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At least 289 records · Page 16Linked to original sources
PubMed1999

The large vestibular aqueduct syndrome: the role of cochlear implantation in its management.

In recent years the large vestibular aqueduct syndrome has become an increasingly recognized cause of a progressive sensorineural hearing loss. Cochlear implantation, although not correcting the bony abnormality, does offer an avenue for rehabilitation for affected individuals and initial results are encouraging. Of 15 patients identified with the large vestibular aqueduct syndrome we have implanted seven (five adults and two children). All patients underwent an uneventful electrode insertion with uncomplicated postoperative periods. Three of our adult patients are showing very good initial results with BKB speech recognition scores of 100%, 74% and 66% with the implant alone. It is still early days with the children, but initial results are encouraging. A full review of the large vestibular aqueduct syndrome is given along with our results.

Adolescent↗
PubMed2003

[Implication of cochlear implantation in patients with enlarged vestibular aqueduct syndrome].

OBJECTIVE: To evaluate the intraoperative obstacles and post-operative outcomes of cochlear implantation in children with enlarged vestibular aqueduct syndrome. METHODS: Between May 1995 and June 2002, 10 patients with enlarged vestibular aqueduct syndrome received cochlear implantation in Pekin Union Medical College Hospital. The clinical records of 10 cases of enlarged vestibular aqueduct syndrome were analyzed retrospectively. RESULTS: Via cochleostomy pulsatile clear fluid gusher occurred in 8 cases, which were easily controlled by quickly inserting the electrode array and sealing the cochleostomy with muscle tissue in each instance. The implant was inserted without difficulty in all patients. There have been no post-operatively complications, and audiologic outcome of the 10 patients with cochlear implantation were almost the same as the other patients without inner ear malformations. Five cases of them can even speak better than the other prelingual deafness that may be attributed in part to postlingual deafness. CONCLUSIONS: Despite pulsatile clear fluid gusher in cochlear implantation, no post-operatively complications have been found, and the outcome of these 10 patients with cochlear implantation was excellent. Cochlear implantation can be safely and effectively performed in patients with profound sensorineural hearing loss (SNHL) associated with enlarged vestibular aqueduct syndrome.

Adolescent↗
PubMed2005

[Comparing the characteristics of psychophysical tests between cochlear implant children with large vestibular aqueduct syndrome and normal inner ear].

OBJECTIVE: The purpose of this study is to compare the characteristics of psychophysical tests between cochlear implant children with large vestibular aqueduct syndrome and normal inner ear and to analyse the characteristics of psychophysical and impedance tests for cochlear implant patients with large vestibular aqueduct syndrome. METHOD: One hundred and thirty-one cochlear implant pre-lingual deafness patients participated in this study. They received cochlear implant surgery at the age of 1.5 to 12 years old from Nov, 1998 to Aug, 2003. The patients were divided into two groups. Group A had 111 patients with normal inner ear. Group B had 20 patients with radiographically proven large vestibular aqueduct syndrome. All of the patients were inserted with Nucleus 24M. The time of the first programming session was approximately 4 weeks after surgery. The psychophysical tests included T and C-Level tests. The programming techniques used in the test were suitable for the age of the patients. The R116 and R126 software were used to test the impedance of the electrodes automatically. RESULT: Using the independent-samples t-test, the impedance and T/C-Level of each electrode did not differ significantly between group A and B ( P > 0.05). The impedance and T/C-Level were not significantly different between group A and B at the time of switch-on, 1 week, 2 weeks, 3 weeks, 2 months, 3 months, 6 months, 9 months and 1 year after switch-on (P > 0.05). CONCLUSION: The parameters of mapping were not significantly different between cochlear implant children with large vestibular aqueduct syndrome and normal inner ear. The management and procedure of mapping used in cochlear implant children with normal inner ear can be used in the children with large vestibular aqueduct syndrome.

Case-Control Studies↗
PubMed1999

Cochlear implantation in children with enlarged vestibular aqueducts.

