occupying the area of the absent oval window. The stapes was totally absent and a malformed long process of the incus was attached by a fibrous band to the promontory. Manipulation of the facial nerve in association with stapedectomy or vestibulotomy was avoided in order not to injure the nerve. Instead, amplification with hearing aids was recommended to the patient.
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ranking = 18.81138453218
keywords = membrane
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10/40. Acquired atrophy of the long process of the incus.

    Various lesions can cause conductive hearing loss in a patient with a normal tympanic membrane. These include congenital ossicular anomaly, otosclerosis, and congenital or acquired ossicular fixation and discontinuity. We had an experience with a patient who presented with a conductive hearing loss in both ears, in which small pieces of the long process of the incus were absent and had been replaced with fibrous tissues in both ears. No other abnormalities, such as postinflammatory changes or fixation of the ossicles, were found. Because the long process of the incus undergoes remodeling through resorption and rebuilding throughout life, failure of the remodeling or impaired vascular supply to the long process of the incus may have been the cause of the conductive hearing loss in this patient.
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ranking = 18.81138453218
keywords = membrane
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