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1/41. Sensorineural hearing loss associated with otitis media with effusion in children.

    Sensorineural hearing loss (SNHL) is known to occur in various types of otitis media. Although the mechanism by which SNHL develops in association with otitis media with effusion (OME) is unknown, several hypotheses have been advocated up to now. We reviewed the clinical records of children with otitis media with effusion (OME) to reveal the association with sensorineural hearing loss. The material consisted of 71 children (119 ears) who were diagnosed as having OME and gave reliable audiograms in our clinic during an 11 month period from February 1997 through January 1998. From these cases those which showed bone conduction loss of 25 dB or higher at any one of the frequencies of 250 through 4 kHz were selected and considered to be cases of SNHL. Eight cases (9%) which had temporary threshold shift (TTS) or permanent threshold shift (PTS) were considered to be etiologically related to OME. The clinical course in each of these cases with SNHL was reviewed and evaluated in detail. We noted that all children with TTS improved completely. The result of this study indicates that we have to be aware of a possible development of SNHL during the course of OME.
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keywords = sensorineural hearing loss, hearing loss, sensorineural hearing, sensorineural, hearing
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2/41. Spontaneous cerebrospinal fluid otorrhea from a tegmen defect: transmastoid repair with minicraniotomy.

    Spontaneous cerebrospinal fluid (CSF) otorrhea is a rare condition that presents in 2 clinical categories. In congenital labyrinthine malformations, it leads to bouts of meningitis in a hearing-impaired child. In the adult age group, a spontaneous CSF leak almost always results from a dural and bony defect in the tegmen area. Possible pathogenic mechanisms include progressive sagging and rupture of dura through a congenital tegmen dehiscence and progressive bone erosion by aberrant arachnoid granulations. These patients usually present with a middle ear effusion, resulting in clear discharge after myringotomy with tube insertion. Based on 4 patients with a CSF leak from a tegmen defect, this report reviews the clinical findings and diagnostic approach. The surgical management by a 5-layer closure using a transmastoid approach with minicraniotomy is outlined. This procedure offers a relatively simple and reliable method for repair without the inherent risks of a middle fossa craniotomy.
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ranking = 0.041833522539428
keywords = hearing
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3/41. A possible otological complication due to maxillary expansion in a cleft lip and palate patient.

    OBJECTIVE: We present a possible negative side effect of a sudden onset of secretory otitis media in a 12-year-old boy with unilateral cleft lip and palate who underwent maxillary expansion. The secretory otitis media caused a temporary hearing loss developed during the activation of the expander appliance. The possible causes for this complication are discussed. CONCLUSIONS: Clinicians should be aware of the possible association between maxillary expansion and secretory otitis media.
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ranking = 0.13311743767266
keywords = hearing loss, hearing
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4/41. Wegener's granulomatosis: diagnostic dilemma.

    Wegener's granulomatosis is a systemic disease characterized by a granulomatous lesion that can affect any organ throughout the body. This case-report illustrates the problem posed by a patient presenting with bilateral serous otitis media with marked sensorineural hearing loss, facial nerve paralysis and an atypical serological picture. The importance of early diagnosis and the protocol for the management of a patient with an uncertain diagnosis is discussed. Due to atypical presentations, only a high index of suspicion will ensure an early diagnosis.
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ranking = 0.33441281163672
keywords = sensorineural hearing loss, hearing loss, sensorineural hearing, sensorineural, hearing
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5/41. Acute coalescent mastoiditis and acoustic sequelae in an infant with severe congenital neutropenia.

    We report a 2-month-old boy with severe congenital neutropenia (SCN), who developed acute necrotizing otitis media and coalescent mastoiditis due to methicillin-sensitive staphylococcus aureus. The infection fulminantly progressed within a day to a subtotal perforation of the tympanic membrane, destructive bony changes of ossicles, lateral subperiosteal abscess, and suppurative labyrinthitis. Despite the combined treatment with intravenous antibiotics and granulocyte colony-stimulating factor, the infection resulted in mixed hearing impairment. Much attention should be given to prompt diagnosis of otomastoiditis in SCN, a rare congenital disorder, since resultant acoustic sequelae may affect subsequent speech development and intellectual ability.
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ranking = 0.041833522539428
keywords = hearing
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6/41. Clinical characteristics of so called eosinophilic otitis media.

    OBJECTIVE: Although "eosinophilic otitis media" is not as uncommon a condition as was previously believed, its cause and pathogenesis are not yet fully understood. The purpose of this study was to describe the clinical characteristics in patients with "eosinophilic otitis media" to clarify its pathogenesis. methods: Seven adult patients with persistent and intractable otitis media with viscous middle ear effusion containing many eosinophils, who were also under treatment for bronchial asthma, were studied. The following examinations were conducted: nasopharyngeal endoscopy, pure-tone audiometry, eustachian tube function test, temporal bone CT scan, blood analysis, bacterial and fungal culture of middle ear effusion, histological study of the middle ear and nasal specimens, and measurement of eosinophilic cationic protein (ECP) in middle ear effusion. RESULTS: Some patients had persistent perforation with papillomatous granulation tissue arising from the mesotympanic mucosa, and all the patients had nasal polyposis. The pure-tone audiometry showed the mixed-type of hearing loss in all the patients, and the hearing level deteriorated progressively during the course in some patients. The eustachian tube function was not always poor but was patulous in some cases. The most severely diseased areas were in the eustachian tube and mesotympanum by temporal bone CT images. All the seven patients had the high levels of total serum IgE, but the RAST scores were negative in three patients and low grade in three patients. The accumulation of eosinophils was observed in middle ear effusion, middle ear mucosa and nasal polyps, and the eosinophils were highly activated with degranulation. High level of ECP was also recovered from middle ear effusion. CONCLUSIONS: Active eosinophilic inflammation occurs in the entire respiratory tract, including the middle ear in these patients. From our present investigation, we propose the criteria and clinical characteristics of "eosinophilic otitis media".
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ranking = 0.17495096021208
keywords = hearing loss, hearing
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7/41. Otologic manifestations of Wegener's granulomatosis.

