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Biomedical subjects

John K Niparko

Publications and source records attributed to John K Niparko.

14 recordsLinked to original sources
PubMed2003

Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial.

CONTEXT: A number of therapies have been proposed for the long-term management of corticosteroid-responsive, rapidly progressive, bilateral sensorineural hearing loss (autoimmune inner ear disease [AIED]). Methotrexate has emerged as the benchmark agent but has not been rigorously evaluated for hearing improvement in patients with AIED. OBJECTIVE: To assess the efficacy of long-term methotrexate in maintaining hearing improvements achieved with glucocorticoid (prednisone) therapy in patients with AIED. DESIGN, SETTING, AND PARTICIPANTS: A randomized, double-blind, placebo-controlled trial conducted from February 3, 1998, to November 5, 2001, of 67 patients with rapidly progressive, bilateral sensorineural hearing loss at 10 tertiary care centers in the United States. INTERVENTION: Randomization to either oral methotrexate (15 to 20 mg/wk; n = 33) or placebo (n = 34), in combination with an 18-week prednisone taper. Follow-up examinations, including audiometric evaluation, were performed at 4, 8, 12, 24, 36, 48, and 52 weeks, or until hearing loss was documented. MAIN OUTCOME MEASURE: Maintenance of hearing improvement achieved from prednisone treatment. RESULTS: Sixty-seven patients (57.8%) enrolled in the prednisone challenge experienced hearing improvement. Twenty-five patients (37%) experienced hearing improvements in both ears. Of the individuals who reached study end points, 24 (80%) of 30 end points were because of measured hearing loss in the methotrexate group and 29 (93.5%) of 31 end points were because of measured hearing loss in the placebo group (P =.15). Methotrexate was no more effective than placebo in maintaining the hearing improvement achieved with prednisone treatment (hazard ratio, 1.31; 95% confidence interval, 0.79-2.17; P =.30). CONCLUSION: Methotrexate does not appear to be effective in maintaining the hearing improvement achieved with prednisone therapy in patients with AIED.

Audiometry↗
PubMed2003

Developmental, audiological, and speech perception functioning in children after cochlear implant surgery.

OBJECTIVES: To examine changes in audiological, speech perception, and developmental functioning subsequent to cochlear implantation in children with severe to profound hearing impairment, and to identify factors related to those changes. DESIGN: Prospective, longitudinal analysis to compare functioning of pediatric patients who underwent cochlear implantation before and 1 year after surgery. SETTING: Outpatient pediatric cochlear implantation program in an academic institution (The Listening Center, The Johns Hopkins University School of Medicine, Baltimore, Md). Patients Forty consecutive pediatric patients between 1(1/2) and 9 years of age who received a cochlear implant between April 1, 1996, and August 31, 1998, and who also underwent psychological, audiological, and speech perception evaluations immediately before and 1 year after implantation. MAIN OUTCOME MEASURE: Scores on the Bayley Scales of Infant Development-Second Edition, Stanford-Binet Intelligence Scale-Fourth Edition, Developmental Profile II, Child Behavior Checklist, speech perception categories, and audiological pure-tone thresholds. RESULTS: Mean (SD) duration of hearing impairment was 37.78 (27.94) months, mean (SD) age at surgery was 50.72 (27.66) months. Significant improvements were found 1 year after surgery in audiological, speech perception, and developmental functioning, but not in nonverbal intelligence or behavior. Greater benefits in audiological and developmental functioning were associated with younger age (<48 months) at implantation. CONCLUSIONS: Patients showed significant improvement in audiological status, overall developmental functioning, and speech perception skills in a short time after surgery. Greatest improvement in speech perception was for children with the least initial impairment, and greatest developmental gains were associated with young age at implantation.

Age Factors↗
PubMed2003

Cochlear implants in young children.

