Cochlear implantation in elderly patients: stability of outcome over time.

BACKGROUND: Cochlear implantation is the standard of care for treating severe to profound hearing loss in all age groups. There is limited data on long-term results in elderly implantees and the effect of ageing on outcomes. This study compared the stability of cochlear implantation outcome in elderly and younger patients. METHODS: A retrospective chart review of cochlear implant patients with a minimum follow up of five years was conducted. RESULTS: The study included 87 patients with a mean follow up of 6.8 years. Of these, 22 patients were older than 70 years at the time of implantation. Hearing in Noise Test scores at one year after implantation were worse in the elderly: 85.3 (aged under 61 years), 80.5 (61-70 years) and 73.6 (aged over 70 years; p = 0.039). The respective scores at the last follow up were 84.8, 85.1 and 76.5 (p = 0.054). Most patients had a stable outcome during follow up. Of the elderly patients, 13.6 per cent improved and none had a reduction in score of more than 20 per cent. Similar to younger patients, elderly patients had improved Short Form 36 Health Survey scores during follow up. CONCLUSION: Cochlear implantation improves both audiometric outcome and quality of life in elderly patients. These benefits are stable over time.

CT grading of otosclerosis.

BACKGROUND AND PURPOSE: The CT grading system for otosclerosis was proposed by Symons and Fanning in 2005. The purpose of this study was to determine if this CT grading system has high interobserver and intraobserver agreement. MATERIALS AND METHODS: All 997 petrous bone CTs performed between December 2000 and September 2007 were reviewed. A total of 81 subjects had CT evidence of otosclerosis on at least 1 side; 68 (84%) had bilateral disease. Because otosclerosis was clinically suspected in both ears of all 81 subjects even if CT evidence was only unilateral, both petrous bones (162 in total) were included. Two blinded neuroradiologists independently graded disease severity using the Symons/Fanning grading system: grade 1, solely fenestral; grade 2, patchy localized cochlear disease (with or without fenestral involvement) to either the basal cochlear turn (grade 2A), or the middle/apical turns (grade 2B), or both the basal turn and the middle/apical turns (grade 2C); and grade 3, diffuse confluent cochlear involvement (with or without fenestral involvement). One reviewer repeat-graded the petrous bone CTs to determine intraobserver agreement with a 7-month intervening delay to mitigate recall bias. RESULTS: There were 154 agreements (95%) comparing the first grading of reviewer 1 with that of reviewer 2 (kappa = 0.93). When the repeat 7-month delayed grading of reviewer 1 was compared with that of reviewer 2, there were 151 (93%) agreements (kappa = 0.90). Therefore, mean interobserver agreement was excellent (mean kappa = 0.92). There were 155 agreements (96%) comparing the original grading of reviewer 1 with the delayed grading (kappa = 0.94), demonstrating excellent intraobserver agreement. CONCLUSIONS: A recently published CT grading for otosclerosis on the basis of location of involvement yielded excellent interobserver and intraobserver agreement.

Cochlear implantation in cochlear otosclerosis.

OBJECTIVES/HYPOTHESIS: The objective was to correlate implant performance in cochlear otosclerosis to 1) matched control samples, 2) severity of otic capsule involvement, 3) prior ipsilateral surgery, and 4) programming issues. STUDY DESIGN: Retrospective case controlled study. METHODS: Study cohort comprised 30 individuals. Diagnosis was based on prior ear surgery (stapedectomy [n = 18] or fenestration [n = 2]) and/or pathognomonic radiological findings. High-resolution computed tomography images of the temporal bones were assessed by two radiologists and graded (range, 0-3) for the extent of otosclerosis. Operative records were reviewed. Performance, programming visits, and the number of electrode deactivations at 6 months and at 1 year after implantation were determined for the individuals with otosclerosis and compared with a group of matched control subjects. A within-group comparison correlating severity of otosclerosis to the above was carried out. RESULTS: Implant performance in individuals with cochlear otosclerosis was not significantly different from those without. Previous surgery on the side of implantation did not alter performance. Programming difficulty as reflected in the number of visits and electrode deactivation for sound quality reasons were comparable. Deactivation for facial nerve stimulation occurred exclusively in otosclerotics with the most severe radiological disease (grade 3) and was only with non-modiolar hugging electrodes (n = 5). There was no observed difference between the radiological extent of otosclerosis and implant performance. CONCLUSION: Individuals with severe otosclerosis considering cochlear implantation can be counseled to expect similar benefit to those without, regardless of whether prior surgery occurred on the side of implantation or of severity of otic capsule involvement. There is a significant risk of facial nerve stimulation in otosclerotics with grade 3 disease.

