Post headshake nystagmus: further correlation with other vestibular test results.

BACKGROUND: It is generally accepted that headshake nystagmus (HSN) is generated from an asymmetrical peripheral vestibular input and a correlation exists between HSN and canal paresis. There have been limited reports, however, how HSN correlates with the more recently introduced tests of vestibular function. AIMS/OBJECTIVES: To evaluate the correlation between HSN and unilateral weakness on caloric testing, high-frequency vestibulo-ocular reflex (VOR) function on video head impulse testing (VHIT) and otolith function determined by vestibular evoked myogenic potentials (VEMPs). METHODS: A retrospective study of all patients who underwent complete vestibular function testing at our tertiary referral center from 2016 to 2019 was performed. Demographic data, clinical diagnosis (where available), the results of video-nystagmography, vHIT, and VEMPs were evaluated. RESULTS: Of the 1499 patients in the study period, 101 (6.7%) had HSN. Vestibular test abnormalities were more common in patients with HSN. The sensitivity of HSN for underlying pathology was low in comparison with vestibular testing. However, HSN demonstrated a high specificity. CONCLUSIONS AND SIGNIFICANCE: HSN generation is likely more complex than caloric-induced nystagmus, probably due to additional variables within the vestibular system. HSN may provide clinicians with additional information concerning high-frequency VOR function involving lateral semicircular and otolith dysfunction.

Primary epidermoid cysts of the mastoid: clinical and treatment implications.

Epidermoid cysts of the temporal bone are extremely rare and such lesions arising in isolation within the mastoid bone have never been reported in literature. We report and describe the first two unique cases of primary epidermoid cysts arising in the mastoid bone. Of the two cases, one presented with progressive headache and imbalance and the other with unilateral hearing loss and tinnitus. Both cases needed CT and MRI scans and needed surgical management. We review the clinical presentations, histology, pathogenesis, radiological findings and management of these challenging cases. The diagnosis of an epidermoid cyst is based on clinical presentation, physical examination and especially the radiological, histological and intraoperative findings. Total removal of the lesion along with its capsule is recommended to prevent recurrence and to allow for a good long-term prognosis.

Whole stimulated salivary flow: correlation with the pathology of inflammation and damage in minor salivary gland biopsy specimens from patients with primary Sjögren's syndrome but not patients with sicca.

OBJECTIVE: To determine which measure of the salivary flow rate, stimulated or unstimulated, is most strongly associated with pathologic changes in minor salivary gland (MSG) biopsy specimens, and to explore the correlation of salivary flow with oral surface damage, disease duration, and symptom severity in patients with primary Sjögren's syndrome (SS). METHODS: In all patients (n = 32), a biopsy of the MSG was performed, and stimulated salivary flow was assessed. Beginning in 2002, unstimulated salivary flow was also assessed. Scores for the severity of symptoms, according to the decayed/missing/filled teeth (DMF) index, were recorded. Associations between measures of salivary flow and covariates characterizing pathology were examined. RESULTS: A definite association between stimulated salivary flow and the MSG focus score, the grade of MSG fibrosis, the duration of dry mouth symptoms, and the DMF score was observed. In contrast, unstimulated salivary flow was not associated with fibrosis, atrophy, the DMF score, or the duration of dry mouth symptoms. In patients with primary SS, the DMF score was associated with pathologic changes in the MSG. Among patients with sicca, 57.9% had an abnormal unstimulated salivary flow rate (versus 82.4% of patients with primary SS), and 15.2% had an abnormal stimulated salivary flow rate (versus 61.8% of patients with primary SS). Among patients with sicca, neither stimulated salivary flow nor unstimulated salivary flow was associated with the degree of fibrosis or atrophy or with the DMF score. CONCLUSION: Compared with unstimulated salivary flow, stimulated salivary flow appeared to be a better measure of inflammation (according to the focus score) and fibrosis. In patients with sicca, the unstimulated salivary flow rate appeared to be abnormal more commonly compared with the stimulated salivary flow rate. In the future, stimulated salivary flow may serve as a noninvasive surrogate biomarker of inflammation and fibrosis as well as a measure of response to treatment in patients with primary SS.

