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At least 343 records · Page 19Linked to original sources
PubMed1999

Progressive cochleovestibular impairment caused by a point mutation in the COCH gene at DFNA9.

OBJECTIVES: Analysis of phenotype-genotype correlation. STUDY DESIGN: Family study. METHODS: Auditory and vestibulo-ocular functions were examined in a Dutch family with autosomal dominantly inherited sensorineural hearing impairment caused by a 208C > T mutation in the COCH gene, located in chromosome 14q12-q13 (DFNA9). Linear regression analysis of individual longitudinal hearing threshold data (n = 11) on age was performed. RESULTS: Fifteen of the 16 genetically affected persons could be evaluated. They all developed hearing and vestibular impairment symptoms--and in many cases also cardiovascular disease--in the fourth to fifth decade. At the low frequencies (0.25-2 kHz), hearing loss started at the age of about 40 years and showed an average annual progression of approximately 3 dB, finally resulting in profound hearing losses. In two exceptional cases, annual progression attained levels of up to 24 dB. At the high frequencies (4-8 kHz), the average threshold increased from about 50 dB at the age of 35 years to about 120 dB at the age of 75 years (which amounts to 1.8 dB annual threshold increase). All affected individuals tested showed normal ocular motor functions. The patients older than 46 years generally showed absence of the vestibulo-ocular reflex, but their cervico-ocular reflex was enhanced compared with normal subjects, whereas those aged 40 to 46 years showed either severe vestibular hyporeflexia or unilateral caloric areflexia. CONCLUSION: These findings suggest a gradual development of cochleovestibular impairment caused by the new mutation found.

Adult↗
PubMed1999

Non-insulin-dependent diabetic microangiopathy in the inner ear.

UNLABELLED: Hearing loss has long been associated with diabetes mellitus. Microangiopathy, associated with thickening of the basement membranes of small vessels, has been implicated as a major source of multiple system organ disease. OBJECTIVE: This study was designed to evaluate changes in basement membrane thickness in the inner ear of laboratory animals suffering from non-insulin-dependent diabetes mellitus (NIDDM) with, and without, exposure to moderate intensity noise exposure in an attempt to extrapolate the same disease process in humans. DESIGN: Spontaneously hypertensive-corpulent non-insulin-dependent rats (SHR/N-cp) were selected as a genetic model for the above study. Both lean and obese rats were used in this study. A genetically similar control group of animals (LA/N-cp) were used as controls. These animals express both the lean and obese phenotypes, but they lack the NIDDM gene. Forty-eight animals in each group were sacrificed at the end of the study. The cochleas were dissected and fixed. The basement membrane of the stria vascularis was examined using transmission electron microscopy. SETTING: This study was a laboratory-based, standard animal study. MAIN OUTCOME: This study was designed to show microangiography of the inner ear as related to NIDDM with, and without, obesity and noise exposure. RESULTS/CONCLUSIONS: NIDDM alone does not cause statistically significant basement membrane thickening; however, NIDDM in combination with obesity and/or noise exposure did show significant thickening and the combination of all three showed the greatest thickening. NIDDM appeared to be the greatest contributing factor.

Animals↗
PubMed2006

Summating potential-action potential waveform amplitude and width in the diagnosis of Menière's disease.

OBJECTIVES: It has been suggested that analyzing the width and amplitude of the summating potential-action potential (SP-AP) waveforms can increase the sensitivity of electrocochleography. The objective of this study was to evaluate the ratio of SP to AP amplitude (SP/AP) and SP-AP waveform width, as well as the AP latency difference to condensation and rarefaction clicks, for the diagnosis of Menière's disease. STUDY DESIGN: This was a prospective, controlled study. METHODS: We used transtympanic electrocochleography to evaluate 21 patients with definite Menière's disease and 19 normal-hearing patients with other cochleovestibular disorders, comparing SP/AP, AP latency difference, and SP-AP waveform widths as well as calculating the diagnostic sensitivity of these parameters. RESULTS: Mean SP-AP waveform width was 1.89 ms in the study group and 1.58 ms in the control group. Mean SP/AP was 0.37 in the study group and 0.22 in the control group. The mean product of SP-AP waveform width and SP/AP was 75.26 ms% in the study group and 34.60 ms% in the control group. Mean AP latency difference was 0.13 ms in the study group and 0.07 ms in the control group. For the parameters evaluated, the differences between the groups were statistically significant. In the study group, the sensitivities for the width of the SP-AP waveform, the SP/AP, and the AP latency difference were 33.3%, 52.4%, and 23.8%, respectively. CONCLUSIONS: The use of the parameters evaluated did not increase the sensitivity of the electrocochleography, whether used in isolation or in conjunction with the SP/AP. Determining SP/AP presented the greatest sensitivity.