The cochlear implant is best characterized as a device that provides access to the sound environment. The device enables the hearing pathway to respond to environmental and speech sounds, providing informational cues from the surroundings and from others that may escape visual detection. As the developmental effects of a profound hearing loss are multiple, cochlear implants have been applied to ever younger children in an attempt to promote a more normal level of developmental learning through audition. In deafness, transducer elements of the inner ear fail to trigger auditory nerve afferent nerves in the presence of sound input. However, large reserves of afferent fibers exist even in the auditory nerve of a profoundly deaf patient. Furthermore, these nerve fibers retain the ability to respond to prosthetic activation. Through developmental learning in the early, formative years, auditory centers of the brain appear capable of processing information from the implant to provide speech comprehension and oral language development. Multichannel implants have replaced original single channel designs. multichannel devices enable larger percentages of recipients to recognize the spoken word without visual cues because they provide spectral information in addition to temporal and intensity cues. Testing under conditions of auditory (implant)-only input reveals significant open-set speech understanding capabilities in more than 75% of children after three years of device use. The benefit provided by implants may vary with a number of conditions including: hearing history, age of deafness onset, age at implantation, etiology of deafness, linguistic abilities, and the presence of a motivated system of support of oral language development. Patient variables should be given individual consideration in judging candidacy for a cochlear implant and in planning rehabilitative and education services after surgery and activation of the device.

Child↗
PubMed2003

Cochlear implantation update.

Cochlear implantation is recognized as a valuable intervention with important implications for the acquisition of speech perception and verbal language in children with severe to profound hearing impairment. Auditory rehabilitation, language intervention, and close coordination between parents, schools, and the implant center are necessary to maximize efficacy. Early identification of hearing loss, early hearing aid use and language intervention, and cochlear implantation by 2 years of age are positive predictors for language acquisition that can approach the levels of normal-hearing children. There are early indications that increased access to mainstream education and gains in quality of life are long-term benefits that render cochlear implantation a cost-effective intervention.

Auditory Pathways↗
PubMed2003

Transcranial contralateral cochlear stimulation in unilateral deafness.

OBJECTIVES: The purpose of this study is to evaluate the effectiveness of Bone Anchored Cochlear Stimulator (BAHA) in transcranial routing of signal by implanting the deaf ear. Study design and settings Eighteen patients with unilateral deafness were included in a multisite study. They had a 1-month pre-implantation trial with a contralateral routing of signal (CROS) hearing aid. Their performance with BAHA was compared with the CROS device using speech reception thresholds, speech recognition performance in noise, and the Abbreviated Profile Hearing Benefit and Single Sided Deafness questionnaires. RESULTS: Patients reported a significant improvement in speech intelligibility in noise and greater benefit from BAHA compared with CROS hearing aids. Patients were satisfied with the device and its impact on their quality of life. No major complications were reported. Conclusion and significance BAHA is effective in unilateral deafness. Auditory stimuli from the deaf side can be transmitted to the good ear, avoiding the limitations inherent in CROS amplification.

Acoustic Stimulation↗
PubMed2003

Task performance in stapedotomy: comparison between surgeons of different experience levels.

OBJECTIVES: Two steps in stapedotomy are particularly challenging: (1) micropick fenestration of the stapes footplate (SF) and (2) crimping of the stapes prosthesis (SP) to the incus. We conducted trials to determine if experience correlates with differences in performance for these tasks. METHODS: In a surgical model of stapedotomy, performance was measured for 3 experienced and 3 novice surgeons. For fenestration, we measured ability to target the fenestration and force applied to the SF. For crimping, we measured crimp quality, movement of the SP during crimping, and force applied to the SF. RESULTS: Experienced surgeons demonstrated significantly better ability to target the fenestration and, during crimping, caused less SP movement and a significantly lower rate of SP dislodgment. CONCLUSIONS: Clear differences in task performance are measurable between more and less experienced surgeons during critical steps of stapedotomy. CLINICAL SIGNIFICANCE: The observed differences in task performance may contribute to an understanding of maneuvers that increase the risk of inadequate prosthesis placement and cochlear trauma-factors likely responsible for variable hearing results with strapedotomy.

Clinical Competence↗
PubMed2003

Comparison of the bone anchored hearing aid implantable hearing device with contralateral routing of offside signal amplification in the rehabilitation of unilateral deafness.