Receiving Long-Term Disability or Pursuing a Legal Claim May Prevent Recovery From Chronic Dizziness.

OBJECTIVES/HYPOTHESIS: Chronic dizziness (CD) and imbalance have multiple etiologies. CD is strongly linked with psychiatric and psychological comorbidities, thus an interdisciplinary approach, including psychopharmacological interventions, is recommended. Despite the use of this comprehensive treatment approach, the recovery of individuals with CD that pursue long-term disability (LTD) insurance or legal claims (LC) appears hampered. As such, we aimed to compare symptom recovery from CD in an interdisciplinary setting between patients receiving LTD/LC versus those who were not, and to explore the factors that may contribute to changes in symptom severity. STUDY DESIGN: Retrospective cohort study. METHODS: Dizziness-related diagnoses were extracted from the charts of 195 adults in an outpatient interdisciplinary neurotology clinic in Toronto, Canada. Patients with baseline Dizziness Handicap Inventory (DHI) and Dizziness Catastrophizing Scale (DCS) assessments between August 2012 and July 2018 and a mean follow-up visit within approximately 10 months were included. The study participants were categorized as "LTD/LC+" (n = 92) or "LTD/LC-" (n = 103), referring to either receiving or pursuing LTD/LC or not, respectively. RESULTS: There were differences in the mean percentage changes in DHI (t[187] = 3.02, P = .003) and DCS (t[179] = 2.63, P = .009) scores between LTD/LC+ and LTD/LC- patients. LTD/LC+ patients showed 8.0% and 7.6% mean increases in DHI and DCS scores, respectively, whereas LTD/LC- patients showed 21.5% and 25.9% reductions in DHI and DCS scores, respectively, controlling for age, sex, and baseline illness severity. CONCLUSIONS: Patients receiving or pursuing LTD insurance or a legal claim did not improve from CD and dizziness catastrophizing compared to those who were not. Future studies are required to test these findings prospectively and to determine the factors that may contribute to symptom recovery, including the anxiety-aggravating effects of the LTD/LC process and the deleterious consequences of developing a sick-role while afflicted with a chronic illness. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:655-661, 2022.

Gentamicin Vestibulotoxicity: Further Insights From a Large Clinical Series.

OBJECTIVE: To review insights gained from a 21-year experience with gentamicin-induced vestibulotoxicity including differences in vestibulotoxicity between single daily dosing (SDD) and multiple daily dosing (MDD) regimens. STUDY DESIGN: Retrospective case series. SETTING: Tertiary care center. PATIENTS: Patients with gentamicin vestibulotoxicity referred to the Hertz Multidisciplinary Neurotology Clinic between January 1993 and September 2014. INTERVENTION: None. MAIN OUTCOME MEASURES: Spectrum of vestibular dysfunction measured using videonystagmography, vestibular evoked myogenic potentials, video head impulse testing, and magnetic scleral search coil testing. RESULTS: Of 53 patients with gentamicin-induced vestibulotoxicity, 24 received SDD and 29 received MDD treatment. The most common indications for treatment were sepsis, endocarditis, and osteomyelitis. Angular acceleration receptor function (semicircular canals) was more commonly affected than linear acceleration receptor function (otolithic organ of the saccule; 100% vs. 62%). A significant proportion of patients (53%) developed vestibulotoxicity in the absence of nephrotoxicity and 40% experienced vestibulotoxicity in a delayed fashion up to 10 days posttreatment cessation (mean 3.9 ±â€Š0.7). Therapeutic monitoring did not necessarily prevent delayed vestibulotoxicity. Nephrotoxicity was less common for SDD compared with MDD (60% vs. 35%, p = 0.01). However, the SDD group experienced vestibulotoxicity at a lower cumulative dose (6.3 vs. 7.0 g, p = 0.04) and shorter duration of therapy (20.7 vs 29.4 d, p = 0.02). CONCLUSIONS: Our study further highlights important insights regarding gentamicin-induced vestibulotoxicity. While SDD is associated with decreased risk for nephrotoxicity compared with MDD, it confers a higher risk for vestibulotoxicity.

Interdisciplinary integration of nursing and psychiatry (INaP) improves dizziness-related disability.