OBJECTIVES: This report details our experience with cochlear implantation in children with both profound sensorineural HL (SNHL) and enlarged vestibular aqueducts (EVAs). It seeks to determine if the abnormal anatomy of EVA predisposes to any adverse events during or after cochlear implantation. STUDY DESIGN: A retrospective review. METHODS: Charts were reviewed for details of the procedure, complications, and audiologic outcome. RESULTS: Between 8/25/93 and 9/16/98, 10 children with EVAs received cochlear implants, of whom 8 children (5 males, 3 females; mean age 7.8 y) had audiologic follow-up of at least 6 months. The implant was inserted without difficulty in all patients. Pulsatile clear fluid via the cochleostomy was observed in five patients, but was easily controlled in each instance. There have been no major complications, although two patient had short-lived postoperative vestibular symptoms and one child has experienced an intermittent pulsing sensation in her head. Speech perception measures were obtained using a battery of tests that assessed the children's ability to perceive speech in both open- and closed-set formats. Two patients were excluded because the implant was placed within the last 6 months. Of the remaining eight children identified with EVAs, seven (86%) demonstrated open-set word recognition. CONCLUSIONS: These favorable results may be attributed in part to HL that occurs relatively late in childhood, allowing implantation in postlingual candidates. Cochlear implantation can be safely and effectively performed in children with SNHL associated with EVAs.

Adolescent↗
PubMed2004

Sudden hearing loss due to large vestibular aqueduct syndrome in a child: should exploratory tympanotomy be performed?

A 16-year-old girl applied to our ENT clinic with a 3-day history of right hearing loss, tinnitus, and pressure in the right ear. She had had surgery for right perilymph fistula two times, one at the age of 7 and the second at the age of 9. She had recovered after both of these surgeries. This time she had exploratory tympanotomy and perylymh fistula was detected. Computerized tomography investigation obtained after 5 days postoperatively showed bilateral large vestibular aqueducts and otherwise normal inner ear structures. Thyroid function tests and neck palpation were normal. It was an unusual case with both large vestibular aqueduct syndrome (LVAS) and simultaneous spontaneous perilymph fistula.

Adolescent↗
PubMed1999

Multichannel cochlear implantation in children with large vestibular aqueduct syndrome.

This study examined the postoperative performance of multichannel cochlear implantation in children with severe-to-profound sensorineural hearing loss secondary to large vestibular aqueduct syndrome. Five children between ages 4 and 13 years who had large vestibular aqueducts confirmed by computed tomography scans underwent implantation of a CLARION Multi-Strategy Cochlear Implant between January 1995 and June 1996 at Boys Town National Research Hospital. In addition to preoperative evaluations, they were examined postoperatively with their implants on a battery of open-set speech recognition tests at 3, 6, 12, and 18 months following initial stimulation. Results indicated a noticeable improvement in open-set speech recognition within the first 3 months of implant use compared to preimplant performance with hearing aids. All 5 patients demonstrated substantial benefit from their implants, but there was variation among the children in the rate and amount of improvement in speech recognition skills.

Child↗
PubMed2003

[Cochlear implants in children with enlargement of vestibular aqueduct].

OBJECTIVE: To describe the clinical experiences with multi-channel cochlear implantation in the children with enlargement of vestibular aqueduct. METHOD: Fifteen patients were diagnosed with enlargement of vestibular aqueduct and received multi-channel cochlear implantation at Beijing Tongren Hospital. A cochleostomy was performed for all patients by a standard facial recess approach to anterior inferior to the round window niche. RESULTS: (1) Most of the electrodes were totally inserted in the cochlear, and gusher appeared in 6 implantations. (2) No serious complications occurred after implantation. (3) All patients have auditory sensations after implantations. (4) The hearing threshold in this series were similar to that of the normal cochlear implantation (the average threshold was 30 dB). (5) The rehabilitation concept was developed by Rehabilitation Research Center for Deaf Children in Beijing and Beijing Tongren Hospital. The training sessions included sound detection, word discrimination, auditory comprehension and spoken language skill development. The abilities of speech discrimination and spoken language were improved comparing with that of pre-operation. Two of the patients could go to the normal school after implantations. CONCLUSION: The outcome of hearing rehabilitation for children with enlargement of vestibular aqueduct were similar to those children with "normal" cochlear structure followed the multi-channel cochlear implantation.

Adolescent↗
PubMed1999

Cochlear implantation in children with large vestibular aqueduct syndrome.

