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[Indications and results of cochlear implants in young children].

In order to determine the criteria for patient selection and the preoperative prognostic factors for hearing recovery after cochlear implants in young totally dead children, the results of hearing rehabilitation were studied in 15 children who had undergone cochlear implantation at from 2 to 9 years of age. The choice of implant is determined by the permeability of the cochlear duct. A multi-system should be used, except when the cochlea is totally ossified. The reliability and efficacy of mono and multi system implants make it possible to offer a solution to the urgent therapeutic problems posed by total deafness in the young child.

Age Factors↗

Binaural cochlear implants.

With the success of monaural cochlear implantation, patients frequently ask about having a second implant. We have performed binaural cochlear implants in 12 adult patients. Desire not to disrupt a functioning implant was the primary consideration in implanting the contralateral ear. Seven patients received a second 3M/House single-channel implant to upgrade to a magnetic external receiver. Four patients with a 3M/House device in one ear elected to place a Nucleus multichannel implant in the opposite ear. One patient with a poorly functioning Nucleus device elected to have a second Nucleus device. Four patients with a Nucleus and a 3M/House implant, one with binaural 3M/House implants, and one with binaural Nucleus implants were tested for auditory discrimination in order to quantify monaural versus binaural differences. The functional benefit of the second implant was mixed, but all patients showed some degree of objective improvement on one or more tests. Five of the six are regular users of both devices; the patient with binaural Nucleus implants wears only one. Despite the differing processing schemes, patients with a Nucleus device in one ear and a 3M/House device in the other ear are able to combine the two signals to advantage. We feel that cochlear implantation in the contralateral ear is an acceptable option in selected patients desiring an upgraded implant without placing a functioning implant at risk.

Aged↗

[Susac syndrome: a report of two cases].

OBJECTIVE: To investigate the clinic characters and diagnosis of Susac syndrome. METHODS: Two cases were systematically studied by the authors. RESULTS: All the two cases were young women. The clinical manifestations include acute and subacute multifocal and diffuse encephalopathic symptoms, hearing loss, and visual loss. Diagnosis is facilitated by demonstration of retinal arteriolar occlusions without uveitis or keratoconjunctivitis, mid-to-low frequency unilateral or bilateral sensorineural hearing loss, and numerous small foci of increased signal in the white and gray matter on T2-weighted brain magnetic resonance imaging. CONCLUSIONS: This rare syndrome often can be identified at an early stage with a careful history and physical examination. MRI, SPECT, retinal fluorescein angiography and audiometry will contribute to diagnosis.

Adult↗

Surgical considerations in cochlear implantation in children and adults: a review of 342 cases in Vienna.

CONCLUSIONS: Our data represent the experience of the largest cochlear implant program in Austria. In conclusion, cochlear implantation is a safe procedure, associated with a low rate of intra- and postoperative complications. Nevertheless, patients should be informed about possible problems and especially about the risk of a reoperation due to device failure. OBJECTIVES: To evaluate the cause of deafness, the intraoperative findings and the complication rate for all cochlear implant operations performed consecutively between 1994 and 2003 at Vienna General Hospital. MATERIAL AND METHODS: Including all surgeries for bilateral implantation and revision, a series of 342 operations performed on 164 adults (age range 14.5-81 years; mean age 50.79 years) and 128 children (age range 0.75-14 years; mean age 5.00 years) was retrospectively analyzed. RESULTS: The etiology of deafness was predominantly congenital or progressive (66.89%). The routine mastoidectomy approach was chosen in 300 patients (87.72%) and the suprameatal approach in 42 (12.28%). Intraoperatively, 4 children (2.53%) had a cerebrospinal fluid fistula and 35 patients (10.23%) showed cochlear ossification. Three adults (1.63%) and two children (1.27%) had facial nerves with an aberrant course. The overall complication rate was 12.2%, the rate of major complications was 4.97% and the rate of minor complications was 4.09%. There were no cases of either postoperative meningitis or facial nerve palsy. Both flap necrosis and electrode dislocation occurred in one adult patient (0.54%), but in none of the children. Formation of cholesteatoma was found in one adult (0.54%) and one child (0.63%). The rate of device failure was 7.07% for adults and 13.92% for children.

