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Results for “Cochlear Diseases”

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At least 307 records · Page 17Linked to original sources

PubMed2003

A review of cochlear implantation in mitochondrial sensorineural hearing loss.

OBJECTIVE: Mitochondrial sensorineural hearing loss (SNHL) may be nonsyndromic (occurring in isolation), associated with the A1555G mutation in the MTRNR1 gene. Mitochondrial SNHL may also be syndromic, associated with the A3243G point mutation in the MTTL1 gene. In syndromic cases-mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS), maternally inherited diabetes and deafness, Kearns-Sayre syndrome, and chronic progressive external ophthalmoplegia-the SNHL compounds already existing disabilities. The genetic basis for mitochondrial SNHL and postulated sites of pathologic changes are discussed. DATA SOURCES: Sources used were relevant clinical and basic science publications. STUDY SELECTION: A search of the entire databases of Medline and Web of Science, using various subject headings and free-text terms, was used to identify patients with mitochondrial disease having cochlear implants. DATA EXTRACTION: The data from publications were critically reviewed and tabulated to assess implantation outcomes. DATA SYNTHESIS: The data were not amenable to formal meta-analysis or valid data summarization, other than descriptive statistics. CONCLUSIONS: There is an increasing awareness of the prevalence of mitochondrial SNHL and its progressive nature. High-risk candidates warrant genetic testing and family screening. Correlating the data for mitochondrial SNHL as a treatable entity is important, and the authors present an overview of these patients successfully rehabilitated by cochlear implantation.

Adult↗

PubMed2002

Cochlear implantation in chronic suppurative otitis media.

OBJECTIVE: To evaluate management options for cochlear implantation in patients with chronic suppurative otitis media. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center with a large cochlear implant program. PATIENTS: Ten patients with chronic suppurative otitis media who received cochlear implants were identified. Case history, etiology of hearing loss, and management of the ear with the implant were evaluated. INTERVENTIONS: Cochlear implantation and subsequent rehabilitation. MAIN OUTCOME MEASURES: Early and late complications were evaluated. RESULTS: No early or late complications were identified in this group of patients after an average follow-up period of about 4 years. CONCLUSION: Cochlear implantation can be safely achieved in patients with chronic suppurative otitis media. There are several options for the management of these patients, and the approach chosen should be individualized on the basis of clinical findings.

Adolescent↗

PubMed1995

[Interstitial keratitis and Cogan syndrome].

Cogan's syndrome consists of non syphilitic interstitial keratitis associated with vestibuloauditory dysfunction. This syndrome is rare and very few cases have been reported since Cogan's first characterization in 1945. We present the case of a 24-year-oldman with a history of recurrent access of arthralgia and glomerulonephritis. Bilateral corneal disease consists of peripheral whitish patchy opacities in deep stroma. The patient also shows bilateral severe deafness, fever, meningeal and tetrapyramidal syndrome. Ocular involvement responds well to corticosteroids therapy but hearing loss is severe and often irreversible.

Adult↗

PubMed2002

Mpv37 mouse strain--a model for the relationship between the kidney and the inner ear.

Using the Mpv17-negative mouse strain, which developed inner ear and kidney dysfunction, we confirm a strong relationship between the kidney and the inner ear. Both organs have specialized epithelia involved in active ion transport, which are separated from the vessels by a basement membrane of similar composition. Our recent results indicate that the glomerular and the stria vascularis basement membrane are simultaneously affected in early stages. Concomitant deposits of IgG during the progressive development of the disease support the idea of a shared antigen. Understanding the pattern of the development of the degeneration will provide a basis towards understanding the essential role of the Mpv17 protein in the structures of both organs and may provide a basis for future therapeutic intervention.

Animals↗

PubMedDate not supplied

Aberrant frequency tuning and early stereociliary derangement in genetic inner ear disease.

We have developed an auditory brainstem response (ABR) based method for frequency selective forward masking tuning curve technique. Normative data from CBA/CBA mice are compared with tuning curves (TCs) from heterozygote dancer mice which in most cases initially had a normal ABR threshold. The sharpest tuning in normal mice was obtained at 10 kHz (Q10 = 7.6) and 12 kHz (Q10 = 10.9). With increasing age the mutants showed a broadening of the TC tip before ABR threshold impairment occurred. Morphological analysis of the cochleae from dancer mutants revealed an early disarray of hair cell stereocilia which became increasingly more severely affected with advancing age. We conclude that the broadening of the TC tip is an early finding in the genetically induced cochlear susceptibility which can be correlated to stereociliary dysfunction.