Does choosing the "worse" ear for cochlear implantation affect outcome?

OBJECTIVE: To determine whether choosing the "better" ear or the "worse" ear for cochlear implantation impacts performance outcome. DESIGN: Retrospective cohort study. SETTING: University teaching hospital-cochlear implant program. METHODS: Two groups of cochlear implantees were selected and matched based on clinical parameters, including duration of deafness/age at implantation, implant types, and processing strategies. Nineteen patients received an implant in his or her "better" ear of the two that had been amplified. An equal number of patients received an implant in the "worse" ear--an ear that was not amplified or was chosen to avoid causing oscillopsia; or if the patient was not willing to relinquish his or her hearing aid in the "better" ear based on subjective or objective criteria. Standard speech perception testing was performed. RESULTS: The average open-set speech perception responses at 1 year after implantation were as follows: word recognition score 40.4% and sentence recognition score 81% in the aided subjects (better ears); word recognition score 41.5% and sentence recognition score 84.5% in the unaided group (worse ears). CONCLUSION: No differences were found between the two groups of implantees. Choosing the "worse" ear for implantation did not appear to have a negative impact on performance outcome in this match-paired study.

Cerebrospinal fluid leak after acoustic neuroma surgery: influence of tumor size and surgical approach on incidence and response to treatment.

OBJECT: The aims of this study were to review the incidence of cerebrospinal fluid (CSF) leakage complicating the removal of acoustic neuroma and to identify factors that influence its occurrence and treatment. METHODS: Prospective information on consecutive patients who underwent operation for acoustic neuroma was supplemented by a retrospective review of the medical records in which patients with CSF leaks complicating tumor removal were identified. This paper represents a continuation of a previously published series and thus compiles the authors' continuous experience over the last 24 years of practice. In 624 cases of acoustic neuroma the authors observed an overall incidence of 10.7% for CSF leak. The rate of leakage was significantly lower in the last 9 years compared with the first 15, most likely because of the abandonment of the combined translabyrinthine (TL)-middle fossa exposure. There was no difference in the leakage rate between TL and retrosigmoid (RS) approaches, although there were differences in the site of the leak (wound leaks occurred more frequently after a TL and otorrhea after an RS approach, respectively). Tumor size (maximum extracanalicular diameter) had a significant effect on the leakage rate overall and for RS but not for TL procedures. The majority of leaks ceased with nonsurgical treatments (18% with expectant management and 49% with lumbar CSF drainage). However, TL leaks (especially rhinorrhea) required surgical repair significantly more often than RS leaks. This has not been reported previously. CONCLUSIONS: The rate of CSF leakage after TL and RS procedures has remained stable. Factors influencing its occurrence include tumor size but not surgical approach. The TL-related leaks had a significantly higher surgical repair rate than RS-related leaks, an additional factor to consider when choosing an approach. The problem of CSF leakage becomes increasingly important as nonsurgical treatments for acoustic neuroma are developed.

Reliability of the Sunnybrook Facial Grading System by novice users.

OBJECTIVES: To evaluate the intrarater and inter-rater reliability of the Sunnybrook Facial Grading System (SFGS) by novice users. DESIGN: Prospective interval study using one measurement instrument. SETTING: Tertiary teaching hospital (Sunnybrook & Women's College Health Science Centre). METHODS: Twenty-two patients with a wide spectrum of facial dysfunction recorded on videotapes were rated using the SFGS by eight novice observers independently in two different sittings separated by 3 weeks. The order of patients was randomized for the second sitting. Intraclass correlation coefficients were calculated for component scores and for total scores within and between raters. RESULTS: The intrarater reliability coefficients for the eight raters ranged from .838 to .929. This largely overlaps with the data obtained in previous studies with expert raters. The inter-rater reliability for all eight raters at time 1 was .982 and for time 2 was .970. This is higher than what was previously obtained with expert raters. CONCLUSION: The SFGS is as reliable when applied by novice users as by expert users.

Surgical outcome after paediatric cochlear implantation: diminution of complications with the evolution of new surgical techniques.

OBJECTIVE: Cochlear implantation is a commonly performed surgical procedure with specific aspects in the paediatric population. The surgical outcome associated with this procedure in a paediatric population is analyzed. METHOD: A retrospective study was performed of all children receiving a cochlear implant at The Hospital for Sick Children from 1990 to 1998. During this period, 104 patients received a cochlear implant. RESULTS: The complications encountered were classified as major (4%), minor (3%), and device failure (2%). The surgical techniques developed to decrease complications are discussed. CONCLUSION: Paediatric cochlear implantation is a safe procedure with a low incidence of complications. Nevertheless, the surgeon is now facing new challenges with cochlear implantation performed in patients with an abnormal cochlea, who carry a higher rate of complications, and cochlear reimplantation in case of device failure.