OBJECTIVES/HYPOTHESIS: The traditional medical care model of "assess and refer" in a sequential fashion fails to recognize the complexities that arise due to overlapping physical and psychiatric comorbidities experienced by patients with chronic dizziness or imbalance, thus resulting in inadequate treatment outcomes. We aimed to evaluate the impact of a novel interdisciplinary approach to care that integrates nursing and psychiatry (INaP) on dizziness-related disability. STUDY DESIGN: Retrospective cohort study. METHODS: We compared the change in clinical assessment scores (i.e., Dizziness Handicap Inventory [DHI], Dizziness Catastrophizing Scale) at approximately 8 months follow-up between those who did (INaP+) and did not receive INaP (INaP-). Data from 229 patients with dizziness or imbalance referred to an interdisciplinary neurotology clinic in Toronto, Ontario, Canada were acquired from August 2012 to December 2016 and January 2011 to December 2013 for the INaP+ and INaP- groups, respectively. RESULTS: A mean group difference in the percentage change in DHI scores was found, with greater reductions in dizziness-related disability in the INaP+ group (n = 121) versus the INaP- group (n = 108). This remained significant after controlling for age, gender, baseline illness severity, and duration between baseline and follow-up visits. CONCLUSIONS: The novel interdisciplinary approach of incorporating INaP appears to be more effective than interdisciplinary care without INaP in reducing dizziness-related disability in patients with chronic dizziness or imbalance. Clinical settings should consider the addition of INaP to achieve better patient outcomes. Future studies are required to test the hypothesis that INaP is more efficient and cost-effective than the traditional model of care. LEVEL OF EVIDENCE: 3 Laryngoscope, 130:1800-1804, 2020.

Interdisciplinary integration of nursing and psychiatry (INaP) for the treatment of dizziness.

OBJECTIVES: The traditional medical care model of "assess and refer" requires revamping to address the multifaceted needs of patients with chronic dizziness and imbalance by adopting an interdisciplinary approach to care that integrates nursing and psychiatry (INaP). We aim to present a novel interdisciplinary approach that incorporates INaP in the care of patients with chronic dizziness and imbalance. METHODS: Presentation of an interdisciplinary model of care that incorporates INaP provided at the Toronto General Hospital in Toronto, Canada. RESULTS: Interdisciplinary care incorporating INaP, which includes the provision of support from an interdisciplinary health care team (ie, neurotologist, neurologist, psychiatrist, physiotherapist, and nurse clinician), psychoeducation about the interaction between chronic dizziness and psychiatric comorbidities, and ongoing access to medical and psychosocial assessment and intervention, addresses the physical and emotional aspects of patients' experience with chronic dizziness. CONCLUSIONS: The novel comprehensive interdisciplinary approach incorporating INaP may be more effective than interdisciplinary care without INaP in improving clinical outcomes in patients with chronic dizziness. In the subsequent study, we present data comparing patients treated for chronic dizziness and imbalance with and without the integration of INaP in an interdisciplinary setting. LEVEL OF EVIDENCE: 5 Laryngoscope, 130:1792-1799, 2020.

Vestibular Outcomes in Bilateral Posterior Semicircular Canal Occlusion for Refractory Benign Positional Vertigo.

INTRODUCTION: Bilateral posterior semicircular canal (PSCC) occlusion is a successful treatment of bilateral benign positional vertigo (BPV) refractory to particle repositioning manoeuvers. Little about the effects on the vestibular ocular reflex (VOR) postoperatively, which is the intent of this study. METHODS: All patients who underwent bilateral posterior canal occlusion for BPV at the University Health Network by the senior author (J.A.R.) between 2001 and 2017 with pre- and postoperative vestibular testing were included in the study.All patients underwent a detailed history and neuro-otological examination including dynamic visual acuity (DVA). Laboratory testing including video head impulse testing or magnetic scleral search coil testing, video nystagmography, cervical and ocular vestibular evoked myogenic potentials and audiological testing before and following their procedure at 1, 6, and 12 months postoperatively. RESULTS: Three patients were included in the study, all females, with a mean age of 41 years (range 36-44 yr). All patients had developed bilateral BPV after head trauma. Mean length of follow-up was 26 months (range 6-84 mo). All patients demonstrated a reduction in the vertical VOR of between 0.37 and 0.57/s at 1 month postocclusion. Between 6 and 12 months postoperatively, an improvement in their vertical VOR between 0.45 and 0.75/s was observed. Clinically, all patients reported complete resolution of their positional vertigo with a negative Dix-Hallpike bilaterally at follow-up. None reported oscillopsia, which was confirmed with DVA testing. CONCLUSION: Bilateral PSCC occlusion results in a reduction in the vertical VOR of the PSCCs.Over a 6 to 12 month time period improvement in the VOR gain can be demonstrated, most likely due to central compensation. There is clinical correlation with improvement in DVA testing. Bilateral PSCC occlusion is a safe and effective treatment for bilateral BPV proved refractory to particle repositioning manoeuvers.