Animals↗

PubMed2005

An unusual disease presenting at an unusual age: Susac's syndrome.

Susac's syndrome is a rare disease of unknown aetiology affecting the small vessels of the retina, brain, and cochlea. We present the case of a 55-year-old female, the oldest patient yet described with the condition, and highlight the syndrome's clinical features.

Anti-Inflammatory Agents↗

PubMed2003

Vibration-induced nystagmus in patients with vestibular disorders.

OBJECTIVE: Our goal was to clarify the clinical significance of vibration-induced nystagmus (VIN). METHODS: One hundred patients with unilateral vestibulocochlear disorders were enrolled into this study. However, patients with spontaneous nystagmus were excluded. Vibratory stimuli (approximately 100 Hz) were presented to the mastoids and the forehead. Patients also underwent caloric testing and vestibular evoked myogenic potential testing. RESULTS: Of the 100 patients, 60 (60%) showed VIN. The nystagmus was mainly horizontal. VIN was more frequently evoked on the mastoids than the forehead. In the majority of patients, the direction of VIN was toward the healthy side, whereas some patients, especially patients with Meniere's disease, showed nystagmus toward the affected side. VIN was frequently evoked in patients with severe unilateral vestibular damages (canal paresis >50%) (39 of 43, or 90%). CONCLUSION: VIN testing is a simple and sensitive clinical test that indicates unilateral vestibular dysfunction.

Adolescent↗

PubMed1993

Otoacoustic emissions in children with normal ears, middle ear dysfunction, and ventilating tubes.

The clinical utility of otoacoustic emissions (OAEs) has been well established in adults. The purpose of this investigation was to determine the efficacy of OAE testing in children. Distortion-product OAE (DPOAE) audiograms, response/growth functions, and transiently evoked OAEs elicited with clicks were measured from the ears of both healthy volunteers, aged 4 to 13 years, and children with confirmed middle ear disorders. These measures established the means and variabilities for DPOAE and noise-floor amplitudes of normal and diseased young ears. Compared with adult emissions, the healthy young ears exhibited greater mean DPOAE and noise-floor amplitudes. In contrast, ears with type B and type C tympanogram patterns showed absent or markedly reduced OAE amplitudes, when compared with emissions measured in their control counterparts. Finally, ears with ventilating tubes exhibited OAE amplitudes lower than amplitudes from healthy ears, but higher than those of the untreated diseased ears. Although these findings imply that using OAEs to test the outer hair-cell reserve of infected ears is problematic, emitted responses provide useful information concerning the normalcy of middle ear function.

Acoustic Impedance Tests↗

PubMed1991

Pathogenesis and pathophysiology of Meniére's disease.

Meniére's disease, neither spontaneous nor inducible in animals, is studied only in patients. Natural history (epidemiology) shows a triad of major symptoms: vestibular, auditory, and aural pressure. One in 3 patients has bilateral Meniére's; over full lifespans, bilaterality approaches 50%. Aural pressure (74.1%) and positional vertigo during/between attacks (85.9%) are common. Clinical variants can persist for 25+ years. All forms have delayed onset and can occur years after incitement, after otosclerosis, infections like otitis media, syphilis, or trauma. Endolymphatic hydrops is found in all, most importantly in pars inferior (cochlear duct and saccule). Some (not most) cases show ruptures. The saccule can distend into the lateral semicircular canal. Symptomatic attacks are explained on physical/biochemical bases. Both longitudinal (slow) and radial (fast) flow seem operational, longitudinal in advanced Meniére's where membranous labyrinth replaces perilymph in scala vestibuli and vestibule. All forms result from endolymphatic absorptive dysfunction (in duct and sac), with mastoid and periaqueductal hypocellularity, hypodevelopment of Trautmann's triangle, and anterior displacement of lateral sinus. Secondary obstructions in ductus reuniens or utricolo-endolymphatic valve may explain atypical Meniére's (vestibular or cochlear alone).

Cochlear Diseases↗

PubMed1993

[A case of superficial siderosis of the central nervous system with total deafness].

