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1.
N Engl J Med ; 384(6): 521-532, 2021 02 11.
Article in English | MEDLINE | ID: mdl-33567192

ABSTRACT

BACKGROUND: Bilateral vestibular hypofunction is associated with chronic disequilibrium, postural instability, and unsteady gait owing to failure of vestibular reflexes that stabilize the eyes, head, and body. A vestibular implant may be effective in alleviating symptoms. METHODS: Persons who had had ototoxic (7 participants) or idiopathic (1 participant) bilateral vestibular hypofunction for 2 to 23 years underwent unilateral implantation of a prosthesis that electrically stimulates the three semicircular canal branches of the vestibular nerve. Clinical outcomes included the score on the Bruininks-Oseretsky Test of Motor Proficiency balance subtest (range, 0 to 36, with higher scores indicating better balance), time to failure on the modified Romberg test (range, 0 to 30 seconds), score on the Dynamic Gait Index (range, 0 to 24, with higher scores indicating better gait performance), time needed to complete the Timed Up and Go test, gait speed, pure-tone auditory detection thresholds, speech discrimination scores, and quality of life. We compared participants' results at baseline (before implantation) with those at 6 months (8 participants) and at 1 year (6 participants) with the device set in its usual treatment mode (varying stimulus pulse rate and amplitude to represent rotational head motion) and in a placebo mode (holding pulse rate and amplitude constant). RESULTS: The median scores at baseline and at 6 months on the Bruininks-Oseretsky test were 17.5 and 21.0, respectively (median within-participant difference, 5.5 points; 95% confidence interval [CI], 0 to 10.0); the median times on the modified Romberg test were 3.6 seconds and 8.3 seconds (difference, 5.1; 95% CI, 1.5 to 27.6); the median scores on the Dynamic Gait Index were 12.5 and 22.5 (difference, 10.5 points; 95% CI, 1.5 to 12.0); the median times on the Timed Up and Go test were 11.0 seconds and 8.7 seconds (difference, 2.3; 95% CI, -1.7 to 5.0); and the median speeds on the gait-speed test were 1.03 m per second and 1.10 m per second (difference, 0.13; 95% CI, -0.25 to 0.30). Placebo-mode testing confirmed that improvements were due to treatment-mode stimulation. Among the 6 participants who were also assessed at 1 year, the median within-participant changes from baseline to 1 year were generally consistent with results at 6 months. Implantation caused ipsilateral hearing loss, with the air-conducted pure-tone average detection threshold at 6 months increasing by 3 to 16 dB in 5 participants and by 74 to 104 dB in 3 participants. Changes in participant-reported disability and quality of life paralleled changes in posture and gait. CONCLUSIONS: Six months and 1 year after unilateral implantation of a vestibular prosthesis for bilateral vestibular hypofunction, measures of posture, gait, and quality of life were generally in the direction of improvement from baseline, but hearing was reduced in the ear with the implant in all but 1 participant. (Funded by the National Institutes of Health and others; ClinicalTrials.gov number, NCT02725463.).


Subject(s)
Bilateral Vestibulopathy/surgery , Gait/physiology , Hearing Loss/etiology , Implantable Neurostimulators , Postural Balance/physiology , Quality of Life , Vestibule, Labyrinth/surgery , Aged , Bilateral Vestibulopathy/chemically induced , Bilateral Vestibulopathy/complications , Dizziness/etiology , Female , Gait Disorders, Neurologic/etiology , Humans , Implantable Neurostimulators/adverse effects , Male , Middle Aged , Postoperative Complications , Prospective Studies , Semicircular Canals/innervation , Vestibular Nerve/drug effects
2.
Otol Neurotol ; 40(2): 204-212, 2019 02.
Article in English | MEDLINE | ID: mdl-30570606

ABSTRACT

OBJECTIVE: To identify predictors of near dehiscence (ND) or thin rather than dehiscent bone overlying the superior semicircular canal in patients with signs and symptoms suggestive of superior semicircular canal dehiscence syndrome (SCDS), as well as postoperative outcomes. STUDY DESIGN: Retrospective case-control study. SETTING: Tertiary referral center. PATIENTS: All 288 patients who underwent middle cranial fossa approach for repair of SCDS (1998-2018) were reviewed for cases of ND. Demographics, symptoms, and clinical signs including nystagmus, ocular vestibular-evoked myogenic potential (oVEMP) amplitude, cervical vestibular-evoked myogenic potential (cVEMP) thresholds, and low-frequency air-bone gap were compared before and after surgery. MAIN OUTCOME MEASURE: Presence of preoperative ND and postoperative symptoms and physiologic measures. RESULTS: Seventeen cases of ND (16 patients, 17 ears) and 34 cases (34 ears) of frank SCDS were identified. ND cases differed from frank dehiscence cases in that they were less likely to have nystagmus in response to ear canal pressure or loud sounds, OR = 0.05 (95% CI 0.01-0.25) and Valsalva, OR = 0.08 (0.01-0.67), smaller peak-to-peak oVEMP amplitudes, OR = 0.84 (0.75-0.95), and higher cVEMP thresholds, OR = 1.21 (1.07-1.37). Patients with ND had similar symptoms to those with frank SCDS before surgery, and after surgery had outcomes similar to patients with frank SCDS. CONCLUSIONS: In patients with symptoms consistent with SCDS, predictors of ND include absence of nystagmus in response to pressure/loud sounds, greater cVEMP thresholds, and smaller oVEMP amplitudes. We propose ND is on a spectrum of dehiscence that partially accounts for the diversity of clinical presentations of patients with SCDS.


Subject(s)
Labyrinth Diseases/pathology , Labyrinth Diseases/physiopathology , Semicircular Canals/pathology , Adult , Case-Control Studies , Female , Humans , Labyrinth Diseases/surgery , Male , Middle Aged , Nystagmus, Pathologic/epidemiology , Nystagmus, Pathologic/etiology , Retrospective Studies , Semicircular Canals/surgery , Syndrome , Vestibular Evoked Myogenic Potentials/physiology
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