Faculty Bibliography

OBJECTIVE: To evaluate the efficacy of cochlear implants in human immunodeficiency virus-infected individuals and correlate these results with a proposed pathophysiological mechanism of human immunodeficiency virus-associated hearing loss. STUDY DESIGN: Retrospective case series and temporal bone analysis of deceased human immunodeficiency virus-positive patients. SETTING: Tertiary care hospital. PATIENTS: Seven human immunodeficiency virus-positive individuals with profound sensorineural hearing loss. INTERVENTION: Cochlear implantation at New York University Medical Center. METHODS: The surgical outcomes and complications were analyzed. Additionally, electron microscopic and immunohistochemical findings of cadaver temporal bone specimens of other known human immunodeficiency virus-positive individuals were reviewed. The performance results of the human immunodeficiency virus-positive cochlear implant patients were then correlated with the previously hypothesized pathophysiological mechanism of human immunodeficiency virus-associated hearing loss. RESULTS: The patients had a varied performance with cochlear implantation, and as a group performance was good. There were no surgical complications or postoperative complications. The good performance of these patients supports the hypothesis that the mechanism of human immunodeficiency virus-associated deafness involves infiltration, malfunction, and premature degeneration of the hair cells and supportive cells of the cochlea. CONCLUSIONS: Human immunodeficiency virus-positive individuals benefit from cochlear implantation without increased surgical risk

HYPOTHESIS: Real-time intraoperative fluoroscopy is a useful adjunct to cochlear implantation in selected cases. The advantages include the avoidance of complications such as extracochlear array placement, intrameatal array insertion, and avoidance of significant bending or kinking. This is particularly useful when implanting a severely abnormal cochlea. BACKGROUND: The technique was initially developed for laboratory study of electrode prototypes in cadaver temporal bones to evaluate insertion dynamics and mechanisms of intracochlear trauma. The technique was subsequently adapted for use in live surgeries. METHODS: Live surgeries were performed using fluoroscopic guidance on nine patients. RESULTS: Five patients were implanted with the Nucleus 24 RCS during preclinical trials. Two patients with severe cochlear malformations were implanted with a Nucleus CI24M straight array. Two patients with severe cochlear ossification were implanted with the Nucleus CI24 double array. Appropriate insertions were achieved without electrode damage in all cases. CONCLUSIONS: Intraoperative fluoroscopy is a useful adjunct to cochlear implantation, which can be performed with minimum risk to the patient and operating room staff if the outlined precautions are taken. Intraoperative fluoroscopy is indicated in cases where the intracochlear behavior of the electrode array cannot be predicted, a condition encountered when implanting new electrode designs, cases with severely malformed inner ears, or cases of severe intraluminal obstruction requiring a double-array insertion

OBJECTIVE: Since 1987, when the use of multichannel cochlear implants was initiated in children, candidacy has expanded; many thousands of children have received these devices, and results have revealed a wide range of performance. However, few long-term studies exist on a large population of these children. There have been concerns expressed that cochlear implant function might degrade over time, that devices and electrodes might migrate and extrude in the growing child, or that there might even be a deleterious effect of long-term stimulation of the cochlear nerve. The purpose of this study was to explore the long-term effects of implantation as a function of performance over time, reimplantation, and educational factors. STUDY DESIGN AND SETTING: We studied 81 children who received implants at a major academic medical center and were followed for 5 to 13 years. RESULTS: Results revealed significant gains in speech perception, use of oral language, and ability to function in a mainstream environment. There was no decrease in performance over time and no significant incidence of device or electrode migration or extrusion, and device failure did not cause a deterioration in long-term outcome. CONCLUSIONS: Multichannel cochlear implants in children provide perception, linguistic, and educational advantages, which are not adversely affected by long-term electrical stimulation