Action Potentials↗
PubMed1992

[Itching following therapy with hydroxyethyl starch (HES) in otoneurological diseases].

It is generally assumed, that a disturbance of microcirculation is the common pathogenetic end factor in various cochleovestibular disorders of different etiology. Therefore improvement of microcirculation is an important therapeutic goal. Several studies demonstrated, that hydroxyethylstarch (HES) has better haemorheological effects than Dextran and less side effects. For this reason we have changed the therapy with Dextran since 1987 to hydroxyethylstarch in several oto-neurological disorders (as sudden hearing loss, neuronopathia vestibularis, idiopathic facial palsy). As after the therapy with HES--generally after dismissal from the ENT-department--some patients complained of general pruritus, so we performed a retrospective study with a standardized interview-protocol. Of 481 treated patients we investigated 237 (49%): of 149 patients treated with HES 200/0.5, 43 patients (28.8%) complained of pruritus; from 88 patients treated with Dextran 40, only 5 patients (5.7%) reported pruritus. The difference is significant (p less than 0.0001). In nearly half of the patients (more than 40%) the pruritus started in normal skin 1 to 3 weeks after the HES-therapy and lasted for 6 weeks to 6 months; the itching was very resistant to therapy (f.e. with antihistaminics). We want to draw the attention to this possible, in the literature until now quite neglected, for some patients extremely uncomfortable and socially embarrassing side effects after HES-therapy when given in relatively high doses. It is therefore suggested therapeutic recommendations should be developed to prevent this undesired side effect.

Cochlear Diseases↗
PubMed1998

Speech discrimination scores of postlingually deaf adults implanted with the Combi 40 cochlear implant.

The aim of the study was to assess the speech discrimination ability of postlingually deaf adults implanted with the Combi 40 cochlear implant and to compare the results with the postoperative data published for other devices. The postoperative open and closed set speech perception performance of 21 consecutive patients was tested using a standardized test battery comprising a number, monosyllable, sentence, consonant and vowel discrimination test as well as a rhyme test in the sound only condition. Mean values achieved for each test 1, 6 and 12 months after "switch on" were evaluated. The results demonstrate that all patients have a substantial benefit from their implant and show a continuous improvement in their speech perception abilities with increased device experience. The mean percentages of correct answers after 12 months were 93.4 for numbers, 44.6 for monosyllables, 78.5 for sentences, 67.6 for the rhyme test, 59.8 for vowel, and 67.3 for consonant discrimination. Preoperatively, the mean discrimination score for monosyllables was 0%. The speech discrimination scores of our patients were similar or higher than described for similar patient groups implanted with other devices. The high stimulation rate of the implant system using the continuous interleaved speech processing strategy as well as a deep atraumatic electrode insertion into the apicalmost regions of the scala tympani may be the reason for good performance.

Adult↗
PubMed2005

Cochlear implantation in chronic otitis media.

Chronic otitis media (COM) patients who had a multichannel device implanted were evaluated regarding surgical problems and technical modifications. In a multicentric study, implantees whose aetiology was COM were retrospectively evaluated. Patients were operated on and evaluated at three different tertiary referral centres: SSK Izmir Hospital Cochlear Implantation (CI) Center (32 cases), Istanbul Marmara University ENT Clinic (six cases), Eskişehir Anadolu University CI Center (one case). Thirty-nine implantees were evaluated with respect to surgical problems, technical modifications, complications and hearing results. All patients had lost their hearings as a result of COM. Three out of 39 cases were children. Thirty-seven of the patients either had a radical cavity or ear converted to radical at the first stage or concomittantly with the implantation. Patients were evaluated in order to find out the best possible surgical solutions to specific problems caused by COM. In seven cases electrode array disrupted the epithelial lining of the cavity despite specific measures. Five of those cases were re-implanted, passing the electrode array through a tunnel under the facial nerve. Seven recent cases were also implanted with the same subfacial route. In all patients but one, satisfactory hearing results were achieved one to five years after implantation (SDS scores with monosyllabic word list were between 59 and 89 per cent, median 67.4 per cent). CI in COM patients necessitates technical modifications. In radical cavities subfacial implantation seems to be a good solution for the protection of the electrode array.

