Cochlear implants: a 30-year case study.
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Biomedical subjects
Publications and source records attributed to R A Schindler.
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BACKGROUND: The incidence of complications resulting from suppurative otitis media has significantly decreased since the introduction of antibiotics. At the start of the 20th century 50% of all cases of otitis media developed a coalescent mastoiditis. By 1959, the incidence had fallen to 0.4%. Recent studies suggest a current incidence of only 0.24%. Additionally, during the time of Friedrich Bezold (1824-1908), 20% of patients with mastoiditis developed subperiosteal abscess. Interestingly, this has incidence increased; today nearly 50% of patients diagnosed with coalescent mastoiditis have subperiosteal abscess. OBJECTIVE: To review the contemporary presentation, diagnosis, and management of a spectrum of mastoid abscesses. DESIGN: Retrospective case series. SETTING: Hospitals associated with the Department of Otolaryngology/Head and Neck Surgery at the University of California, San Francisco. PATIENTS: Three patients with mastoid abscesses are reported. One patient displayed "classic" Bezold's abscess, with pus escaping the mastoid near the incisura digastrica and tracking along the digastric and sternocleidomastoid muscles into the neck. The second and third patients exhibited temporoparietal swelling secondary to mastoid abscess eroding the root of the zygomatic process, a complication noted by Bezold in 1908 as occurring "in only very rare cases." RESULTS AND CONCLUSIONS: Since only one third of patients show pathologic tympanic membrane changes, and since complaints of otalgia, fever, and tenderness are inconstant, subperiosteal mastoid abscess is frequently a delayed diagnosis. The clinical presentation, pathogenesis, and routes of abscess spread are presented with photographic and radiographic illustration. Medical and surgical management is reviewed, and methods for accurate diagnosis are emphasized.
OBJECTIVE: This study aimed to review a single institution's experience with Clarion Multi-Strategy Cochlear Implant with respect to surgical technique, surgical complications, and rehabilitative outcome. STUDY DESIGN: This study was a review of patients implanted with the Clarion Multi-Strategy Cochlear Implant. SETTING: The setting was a tertiary referral center with care delivered in the inpatient and outpatient environment. PATIENTS: The first 37 patients were implanted under an Investigational Device Exemption as part of the Food and Drug Administration (FDA) clinical trial of the Clarion implant. Subsequent patients were implanted after the device received FDA approval. Patients met the following criteria for implantation: 18 years of age or older, psychological and emotional stability, profound postlingual deafness without evidence of middle ear disease, one cochlea at least partially patent, and marginal or no benefit from conventional hearing aids. INTERVENTION: Patients received implantation with the Clarion Multi-Strategy Cochlear Implant. MAIN OUTCOME MEASURES: Measured were presence or absence of surgical complications and auditory performance with open- and closed-set word and sentence recognition testing. RESULTS: A total of 47 patients have been implanted. Three patients have suffered complications: two cases of delayed-onset facial palsy both resolved with steroid therapy and one case of internal cochlear stimulator migration required refixation. Significant improvement in speech understanding has been seen in the majority of patients who were implanted within the first 6 months of device use. Specifically, at 6 months, scores on CID (Central Institute for the Deaf) sentences (implant alone) improved from a preoperative mean of 9% to a mean of 72%, and scores on the NU-6 (Northwestern University) monosyllabic word test increased from a preoperative mean of 3% (range, 0-20%) to a mean of 32% (range, 0-70%). More than two thirds (68%) of the adults were able to understand at least 50% of sentences over the telephone, and half were able to understand at least 75% of the sentence material. CONCLUSIONS: The authors' institutional experience with the Clarion Multi-Strategy Cochlear Implant shows minimal surgical morbidity and significant improvement on all open-set test measures of sentence and word recognition.
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The endolymphatic sac (ELS) has been shown to respond rapidly to sudden disruptions in fluid balance such as labyrinthectomy or systemic administration of hyperosmolar agents. The present study was designed to determine the ELS response to slower changes in fluid dynamics by occluding the endolymphatic duct (ELD), thereby interrupting the longitudinal flow of endolymph to the ELS. Morphologic studies and autoradiographic techniques were used to evaluate the effects of ELD obstruction on the structure and function of the ELS after 48 hours. There were no significant changes in cellular morphology and a slight decrease in the incorporation of radiolabeled glucose when compared with normal ELS cells. We conclude that it is rapid change in fluid balance that triggers the ELS response, which is not seen with disruption of longitudinal flow.
Light microscopic autoradiographs were prepared of isolated guinea pig endolymphatic sac (ES) tissue incubated with iodine (I)-125-labeled insulin (insulin I 125). These demonstrated specific binding of the insulin I 125 to receptors in the ES. Receptor-mediated endocytosis of the insulin-receptor complex is postulated to occur, and the relevance of insulin receptors in the ES is discussed, with particular emphasis on the previously observed synthesis of hyaluronan from glucose by the ES.