Superficial siderosis of the central nervous system (SSCN) in a disease characterized by chronic deposition of hemosiderin in the leptomeninges, subpial tissue, spinal cord, and cranial nerves. Previously the diagnosis of SSCN could only be made at autopsy or during a neurosurgical procedure. Now, however, a diagnosis of SSCN can be made non-invasively by magnetic resonance imaging (MRI). We present the case of a 50-year-old male with SSCN accompanied by bilateral sensorineural hearing loss which gradually progressed to total deafness over a seven year period. This patient also had associated bilateral caloric weakness with episodes of severe recurrent headaches over the two preceding years. The deafness and gait disturbance, which were his chief complaints, were followed by other neurological manifestations including pyramidal tract signs, anosmia, and ageusia. High-field MRI on T-2 weighted images of the CNS showed diffuse marginal hypointensity of the cerebrum, brain stem, and cerebellum. Atrophy of the cerebellum and brain stem was also apparent. Low signal intensity along the proximal segment of the acoustic nerve and the facial nerve was noted from the cistern to the internal auditory canal. Neither bilateral transtympanic promontory nor round window electrical stimulation elicited any sound sensation. In this case it was thought that the acoustic nerve alone or both the acoustic nerve and the cochlea were affected by this disease. Consequently, cochlear implantation was not indicated. The source of bleeding into the subarachnoid space could not be detected despite thorough examination. This diagnosis will be made only by physicians who are aware of this rare entity and have knowledge of the characteristic clinical pathology.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗

PubMed1997

[Is otoacoustic emission useful in the differential diagnosis of occupational noise-induced hearing loss?].

It is very difficult to distinguish between occupational noise-induced hearing loss and other diseases with cochlear hearing loss by the means of conventional audiometric tests. Otoacoustic emission measurement is a relatively new tool for assessing the inner ear function. It gives an opportunity of monitoring the status of the outer hair cells, the elements which are the most sensitive to noise-induced damage. In this study the results of distortion product otoacoustic emission (DPOAE) measurement in subjects with industrial noise-induced hearing loss, presbycusis and hearing impairment caused by gentamycin, are presented. In all these cases pure-tone audiometry revealed similar shape of the audiograms with cochlear hearing loss mainly at high frequencies. In the cases of industrial noise-induced hearing loss, DPOAE measurements demonstrated a very typical shape of DP-gram with the decrease (notch) in otoacoustic primarily at the frequencies of 3-4 kHz. Such a notch in DP-grams was not observed in the cochlear hearing loss caused by factors other than noise. The data indicate that otoacoustic emissions may be useful in the differential diagnosis of occupational noise-induced hearing loss.

Acoustic Impedance Tests↗

PubMed1996

[Endaural reflex diagnosis of cochleovestibular dysfunctions in patients with vascular brain diseases].

The analysis of electrical conductivity of acupuncture points was performed in 225 patients with cochleovestibular dysfunctions against a background of cerebrovascular pathology. Original highly sensitive express-method with application of special registering device was proposed for endaural reflex diagnosis. The system was elaborated for interpretation of examination's results. It permitted either to confirm or to define more precisely both the location (side and level) and the stage of cochleovestibular damage.

Acupuncture Points↗

PubMed1993

Correcting for factors affecting ABR wave V latency.

The latency of auditory brainstem response (ABR) wave V has been shown to be affected by several subject and technical factors. Previous studies have often concluded with a recommendation to account or correct for one or more of these factors when latency is used as a means of detecting retrocochlear dysfunction. This study investigated the effect of subject age, sex, hearing loss and stimulus intensity on ABR wave V latency in a group of 189 clinical subjects in whom retrocochlear disease was effectively excluded. Regression analysis showed that all four factors exerted a significant influence on latency and that hearing loss and intensity were most effectively represented when combined to form a sensation level variable, together with a measure of audiogram slope.

Acoustic Stimulation↗

PubMed1988

Hyperbaric effects on sensorimotor reactivity studied with acoustic startle in the rat.

In three experiments, startle responses to brief intense tone-bursts (30 msec, 110 dB, 6000 Hz) and single pulse (0.1 msec) stimulation of the cochlear nucleus and the nucleus reticularis pontis caudalis are studied under high pressures of heliox (from 0 bar to 50 bars) in the rat. For each rat (N = 12), mean amplitude and latency changes in startle responses (nuchal electromyography and whole-body accelerometry) are compared at normobaric pressure and during compression, at a speed of 100 bar/H. The results indicate that high pressures decrease (50% of control size) tone evoked startle by acting on the peripheral auditory organ, probably through middle and/or inner ear barotrauma. The large increases in electrically-elicited startle (250% of control size) from the cochlear and reticular nuclei, under hyperbaric conditions, suggest that high pressures affect sensorimotor reactivity by excitatory action on synaptic transmission in the relays of the acoustic startle reflex arc at the lower brainstem and spinal levels. Startle latencies remain unaffected by the heliox high pressures studied here.