Adolescent↗
PubMed2005

Is mastoidectomy indispensable in cochlear implant surgery?

OBJECTIVE: To challenge the need for mastoidectomy in cochlear implant (CI) surgery by comparing the advantages and disadvantages of the classic technique for CI, the mastoidectomy posterior tympanotomy approach (MPTA), with the suprameatal approach (SMA), a nonmastoidectomy approach. STUDY DESIGN AND SETTING: A retrospective study of 290 patients who underwent cochlear implantation in our department between 1989 and 2002. One hundred fifty-seven of them underwent the MPTA and 133 underwent the SMA. The ability of the electrode to expand in the MPTA patients who were reoperated on was examined, as was the influence of mastoidectomy on the course of chronic secretory otitis media (SOM) in a group of 56 children who suffered from chronic SOM prior to implantation. RESULTS: In all 4 reoperated children, in whom the MPTA was used, the mastoid was completely closed by bony regrowth on the cortical portion of the mastoid and the mastoid cavity was obliterated. The electrode had been embedded in dense fibrous tissue and bony spicule, preventing electrode expansion. Mastoidectomy was found to have no influence on the course of chronic SOM. The rate of postoperative SOM was found to be equal in 40 children who were operated on using the MPTA compared with 16 operated on using the SMA. CONCLUSION: Using a nonmastoidectomy approach, such as the SMA, provides a wide exposure of the middle ear and promontory and thus enables a well-controlled cochleostomy site and safe insertion of the electrode into the cochlea. Mastoidectomy in CI surgery is not indispensable; it may cause more disadvantages than advantages.

Adolescent↗
PubMed1998

Hearing results of intratympanic steroid treatment of endolymphatic hydrops.

OBJECTIVES/HYPOTHESIS: This study evaluated the effectiveness of a single application of steroids to the open middle ear in improving short-term hearing in patients with Meniere's disease and cochlear hydrops. STUDY DESIGN: Retrospective study in which each patient's pretreatment hearing served as the control compared with posttreatment hearing. METHODS: Patients were treated with a single application of dexamethasone, 8 mg, in hyaluronan. Following tympanotomy and lysis of round window adhesions, steroids were placed in the round window niche with absorbable gelatin sponge and the remainder of the middle ear was then filled with the steroid solution. Systemic steroids were not administered. Audiograms were performed within 1 month before surgery and at least 1 month after surgery. RESULTS: Between September 1996 and July 1997, 21 ears in 19 patients underwent intratympanic steroid treatment. The criterion for hearing change was a 10-dB or greater change in pure-tone average (PTA), or a 15% change in speech discrimination score (SDS). Of the 15 ears meeting inclusion criteria for this study, five (33%) demonstrated hearing improvement and three (20%) demonstrated hearing deterioration. Maximum improvement was a 38-dB improvement in PTA and a 32% improvement in SDS. CONCLUSION: A single application of intratympanic dexamethasone/hyaluronan solution directly to the round window did not produce dramatic short-term hearing improvement in patients with endolymphatic hydrops. Although the theoretical basis for intratympanic steroid treatment of endolymphatic hydrops is appealing, we urge close evaluation of the results of specific protocols of intratympanic steroid administration before widespread utilization of this treatment. The choice of steroid, route of administration, frequency of application, and need for simultaneous systemic administration require standardization to adequately assess the efficacy of this treatment.

Adjuvants, Immunologic↗
PubMed2005

Auditory electrical tinnitus suppression in patients with and without implants.