OBJECTIVE: This study describes the effectiveness of a multielectrode cochlear implant prosthesis (Cochlear; Cochlear Pty., Lane Cove, Australia) for providing hearing to children with deafness caused by large vestibular aqueduct syndrome (LVAS). STUDY DESIGN: The study design was a retrospective study. SETTING: All the children attended The Children's Cochlear Implant Center (NSW), which is a specialist center that provides audiologic testing, speech therapy, habilitation, and medical assistance for children with cochlear implants. PATIENTS: Ten children were studied who had profound hearing loss and radiologic evidence of a vestibular aqueduct larger than 2 mm in width in its intraosseous portion. INTERVENTION: The children received a multielectrode (Cochlear) cochlear implant prosthesis, and the associated programming of the device and habitation were performed postoperatively. No significant problems were encountered at any of the surgeries, although there was an initial gush of perilymph when the otic capsule was opened in 7 ears. MAIN OUTCOME MEASURES: Postoperative audiologic performance at six monthly intervals and school performance were assessed. RESULTS: The postoperative auditory performance was improved in all children. At 6 months, their average BKB score had increased from 31% to 79%; average word score, from 8% to 43%; and average phoneme score, from 38% to 70%. The older children were able to continue their education in their usual setting with less reliance on hearing support staff. CONCLUSION: Children with a deteriorating hearing loss caused by LVAS can derive considerable benefit from a cochlear implant.

Child↗
PubMed1986

Anatomic basis for labyrinthine preservation during posterior fossa acoustic tumor surgery.

Earlier diagnosis of acoustic tumors promises to increase our opportunity to identify patients with serviceable hearing. Critical to a posterior fossa transmeatal approach for acoustic tumor resection is preservation of the underlying labyrinth. Although the labyrinth has been recognized as a limiting factor in exposure of tumor in the internal auditory canal, few reports have detailed the microscopic surgical anatomy posterior to the internal auditory canal. An anatomic study was undertaken to determine consistent relationships between critical structures within the temporal bone relevant to hearing preservation surgery. The results of this study indicate that, whereas topographic landmarks are helpful for orientation, the more consistent relationship of the labyrinth to the vestibular aqueduct and singular canal allows a more accurate localization of the underlying labyrinth. Although the vestibule frequently prevents direct visualization of the transverse crest, a dissection based upon the microsurgical anatomy will maximize visualization of the lateral fundus while preserving the integrity of the labyrinth.

Cochlea↗
PubMed2002

Changes in quality of life and the cost-utility associated with cochlear implantation in patients with large vestibular aqueduct syndrome.

OBJECTIVE: A group of 20 patients with large vestibular aqueduct syndrome was identified at the Indiana University School of Medicine. The major objective of this study was to explore the improvements in quality of life associated with cochlear implantation in patients with large vestibular aqueduct syndrome, as well as the cost-utility of cochlear implantation in this group. SETTING: A total of 70 patients were identified with large vestibular aqueduct syndrome through analysis of thin-section computed tomography of the temporal bone over the past 6 years at this medical center. Data collected from the medical records for each patient included demographic data, hearing-related statistics, implantation data, and audiometric data. Sixteen children and adults with large vestibular aqueduct syndrome had undergone cochlear implantation before the beginning of this study, and the remaining 54 children and adults were identified as undergoing treatment of progressive or fluctuant sensorineural hearing loss. Health utility indexes used in this analysis were taken through the use of the Ontario Health Utility Index, Mark III. The range of costs used for cost-utility analysis was derived from the costs of cochlear implantation at this medical center, as well as from costs associated with implantation published in the medical literature. METHODS: Participants were selected from the total population of patients with large vestibular aqueduct syndrome at this center who were postlingually deafened and who currently had severe hearing loss. Two groups were formed. These groups comprised either cochlear implant patients with large vestibular aqueduct syndrome or patients with large vestibular aqueduct syndrome currently using hearing aids. Ten of the 16 cochlear implant patients and 10 of the remaining 54 patients with large vestibular aqueduct syndrome met these criteria. Mark III health utility indexes were distributed to patients in each group and scored. Those health utility indexes not completed by the patients were scored by proxy, using the audiologist at this center who was the most familiar with the patient. Changes in quality of life associated with cochlear implantation were derived by comparison of the health utility index results of the two groups. Cost-utility measures were then made using discounted costs per quality-of-life years (QALYs) (5%), and a sensitivity analysis was performed that evaluated changes in scoring done by proxy. The cost-utility results were then compared with the cost-utilities derived from similar studies and associated with other disease states. RESULTS: Although both groups of patients had significant hearing loss, the hearing aid group had a better mean pure-tone average. The mean pure-tone average for the hearing aid group was 70.8 dB (SD 24.4), and the mean pure-tone average for the cochlear implant group was 107.0 dB (SD 21.7). Seven of the 20 health utility indexes were scored by proxy. Results from the base case indicate a 0.20 gain in health utility from cochlear implantation (hearing aid = 0.62, cochlear implant = 0.82, p = 0.037), resulting in a discounted cost per QALY of $12,774. Sensitivity analysis of the proxy scoring indicated that decreasing the hearing score one level on the health utility index resulted in a gain in health utility with cochlear implantation of 0.15, resulting in a discounted cost per QALY of $17,832. A decrease of the hearing score by two levels on the health utility survey resulted in no significant gain in quality of life with cochlear implantation. CONCLUSION: This study found an improvement in quality of life associated with cochlear implantation in postlingually deafened patients with large vestibular aqueduct syndrome. By weighing this improvement in quality of life against the significant difference noted between the pure-tone averages of each group, further strength can be given to this conclusion. This gain in quality of life, as well as the results derived for the cost-utility of cochlear implantation, was similar to that in previous published studies of cochlear implantation in all types of patients. These results also indicate a favorable cost-utility when compared with published data about other disease states. As patients with large vestibular aqueduct syndrome progress to profound levels of hearing loss, these results indicate that cochlear implantation can be offered as a beneficial, life-improving therapy.