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Survival of auditory neurons in the cochlea is thought to be an important factor in the success of cochlear implantations. Damage to the cochlear end-organ often produces loss of dendrites in the osseous spiral lamina. The authors have established a method that delivers water soluble pharmacologic agents to the cochlea using a mini osmotic pump with a polyethylene cannula that is inserted into the scala tympani via cochleostomy. The cannula is filled with an ototoxic agent, neomycin, that destroys the end-organ over the first 24 hours after insertion. Nerve growth factor (NGF), or a control substance, is placed in the pump reservoir and is pumped into the cochlea over a 2-week period. In this study, auditory nerve fibers in the osseous spiral lamina were counted in the apical, middle, and basal turns of the cochlea in 16 guinea pigs. At each location, fiber counts were significantly higher when neomycin was followed by perfusion with NGF, than when it was followed by infusion with control substances. The study demonstrates that NGF confers some protection against ototoxic induced degeneration of the auditory nerve in vivo.
Intracochlear survival of auditory neurons is thought to be a factor contributing to the success of cochlear implantations. Damage to the cochlear end-organ is frequently associated with loss of neurites in the osseous spiral lamina. The authors have previously reported a model for long-term intracochlear administration of pharmacologic agents using a technique and device analogous to a cochlear implant. These studies suggest that nerve growth factor (NGF) protects the auditory nerve from the immediate neurotoxic effects of neomycin. The intent of the present investigation is to determine whether NGF can protect auditory neural elements from neomycin-induced degeneration after several weeks have elapsed between the neomycin perfusion and the administration of NGF. Neomycin was infused over 24 hours through an indwelling, intracochlear cannula attached to a mini osmotic pump to unilaterally destroy the cochlear end-organ. The pump perfused the cochlea with artificial perilymph for 2 weeks. Then, the pump reservoirs were surgically replaced; the new reservoirs delivered either NGF or artificial perilymph for an additional 2 weeks. Spiral ganglion cell densities were measured along Rosenthal's canal in the basal, middle, and apical cochlear turns. This investigation revealed that NGF prevented auditory nerve degeneration over the 2-week period, when compared to controls, and that the protective effect was greatest in the neural elements closest to the source of NGF.
A study was carried out to compare the consonant perceptions of five Nucleus multichannel cochlear implant users, five Clarion-CIS users, and four Clarion-CA users. All subjects were administered the Iowa Medical Consonant Recognition Test in the auditory-only condition. The groups averaged approximately 1 year of implant use at the time of testing, and the average age at implantation was 47 years for the Nucleus users, 60 years for the Clarion-CIS users, and 56 years for the Clarion-CA users. The percent-corrected scores for all three devices were variable and ranged from 34% to 84%, with each group averaging about 50% correct consonant recognition. Consonant feature information transmission was calculated for five features: manner of articulation, nasality, place of articulation, duration, and voicing. The highest information transmission in the Nucleus group was for the features of duration (71%), nasality (60%), and voicing (57%), which agrees with previous studies. The Clarion-CIS group had the highest information transmission for duration, place of articulation, manner of articulation, and nasality (50%, 29%, 28%, and 27%). The Clarion-CA group had the highest information transmission for the features voicing, place of articulation, and duration (41%, 40%, and 37%). This study indicates that the Clarion device may carry some acoustic information related to place of articulation, a feature that has not been found to be encoded well by other multichannel cochlear implants.
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A technique is described that provides a method to study the effects of intracochlear pharmacologic agents on the degenerating auditory nerve. The model involves implanting a guinea pig with an osmotic minipump that allows slow perfusion of the cochlea over a period of hours to days with up to two water soluble substances. Neomycin and horseradish peroxidase, whose histopathologic effects on the cochlea are well known, are used in the present study to validate the efficacy of the system.
Under phase I of an FDA-approved Investigational Device Exemption (IDE), six patients at the University of California, San Francisco (UCSF) have been implanted with the Clarion, a new multichannel, multiprogrammable cochlear implant system. Over a period of 5 years, the Clarion has been collaboratively developed by UCSF, the Research Triangle Institute (RTI) of North Carolina, and the device manufacturer and sponsor, MiniMed Technologies of Sylmar, California. For UCSF, it represents the culmination of 21 years of cochlear implant research. The Clarion incorporates the 16-contact electrode array developed at UCSF. This electrode is designed with a gentle curve and an elastic memory that allow it to hug the modiolar wall of the scala tympani. In addition, the electrode has mechanical features that prevent it from rotating upward and injuring the basilar membrane. One of the innovations necessary to allow broad clinical application of this device has been the development of an electrode insertion tool to assist the surgeon in safe placement. In each of the six UCSF cases, the insertion tool was used and electrode placement was achieved with ease. With the availability of a variety of speech processing strategies, the Clarion has also provided the opportunity for all of the patients tested to date to achieve some degree of open-set, auditory-only speech recognition. Surgical considerations and preliminary audiologic data demonstrate that the design rationale and objectives of the Clarion have been met.
