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Results for “Cochlear Diseases”

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At least 289 records · Page 16Linked to original sources

PubMed1997

Pathology of the cochlea following a spontaneous mutation in DBA/2 mice.

The DBA/2 strain of mice usually presents with noise-induced epileptic seizures and hearing disorders. After a spontaneous mutation a strain with early hearing loss and circling behaviour was produced. This strain presents with clinical symptoms found in diseases connected to inner ear disorders. These animals do not suffer from periodical disorders, however, but have functional disturbances continuously and can therefore serve as an animal model for diseases originating from both parts of the inner ear. The genetic inheritance appears to be autosomal recessive. Offspring showed circling behaviour and severe pathology in the vestibular part of the inner ear. In the present study pathology of the cochlear part of the inner ear was visualized using conventional microscopical techniques. The content of actin and fodrin was labelled immunohistochemically, and hearing was assessed with auditory brainstem recordings. After 1 month the animals showed deterioration of the cochlear part of the inner ear. At 6 months no organ of Corti remained and the animals were deaf. Transmission and scanning electron microscopy revealed severe apical hair cell changes. The content of alpha-actinin and fodrin in the DBA/2 mouse was already fainter than that in age-matched CBA control mice at the age of 1 month. Labelling of antibodies against fodrin increased in the supporting cells of the older animals, probably owing to the replacement of hair cells.

Actins↗

PubMed1993

Otosyphilis: diagnostic and therapeutic update.

The thorough investigation of patients presenting with sudden or fluctuating hearing loss, ringing or vertigo includes serology to exclude otosyphilis. Treatment of otosyphilis with penicillin and corticosteroids has achieved improvement in hearing, tinnitus and vertigo, but not in all patients. Selecting which patient with positive serology will benefit from treatment remains a difficult clinical problem. All patients presenting to The New York Eye and Ear Infirmary with cochleovestibular dysfunction of unknown aetiology and positive syphilis serology were assumed to have otosyphilis and were treated with intravenous penicillin, if non-allergic, and steroids. Lumbar puncture and HIV testing were performed. Eighteen patients were treated. Hearing (SRT and/or discrimination) improved in 4 of 16 patients with hearing loss (25%), tinnitus decreased in 10 of 14 (71%) and dysequilibrium improved in 6 of 9 (66%). Factors associated with a good response included fluctuating symptoms, especially hearing, hearing loss less than five years, and age less than 60. Improvement was unrelated to the severity of the loss or previous therapy. Patients with CSF abnormalities, including two patients with HIV disease, had subjective improvement. A summary of our results and a treatment protocol are presented.

Acquired Immunodeficiency Syndrome↗

PubMed2006

Cochlear implantation in superficial siderosis.

Superficial siderosis (SS) of the central nervous system has been thought to be a rare condition that generates progressive hearing loss, ataxia, pyramidal signs, and dementia. The main cause of hearing loss by SS is thought to be neuronal. Because there is no histopathologic report of the human temporal bone in SS, there is a debate about the possibility of cochlear involvement. We present a 25-year-old man who was investigated for bilateral progressive sensorineural hearing loss and vestibular failure after head trauma. On brain MRI, SS of the central nervous system was detected. Distortion product otoacoustic emission (DPOAE) and auditory brainstem response (ABR) showed no response on both sides. However, integrity of the eighth nerve was proved by the electrical ABR test on the right side and the patient benefited significantly from cochlear implantation. The sensorineural hearing loss in SS seems to be related to cochlear damage as well as neuronal damage. So, cochlear implantation would be a hearing rehabilitation modality for the sensorineural hearing loss caused by SS.

Adult↗

PubMed2000

[Clinical utilization of magnetic resonance imaging for patients with cochlear implants].

To date, MR imaging is absolutely contraindicated in cochlear-implanted patients. The aim of this study is to document the potential value of MR imaging in such patients, without injurious or side effects and without technical device failure. After in vitro experimental testing, 25 cochlear implanted patients underwent MRI. Radiological evaluation was also possible. The use of 1-Tesla fast spin echo sequences minimised artifacts. All cochlear implants are still in working order; no changes in fitting data or a reduction in speech understanding were observed. MR helped to avoid other invasive medical procedures.

Austria↗

PubMed1992

Otosyphilis: a diagnostic and therapeutic dilemma.