The aim of this study was to evaluate the effectiveness of electrical tinnitus suppression in two groups of chronic severe tinnitus sufferers. Through standard tinnitus questionnaires, we compared the effectiveness of extratympanic and intratympanic auditory electrical stimulation (AES) by cochlear implants (CI) for the suppression or abolition of the perception of tinnitus and the decrease of its associated complaints. We made otolaryngological and comprehensive audiological assessment and also tinnitus measurement in each group of patients before and after AES and 50 days later. We investigated the dimensions of psychological complaints due to chronic and disabling tinnitus by means of the tinnitus questionnaire (TQ). The control examination during at least seven sessions (50 days) after AES in the group of patients without implants showed improvement in 20 of 32 patients (62.5%); 12 (37.5%) did not notice any change. In the comparative group of patients with implants, improvement occurred in 16 of 20 patients (75%); during the switch-on of the speech processor, these patients reported significant attenuation or complete suppression of their tinnitus. Complete suppression of the tinnitus after CI was observed for 11 patients (55%), and 5 patients (25%) demonstrated significant attenuation of tinnitus. Nonsuppression of tinnitus was observed for only 4 patients (25%). None of our patients was affected by an increment in the tinnitus owing to CI. The differences of means of scores in the standard TQ were significant in both groups of patients. A comparison of TQ score differences between patients with and without implants showed no significance. We concluded that AES is a useful and effective therapeutic intervention in patients with tinnitus. Extratympanic AES reduces the effects of the tinnitus but presents limitations, mainly owing to the short duration of the electrical residual inhibition of the tinnitus. CI is shown to be more efficient for the treatment of tinnitus, mainly because the electrical stimulation affects a wider area of the cochlea and is presented for longer sessions. Therefore, patients affected by incapacitating tinnitus should be considered for continuous use of electrical stimulation.

Adolescent↗
PubMed1999

Short tone burst-evoked myogenic potentials on the sternocleidomastoid muscle: are these potentials also of vestibular origin?

OBJECTIVES: To show that short tone bursts (STBs) evoke myogenic potentials from the sternocleidomastoid muscle (SCM) that are of vestibular origin. DESIGN: Evoked potential activity was recorded from the SCMs of normal volunteers and from patients with vestibulocochlear disorders. SETTING: This outpatient study was conducted at the Department of Otolaryngology, University of Tokyo, Tokyo, Japan. SUBJECTS: Nine normal volunteers and 30 patients (34 affected ears) with vestibulocochlear disorders were examined. INTERVENTION: Diagnostic. OUTCOME MEASURES: Sound-evoked myogenic potentials in response to STBs were recorded with surface electrodes over each SCM. Responses evoked by STBs in patients were compared with responses evoked by clicks. RESULTS: In all normal subjects, STBs (0.5, 1, and 2 kHz) evoked biphasic responses on the SCM ipsilateral to the stimulated ear; the same was true for clicks. Short tone bursts of 0.5 kHz evoked the largest responses, while STBs of 2 kHz evoked the smallest. In patients with vestibulocochlear disorders, responses to STBs of 0.5 kHz were similar to responses evoked by clicks. Thirty (88%) of the 34 affected ears demonstrated the same results with 0.5-kHz STBs and with clicks. Responses were present in patients with total or near-total hearing loss and intact vestibular function. Conversely, patients with preserved hearing but with absent or severely decreased vestibular function had absent or significantly decreased myogenic potentials evoked by STBs. CONCLUSIONS: Short tone bursts as well as clicks can evoke myogenic potentials from the SCM. Myogenic potentials evoked by STBs are also probably of vestibular origin.

Acoustic Stimulation↗
PubMed2002

Otoacoustic emissions for monitoring aminoglycoside-induced ototoxicity in children with cystic fibrosis.