The endolymphatic sac is believed to play a major role in membranous labyrinth homeostasis by controlling the volume of endolymph, removing debris, and participating in the immune response of the inner ear. The endolymphatic sac is postulated to absorb endolymph and to synthesize and secrete high-molecular-weight and osmotically active glycosaminoglycans (GAGs). The present study examines the ability of in vitro adult guinea pig endolymphatic sac cells to synthesize complex proteins and polysaccharides. The intent is to characterize the nature of these compounds by studying carbon-14 (14C) glucose incorporation in tissue cultured endolymphatic sac specimens using autoradiographic and specific enzymatic digestion techniques. Our results suggest that sac cells can synthesize GAGs and proteins in vitro in proportionately larger amounts than surrounding connective tissue and dura. The principal GAG synthesized by the endolymphatic sac appears to be hyaluronan.
A new cochlear implant has become available. This device, called the Clarion Multichannel Cochlear Implant, is the result of collaborative efforts between the University of California, San Francisco (UCSF), the Research Triangle Institute (RTI), Research Triangle Park, North Carolina, and the device manufacturer and sponsor, MiniMed Technologies of Sylmar, California. The Clarion represents a new generation of multi-programmable cochlear implant systems. Options in waveform, the stimulation mode, and the temporal distribution of the signal permit the device to be optimally customized for each individual patient. The speech coding scheme offering the greatest opportunity for speech recognition can be selected. The first Clarion recipient has recently been implanted at UCSF, initiating the Clarion's investigational clinical trials. A description of the device and preliminary patient results are presented.
The endolymphatic sac (ELS) has been the subject of investigation for many years and yet its overall function remains unclear. It is believed mainly to be involved in the regulation of endolymph through fluid resorption and secretion of osmotically active substances. The present study was performed using in vitro cultured, fetal ELSs of 18 to 19 day gestational mice, to assess whether the ELS cells can synthesize the osmotically active polysaccharide, hyaluronan (HA). The ELS and portions of the membranous labyrinth were dissected from whole otocyst specimens and placed in 14C glucose-enhanced tissue culture media. A light microscopic (LM), autoradiographic study was performed to assess whether 14C glucose could be incorporated by the tissue into HA. Both the ELS cells and the adjacent cartilage demonstrated radiolabel incorporation within 4 hours of incubation in tissue culture medium, with increased radiolabel density in ELS cells after 24 hours of incubation. HA-specific hyaluronidase (HAase) resulted in removal of HAase-sensitive compounds in the ELS in both 4-hour and 24-hour cultured specimens when compared to adjacent cartilage cells (p = 0.001). Approximately 43 percent of the radiolabel was incorporated into HA in ELS specimens, as compared to a 22 percent HA synthesis in the adjacent cartilage tissue, suggesting preferential synthesis by ELS cells. The dissected murine otocysts demonstrate viability in vitro as measured by their ability to incorporate 14C glucose from tissue culture medium. Under these conditions the cultured ELS demonstrates an ability to synthesize HA. A theory of ELS function is proposed.
The endolymphatic sac (ELS) has been implicated in the maintenance of endolymph volume and pressure in the membranous labyrinth through fluid resorption and secretion of osmotically active substances, known as glycosaminoglycans (GAGs). To assess whether the ELS cells synthesize the GAG, hyaluronan (HA), and to further elucidate the secretory function of the ELS, a series of experiments were carried out on in vitro tissue-cultured, fetal murine ELSs. In phase 1 of the investigation, the ELSs that were attached to a small portion of the posterior labyrinth, were resected from whole otocyst specimens and studied in tissue culture. This model was chosen to determine whether a change in endolymph homeostasis affects ELS activity. Radiolabeled 14C glucose incorporation was used to evaluate HA synthesis by ELS cells when cultured in vitro. Approximately 43 percent of the incorporated 14C glucose radiolabel was digested by Streptomyces hyaluronidase (an HA-specific hyaluronidase), suggesting HA synthesis by sac cells. In phase 2 of our experiments, the ELSs were not resected from the otocysts. Instead, they were left attached to intact membranous labyrinths, and whole otocysts were cultured. Studies analogous to those of phase 1, assessing the ability of the ELS cells to incorporate 14C glucose into HA, were performed on these specimens. Streptomyces hyaluronidase treatment of these ELS specimens resulted in a reduction in the removal of radiolabel. Therefore, the ELS cells of intact otocysts incorporated less 14C glucose into HA when compared to the ELS cells of the resected specimens.(ABSTRACT TRUNCATED AT 250 WORDS)