Traditional treatment of otosyphilis with penicillin and corticosteroids has achieved hearing improvement; however, selecting which patients with a positive fluorescent treponemal antibody absorption (FTA-ABS) test will benefit from treatment remains a problem. In order to study this problem, 18 patients with cochleovestibular dysfunction of unknown etiology and positive syphilis serology were treated with intravenous penicillin and corticosteroids. In addition, lumbar puncture and human immunodeficiency virus (HIV) testing were performed on all patients. Hearing improved in 5 (31%) of 16 patients, tinnitus decreased in 11 (85%) of 13, and vertigo improved in 6 (86%) of 7. Factors associated with hearing improvement were hearing loss present less than 5 years, fluctuating hearing, and age less than 60. Improvement was unrelated to the severity of the loss or previous therapy. All patients with cerebrospinal fluid abnormalities, including two patients with HIV disease, had subjective improvements. A diagnostic and treatment protocol is presented.

Adult↗

PubMed2005

Cochleovestibular dysfunction caused by cerebrovascular diseases.

When a vascular disorder of the cochleovestibular system is considered, the diagnosis is based on exclusion of other diseases. As arteries of the cochleovestibular system cannot be directly visualized, physicians must deduce vascular cochleovestibular disease from the vascular risk factors and vascular lesions of other territories. With my colleagues at Semmelweis University, I analyzed neurootological and audiological examination results in 65 patients with vascular vertigo. Cochleovestibular system disorders can be considered to be of vascular origin if the examinations exclude other diseases, if the patients have vascular risk factors, and if other territories of the brain accessible for imaging methods show vascular disorders.

Carotid Artery, Internal↗

PubMedDate not supplied

Successful cochlear implantation in a patient with superficial siderosis.

OBJECTIVE: To define the presentation and outcome of cochlear implantation in a patient with profound sensorineural hearing loss due to superficial siderosis. STUDY DESIGN: Retrospective case review. RESULTS: Postimplantation speech reception threshold was 34 dB, and hearing in noise testing sentences were 71% demonstrating a successful outcome. These results have been maintained for 5 years after implantation. CONCLUSION: Cochlear implantation is a potentially successful rehabilitation option for certain patients with profound sensorineural hearing loss due to superficial siderosis.

Adult↗

PubMed2002

Neurotologic manifestations of the fibromyalgia syndrome.

The purpose was to assess otologic symptoms, and audiologic and vestibular findings in fibromyalgia (FM) syndrome. Twenty-four female patients with FM syndrome (FMS) were included in the study. The assessments were based on history, physical examination, audiometry, bithermal caloric testing and auditory brainstem response (ABR) testing. Dizziness was the most common complaint of the patients, and was followed by tinnitus, hearing loss and vertigo. Almost 50% of the patients had some sort of otologic symptoms.Dix-Halpike maneuver proved positional rotary vertigo in 5 (20.8%) patients. The audiometry results of 23 patients were normal. None of the patients had abnormal bithermal caloric testing. Although there were a variety of ABR abnormalities, the ABR results of the patients with and without cochleovestibular symptoms were not significantly different (p<0.05). In conclusion, FM patients can complain otologic symptoms even though they do not have any clinically or audiologically detectable ear disease. A neural disintegration or some other events related to neural mediators may be the mechanisms involved in the pathogenesis of otoneurologic as well as systemic manifestations of the disease that possibly leads to abnormal perception of the stimuli coming from internal or external environment.

Adult↗

PubMed2006

Cochlear implant explantation as a sequela of severe chronic otitis media: case report and review of the literature.

INTRODUCTION: In the 1980s, intracranial and inner ear infections were feared complications in patients with recurrent or chronic otitis media (COM) who had undergone cochlear implantation. Current studies show a low incidence of such complications. We present a case of a patient who developed severe COM requiring cochlear explantation. CASE: Our patient had a previous cleft palate repair and as a three-year-old was implanted with a Nucleus-24 implant. She developed chronic otorrhea in the implanted ear, which was managed by her pediatrician until her cochlear implant stopped functioning. Radiographic imaging revealed erosion of the cochlea and extrusion of the distal electrode medially in the petrous apex. SETTING: Tertiary care university hospital. INTERVENTION/RESULTS: The patient underwent cochlear explantation, subtotal petrosectomy, obliteration of ear, and intravenous antibiotic therapy. One month later she was implanted in the contralateral ear. CONCLUSION: COM poses potentially severe complications in patients receiving cochlear implants. Patients receiving cochlear implants who are at high risk for COM require follow-up for an extended period of time.

Anti-Bacterial Agents↗

PubMed2003

Prediction of progression from atypical to definite Ménière's disease using electrocochleography and glycerol and furosemide tests.