Association Between Catastrophizing and Dizziness-Related Disability Assessed With the Dizziness Catastrophizing Scale.

Importance: Catastrophizing is a maladaptive thought process that involves irrational fear and worry about anticipated or actual symptoms. Although clinically relevant, the role of catastrophizing in patients with chronic dizziness or imbalance has not yet been explored to our knowledge. Objectives: To validate a measure of dizziness catastrophizing and to assess its association with dizziness-related disability compared with other negative affect constructs (eg, anxiety and depression). Design, Setting, and Participants: For this retrospective medical record review, the Dizziness Catastrophizing Scale (DCS), a dizziness-specific catastrophizing assessment tool, was adapted from the previously validated Pain Catastrophizing Scale. Psychometric evaluation of the DCS was performed. In addition, the associations of dizziness catastrophizing and positive and negative affectivity with dizziness-related disability were assessed using structural equation modeling and regression analyses. Data were collected using a retrospective medical record review from April 27, 2010, to June 25, 2014. The dates of analysis were June 3 to August 15, 2017. The setting was the Multidisciplinary Neurotology Clinic at the Toronto General Hospital (Toronto, Ontario, Canada). Participants were 457 adult outpatients with dizziness or imbalance who were referred to the clinic. Main Outcomes and Measures: Psychometric properties of the DCS and its association with dizziness-related disability, as measured with the Dizziness Handicap Inventory. Results: Among 457 patients (mean [SD] age, 53.4 [15.4] years; 154 [33.7%] male), the DCS demonstrated good convergent (r = 0.78, P < .001) and discriminant validity (r = -0.40, P < .001) with the negative and positive affectivity, respectively; internal consistency (α = .95); and test-retest reliability (intraclass correlation coefficient, 0.92; P < .001 at the 95% CI). An exploratory dimension reduction analysis revealed a single latent component of the DCS. The results of the structural equation modeling and regression analyses revealed that dizziness catastrophizing, although associated with negative affectivity (eg, symptoms of anxiety and depression), was independently associated with dizziness-related disability (standardized ß = 0.378; P < .001). Furthermore, a strong association was found between catastrophizing and dizziness-related disability across different dizziness-related diagnoses (r ≥ 0.6; P < .001). Conclusions and Relevance: In this study, the DCS was a valid and reliable measure for evaluating catastrophic thinking in patients with dizziness, which was independently associated with dizziness-related disability. Future studies should investigate the influence of alleviating symptoms of catastrophizing on functional outcomes in patients with dizziness or imbalance, the results of which will help guide novel approaches to the clinical care of patients with chronic dizziness.

Transmastoid partial labyrinthectomy for brainstem vascular lesions: clinical outcomes and assessment of postoperative cochleovestibular function.

OBJECTIVES: To discuss the transmastoid partial labyrinthectomy approach for brainstem vascular lesions, with respect to hearing and balance preservation. DESIGN: Retrospective case series. SETTING: Tertiary referral center (University Health Network, Toronto). PARTICIPANTS: Nine consecutive surgical patients between 1999 and 2004. MAIN OUTCOME MEASURES: Clinical, audiometric, and electrophysiological vestibular data. RESULTS: Nine transmastoid partial labyrinthectomy procedures (all females) were performed. In seven patients the underlying pathology was an intra-axial brainstem cavernous malformation. Two patients were treated for a basilar artery aneurysm. All patients had progressive neurological signs. Serviceable hearing (pure tone average (PTA): < 50 dB; speech discrimination score (SDS): > 50%) was preserved in seven patients. Partial vestibular function (clinical and electrophysiological) was maintained in most patients. CONCLUSIONS: The partial labyrinthectomy approach to the skull base provides excellent exposure while preserving cochleovestibular function in most patients.

