Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence.

Objective: This communication is the first assessment of outcomes after surgical repair of cochlea-facial nerve dehiscence (CFD) in a series of patients. Pre- and post-operative quantitative measurement of validated survey instruments, symptoms, diagnostic findings and anonymous video descriptions of symptoms in a cohort of 16 patients with CFD and third window syndrome (TWS) symptoms were systematically studied. Study design: Observational analytic case-control study. Setting: Quaternary referral center. Patients: Group 1 had 8 patients (5 children and 3 adults) with CFD and TWS who underwent surgical management using a previously described round window reinforcement technique. Group 2 had 8 patients (2 children and 6 adults) with CFD who did not have surgical intervention. Interventions: The Dizziness Handicap Inventory (DHI) and Headache Impact Test (HIT-6) were administered pre-operatively and post-operatively. In addition, diagnostic findings of comprehensive audiometry, cervical vestibular evoked myogenic potential (cVEMP) thresholds and electrocochleography (ECoG) were studied. Symptoms before and after surgical intervention were compared. Main outcome measures: Pre- vs. post-operative DHI, HIT-6, and audiometric data were compared statistically. The thresholds and amplitudes for cVEMP in symptomatic ears, ears with cochlea-facial nerve dehiscence and ears without CFD were compared statistically. Results: There was a highly significant improvement in DHI and HIT-6 at pre- vs. post-operative (p < 0.0001 and p < 0.001, respectively). The age range was 12.8-52.9 years at the time of surgery (mean = 24.7 years). There were 6 females and 2 males. All 8 had a history of trauma before the onset of their symptoms. The mean cVEMP threshold was 75 dB nHL (SD 3.8) for the operated ear and 85.7 dB (SD 10.6) for the unoperated ear. In contrast to superior semicircular canal dehiscence, where most ears have abnormal ECoG findings suggestive of endolymphatic hydrops, only 1 of 8 operated CFD ears (1 of 16 ears) had an abnormal ECoG study. Conclusions: Overall there was a marked improvement in DHI, HIT-6 and symptoms post-operatively. Statistically significant reduction in cVEMP thresholds was observed in patients with radiographic evidence of CFD. Surgical management with round window reinforcement in patients with CFD was associated with improved symptoms and outcomes measures.

Comorbidities confounding the outcomes of surgery for third window syndrome: Outlier analysis.

Objective: Patients with third window syndrome and superior semicircular canal dehiscence (SSCD) symptoms whose surgical outcomes placed them as outliers were systematically studied to determine comorbidities that were responsible for their poor outcomes due to these confounding factors. Study Design: Observational analytic case-control study in a tertiary referral center. Methods: Twelve adult patients with clinical SSCD syndrome underwent surgical management and had outcomes that did not resolve all of their subjective symptoms. In addition to one of the neurotologists, 2 neurologists (one specializing in migraine and the other a neuro-ophthalmologist), and a psychologist clinician-investigator completed comprehensive evaluations. Neuropsychology test batteries included: the Millon Behavioral Medicine Diagnostic; Patient Health Questionnaire (PHQ-9) and Generalized Anxiety Disorder Screener (GAD-7); Adverse Childhood Experiences Scale; the Wide Range Assessment of Memory and Learning, including the 3 domains of verbal memory, visual memory, and attention/concentration; Wechsler Adult Intelligence Scale; and the Delis-Kaplan Executive Function System. The control cohort was comprised of 17 participants who previously underwent surgery for third window syndrome that resulted in the expected outcomes of resolution of their third window syndrome symptoms and cognitive dysfunction. Results: There was a high rate of psychological comorbidity (n = 6) in the outlier cohort; multiple traumatic brain injuries were also a confounding element (n = 10). One patient had elevated cerebrospinal fluid (CSF) pressure requiring ventriculoperitoneal shunting to control the recurrence of dehiscence and one patient with a drug-induced Parkinson-like syndrome and idiopathic progressive neurological degenerative process. Conclusions: Components of the Millon Behavioral Medicine Diagnostic, PHQ-9 and GAD-7 results suggest that these instruments would be useful as screening tools preoperatively to identify psychological comorbidities that could confound outcomes. The identification of these comorbid psychological as well as other neurological degenerative disease processes led to alternate clinical management pathways for these patients. Level of Evidence: 2b.

Longitudinal Cognitive and Neurobehavioral Functional Outcomes Before and After Repairing Otic Capsule Dehiscence.