OBJECTIVE: To investigate whether electrocochleography (ECochG) and glycerol and furosemide tests could predict progression from atypical to definite Ménière's disease (MD). MATERIAL AND METHODS: ECochG and glycerol and furosemide tests were performed in 1569 patients with various cochleovestibular diseases, including definite MD, atypical MD, syphilitic labyrinthitis, delayed endolymphatic hydrops, sudden hearing loss, cochleovestibulopathy and sensorineural hearing loss. Patients with atypical MD were divided into five categories based on their symptoms. RESULTS: A total of 115/118 patients (97%) with definite MD who underwent all 3 tests showed a positive result in at least 1 test. Ninety-nine patients who did not satisfy the diagnostic criteria of definite MD but had vertigo and/or hearing loss at the first visit subsequently progressed to definite MD. It was retrospectively found that 92% of patients showed at least 1 positive finding in these 3 tests at the initial stage. In those patients who showed a negative test result in either ECochG or the glycerol test, the possibility of progression to definite MD was low. CONCLUSION: The combination of ECochG and the glycerol and furosemide tests was helpful in diagnosing endolymphatic hydrops (ELH). ECochG and the glycerol test were effective tools for predicting the progression to definite MD in patients with atypical MD, sudden hearing loss and other cochleovestibular diseases. Our test results also indicated that the pathological state of atypical MD included both non-ELH and ELH.

Adolescent↗

PubMed1990

[High-resolution computed tomography in diseases of the inner ear].

High-resolution CT of the petrous bone was carried out in 259 patients with clinically diagnosed abnormalities of the inner ear; in some the lesions were bilateral. In 62 patients (approximately 20%), the scans showed findings which related to the signs and symptoms. Patients with hearing loss and vestibular neuronitis showed no abnormalities. Amongst the remaining patients, the most common findings were acoustic neuromas (17 cases), cochlear otospongiosis (14 cases), malformations (13 cases) and tumours or cholesteatomas of the labyrinth (13 cases) as well as single rare isolated lesions.

Cochlear Diseases↗

PubMed2007

[Intracochlear schwannoma as a cause of a deafness: a case report].

Inner ear extension caused by schwannomas, which can cause hearing loss, tinnitus and vertigo, is an extremely rare finding. We report a male patient who presented with progressive hearing loss as well as tinnitus in the right ear. Despite rheologic infusion therapy with glucocorticosteroids at another hospital, the patient showed an explicit hearing loss and vertigo related to the right ear. In the pure tone audiogram, we found complete deafness of the right ear, the equilibriometry with caloric testing showed complete loss of the labyrinth. In the HR-MRI, a structure appeared in ranges of the basal and middle cochlea with enhancement of the contrast medium. From the MRI aspect, this appeared to be a small intracochlear schwannoma. As a differential diagnosis, a chronic granulation is also possible. In agreement with the patient, we decided to control the structure clinically every 6 months using MRI; no further growth of the structure appeared. A precise imaging via HR-MRI is required to detect intracochlear schwannomas. Observation is an appropriate option for patients who have serviceable hearing. Surgical exploration can be recommended in cases of progressive diseases and growth into the inner ear with recurrent vertigo attacks. The option of radiotherapy for the treatment of intrameatal acoustic neurinomas should be considered.

Adult↗

PubMed2004

Cochlear implantation in patients with a history of chronic otitis media.

OBJECTIVE: To propose management options for cochlear implantation in chronic otitis media based on our experiences. MATERIAL AND METHODS: A retrospective review of 418 cochlear implantations performed by the 2 senior authors between November 1988 and February 2004 was conducted. Nine patients who had chronic otitis media in the ear to be implanted were included. Of these, three showed active inflammation at presentation; the other six cases had undergone previous tympanomastoidectomy surgery and did not show active inflammation at presentation. RESULTS: Five patients with active inflammation or without an adequate soft tissue layer in the mastoid bowl underwent a two-stage procedure. Four cases who showed inactive inflammation and had an adequate tissue layer to protect the electrode array underwent a single-stage technique, although two of them showed dry tympanic membrane perforation. No local or intracranial inflammation recurred. The electrode was exposed in the mastoid bowl in one case, who was managed with revisional mastoid obliteration with soft tissue. CONCLUSION: Complete eradication of inflammation and the securing of a strong protective soft tissue layer over the electrode are prerequisites for cochlear implantation in ears with chronic otitis media.

Adult↗

PubMed2001

Evidences of vascular origin of cochleovestibular dysfunction.