OBJECTIVE: Monaural hearing imposes constraints under many listening conditions. The authors compared the effects of a semi-implantable bone conductor, the Entific bone anchored hearing aid, with conventional contralateral routing of offside signal amplification to assess rehabilitative benefit in adults with unilateral deafness. DESIGN: Prospective trials of subjects with unilateral deafness using benefit surveys, source identification testing, and hearing in noise testing. SETTING: Tertiary referral center, outpatient surgical and audiologic services. PATIENTS: Adults with unilateral deafness (pure tone average >90 dB, SD <20%) after acoustic neuroma excision (n = 7), meningitis (n = 1), sudden sensorineural hearing loss (n = 1), and sudden sensorineural hearing loss with chronic suppurative otitis media (n = 1). Entry criteria included normal hearing in the contralateral ear (pure tone average <25 dB, SD >80%). INTERVENTION: Subjects were fitted with contralateral routing of offside signal amplification devices for 1 month and tested with contralateral routing of offside signal before mastoid implantation of the deaf ear, fitting, and testing for bone anchored hearing aid. OUTCOME MEASURES: Subjects' assessment of experience with their devices and patterns of use, 2) source azimuth identification in noise test, and 3) speech discrimination in quiet and in noise under conditions of noise-front, noise-to-normal-ear, and noise-to-deaf-ear. RESULTS: There was consistent satisfaction with bone anchored hearing aid implantation and amplification, and poor acceptance of contralateral routing of offside signal amplification. Sound localization was poor at baseline and with both bone anchored hearing aid and contralateral routing of offside signal. Relative to baseline, contralateral routing of offside signal and bone anchored hearing aid produced significantly better speech recognition in noise under most conditions. The bone anchored hearing aid enabled significantly better speech recognition than contralateral routing of offside signal in quiet and in a composite of noise conditions. The advantages may relate to averting the interference of speech signals delivered to the better ear, as occurs with conventional contralateral routing of offside signal amplification. CONCLUSIONS: Preliminary data in subjects with normal monaural hearing indicate that vibromechanical stimulation with the bone anchored hearing aid overcomes some of the negative head shadow effects in unilateral deafness. The bone anchored hearing aid system, when placed on the side of a deaf ear, yields greater benefit in subjects with normal monaural hearing than does contralateral routing of offside signal amplification. It seems that this rehabilitative approach can expand the sound field of monaural listeners in further enhancing speech understanding. Observations suggest that further understanding of bone conduction as implemented in transcranial stimulation will guide further options for patients with monaural hearing. Longer follow-up will help to determine whether communicative skill improvements with the bone anchored hearing aid outweigh the disadvantages of implantation surgery, costs, and device maintenance.

Adult↗
PubMed2003

Cochlear implantation in patients with bilateral Ménière's syndrome.

OBJECTIVE: To evaluate the indications and clinical outcomes (audiologic and vestibular) in patients with Ménière's syndrome who have undergone cochlear implantation. STUDY DESIGN: This is a retrospective review of patients at a large tertiary academic medical center. PATIENTS: Nine patients were included in the study with AAO-HNS criteria for diagnosis of Ménière's syndrome as well as bilateral severe to profound sensorineural hearing loss as an indication for undergoing cochlear implantation. Audiologic criteria for implantation were considered in the context of speech recognition performance with well-fit, powerful hearing aids noting large fluctuations in performance levels in some patients. In all cases, the poorer hearing ear was implanted. Seven subjects had bilateral disease and had progressed to profound sensorineural hearing loss. The average age of the patients was 61 years. Six patients had undergone previous surgery to control vertigo, including endolymphatic shunt surgery and vestibular nerve section. No patient had received previous treatment with intra-tympanic gentamicin. Symptoms of Ménière's syndrome had been present in all patients for at least 10 years before implantation. INTERVENTION: Cochlear Implantation. MAIN OUTCOME MEASURES: Pre- and Postoperative audiometric scores (monosyllable words/phonemes, Central Institute for the Deaf (CID) sentences, Hearing in Noise Test (HINT) in quite/noise (+10 db)), pre- and postoperative vestibular symptoms (number of vestibular attacks, aural fullness, tinnitus). RESULTS: Follow-up after implantation ranged from 1 to 5 years. Average 6 month postimplantation scores were: monosyllable words/phonemes = 52%/65%, CID sentences = 82%, HINT in quiet/noise = 70%/50%. Average 1-year postimplant scores were: monosyllable words/phonemes = 60%/76%, CID sentences = 97%, HINT in quiet/noise = 89%/78%. Postoperative speech recognition scores were, on average, substantially greater than preoperative scores. While there were few complications associated with implantation, some patients experienced alterations in their implant performance in association with fluctuations in vestibular symptoms. CONCLUSIONS: Patients with advanced binaural involvement with Ménière's Disease may present a challenge to conventional criteria for cochlear implant candidacy because of fluctuating symptoms. We observed significant benefit over baseline in a consecutive series of patients with Ménière's syndrome who progressed to bilateral, severe-to-profound sensorineural hearing loss and underwent cochlear implantation. Further, previous vestibular surgery, including labyrinthectomy, does not contraindicate cochlear implantation.