Adolescent↗

Temporal bone histopathology of a patient with a nucleus 22-channel cochlear implant.

The temporal bone histopathology of a patient with profound sensorineural hearing loss attributable to cochlear otosclerosis is presented. The patient had a Nucleus 22-channel cochlear prosthesis implanted in the right ear prior to his demise. Unintentional scala media insertion of the electrode array resulted from labyrinthitis ossificans obliteration of the scala tympani. Inadvertent facial nerve stimulation necessitated program exclusion of electrodes 11 to 16 located in proximity to the labyrinthine and geniculate segments of the facial nerve. The patient received significant benefit from use of the device. The histopathologic findings are correlated with psychophysical and speech perception results.

Cochlear Diseases↗

Pediatric cochlear implantation: experience in Thai patients.

OBJECTIVE: This study was to evaluate the result of cochlear implantation in Thai pediatric patients from 1999-2003. DESIGN: A prospective, open-labeled study. SETTING: University teaching hospital. MATERIAL AND METHOD: Long term surgical and audiologic results comparison after cochlear implantation in 2 cases with Mondini anomality, 7 normal cochlea cases and a case of post meningitis post lingual child. MAIN OUTCOME MEASURES: Speech perception tests battery. RESULTS: Among 10 children, 8 were implanted with Nucleus 22/24; the other two were implanted with Med-el device system. The pre-operated cochlea in 2 cases showed Mondini deformity; both had gushers during the operation with a good outcome. The other 8 cases had normal cochlea although one was deaf from bacterial meningitis. There were 3 re-implantations. Speech production and perception was improved faster in the two children implanted with Med-el than those implanted with Nucleus devices. CONCLUSION: Cochlear implantation in Mondini cases can be successful in speech production and perception. Speech perception ability depended on the age of implantation and preoperative rehabilitation.

Adolescent↗

[Psychosomatic status of patients with cochleovestibular disorders. Correction methods].

Psychosomatic status was studied in 106 patients aged 18-65 years with acute and chronic cochleovestibular disorders. Hearing problems, noise in the ears and vestibular disorders have a negative psychogenic effect in patients with cochleovestibulopathy. The majority of the patients need psychological and psychopharmacological care to relieve anxiety and form adequate attitude to the disease. Correction of psychosomatic disorders raises efficacy of the treatment of cochleovestibular diseases.

Adolescent↗

[Cochlear prosthesis:approaches and results].

This paper describes the development of a prototype of a verbal processor for electric cochlear stimulation. This is a four-channel verbal processor that allows electric stimulation of the hearing organ with acoustic signals, e.g., verbal speech. Preliminary results of psychophysical investigations into the perception of electric signals by deaf patients are presented. It is concluded that devices of this type can be used for research purposes and cochlear implantation.

Cochlear Diseases↗

[Cochlear implants and total cochlear obstruction].

Total cochlea obstruction is not a contraindication for cochlear implant. We report our experience owing to the single channel cochlear implant Monosonic (9 cases), partial insertion of the electrodes-array (3 cases: 1 Digisonic and 2 minisystem) and, more recently the separate electrodes version of the Digisonic (6 cases). This new device is encouraging because it supplies 2/5 patients with telephone use.

Adolescent↗

[Relationship between summating potentials and perilymphatic fistula].

Serial electrocochleogram (ECochG) recordings were obtained within 24 hours from 40 guinea pigs with experimentally induced PLF, and the cochlears of 30 guinea pigs (60 ears) were histopathologically observed under light microscope. -SP occurred in 100% experimented ears within 1-3 hours after PLF, but distention of Ressner's membrane was found only in 30% ones. CAP thresholds and N1 latencies of 10 animals were improved, accompanying disappearance of -SP in 6 of the 10 animals in 24 hours after PLF. It is presumed that -SP is not a specifically electrophysiologic indicator of ELH, and that the generation of -SP may chiefly result from reversible damage of cochlear hair cells. The mechanical factor of basilar membrane displacement can hardly be considered as a main cause of SP abnormality. The mechanism of the ELH following PLF was also discussed.