Treating Benign Paroxysmal Positional Vertigo in the Patient With Traumatic Brain Injury: Effectiveness of the Canalith Repositioning Procedure.

OBJECTIVE: The aim of this study was to determine the effectiveness of the canalith repositioning procedure (CRP) in the treatment of benign paroxysmal positional vertigo (BPPV) among patients after mild-to-moderate traumatic brain injury. METHODS: An unblinded, nonrandomized, case comparison interventional study with repeated measures (1, 5, 9, and 12 weeks postenrollment) of three groups of patients with traumatic brain injury (BPPV, n = 21; nonspecific dizziness, n = 23; no dizziness, n = 12) was conducted. Patients in the BPPV group received the CRP at baseline and repeatedly until a negative Dix-Hallpike Maneuver was observed. Participants in the other two groups did not receive the CRP. RESULTS: Symptom resolution at the 12-week follow-up was observed in 75% of patients in the BPPV group versus 8.3% in the nonspecific dizziness group (p = .0006). A significant Group × Time interaction was observed for the Dizziness Handicap Inventory (F = 4.2, p = .003) and 36-item Short Form Health Questionnaire physical component scores (F = 2.16, p = .035) with the BPPV group showing significantly improved scores by the 12-week follow-up. Although there were between-group differences on the 36-item Short Form Health Questionnaire mental health component scores (F = 4.06, p = .022), changes over time were not significant in the groups. CONCLUSIONS: Treatment with the CRP for posttraumatic BPPV resulted in significant symptom resolution and improvement in perceived physical health status.

Ménière's Syndrome or Disease: Time Trends in Management and Quality of Evidence Over the Last Two Decades.

OBJECTIVE: To evaluate the evidence and trends in published literature on the treatment of Ménière's syndrome or disease (MS/D) by comparing studies published in the last two decades. DATA SOURCES: A literature search was performed on AMED, EMBASE, HMIC, MEDLINE, PsycINFO, BNI, CINAHL, HEALTH BUSINESS ELITE, CENTRAL and Cochrane Ear, Nose and Throat disorders groups trials register using a combination of MeSH. The date of last search was October 2014. STUDY SELECTION AND DATA EXTRACTION: Two hundred five studies (104 in decade I and 101 in decade II) were evaluated to report trends in the management of this condition, the differing levels of evidence published for each treatment modality, evaluate whether the guidelines published by the AAOHNS-Committee on Hearing and Equilibrium had been correctly employed, and whether the randomized controlled trials (RCTs) were compliant with the CONSORT guidelines. RESULTS: The number of published RCTs almost tripled from decade I (1994-2003) to decade II (2004-2103). There was a significant decline in the use of surgical intervention (p = 0.013); however, the number of studies involving the use of intratympanic injection remained largely unchanged. There was a shift in the level of evidence in published studies; studies with level 1 evidence tripled from decade I to II (4.8 to 17.8%, respectively) (p = 0.03); however, compliance with the AAOHNS-CHE criteria for reporting treatment outcomes and the CONSORT checklist was still poor. CONCLUSION: Although the evidence base on published literature on MS/D has improved over the last decade with an increase in emphasis on RCTs and quality of life (QoL) studies, a significant number of studies failed to follow AAOHNS-CHE criteria for reporting treatment outcome and the CONSORT criteria for reporting RCTs.

Intratympanic gentamicin for Menière's disease: a meta-analysis.