    OBJECTIVE/HYPOTHESIS: To evaluate the clinical features, treatment, and outcomes of otologic manifestations in Wegener's granulomatosis (WG) treated at Hokkaido University Graduate School of medicine, Sapporo, japan. STUDY DESIGN: We retrospectively reviewed 15 cases of WG with ear involvement. methods: Twenty-five patients with WG were treated at Hokkaido University Graduate School of medicine between 1992 and 2001. Fifteen of these patients had otologic symptoms. We evaluated the clinical course, method of therapy, and outcomes in all cases. Diagnosis of WG was made when the patients had clinical findings and a positive titer of cytoplasmic pattern antineutrophil cytoplasmic antibodies (c-ANCA), or when there were clear histologic findings. We also present three case reports. RESULTS: In 15 cases, the most frequent finding was chronic otitis media. Sensorineural hearing loss was present in 2 patients. In 7 patients whose otologic manifestations were the primary involvement of WG, all were confirmed positive for c-ANCA and were treated with glucocorticoids and immunosuppressive drugs. Three patients who could be treated within 1 month of symptom onset showed marked improvement. CONCLUSIONS: In localized cases, biopsy specimens are often small, and it is frequently difficult to make a histologic diagnosis. The prognosis for hearing was poor when appropriate treatment was not given in the early stages of the disease. Therefore, WG should be included in the differential diagnosis in cases of atypical inflammatory states of the ear. early diagnosis and appropriate treatment are important to prevent irreversible changes in the middle ear and inner ear.
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ranking = 0.17495096021208
keywords = hearing loss, hearing
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8/41. hearing loss due to mannosidosis and otitis media with effusion. A case report and review of audiological assessments in children with otitis media with effusion.

    A case of a child with mannosidosis and bilateral otitis media with effusion (OME) is reported here along with some discussion of relevant literature to emphasize the need for age appropriate audiometric assessment before and after insertion of grommets for glue ear (OME). There is a need for multidisciplinary teamwork in the management of children with hearing loss. If OME is treated surgically, age-appropriate hearing assessment is required before and after insertion of grommets. The need for audiological assessments will be relevant even if children had passed the newborn hearing screening test.
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ranking = 0.21678448275151
keywords = hearing loss, hearing
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9/41. endolymphatic sac tumor in a 4-year-old boy.

    INTRODUCTION: endolymphatic sac tumors (ELST) are rare, low-grade, locally aggressive papillary neoplasms. We present a case of a 4-year-old boy with an ELST, the youngest described in the literature. CASE: A boy presented with a right-sided serous otitis media and sudden-onset right facial nerve palsy. An audiogram revealed right-sided profound sensorineural hearing loss. Radiographic imaging demonstrated a 3-cm expansile lytic lesion along the posterior face of the petrous bone. INTERVENTION/RESULTS: The patient initially underwent a right transmastoid-infralabyrinthine biopsy. Pathologic examination revealed a papillary lesion suspicious for an ELST. Subsequently, a transtemporal-transcochlear approach with intra-and extradural resection of the tumor was performed. The facial nerve was dissected and transposed anteriorly and preserved. Histopathologic and immunohistochemical studies confirmed the ELST. At his 6-month follow up, there is no evidence of recurrence and the facial nerve function has returned to Grade II palsy. CONCLUSION: ELST are rare tumors of the temporal bone. This is the youngest case of ELST reported. Presentation, evaluation, and management of ELST is discussed.
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ranking = 0.33441281163672
keywords = sensorineural hearing loss, hearing loss, sensorineural hearing, sensorineural, hearing
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10/41. Management of brain herniation and cerebrospinal fluid leak in revision chronic ear surgery.

    OBJECTIVES/HYPOTHESIS: brain herniation and cerebrospinal fluid (CSF) leakage into the middle ear and mastoid are rare but described complications of chronic ear disease. This paper will discuss the presentation and management of brain herniation and/or CSF leak encountered in revision chronic ear surgery. STUDY DESIGN: Retrospective chart review. methods: Twelve of 1,130 cases of revision chronic ear surgery in which brain herniation or CSF leak was diagnosed were identified and analyzed. RESULTS: Ten (83%) patients' initial diagnosis was tympanic membrane (TM) perforation with cholesteatoma and two (17%) with TM perforation without cholesteatoma. Initial revision procedures included one (8.3%) tympanoplasty with canal-wall-up mastoidectomy maintaining ossicular continuity, two (17%) tympanoplasties with canal-wall-down (CWD) mastoidectomies with ossicular chain reconstruction (OCR), and nine (75%) tympanoplasties with CWD mastoidectomies without OCR. Three (25%) required a second procedure, two (17%) a third, and one (8.3%) a fourth, finally resulting in four (33%) with an ossicular reconstruction and eight (67%) without. brain herniation and/or CSF leak were repaired by way of transmastoid and middle fossa approaches. Preoperative and postoperative pure-tone average air-bone gaps were statistically similar (33.1 and 28.1 dbHL, respectively; P = .464). CONCLUSIONS: brain herniation and/or CSF leak appear to be rare complications of surgery for revision chronic ear disease. Their management require adherence to the principles of establishing a safe ear with hearing restoration as a secondary goal.
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ranking = 0.041833522539428
keywords = hearing
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