Animals↗

[The characteristics of vestibular-visual interaction in patients with cochleovestibular disorders under galvanic stimulation].

The paper deals with the problem of vestibular-visual interactions in pathogenesis of cochleovestibular diseases. The investigations were made on the original unit for registration of visual illusions in response to galvanic stimulation of vestibular structures. The examination covered patients with Meniere's disease, peripheral and central cochleovestibular syndrome, otogenic arachnoiditis. Emergence of visual illusions was related to the degree of vestibular abnormality and threshold stimulation. The greatest compensation of the equilibrium function at the expense of vision was reported in patients with central vestibular disorders. Further studies into the vestibular reactions seem important for elucidation of cochleovestibulopathy pathogenesis.

Adult↗

Low-frequency sensorineural loss: clinical evaluation and implications for hearing aid fitting.

Spread of excitation in the cochlea places fundamental limits on the interpretation of audiometric pure-tone hearing loss as a simple map of dysfunction along the cochlear partition, and histologic evidence from human temporal bones will be presented to demonstrate the insensitivity of the audiogram to variations in pathology in the case of low-frequency hearing loss. This article will describe a clinical procedure using simultaneous pure-tone masking to improve upon the localization of cochlear disease, particularly for low-frequency hearing losses, and a model for using the Articulation Index (AI) to develop prognoses for hearing aid performance in these cases, which can then be tested. Fourteen patients with low-frequency hearing loss were divided into two groups based upon threshold shifts caused by a pure-tone masker: those that showed normal low-frequency threshold shifts and those that showed marked shifts at frequencies below the masker, indicating greater loss of function than shown by the unmasked audiogram. Hypothetical audiograms were then generated to model a complete loss of apical function for all patients. Measured speech recognition scores were then compared to AI predictions for the actual and hypothetical audiograms. Best agreement for the patients showing normal masking shifts was between the measured scores and the AI for the actual audiogram, whereas the best agreement for the patients showing marked shifts was with the AI for the hypothetical audiogram. The implications for hearing aid recommendation and fitting in these cases are discussed.

Acoustic Stimulation↗

Radiologic evaluation of multichannel intracochlear implant insertion depth.

Postoperative plain film x-rays are necessary in all multichannel cochlear implant patients to confirm intracochlear position, detect possible electrode kinking, and provide a reference if postoperative slippage occurs. In addition, precise documentation of multichannel intracochlear electrode insertion depths is necessary for comparison of speech recognition results among patients and may be of use for future speech processing strategies. In the present study, a method has been devised, using a modified Stenver's view, to more accurately document insertion depths of the electrode array and location of individual electrodes on 50 multichannel cochlear implant patients. Surgical estimates of insertion depth are shown to have great variability in regard to distance along the basilar membrane when compared with x-ray documentation. Additionally, there is preliminary evidence that insertion depth, as determined by x-ray studies, has a strong correlation with open-set speech discrimination.

Basilar Membrane↗

[Cochleoscopy].

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Animals↗

Glycine immunoreactivity and receptor binding in the cochlear nucleus of C57BL/6J and CBA/CaJ mice: effects of cochlear impairment and aging.

Glycinergic neurons in the cochlear nucleus (CN) of C57BL/6J (C57) and CBA/CaJ (CBA) mice were studied by using immunocytochemical and receptor-binding techniques. Adult C57 mice exhibit progressive cochlear pathology as they age, whereas aging CBA mice retain good hearing. In the CN of old C57 mice (18 months) with severe hearing loss, the number of glycine-immunoreactive neurons decreased significantly. The number (Bmax) of strychnine-sensitive glycine receptors (GlyR) decreased significantly in the dorsal CN of old C57 mice. Significant effects were not observed in the CN of middle-aged C57 mice (with less-severe hearing loss) or in very old CBA mice (which do not exhibit severe hearing loss). The data suggest that the combination of severe hearing loss and old age results in deficits in one or more inhibitory glycinergic circuits in the CN.

Aging↗