OBJECTIVE: Patients with peripheral vestibular dysfunction because of gravitational receptor asymmetries display signs of cognitive dysfunction and are assumed to have neurobehavioral sequelae. This was tested with pre- and postoperatively quantitative measurements in three cohort groups with superior semicircular canal dehiscence syndrome (SSCDS) symptoms with: 1) superior canal dehiscence (SCD) repaired via a middle cranial fossa craniotomy and canal plugging only; 2) otic capsule defects not visualized with imaging (no-iOCD) repaired with round window reinforcement (RWR) only; or 3) both SCD plugging and subsequent development of no-iOCD followed by RWR. STUDY DESIGN: Prospective patient series. SETTING: Tertiary referral center. PATIENTS: There were 13 adult and 4 pediatric patients with SSCDS who had completion of neuropsychology test batteries pre- and every 3 months postoperatively. Eight patients had no-iOCD and RWR exclusively, 5 had SCD and plugging exclusively, and 4 had both SCD plugging and then development of no-iOCD with RWR. These cohorts included SSCDS with 2 different dehiscence locations. INTERVENTIONS: Completion of a neuropsychology test battery preoperatively and at 3, 6, 9, and 12 months postoperatively that included: Beck Depression Inventory-II (BDI); Wide Range Intelligence Test (WRIT FSIQ) including average verbal (crystallized intelligence) and visual (fluid intelligence); Wide Range Assessment of Memory and Learning (WRAML), including the four domains of verbal memory, visual memory, attention/concentration, and working memory; and Delis-Kaplan Executive Function System (D-KEFS). The Dizziness Handicap Inventory (DHI) and the Headache Impact Test (HIT-6) were also completed to assess the impact of their disease on activities pre- and postoperatively. MAIN OUTCOME MEASURES: Quantitative and statistical analysis of their cognitive and neurobehavioral function. RESULTS: The pattern of differences between the SCD group and the no-iOCD group from WRAML verbal, visual, and attention test performance indicate different postoperative clinical trajectories. For the WRAML, there was a statistically significant improvement for visual memory and verbal memory for the no-iOCD only and both (SCD and subsequent no-iOCD) groups, but no mean improvement for the SCD only group. By contrast, the no-iOCD group had significantly lower scores on the WRAML attention test preoperatively, but they recovered postoperatively to match the other groups. The preoperative findings and postoperative outcomes did not differ significantly among patient groups on the WRAML working memory test, D-KEFS motor scores, D-KEFS number and letter scores, or Wide Range Intelligence Test scores. There was a significant decrease in the BDI for all groups. The IQ scores were unchanged. There was a statistically significant improvement in the DHI and HIT-6 scores postoperatively in all groups. CONCLUSIONS: There was a marked overall improvement in cognitive and neurobehavioral function postoperatively. Variability may result from duration of underlying disease before intervention. The initial decrement or delay in performance improvement measured in several patients may represent brain reorganization. Greater longitudinal data and greater subject numbers are necessary to better understand and optimize cognitive recovery.

Otic capsule dehiscence syndrome: Superior semicircular canal dehiscence syndrome with no radiographically visible dehiscence.

We conducted a prospective longitudinal study of two cohorts of patients who had superior semicircular canal dehiscence syndrome (SSCDS); one group had radiographically confirmed superior canal dehiscence (SCD), and the other exhibited no identified otic capsule dehiscence on imaging (no-iOCD). We compiled data obtained from prospective structured symptomatology interviews; diagnostic studies; three-dimensional, high-resolution, temporal bone computed tomography; and a retrospective case review from our tertiary care referral center. Eleven adults and 1 child with SSCDS were identified, surgically managed, and followed. Six of these patients-1 man and 5 women, aged 29 to 54 years at first surgery (mean: 41.8)-had radiologically confirmed SCD. The other 6 patients-1 man, 4 women, and 1 girl, aged 1 to 51 years (mean: 32.2)-had no-iOCD. The 6 adults with SCD underwent surgery via a middle cranial fossa approach with plugging procedures. The 5 adults and 1 child with no-iOCD underwent round window reinforcement (RWR) surgery. One SCD patient developed no-iOCD 1.5 years after SCD surgery, and she subsequently underwent RWR surgery. Our main outcome measures were patient symptomatology (with video documentation) and the results of diagnostic studies. Other than the character of migraine headaches, there was no difference in preoperative symptomatology between the two groups. Postoperatively, resolution of SSCDS symptoms ultimately occurred in all patients. Both the SCD and the no-iOCD groups experienced a highly significant improvement in postural control following treatment (Wilcoxon signed rank test, p < 0.001). We conclude that the term otic capsule dehiscence syndrome more accurately reflects the clinical syndrome of SSCDS since it includes both superior semicircular canal dehiscence and no-iOCD, as well as posterior and lateral semicircular canal dehiscence, all of which can manifest as SSCDS. We have also included links to videos in which 4 of the SSCDS patients with no-iOCD in this study discussed their symptoms and the results of their surgery; these links are found in the "References" section in citations 12-15. Links to three other videos of interest are contained in citations 10, 11, and 24.