Adolescent↗
PubMed1991

[Perilymphatic fistula].

Perilymphatic fistulae are the first cause of perceptive deafness that may be amenable to surgical treatment. After a complete study of the literature, we have made a retrospective analysis of 48 ear explorations in 37 children. These children presented with a variable degree of progressive or fluctuating perceptive deafness without any obvious etiology. Computed tomography, especially aimed at detecting an abnormality in the patency of the aqueduct of the cochlea, seems to be the most efficient criterion of selection to establish an indication for surgical exploration. The extent of the perilymphatic fistulae and their location, mainly at the level of the fissura ante fenestram, call to our mind the possibility of an arrest in the differentiation of the otic capsule, with a persistent anomalous patency of the aqueduct of the cochlea. The results of this study mainly demonstrate stabilizations of hearing, while spectacular improvements still are anecdotal. On the other hand, morbidity is very low and mainly results from insufficient surgery to fill in the fistula. Further studies are needed, especially to better understand the pathophysiology of the perilymphatic fistulae, and their results must certainly be appreciated in the longer term.

Adolescent↗
PubMed1994

PEG-IL-2 therapy of advanced cancer in the guinea pig. Impact of the primary tumor and beneficial effect of cyclophosphamide.

The efficacy of tumor therapy using polyethylene-glycol-modified interleukin-2 (PEG-IL-2), alone or in combination with cyclophosphamide, was studied in advanced metastatic disease in the guinea pig. Line 10 (L10) tumor cells appeared in the axillary lymph node only 7 days after intradermal tumor-cell inoculation, and lymph-node leukocytes were almost completely replaced by tumor cells on day 28. Local treatment of the intradermally growing L10 hepatocarcinoma in the guinea pig with a relatively low dose of PEG-IL-2 resulted in regression of the primary tumor and prevention of lymph-node metastases. Therapy was completely curative (4 out of 5 animals) when started on day 7 or 14 after tumor-cell inoculation. When started on day 21, therapy was effective in only some (2 out of 5 cured) of the treated animals. Anti-tumor effects against the primary tumor and against lymph-node metastases were observed only after intratumoral (i.t.) administration of PEG-IL-2. Injection of the agent into or near lymph-node metastases in the absence of the primary tumor had no curative effect. In PBS/BSA-treated control animals the primary tumor and metastases grew progressively. In the treatment of far advanced metastatic disease, the combination of i.t. administration of PEG-IL-2 and i.p. injection of cyclophosphamide (Cy) resulted in improved anti-tumoral effects (5/5 guinea pigs were cured) when compared with monotherapy using either agent (one and none out of 5 animals cured, respectively). PBS/BSA heated controls showed progressive tumor-growth. We conclude that large primary tumors and lymph-node metastases can be treated effectively with PEG-IL-2. The i.t. route of administration is of major importance in the treatment of metastases, since administration of PEG-IL-2 near or into the lymph node had no therapeutic effect. Combination of PEG-IL-2 therapy with systemic injections of Cy significantly improved the curative effects of the treatment of advanced metastatic cancer.

Animals↗
PubMed1979

The permeability of 51Cr between cerebrospinal fluid and internal ear.

Radioactively labelled chromium was instilled in the cerebrospinal fluid of guinea pigs. After different intervals, the radioactivity of inner ear fluids was measured and compared with those of other regions. The results lead to kinetic information about the permeability of 51Cr between cerebrospinal and inner ear fluids.

Animals↗