OBJECTIVES: To systematically review the published experience on intratympanic gentamicin treatment for intractable Menière's disease. STUDY DESIGN: Meta-analysis using a random effect model. METHODS: A comprehensive literature search was performed for articles using intratympanic gentamicin as a sole treatment modality with reporting of results according to the American Academy of Otolaryngology Head and Neck Surgery (AAO-HNS) guidelines for Menière's disease. Two reviewers independently assessed trial quality and extracted data. RESULTS: Fifteen trials with 627 patients met the inclusion criteria. All trials reported "before-after" outcome measures, using patients as their own controls. No double-blind or blinded prospective control trials were identified. Complete (class A) vertigo control was achieved in 74.7% (confidence interval [CI]95% 67.8-81.5%) of patients, and complete or substantial (class B) control was achieved in 92.7% (CI95% 89.5-96.0%). The success rate was not affected by gentamicin treatment regimen (fixed vs. titration). Hearing level and word recognition were not adversely affected, regardless of gentamicin treatment regimen. Analysis of functional level was not performed because of lack of data in the selected articles. CONCLUSIONS: Intratympanic gentamicin treatment for intractable Menière's disease appears to be effective in the relief of vertigo. Cochleotoxicity and ototoxicity is unlikely to be a major side effect. However, the level of evidence reflected from the eligible articles is insufficient, especially because of relatively poor study design. Therefore, it is prudent that patients eligible for this type of treatment should be selected carefully and titrated with low-dose gentamicin. Further investigation with this treatment modality with control subjects is warranted.

Intentional ablation of vestibular function using commercially available topical gentamicin-betamethasone eardrops in patients with Meniere's disease: further evidence for topical eardrop ototoxicity.

OBJECTIVE: To determine whether the controversial findings of suspected ototoxicity from commercially available gentamicin sulfate and betamethasone sodium phosphate eardrops can be used in a therapeutic fashion to ablate (or attenuate) vestibular function in patients with unilateral Meniere's disease. STUDY DESIGN: Prospective case review. METHODS: At a tertiary care dizziness unit at the University Health Network, Toronto General Hospital, University of Toronto (Toronto, Ontario, Canada), adults with unilateral Meniere's disease undergoing intratympanic ablation therapy were studied. After insertion of a tympanostomy tube with the patient under local anesthesia, patients instilled gentamicin containing eardrops three times daily until they became vertiginous for longer than 24 hours and then for an additional 2 days longer or for 1 month, whichever came first. Electronystagmographic caloric test responses were measured before treatment using bithermal water caloric and after treatment using air caloric tests. Main outcome measures included clinical titration of drops to the onset of prolonged vertigo. As well, post-treatment findings on electronystagmography and audiometry were compared with pretreatment testing. RESULTS: Twenty patients were available for review. Fifteen patients had a significant reduction in caloric test responses compared with pretreatment values; among them, 10 patients had absent air caloric test responses on the treated side. In 10 patients hearing worsened according to the 1995 American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium Guidelines for reporting in Meniere's disease. CONCLUSIONS: Topical gentamicin-betamethasone eardrops can pass through a tube into the middle ear, where they may prove primarily vestibulo-ototoxic patients with Meniere's disease. The study further confirms clinical observations that gentamicin-containing eardrops might prove ototoxic, especially in noninflamed ears with a tympanic membrane defect.

The behavior of mastoidectomy cavities following modified radical mastoidectomy.

OBJECTIVES/HYPOTHESIS: An analysis of the frequency and intensity of postoperative aftercare required for modified radical mastoidectomy (MRM) and patterns of healing in the postoperative period. STUDY DESIGN: A retrospective review of all primary modified radical mastoidectomies carried out for cholesteatoma under the care of the senior author between the years of 2004 and 2009 with minimum follow-up of 2 years. METHODS: The time and number of interventions required to achieve a stable and dry mastoid cavity were collected. Cross-sectional and longitudinal analysis of the behavior of the cavities was carried out. RESULTS: Overall, 73 cases (71 patients) were identified. Patients were followed up for a median of 45.7 months (interquartile range, 31.8-70.5). After initial debridement, most cavities settled rapidly, but this was not always predictable, with a large proportion requiring further clinical intervention after the cavity was stable, sometimes for prolonged periods of time. At the time of analysis, 73% had achieved a stable cavity, 17 (23%) still required attention (nine for wax removal and eight for debridement); two were lost to follow-up. No revision surgeries were required. At 6 months, 36% of cavities were settled, 42% at 1 year, 53% at 18 months, and 62% at 2 years. After two standard postoperative visits, a total of 632 visits were made by these patients. CONCLUSIONS: Following MRM, the majority of patients achieve a dry, self-cleaning mastoid cavity. This might require periods of intense care interspersed with periods of quiescence. These results allow the benefits of this procedure to be put in the context of the entire patient journey.