Adult↗
PubMed2003

Choice of ear for cochlear implantation: the effect of history and residual hearing on predicted postoperative performance.

OBJECTIVE: This study used mathematical formulas predicting cochlear implant outcomes to investigate the effects of implantation in the poorer ear on postoperative speech recognition. DESIGN: Retrospective cohort study with mathematical and statistical analyses. This study used the University of Iowa formula for predicting outcomes derived from implantation of the better ear on the basis of duration of deafness and preoperative speech understanding, applying this predictive model to a cohort of patients undergoing implantation in the poorer ear at The Johns Hopkins Medical Center. SETTING: Tertiary referral center with active cochlear implant program. PATIENTS: Postlingually deafened adults (n = 58) with preoperative Central Institute for the Deaf sentence scores less than or equal to 40%. INTERVENTION: Cochlear implantation with all three Food and Drug Administration-approved devices. MAIN OUTCOME MEASURE: Postoperative monosyllabic word recognition scores and correlations between actual and predicted results. RESULTS: There was good statistical correlation between the predicted postoperative performance using the University of Iowa formula and the actual performance of our cohort of patients undergoing implantation in the poorer ear (r = 0.50, p < 0.0001). In addition, as a population, our cohort had a mean postoperative consonant-nucleus-consonant word score of 41.8%, which was statistically the same as that predicted by the University of Iowa formula (43.6%). CONCLUSIONS: The postoperative performance of cochlear implant patients is most closely correlated with duration of deafness. However, our results indicate that this measure may not be ear specific and is more reflective of the total auditory receptivity of the patient. These observations help to form guidelines for choice of ear for implantation.

Adolescent↗
PubMed2003

Clinical and histopathologic features of recurrent vestibular schwannoma (acoustic neuroma) after stereotactic radiosurgery.

OBJECTIVE: Stereotactic radiosurgery for vestibular schwannoma entails uncertain long-term risk of tumor recurrence and delayed cranial neuropathies. In addition, the underlying histopathologic changes to the tumor bed are not fully characterized. We seek to understand the clinical and histologic features of recurrent vestibular schwannoma after stereotactic radiation therapy. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Four patients who underwent microsurgical resection of vestibular schwannoma after primary stereotactic radiation therapy. INTERVENTION: Patients were treated primarily with gamma knife radiosurgery or fractionated stereotactic radiotherapy followed by salvage microsurgery. Retrosigmoid craniotomy was used in all cases. MAIN OUTCOME MEASURES: Histopathologic review. Preoperative and postoperative facial nerve function was assessed with the House-Brackmann scale. RESULTS: We observed highly inconsistent radiation changes in the cerebellopontine angle and internal auditory canal. Fibrosis outside and within the tumor bed varied markedly, complicating microsurgical dissection. Light microscopy confirmed the presence of viable tumor in all cases. Histopathologic features were typical of vestibular schwannoma, and there was no significant scarring that could be attributed to radiation effect. CONCLUSIONS: The variable fibrosis in the cerebellopontine angle and lack of radiation changes seen histopathologically in irradiated vestibular schwannoma suggest that a uniform treatment effect was not achieved in these cases. Although all four patients with preoperative cranial neuropathies were found intraoperatively to have fibrosis in the cerebellopontine angle, excellent preservation of facial nerve anatomy and function was possible with salvage microsurgical resection. Additional analyses are needed to clarify the histopathologic and molecular characteristics associated with vestibular schwannoma growth after stereotactic radiation.