Intratympanic gentamicin for the treatment of unilateral Meniere's disease.

OBJECTIVE: To determine the efficacy of intratympanic gentamicin instillation as treatment of incapacitating unilateral Meniere's disease, using a predetermined regimen with a fixed dose. STUDY DESIGN: A prospective study from a single institution between 1988 and 1998. METHODS: One hundred fourteen patients were enrolled in this study. Gentamicin (26.7 mg/mL) was administered three times daily for 4 consecutive days. The Committee on Hearing and Equilibrium Guidelines for Reporting Treatment Results in Meniere's Disease of the American Academy of Otolaryngology and Head and Neck Surgery (1985) were used. RESULTS: Comprehensive data were available for 90 individuals. Complete control of vertigo was achieved in 76 (84.4%), substantial control in 8 (9.0%), limited control in 2 (2.2%), and insignificant control in 4 (4.4%) patients. Disability scores at the end of 2 years were as follows: 76 patients (84.4%) had no disability, 5 (5.6%) had mild disability, 2 (22%) had moderate disability, and 7(7.8%) had severe disability. Caloric testing responses, as determined using electronystagmography, were as follows: 71% of the patients had an absent ice-water response, 16% had a positive ice-water response, and in 13% there continued to be present a bithermal response. Hearing was worse in 22 patients (25.6%), unchanged in 41 (48.2%), and improved in 22 (25.6%). CONCLUSIONS: Intratympanic gentamicin administration using this particular protocol is an effective treatment option for patients with disabling unilateral Meniere's disease. Hearing loss is a distinct possibility, and patients should be advised accordingly.

Electrocochleography and gentamicin therapy for Ménière's disease: a preliminary report.

OBJECTIVE: It is widely held that an enlarged summating potential (SP) relative to the eighth nerve action potential (AP) is a reflection of endolymphatic hydrops. Aminoglycosides are an accepted treatment for incapacitating Ménière's disease and are known to affect both sensory and secretory cells of the inner ear. The intent of this study was to determine whether this effect on secretory cells could be objectively confirmed by virtue of changes in the electrocochleogram (ECoG) of patients receiving gentamicin therapy for Ménière's disease. STUDY DESIGN: This was a prospective longitudinal study of repeated ECoG measures in three groups of subjects. Ménière's patients undergoing gentamicin treatment were compared with two control groups: individuals with stable Ménière's disease and normal-hearing control subjects. SETTING: The study was conducted at a tertiary referral center. PATIENTS: The sample included 21 normal-hearing subjects, 15 patients with stable unilateral Ménière's disease, and 12 with disabling unilateral Ménière's disease. INTERVENTIONS: For patients with disabling Ménière's disease, gentamicin was administered transtympanically. Audiograms, impedance tests, and ECoG were performed twice for all subjects. MAIN OUTCOME MEASURES: The SP and AP amplitudes, AP latency, and SP/AP ratio of the EcoG were measured. RESULTS: A statistically significant reduction in the SP/AP ratio was observed after gentamicin administration (analysis of variance interaction effect: F2 = 5.64; p = 0.0065). CONCLUSIONS: The significant reduction in the SP/AP ratio in the gentamicin-treated Ménière's group supports the hypothesis that gentamicin improves the electrophysiologic function of the cochlea, possibly by reducing the severity of the associated endolymphatic hydrops.

Does intratympanic gentamicin treatment for Meniere's disease cause complete vestibular ablation?

OBJECTIVE: To determine changes in vestibular function following intratympanic gentamicin (ITG) treatment for Meniere's disease and to correlate changes with the need for further treatment. STUDY DESIGN: Prospective case series. PATIENTS: One hundred and three patients with disabling unilateral Meniere's disease who had failed a minimum of 6 months medical treatment. MAIN OUTCOME MEASURES: Vestibular function was measured by electronystagmography (ENG) caloric testing before and serially following treatment. Caloric responses were classified as normal (excitability difference [ED] < 25%), bithermal response (ED > or = 25%), positive response to ice water only, and absent ice water response. RESULTS: Twenty-one percent of patients had a bithermal caloric response, 62% had an absent ice water response, and 17% had an ice water response only 1 month following treatment. Mean follow-up was 27.3 months (range = 1-106 months). Eighty-four patients had one treatment course only. Fourteen patients required a further course of treatment due to recurrence of vertigo; 38% of these had recovery of caloric function. Significantly more patients with normal caloric function prior to initial treatment required further treatment compared to those with initial reduced caloric function (p < .05). Patients rendered absent ice water responsive were significantly less likely to require further treatment than those with a persistent caloric response (p < .0001). CONCLUSION: An absent ice water response is highly predictive of adequate vertigo control. Regimens of ITG that aim to completely ablate vestibular function are recommended.