OBJECTIVE: To investigate whether transient-evoked and distortion-product (DP) otoacoustic emissions (OAEs) are more sensitive than pure-tone audiometry (PTA) in revealing gentamicin-induced ototoxicity in children with cystic fibrosis (CF). DESIGN: Prospective case-control study. SETTING: Tertiary referral audiologic center in conjunction with an academic pediatric CF unit. PARTICIPANTS: The study group consisted of a consecutive sample of 12 audiologically normal children with CF and a history of gentamicin exposure (CF-gentamicin group). The control groups consisted of 8 age-matched children with CF and 11 age-matched healthy volunteers. No member of the control groups had a history of aminoglycoside exposure. INTERVENTION: Members of the CF-gentamicin study group received 4 mg/kg of gentamicin per day for a mean of 14.2 days (range, 11-29 days). OUTCOME MEASURES: The PTA thresholds (250-8000 Hz) were the criterion standard. Transient-evoked OAEs' reproducibility at 5 frequency bands (800, 1600, 2400, 3200, and 4000 Hz) and total emission level were measured, as were DP-audiogram (DP-gram) amplitude (1001-6299 Hz), input-output function dynamic range, and detection thresholds at 4004, 6006, and 7996 Hz. Baseline measurements were compared between groups examining the effect of CF and previous gentamicin exposure (2-way analysis of variance). For the CF-gentamicin group, baseline measurements were compared with those at the end of the last gentamicin treatment (paired t test). RESULTS: The PTA findings were normal for all groups at baseline and remained normal in the CF-gentamicin group after treatment. The CF-gentamicin group had significantly lower transient-evoked OAEs total emission level, DP-gram amplitude at 5042 Hz, and input-output dynamic ranges with higher detection thresholds in all frequencies compared with both control groups, which was attributed completely to previous gentamicin exposure (P<.05). After treatment, further decreases in total emission levels, DP-gram amplitudes (>3000 Hz), and dynamic ranges were noted, with increased detection thresholds (P<.05). CONCLUSIONS: Otoacoustic emissions measurement (especially of DP OAEs) proved more sensitive than PTA in revealing minor cochlear dysfunction after gentamicin exposure. They should be used for monitoring patients receiving ototoxic factors such as aminoglycosides.

Adolescent↗
PubMed1990

Effect of topical fosfomycin on polymyxin B ototoxicity.

Fosfomycin is an antibiotic that has been found to reduce the ototoxicity of aminoglycoside antibiotics and cisplatin when systemically coadministered. Polymyxin B, an antibiotic frequently used in ototopical preparations, has been shown to be ototoxic in experimental studies. To investigate the effect of fosfomycin on polymyxin B ototoxicity, topical administration of the two agents into the middle ear cavity was performed. Two groups of chinchillas were used. One group received applications of polymyxin B alone, and the second group received polymyxin B combined with fosfomycin. It was found that application of polymyxin B produces severe damage to the cochlea. However, when polymyxin B was given in combination with fosfomycin, cochlear damage was dramatically reduced. It is likely that in clinical use, a combination of polymyxin B and fosfomycin would demonstrate reduced risk of ototoxicity.

Administration, Topical↗
PubMed1991

Inner ear damage secondary to diabetes mellitus. I. Changes in adolescent SHR/N-cp rats.

The association between diabetes mellitus and hearing impairment has been debated in many previous studies. The spontaneous hypertensive/NIH-corpulent (SHR/N-cp) rat has been shown to be a unique genetic model for non-insulin-dependent diabetes mellitus. Seventeen diabetic and 17 control young male rats were divided into groups according to diet and phenotype. The rats were fed either 54% of sucrose or 54% starch diets for 3.5 months and killed at 5 months. The cochleas were fixed, decalcified, dissected, and stained for hair cell counting. A significant loss of outer hair cells was noted in the diabetic obese (SHR/N-cp) animals when compared with the control obese (LA/N-cp) animals in every group. Although no significant difference was noted between the diabetic obese (SHR/N-cp) animals receiving the starch and sugar diets, the diabetic obese (SHR/N-cp) animals were more severely affected than the nondiabetic lean (SHR/N-cp) rats.

Animals↗
PubMed1991

Surgical management of perilymph fistulas. A new technique.

A wide range of recurrence rates (21% to 47%) for perilymph fistula repairs have been reported in the otology literature. An improved surgical technique developed at the Portland (Ore) Good Samaritan Hospital and Medical Center Neurotology Department was used to repair perilymph fistulas in 58 patients from October 1986 to October 1988. Our recurrence rate was reduced from 27% in a 1982-1985 study to 8% in our study. At 1 year postoperatively, improvements in disequillibrium, dizziness, and vertigo were comparable with results of older surgical techniques. Functional outcomes were also good: 83% of patients returned to normal activities of daily living, and 71% also returned to school or resumed gainful employment outside the home.

Adolescent↗