Aged↗
PubMed2002

Robot-assisted stapedotomy: micropick fenestration of the stapes footplate.

OBJECTIVE: Micropick fenestration of the stapes footplate, a difficult step in stapedotomy, was selected for trials evaluating the potential for robotic assistance (RA) to improve clinical measures of surgical performance. STUDY DESIGN: In a surgical model of stapedotomy, we measured accuracy of fenestration to a desired point location and force applied to the stapes footplate. Performance variables were measured for 3 experienced and 3 less-experienced surgeons. RESULTS: RA significantly reduced the maximum force applied to the stapes footplate. For fenestration targeting, RA significantly improved accuracy for less-experienced surgeons and significantly worsened targeting for more-experienced surgeons. CONCLUSIONS: RA significantly improves performance for micropick fenestration in a surgical model of stapedotomy. For certain tasks, RA differentially affects performance for users of different experience levels. CLINICAL SIGNIFICANCE: These are the first results showing quantitative improvements in performance during simulated ear surgery using RA and differential effects of RA on performance for users of different experience levels.

Clinical Competence↗
PubMed2002

Impact of cochlear implants on the functional health status of older adults.

OBJECTIVES: To assess the impact of cochlear implantation on quality of life changes in older adults aged 50 years and above. STUDY DESIGN: Retrospective study by questionnaire and chart review. METHODS: Forty-seven patients aged 50 to 80 years (mean age, 63.4 y [SD = 8.6 y]), who have multiple-channel cochlear implants received at The Listening Center at Johns Hopkins Hospital, completed the Ontario Health Utilities Index Mark 3 survey and a questionnaire on quality of life changes. Health utility scores before and after cochlear implantation were measured, and audiologic data before implantation and at 6 months and 1 year after implantation were analyzed. RESULTS: Cochlear implantation in older adults is associated with a mean gain in health utility(P <.0001) of 0.24 (SD = 0.33), which corresponds to a favorable cost-utility of $9530 per quality-adjusted life-year. Improvements in hearing and emotional health attributes were primarily responsible for this increase in health-related quality of life measure. There was a significant increase in speech perception scores at 6 months after surgery (P <.0001 for both CID sentence and monosyllabic word tests) and a strong correlation between the magnitude of health utility gains and the postoperative increase in speech perception scores (r = 0.45, P <.05). CONCLUSIONS: Cochlear implants have a significant impact on the quality of life of older deaf patients, and are a cost-effective intervention in this population. Improvements in speech perception are predictive of gains in health-related quality of life and associated emotional benefits after cochlear implantation.

Age Factors↗
PubMedDate not supplied

Osseous sarcoidosis presenting as a destructive petrous apex lesion.

We report a case of sarcoidosis presenting as a destructive petrous apex lesion of the temporal bone. Sarcoidosis is a disease of unknown etiology, featuring granulomatous inflammation in a number of organs and tissues. Osseous involvement of the temporal bone by sarcoidosis is uncommon and rarely encountered clinically. When osseous tissue is involved, it commonly affects the phalangeal bones of the hands and feet with noncaseating granulomas occupying the marrow space and creating a destructive pattern. A similar clinico-pathologic process was found to occur within the petrous apex of the temporal bone in an individual presenting with otalgia, hearing loss, and aural fullness. Imaging of the temporal bones revealed a unilateral destructive lesion of the petrous apex and adjacent skull base. A review of sarcoidosis and petrous apex lesions is presented. Sarcoid involvement of the petrous apex should be considered in the number of pathologies that feature bony destruction of the petrous apex of the temporal bone.

Diagnosis, Differential↗