[Surgical techniques and complications of pediatric cochlear implantation]. / Technique chirurgicale et complications de l'implantation cochléaire pédiatrique.

INTRODUCTION: Cochlear implantation has been performed in children since 1980, with thousands of children already implanted. The incidence of complications is around 7 to 10% in the literature. METHODS: This is a retrospective study of all children receiving a cochlear implant at the Hospital for Sick Children over 9 years. Surgical technique and outcome are analysed. RESULTS: There were 7 complications, all successfully treated, and involving no further consequences. 4 reimplantations were performed for device failure. DISCUSSION: Paediatric cochlear implantation is associated with a low incidence of complications. In this series, no complications were encountered on reimplantation.

Facial nerve grading systems.

Clinicians require a reliable and sensitive facial grading system to determine the status of facial nerve function postinjury as well as to evaluate the efficacy of treatment outcomes. The method of assessment should be easy to administer, reproducible, clinically relevant, and require little time and expense. The grading system should be sufficiently sensitive to detect changes over time or following treatment. The lack of a universally accepted grading system frequently does not allow meaningful comparison when assessing the effectiveness of treatment protocols for facial nerve injury. The aim of this article is to review most of the previously published facial grading systems. It is our contention that a grading system developed and currently used within our institution appropriately addresses the needs as well as many of the deficiencies of most other grading systems.

Depth and quality of electrode insertion: a radiologic and pitch scaling assessment of two cochlear implant systems.

OBJECTIVE: To compare the depth of electrode insertion in two types of cochlear implants, and to assess the ability of the implantees in each group to place-pitch during random electrical stimulation. STUDY DESIGN: This was a prospective clinical study. SETTING: This study was performed at an implant program within a university teaching hospital. PATIENTS: Five consecutive patients with the Clarion (Advanced Bionics, Symlar, CA, U.S.A.) device and 5 with the Nucleus-22 (Cochlear Corporation, Sydney, Australia) implants were enrolled. All 10 implantees had fully active and functioning electrodes. INTERVENTIONS: The depth of insertion was determined using plain anteroposterior skull film and high resolution computed tomography (CT). The quality of electrode insertion was assessed by pitch scaling; electrodes were randomly stimulated to generate subjective pitch responses. OUTCOME MEASURES: The depth of electrode insertion was measured radiographically as degrees of angular rotation within the cochlea. For pitch scaling, the averaged responses to electrical stimulation was plotted against the "place" of the electrodes along the array. Pitch range, plateauing, and reversal of pitches were also noted. Insertion depth was correlated with the result of pitch scaling and open-set speech discrimination at 3 months. RESULTS: The mean insertion depth was 406 degrees for the Clarion device and 254 degrees for the Nucleus device. CT was used to confirm the intracochlear placement of the electrodes and their relationships to the cochleostomy site. It did not confer more information than the plain films unless kinking had occurred. Pitch perception was consistent with the tonotopic organization of the cochlea. The Nucleus-22 recipients displayed a broader range of pitches with less plateaus and reversals than the Clarion implants. The depth of insertion did not compare well with the outcome of pitch scaling or with open-set speech discrimination scores in either group of implantees. CONCLUSION: The preformed spiral array of the Clarion device allowed deeper electrode insertion compared to the Nucleus-22 device. However, depth of insertion did not translate into better pitch placement.

A study of vestibular schwannomas using positron emission tomography and monoclonal antibody Ki-67.

OBJECTIVE: This study aimed to evaluate the role of positron emission tomography (PET) as an in vivo determinant of tumor aggressiveness and growth. STUDY DESIGN: The study design was a prospective pilot study. SETTING: Positron emission tomography was performed at the Clarke Institute of Psychiatry. All patients were treated at the Sunnybrook Health Science Centre. Both institutions are affiliated with the University of Toronto, Toronto, Canada. PATIENTS: The study consisted of five consecutive patients with vestibular schwannomas with tumor size of 1 cm or larger within the cerebellopontine angle. One was a recurrent tumor and four were primary tumors. INTERVENTIONS: Preoperative PET studies were conducted using 18-fluorodeoxyglucose (FDG) as a radionuclide tracer to measure glucose metabolism within tumors. Tumors were processed and immunostained against Ki-67 nuclear antigen; their proliferative potentials were quantified based on immunoreactivity of tumor cells. MAIN OUTCOME MEASURES: Tumor metabolic activity on PET was compared with that of contralateral cerebellum to arrive at an FDG index. This number was compared with clinical parameters and Ki-67 reactivity. RESULTS: On PET, all tumors showed less metabolic activity than the cerebellum. The FDG uptake varied greatly between tumors independent of clinical parameters. All the tumors had a low proliferative index (<5%) with immunohistochemistry; there were quite a bit of intralesional variations in proliferative activities. CONCLUSION: Large tumor size and recurrent disease did not correlate well with increased FDG uptake on PET. Similarly, they did not show increased cellular activities as expressed by Ki-67 immunostaining.