OBJECTIVES: When the vascular disorder of the cochleovestibular system is mentioned, the diagnosis is based on exclusion of other diseases. Since arteries of the cochleovestibular system cannot be directly visualized, physicians must deduce from the vascular risk factors and the vascular lesion of other territories to the vascular cochleovestibular disease. MATERIALS AND METHODS: Authors analyzed the data of 19 patients with vertigo. Detailed blood tests, complete neurootological and audiological examination including ABR, carotid and vertebral artery Doppler sonography, MRI and MRA was performed. RESULTS: Cochleovestibular examination and ABR showed abnormalities in 73.7%, either carotid and vertebral artery Doppler or MRI showed abnormalities in 57.9%. MRA was abnormal in 47.4%. In most of the patients multiple risk factors of cerebrovascular disorder could be found. CONCLUSIONS: The cochleovestibular system disorders can be considered to be of vascular origin if the examinations exclude other diseases, if the patients have vascular risk factors and if other territories of brain accessible for imaging methods show vascular disorders.

Carotid Arteries↗

PubMed1996

Derived-band auditory brain-stem response estimates of traveling wave velocity in humans: II. Subjects with noise-induced hearing loss and Meniére's disease.

Estimates of cochlear traveling wave velocity (TWV) were computed from derived-band auditory brain-stem response (ABR) latencies in subjects with noise-induced hearing loss (NIHL) or Meniére's disease (MD). ABR wave V latencies were determined for each of six derived frequency bands (unmasked-8 kHz, 8-4 kHz, 4-2 kHz, 2-1 kHz, 1 kHz-500 Hz, and 500-250 Hz). Representative frequencies were assigned to the derived bands by estimating their energy midpoints, and cochlear positions corresponding to these frequencies were determined using Greenwood's (1961) place-frequency function for humans. An exponential function of the form I = A + BeCd was fitted to each subject's latency-by-distance data using a least-squares algorithm, and a TWV function was generated by taking the reciprocal of the derivative of the latency function with respect to distance [v = 1/(BCeCd)]. Expected values for subjects' TWV functions were compared to normative data from Donaldson and Ruth (1993) at five cochlear loci. NIHL subjects' TWV estimates fell within normal limits at all cochlear loci, and no relation between severity of high-frequency hearing loss and TWV could be discerned. MD subjects with good low-frequency hearing sensitivity generally yielded normal TWV estimates, whereas MD subjects with low-frequency hearing loss yielded either normal or elevated TWVs. MD subjects' data generally support the hypothesis that endolymphatic hydrops results in increased TWV or, alternatively, a basalward shift in the peak of the traveling wave, in cochleas with presumed normal basilar membrane elasticity.

Adult↗

PubMed1994

Subtotal petrosectomy with external canal overclosure in the management of chronic suppurative otitis media.

Subtotal petrosectomy with external canal overclosure has been used in the management of 10 patients with chronic suppurative otitis media in the past four years. These patients fall into two categories. One group consisted of patients with end-stage chronically discharging mastoid cavities, despite aggressive medical therapy. The other group comprised patients who had developed disabling peripheral vestibular symptoms and had a mastoid cavity from past surgery for chronic otitis media. In both groups the affected ear had absent or poor auditory function. In the second group, a translabyrinthine section of the vestibular and/or cochlear nerves was performed in conjunction with subtotal petrosectomy. The procedure has been very successful in relieving the persistent otorrhoea, giving the patient an ear without a cavity that would need regular care. No restriction on swimming and participation in other water sports is an additional advantage. Illustrative cases are used to discuss the technique and indications.

Adult↗

PubMed1997

Schwannoma in the vestibule and cochlea.

Schwannoma of the vestibule or the cochlea is an unusual lesion. In the past, most examples have been found at autopsy or as unsuspected findings at surgery for vertigo. The symptoms of isolated labyrinthine schwannoma may be indistinguishable from advanced Meniere's disease. Magnetic resonance imaging has led to pre-operative diagnosis in some cases. Two cases of schwannoma within the labyrinth from a series of 339 symptomatic acoustic tumours, are presented and the imaging findings are discussed.

Adult↗

PubMed1999

[Removal of a cochlea implant with chronic granulation labyrinthitis and foreign body reaction].

BACKGROUND: Cochlea Implantation is an accepted treatment for children and adults with profound sensorineural deafness. In rare cases postoperative complications may occur so that removal of the implant is unavoidable. CASE REPORT: In a particular case we present and discuss the case of a 69-year-old patient. CONCLUSION: After removal of a cochlear implant, the electronic array is frequently left in the cochlea as a temporary replacement. This is a common practice, but whether it is advisable should be verified according to the specific intraoperative situation.

Aged↗