Vestibular end-organ dysfunction in children with sensorineural hearing loss and cochlear implants: an expanded cohort and etiologic assessment.

INTRODUCTION: Similarities between the peripheral auditory and vestibular systems suggest that children with sensorineural hearing loss (SNHL) may demonstrate associated vestibular impairments. The current study examines vestibular function in a previously reported cohort (n = 40) augmented by 113 children with profound SNHL. METHODS: The current study is prospective and cross-sectional with repeated measures. Horizontal canal function was assessed in response to caloric and rotational stimuli. Saccular function was examined using vestibular evoked myogenic potentials (VEMP). One hundred fifty-three children were tested; 119 had unilateral cochlear implants (CIs) at time of testing, and 34 were evaluated before CI. RESULTS: Horizontal semicircular canal function was abnormal in response to caloric stimuli in 50% (69/139), with a proportion (18/69, 26%) reflecting mild to moderate unilateral abnormalities. Severe hypofunction or areflexia occurred in 37% (51/139). Horizontal semicircular canal function in response to rotation was abnormal in 47% (64/139). Bilateral reduction in VOR gain was seen across frequencies in 29% (40/139) and 13% (18/139) demonstrated isolated high-frequency (≥ 2 Hz) loss. Saccular function was absent bilaterally in 21% (32/135) and unilaterally in 30% (40/135). All children with meningitis (n = 11) and 46% with radiologic cochleovestibular anomalies (n = 31) had horizontal canal dysfunction, whereas 45% and 46%, respectively, displayed saccular dysfunction. Unilateral dysfunction of the horizontal canal or the saccule was equally distributed between the implanted and nonimplanted ear (14:9 and 22:18, respectively), and the differences in proportions were not statistically significant. CONCLUSION: Vestibular end organ dysfunction occurred in half of all children with profound SNHL. Approximately one-third of the subjects displayed severe abnormalities(bilateral and/or severe loss). The likelihood of vestibular end-organ dysfunction is highly dependent on etiology, with meningitis and cochleovestibular anomalies having the highest rates of severe dysfunction.

Double impairment: clinical identification of 33 cases of cerebellar ataxia with bilateral vestibulopathy.

OBJECTIVE: Following recently described small series of patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV), the authors undertook a careful clinical and laboratory assessment of patients who presented to their unit with characteristics of this syndrome. STUDY DESIGN: Case note review. SETTING: Tertiary, university-based, multidisciplinary neurootology clinic. SUBJECTS: Thirty-three patients whose characteristics fit this syndrome. METHODS: Patients presenting to the Multidisciplinary Neurotology Clinic with characteristics of CABV were entered into a bespoke database. This was analyzed to identify the clinical findings and results of vestibular investigations for this group. RESULTS: Patients presented at a mean age of 54 years (SD, 17.6) with symptoms having been present for a median of 3 years (interquartile ratio, 2.0-9.5). Caloric testing greatly underestimated the disorder, being subnormal in only 18% of patients; the head-thrust test was abnormal and dynamic visual acuity testing was abnormal 88% and 91% of the time, respectively. Of the patients, 76% demonstrated gaze-evoked nystagmus. Impaired smooth pursuit (97% of patients showed low gain with saccadic corrections) and impaired cancellation of the vestibulo-ocular reflex (in 97% of patients) were found. Impaired saccular otolithic function was abnormal in 33%, adding to patient imbalance. CONCLUSION: The unique double-pathway balance impairment in CABV patients causes a high prevalence of subnormal function of both central and peripheral vestibular function. This is an easily missed clinical entity that is often associated with normal caloric investigations. As many patients with this syndrome are poor candidates for vestibular rehabilitation therapy, resources are better devoted to the early implementation of assistance with their safe ambulation and activities of daily living.