Surgical outcomes of paediatric cochlear implantation: the Hospital for Sick Children's experience.

OBJECTIVE: Cochlear implantation in children represents a special challenge for the otologist because of such factors as skull dimensions and postimplantation temporal bone growth. As implant programs accrue increasing numbers of children, surgical factors relating to outcomes can be analyzed. METHOD: A retrospective study was performed of all children receiving a cochlear implant from January 1990 through May 1997 at The Hospital for Sick Children (HSC). The surgical procedure as well as the complications encountered were examined. Fifty two children, 28 females (54%) and 24 males (46%) were implanted. RESULTS: Overall, the average age at time of implantation was 7.0 years (range, 1.9-17.3 yr). The presumed cause of deafness was acquired in five children (10%) and congenital in the remaining 47 patients (90%). A recognized syndrome was associated with the hearing loss in only three patients. There were two minor complications (3.8%) and two major complications (3.8%) with an overall complication rate of 7.6%. There were no episodes of device or electrode migration. Two surgical innovations are also discussed: 1) a new periosteal flap design which decreases device mobility, and 2) a new technique for electrode fixation (NED's knot). CONCLUSION: The HSC experience represents the largest paediatric cochlear implant series in Canada. The findings indicate that the procedure is safe and is associated with infrequent morbidity.

Vestibulo-ocular reflex deficits to rapid head turns following intratympanic gentamicin instillation.

The response of the vestibulo-ocular reflex following unilateral vestibular deafferentation by gentamicin ablation was studied using transient stimuli. The response to these rapid passive head turns showed a strong asymmetry with permanent, reduced gains toward the side of lesion. These gain reductions have large variation (gains of 0.26 to 0.83), which may result from preferential sparing of regularly firing afferent fibers following gentamicin ablation. Based on the size and nature of the nonlinearity, an explanation based on Ewald's second law was discounted.

The Paediatric Cochlear Implant Program at the Hospital for Sick Children, Toronto.

Ontario has a province-wide program for provision of cochlear implants. Toronto's Hospital for Sick Children is one of three designated centres that service the paediatric population. This cochlear implant program was established in 1989. Since that time, 37 children (as of May 1996) have been provided with cochlear implants. The program also services Ontario residents who were implanted elsewhere. In the following, we provide a detailed description of the program, including the processes through which children are selected as candidates, the follow-up studies that we carry out, and the roles of various health care professionals involved. We present a demographic survey of our patient population to date, and discuss some of the important issues relating to candidacy.

Hearing preservation in the removal of intracanalicular acoustic neuromas via the retrosigmoid approach.

In a series of 514 consecutive operations for complete excision of acoustic neuromas, 94 procedures were performed via a retrosigmoid approach to preserve the patient's hearing. Twenty-six of these procedures (5.1%) were performed in cases of intracanalicular tumor and 68 (13.2%) were for larger lesions in which most of the tumor was located medial to the porus acusticus within the cerebellopontine angle. Preservation of useful hearing was achieved in 13 (50%) of 26 patients with intracanalicular tumors and in 20 (29%) of 68 with larger tumors. A trend toward higher success rates in intracanalicular tumors appears to be present, although the difference is not statistically significant (p = 0.09). Normal or near normal facial function (House and Brackmann Grades I and II) was present postoperatively in 25 (96%) of 26 patients. Indications for treatment of intracanalicular acoustic neuromas are considered and treatment alternatives are reviewed. Results from other series reporting removal of intracanalicular acoustic neuromas are considered with respect to hearing conservation and postoperative facial nerve function. Surgical excision of intracanalicular acoustic neuromas in otherwise healthy patients appears to be warranted if preservation of useful binaural hearing is considered a worthwhile objective and if perioperative morbidity can be maintained at an acceptably low level. The retrosigmoid approach is familiar to all neurosurgeons and offers a comparable success rate for hearing conservation and probably a superior outcome in terms of facial nerve function when compared with the middle fossa approach.