OBJECTIVE: To determine the efficacy of cochlear implants with current processing strategies in children and adults with long-term congenital deafness, and to determine whether the growth of auditory perceptual skills in these patients is similar to the data reported for patients who have undergone implantation with earlier devices and coding strategies. STUDY DESIGN: A prospective study of children and adults fulfilling the study inclusion criteria. SETTING: University medical center. PATIENTS: Thirty-five congenitally deaf children who received implants after the age of 8 years and 14 congenitally deaf adults who received implants as adults participated in this study. Length of device use ranged from 6 months to 3 years. MAIN OUTCOME MEASURES: Open set phoneme, word recognition tests, and sentence recognition tests were administered in quiet and noise auditory only conditions preoperatively and postoperatively. Changes in test scores were examined by paired t tests, and differences between groups were compared by Student's t tests. Spearman correlation coefficients were calculated to identify associations between scores and subject characteristics. A two-sided alpha of less than 0.05 was considered statistically significant. RESULTS: The results indicated significant improvement in open set speech perception skills in the children after implantation; the adults demonstrated improved mean scores on both word and sentence recognition. A shorter length of deafness correlated with better postoperative performance, but all subjects continued to improve over time. The improvement in test scores was similar between the devices, and no significant differences were detected between the different processing strategies. CONCLUSION: Children and adults with long-term congenital deafness can obtain considerable open set speech understanding after implantation. Length of deafness (age at implantation), length of device use, and mode of communication contribute to outcome

This paper deals with the Nucleus C124R (CS) (Contour) cochlear Implant: its characteristics, differences compared with the previous generation of devices, the perimodiolar electrode, and the surgical technique used for safe insertion. We also discuss the rationale behind perimodiolar electrodes in general, as well as the results of laboratory studies validating the design and safety of this particular electrode array. The differences in surgical technique between this device and prior Nucleus cochlear implants are as follows: the incision and the size of the well, or recess, for the electronics are smaller; the cochleostomy is larger; the posterior portion is placed in a subpericranial pocket, not tied down, before electrode insertion; and the insertion process itself is quite different, due to the nature of the electrode, its size, shape, and stylet. The technique described is that used by one experienced cochlear implant center, and reflect the authors' practice. Clearly, there are other possible variations on this theme, which may be equally satisfactory in other hands. Most surgeons find this device to be easier to place than previous generations: complications to date have been uncommon

OBJECTIVE: To examine the relationship between histopathology, immunohistochemistry, and clinical behavior in atypical and low-grade malignant vestibular schwannomas. STUDY DESIGN: The study design was a retrospective case review in conjunction with a histopathologic and immunohistochemical proliferation marker study of archival specimens. DATA SOURCES: A tertiary referral center's anatomic pathology and vestibular schwannoma computerized databases. METHODS: The diagnosis of atypical or low-grade malignant vestibular schwannoma was based on the number of mitotic figures present per tumor slide. MIB1 labeling indices were used to compare the proliferative activity of the atypical and low-grade malignant groups with that in an age-matched and size-matched control group. RESULTS: Eight cases of atypical and six cases of low-grade malignant vestibular schwannoma were diagnosed from 1990 to 1998. In the atypical and low-grade malignant groups, respectively, the average patient age was 54.3 years (range, 38-74 yr) and 50 years (range, 38-72 yr), and the average total tumor size was 1.53 cm (range, 0.7-3.5 cm) and 1.55 cm (range, 1.5-2 cm). Two recurrences were identified from the low-grade malignant group, and there was one postoperative House-Brackmann Grade III facial weakness. There were no recurrences or facial palsies in the atypical group. No distant metastasis or aggressive local invasion was observed in either group. MIB1 labeling indices were significantly (p < or = 0.001) higher in the atypical (4.69%) and low-grade malignant (5.23%) groups than in the control group (1.99%). CONCLUSIONS: These findings suggest a tendency for recurrence in proliferative tumors; however, the designation of malignancy should be reconsidered

The insertion of an intrascalar electrode array during cochlear implantation causes immediate damage to the inner ear and may result in delayed onset of additional damage that may interfere with neuronal stimulation. To date, there have been reports on fewer than 50 temporal bone specimens from patients who had undergone implantation during life. The majority of these were single-channel implants, whereas the majority of implants inserted today are multichannel systems. This report presents the histopathologic findings in temporal bones from 8 individuals who in life had undergone multichannel cochlear implantation, with particular attention to the type and location of trauma and to long-term changes within the cochlea. The effect of these changes on spiral ganglion cell counts and the correlation between speech comprehension and spiral ganglion cell counts were calculated. In 4 of the 8 cases, the opposite, unimplanted ear was available for comparison. In 3 of the 4 cases, there was no significant difference between the spiral ganglion cell counts on the implanted and unimplanted sides. In addition, in this series of 8 cases, there was an apparent negative correlation between residual spiral ganglion cell count and hearing performance during life as measured by single-syllable word recognition. This finding suggests that abnormalities in the central auditory pathways are at least as important as spiral ganglion cell loss in limiting the performance of implant users