Acoustic Stimulation↗

PubMed2005

Chronic Pseudomonas infections of cochlear implants.

OBJECTIVE: To discuss chronic, refractory Pseudomonas infections of cochlear implants and their management. DESIGN: Retrospective case series. SETTING: Two university-based cochlear implant programs. PATIENTS: Twenty-eight-year-old (Case 1) and 4-year-old (Case 2), different devices. INTERVENTIONS: Medical and surgical management. MAIN OUTCOME MEASURES: Clinical course. RESULTS: Both patients had delayed presentations, 4 months and 3 years postimplantation, respectively, with fluctuating scalp edema and pain resistant to multiple courses of oral antibiotics. Infections began as localized granulation and progressed to complete encasement of both devices with rubbery, poorly vascularized tissue. In each case, two different strains of multiresistant Pseudomonas aeruginosa were cultured. Infections progressed despite local debridement and targeted antipseudomonal antibiotic coverage, and sensitive organisms continued to appear in cultures of refractory granulation tissue. Both patients underwent partial explantation, with the electrode array left in the cochlea, then received 2 to 3 more months of further medical therapy and observation and then were reimplanted successfully with new devices. Both have shown excellent performance and no sign of recurrent infection. CONCLUSIONS: Infections of cochlear implants are uncommon, and cases of successful conservative management without device explantation have been reported. However, our experience and the implanted device literature suggest that chronic Pseudomonas infections may represent a distinct clinical entity, likely to fail protracted therapy and ultimately require device removal. Fortunately, successful reimplantation is possible.

Adult↗

PubMed1991

[Cochleovestibular syndrome disclosing dolicho-ectasis of the basilar trunk and vertebral artery].

The authors present a case of a cochleovestibular syndrome with a scanner showing a calcified dolico-ektasis of the basilar truncus and the left vertebral artery. The patient had typical Ménière's disease symptomatology. A retrolabyrinthic diagnosis with substantial recruitment was indicated by the results of the audio-vestibular explorations (stapedius reflex, electroculography, early brainstem evoked response, electrocochleography). The electrocochleography with glycerol test indicated labyrinthic hydrops. Finally, some reflections on the role of neurovascular problems in the pathogeny of some cochleovestibular syndromes are put forward.

Aged↗

PubMed2004

Transtympanic perfusion: indications and limitations.

PURPOSE OF REVIEW: To review the most recent literature regarding the application of transtympanic inner ear perfusion in the treatment of inner ear disorders including Meniere disease, sudden sensorineural hearing loss, and autoimmune inner ear disease. RECENT FINDINGS: The use of gentamicin perfusion in the management of Meniere disease with intractable vertigo has been demonstrated to have a very high rate of success, and is much less invasive than alternative surgical procedures such as vestibular nerve section or labyrinthectomy. The technique for achieving the highest rate of success while still minimizing the risk of cochleotoxicity continues to be investigated. Sustained delivery techniques such as the Silverstein MicroWick appear to achieve the best pharmacokinetic profile within the inner ear fluids. The end point of treatment does not necessarily require complete vestibular ablation to cure the patient, and shorter courses of treatment may help to reduce the risk of hearing loss. Cochlear Meniere disease can be treated with dexamethasone 4 mg/cc perfusion of the inner ear, which may improve the hearing, tinnitus, and pressure in the ear. Sudden sensorineural hearing loss has been managed with transtympanic steroid delivery, and this appears to be beneficial for some patients who have failed to respond to oral steroids, or have medical contraindications to systemic steroids. SUMMARY: Inner ear perfusion via transtympanic delivery is an emerging technique in the management of inner ear disease. Improved results are expected over time as research in this area answers questions about dosage and delivery techniques, as well as identifying new applications and pharmaceuticals.

Adult↗

PubMedDate not supplied

[The choice of the method for treating patients with Menière's disease and similar diseases].

The experience gained by the authors in the treatment of Menière's disease and similar affections which are supposed to have 3 stages provided the following approach to their treatment: after examination of the cochleovestibular analyzer involvement good results may be expected of conservative treatment in stage I-II, chordoplexectomy is justified in stage I, drainage or decompression of the endolymphatic sac and vestibulotomy are indicated in stage II and III, respectively.

Cochlear Diseases↗