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Joshua J. Wind, John P. Leonetti, Michael J. M. Raffin, Marc T. Pisansky, Brian Herr, Justin D. Triemstra and Douglas E. Anderson

Object

No extant literature documents the analysis of patient perceptions of hearing as a corollary to objective audiometric measures in patients with vestibular schwannoma (VS), or acoustic neuroma. Therefore, using objective audiometric data and patient perceptions of hearing function as outlined on a questionnaire, the authors evaluated the hearing of patients who underwent VS resection.

Methods

This investigation involved a retrospective review of 176 patients who had undergone VS resections in which hearing preservation was a goal. Both pre- and postoperative audiometry, expressed as a speech discrimination score (SDS) and pure tone threshold average (PTA), were performed, and the results were analyzed. Intraoperative auditory brainstem responses were also recorded. Eighty-seven of the patients (49.4%) completed a postoperative questionnaire designed to assess hearing function in a variety of social and auditory situations. Multiple linear regression analyses were completed to compare available audiometric results with questionnaire responses for each patient.

Results

One hundred forty-two patients (80.7%) had PTA and SDS audiometric data pertaining to the surgically treated ear; 94 of these patients (66.2%) had measurable postoperative hearing, as defined by a PTA < 120 dB or SD > 0%. Eighty-seven patients (49.4%) completed the retrospective questionnaire, and 74 of them had complete audiometric data and thus were included in a comparative analysis. Questionnaire data showed major postoperative subjective hearing decrements, even among patients with the same pre- and postoperative objective audiometric hearing status. Moreover, the subscore reflecting hearing while exposed to background noise, or the “cocktail party effect,” characterized the most significant patient-perceived hearing deficit following VS resection.

Conclusions

The authors' analysis of a patient-perceived hearing questionnaire showed that hearing during exposure to background noise, or the cocktail party effect, represents a significant postoperative hearing deficit and that patient perception of this deficit has a strong relation with audiometric data. Furthermore, questionnaire responses revealed a significant disparity between subjective hearing function and standard audiometrics such that even with similar levels of audiometric data, subjective measures of hearing, especially the cocktail party effect, decreased postoperatively. The authors posit that the incorporation of patient-perceived hearing function evaluation along with standard audiometry is an illustrative means of identifying subjective hearing deficits after VS resection and may ultimately aid in specific and subsequent treatment for these patients.

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Paul Gigante, Michael M. McDowell, Samuel S. Bruce, Genevieve Chirelstein, Claudia A. Chiriboga, Joseph Dutkowsky, Elizabeth Fontana, Joshua Hyman, Heakyung Kim, Dean Morgan, Toni S. Pearson, Benjamin D. Roye, David P. Roye Jr., Patricia Ryan, Michael Vitale and Richard C. E. Anderson

Object

Randomized clinical trials have established that lumbar selective dorsal rhizotomy (SDR) reduces lower-extremity tone and improves functional outcome in children with spastic cerebral palsy. Significant data exist to support a secondary effect on upper-extremity function in patients with upper-extremity spasticity. The effects of SDR on upper-extremity tone, however, are not well characterized. In this report, the authors sought to assess changes in upper-extremity tone in individual muscle groups after SDR and tried to determine if these changes could be predicted preoperatively.

Methods

The authors retrospectively reviewed 42 children who underwent SDR at Columbia University Medical Center/Morgan Stanley Children's Hospital of NewYork-Presbyterian between 2005 and 2011. Twenty-five had upper-extremity spasticity. All underwent pre- and postoperative examination for measuring tone (Modified Ashworth Scale) and assessing functional outcome. Follow-up examinations with therapists were performed at least once at a minimum of 2 months postoperatively (mean 15 months).

Results

In the upper extremities, 23 (92%) of 25 patients had improvements of at least 1 Ashworth point in 2 or more independent motor groups on the Modified Ashworth Scale, and 12 (71%) of 17 families surveyed reported increases in motor control or spontaneous movement. The mean Modified Ashworth Scale scores for all upper-extremity muscle groups demonstrated an improvement from 1.34 to 1.22 (p < 0.001). Patients with a mean preoperative upper-extremity tone of 1.25–1.75 were most likely to benefit from reduction in tone (p = 0.0019). Proximal and pronator muscle groups were most likely to demonstrate reduced tone.

Conclusions

In addition to improvements in lower-extremity tone and function, SDR has demonstrable effects on upper extremities. Greater than 90% of our patients with elevated upper-extremity tone demonstrated reduction in tone in at least 2 muscle groups postoperatively. Patients with a mean Modified Ashworth Scale upper-extremity score of 1.25–1.75 may encounter the greatest reduction in upper-extremity tone.

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Jennifer M. Strahle, Rukayat Taiwo, Christine Averill, James Torner, Chevis N. Shannon, Christopher M. Bonfield, Gerald F. Tuite, Tammy Bethel-Anderson, Jerrel Rutlin, Douglas L. Brockmeyer, John C. Wellons III, Jeffrey R. Leonard, Francesco T. Mangano, James M. Johnston, Manish N. Shah, Bermans J. Iskandar, Elizabeth C. Tyler-Kabara, David J. Daniels, Eric M. Jackson, Gerald A. Grant, Daniel E. Couture, P. David Adelson, Tord D. Alden, Philipp R. Aldana, Richard C. E. Anderson, Nathan R. Selden, Lissa C. Baird, Karin Bierbrauer, Joshua J. Chern, William E. Whitehead, Richard G. Ellenbogen, Herbert E. Fuchs, Daniel J. Guillaume, Todd C. Hankinson, Mark R. Iantosca, W. Jerry Oakes, Robert F. Keating, Nickalus R. Khan, Michael S. Muhlbauer, J. Gordon McComb, Arnold H. Menezes, John Ragheb, Jodi L. Smith, Cormac O. Maher, Stephanie Greene, Michael Kelly, Brent R. O’Neill, Mark D. Krieger, Mandeep Tamber, Susan R. Durham, Greg Olavarria, Scellig S. D. Stone, Bruce A. Kaufman, Gregory G. Heuer, David F. Bauer, Gregory Albert, Jeffrey P. Greenfield, Scott D. Wait, Mark D. Van Poppel, Ramin Eskandari, Timothy Mapstone, Joshua S. Shimony, Ralph G. Dacey Jr., Matthew D. Smyth, Tae Sung Park and David D. Limbrick Jr.

OBJECTIVE

Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors’ goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis.

METHODS

A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°).

RESULTS

Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB–C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude.

CONCLUSIONS

Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.