Cases reported "Hearing Disorders"

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1/19. 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 = 1
keywords = labyrinthitis, otitis
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2/19. 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 = 0.29537266094465
keywords = otitis
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3/19. Long-term results and experience with the first-generation semi-implantable electromagnetic hearing aid with ossicular replacement device for mixed hearing loss.

    OBJECTIVE: To document the long-term surgical and audiologic results of the implantation of a first-generation semi-implantable hearing aid for mixed hearing loss, consisting of a magnetic partial or total ossicular replacement prosthesis and an electromagnetic driver fitted in the ear canal. The short-term results have previously been published as excellent in both surgical and audiologic terms. To analyze and discuss reasons of treatment failure, as well as future perspectives. STUDY DESIGN: Patient file review, follow-up otomicroscopic examination and audiometry, as well as patient interview. SETTING Tertiary referral center at a University hospital. patients: Of nine patients operated on, six with a mixed hearing loss after chronic otitis media could be evaluated primarily. Two underwent implantation with a partial and four with a total ossicular replacement prosthesis. MAIN OUTCOME MEASURES: Use of and satisfaction with the hearing aid, apparatus function and integrity, hearing without electromagnetic driver, prostheses containment, drum abnormalities, nonaudiologic ear symptoms, e.g., discharge. RESULTS: All patients attended follow-up for a mean of 9.5 years after implantation (range 8.8-10 years). No patients used the semi-implantable hearing aid at follow-up. The mean semi-implantable hearing aid using time was 24 months (range 3-60 months). The main reasons for discontinuation of use were problems fitting the electromagnetic driver correctly in the ear canal and prosthesis extrusion or dislocation. CONCLUSIONS: Despite excellent short-term surgical and audiologic results, it is concluded that these first long-term results of the first-generation electromagnetic semi-implantable hearing aids with ossicular replacement prosthesis for mixed hearing loss are disappointing. However, improvements in the surgical approach and in the reshaping, refinement, and overall improvement of the semi-implantable hearing aid could lead to satisfactory treatment of this group of patients.
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ranking = 0.036921582618081
keywords = otitis
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4/19. pneumocystis carinii infection of the middle ear and external auditory canal. Report of a case and review of the literature.

    We present a 50-year-old male with pneumocystis carinii infection involving the middle ear and the external auditory canal as the first manifestation of a previously unknown hiv infection. In case of therapy-resistant otitis with a polypoid mass in the external auditory canal histological evaluation should be considered to rule out malignancy or pathogens, like pneumocystis carinii, that cannot be cultured. Oral or intravenous antiprotozoal agents are the treatment in line with current practice. Exploration of the os petrosum is never required.
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ranking = 0.036921582618081
keywords = otitis
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5/19. Degeneration of speech, language, and hearing in a patient with mucopolysaccharidosis vii.

    mucopolysaccharidosis vii (MPS-VII) is probably the rarest of the mucopolysaccharidoses; literature reveals only 20 cases. We have had the opportunity to study and treat such a child in our clinic, and this paper documents his speech, language, and hearing. Results demonstrated a delay with respect to his chronological age in all cognitive, linguistic, and social domains. He had a mixed hearing loss which could have contributed to his diminishing speech and language abilities; he had chronic otitis media. After 59 h of speech and language intervention (over a period of 19 months), primarily for language treatment, standardized tests revealed that his scores had decreased over time. During this period, both his speech production and his hearing got poorer. At about the time of his 8th birthday, he underwent a permanent tracheostomy, altering further therapy. Although MPS-VII is a very rare disorder, what has been learned here may apply to other MPSs and even to other multiply handicapped patients. We hope that the presentation of our findings may assist others when confronted with complex, degenerative disorders.
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ranking = 0.036921582618081
keywords = otitis
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6/19. Keutel syndrome: a report of four cases.

    We report four new cases of Keutel syndrome. Clinical manifestation included abnormal cartilage ossification, multiple peripheral pulmonary stenosis (PPS), brachytelephalangism, subnormal IQ, repeated respiratory infections, otitis media and hearing loss. All four children have a typical facial appearance and are of consanguineous parents. Father and the fifth offspring exhibit a normal phenotype; the mother has pulmonary stenosis. This observation confirms Keutel syndrome as a distinct autosomal recessive syndrome.
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ranking = 0.036921582618081
keywords = otitis
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7/19. When does secretory otitis media affect language development?

    Secretory otitis media is a very common disorder in early childhood, but its effects on language development are still uncertain. We describe 10 children with secretory otitis media and illustrate the wide range of disability attributable to this. It is suggested that the impact of secretory otitis media on language development depends on at least five factors: age of onset, duration of the episode(s), severity of the hearing loss, intrinsic qualities in the child, and the linguistic environment. The implications of this hypothesis for clinical practice and research are discussed.
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ranking = 0.25845107832657
keywords = otitis
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8/19. Pseudomonas labyrinthitis.

    pseudomonas aeruginosa is the gram-negative bacterial rod which is often isolated from chronic aural discharge. This microorganism may also cause necrotizing infection of the external auditory canal in certain patients with impaired host-defense mechanisms. Involvement of the inner ear by this microbe is extremely rare. In this communication, we report a case of pseudomonas labyrinthitis which resulted from traumatic middle ear injury. infection produced massive granulations and extensive bone destruction of the otic capsule. This case shows that while P.aeruginosa is usually an avirulent opportunistic pathogen, it may also cause a highly destructive labyrinthitis if the inner ear is entered.
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ranking = 5.7784705042915
keywords = labyrinthitis
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9/19. Chronic middle ear disease with intracranial extension--a clinicopathologic case report.

    A case is presented with chronic suppurative otitis media and subsequent tympanogenic labyrinthitis, extradural abscess, and otogenic meningitis. The clinical course is described and pathologic findings demonstrated using serial temporal bone sections. The difficulties of diagnosing and treating this clinical condition are illustrated.
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ranking = 1
keywords = labyrinthitis, otitis
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10/19. Serous otitis media associated with sensorineural hearing loss in children.

    The effect of a conductive hearing loss secondary to serous otitis media in children with sensorineural hearing loss was seen to be an increase in threshold and in one case a decrease in speech discrimination. The diagnosis of the conductive component can be made by means of measurement of impedance, middle ear pressure, the recording of a tympanogram and otoscopy. It is likely that a conductive component can lead to delay in the diagnosis of an underlying sensorineural hearing loss in some children which could result in exacerbation of speech and language deficits. Correction of the conductive component can convert a profound hearing loss to a severe hearing loss, or a severe hearing loss to a moderate hearing loss, and it may also increase speech discrimination.
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ranking = 0.18460791309041
keywords = otitis
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