Cupulolithiasis: A Critical Reappraisal.

Objective: To review the history and pathophysiologic theories for cupulolithiasis and canalith jam in benign paroxysmal positional vertigo. Data Sources: PubMed, Google Scholar. Review Methods: Three PubMed and Google Scholar searches were performed, keywords: "cupulolithiasis," "apogeotropic [and] benign," and "canalith jam," resulting in 187 unique full-text articles in English or with English translation. Figures-Labyrinthine photographs were obtained of fresh utricles, ampullae, and cupulae of a 37-day-old mouse. Conclusions: Freely moving otoconial masses explain most cases (>98%) of benign paroxysmal positional vertigo. Evidence that otoconia adhere strongly or persistently to the cupula is lacking. Apogeotropic nystagmus in the horizontal canal form is often attributed to cupulolithiasis; however, periampullary canalithiasis explains self-limited nystagmus, and reversible canalith jam explains prolonged apogeotropic nystagmus. Treatment-resistant cases can be explained by entrapment of particles in the canals or ampullae, but persistent adherence to the cupula remains theoretical. Implications for Practice: Apogeotropic nystagmus is usually due to freely moving particles and should not be used in studies of horizontal canal benign paroxysmal positional vertigo as the sole method to define entrapment or cupulolithiasis. Caloric testing and imaging may help differentiate jam from cupulolithiasis. Treatment for apogeotropic benign paroxysmal positional vertigo should include maneuvers that rotate the head through 270° to fully clear the canal of mobile particles, using mastoid vibration or head shaking if entrapment is suspected. Canal plugging can be used for treatment failures.

Reversible Canalith Jam of the Horizontal Semicircular Canal Mimicking Cupulolithiasis.

OBJECTIVE: To describe a case of benign paroxysmal positional vertigo (BPPV) resulting in reversible horizontal semicircular canalith jam successfully treated with horizontal canal occlusion. A brief literature review of similar cases was performed. METHODS: Case report and literature review. RESULTS: A 68-year-old female presented with apogeotropic positional nystagmus, attributed to reversible horizontal canalith jam mimicking cupulolithiasis that was refractory to tailored repositioning maneuvers across months. She was unable to work due to the severity of her symptoms. She underwent surgical occlusion of the affected canal with immediate resolution of her symptoms. A literature review revealed similar cases of canalith jam mimicking cupulolithiasis. CONCLUSIONS: Reversible canalith jam, in which particles moving with horizontal head position alternate between obstructing the semicircular canal and resting on the cupula, can mimic signs of cupulolithiasis. This variant of BPPV can be effectively managed with surgical canal occlusion should symptoms fail to resolve after tailored repositioning maneuvers.

The Clinical Spectrum of Dizziness in Sleep Apnea.

OBJECTIVE: Sleep apnea has been associated with dizziness, but the clinical characteristics of this dizziness have not been fully described. This study evaluated clinical descriptions of dizziness, vestibular diagnoses, and the response to treatment of dizzy patients with sleep apnea. PATIENTS: All subjects had a new diagnosis of sleep apnea with a confirmatory pulse oximetry or polysomnogram and received treatment for sleep apnea through our center. Fifty-two patients met these criteria, with a mean age of 55 years and a mean BMI of 31. INTERVENTION: Continuous positive airway pressure or uvulopalatopharyngoplasty. MAIN OUTCOME MEASURES: Reported symptoms of dizziness and vestibular diagnoses were collected retrospectively in all patients. Nineteen patients with a complete resolution of dizziness with treatment of sleep apnea on mean follow-up of 4 years were used to define the clinical syndrome and their symptoms were compared with the remainder of the group. RESULTS: Repeated spells of sudden momentary vertigo were common. Vestibular migraine, Menière's disease, and sudden sensorineural hearing loss occurred in a much higher frequency than in the general population. CONCLUSIONS: Brief spells of nonpositional vertigo that recur throughout the day, phenotypically similar to vestibular paroxysmia, responded to treatment of sleep apnea, and could represent a new vestibular entity. The greatly elevated risk of migraine, Menière's disease and sudden sensorineural hearing loss are likely due to enhanced vascular risks associated with sleep apnea. A history of snoring should be sought in all dizzy patients.

Meniere's disease.

Meniere's disease (MD) is a disorder of the inner ear that causes vertigo attacks, fluctuating hearing loss, tinnitus and aural fullness. The aetiology of MD is multifactorial. A characteristic sign of MD is endolymphatic hydrops (EH), a disorder in which excessive endolymph accumulates in the inner ear and causes damage to the ganglion cells. In most patients, the clinical symptoms of MD present after considerable accumulation of endolymph has occurred. However, some patients develop symptoms in the early stages of EH. The reason for the variability in the symptomatology is unknown and the relationship between EH and the clinical symptoms of MD requires further study. The diagnosis of MD is based on clinical symptoms but can be complemented with functional inner ear tests, including audiometry, vestibular-evoked myogenic potential testing, caloric testing, electrocochleography or head impulse tests. MRI has been optimized to directly visualize EH in the cochlea, vestibule and semicircular canals, and its use is shifting from the research setting to the clinic. The management of MD is mainly aimed at the relief of acute attacks of vertigo and the prevention of recurrent attacks. Therapeutic options are based on empirical evidence and include the management of risk factors and a conservative approach as the first line of treatment. When medical treatment is unable to suppress vertigo attacks, intratympanic gentamicin therapy or endolymphatic sac decompression surgery is usually considered. This Primer covers the pathophysiology, symptomatology, diagnosis, management, quality of life and prevention of MD.

Optimal management of Ménière's disease.

Confusion in the nomenclature of Ménière's disease and lack of a standard definition of the disorder until 1995 has hampered accurate assessment of treatment efficacy since the presently defined disorder was first described in 1938. The lack of a widely accepted mechanism of the disease has also delayed the development of rational treatments. Past treatments have focused on relieving elevated pressures in the hydropic ear and more recently on treatment of underlying migraine. Present dietary methods of control include sodium restriction and migraine trigger elimination. Pharmacologic treatments include diuretics, migraine prophylactic medications, histamine analogs, and oral steroids. Surgical procedures include intratympanic steroid perfusion, shunts, and ablative procedures when conservative treatments fail. External pressure devices are also used. Evidence of efficacy is lacking for most interventions other than ablation. At our institution, Ménière's disease is treated as a cerebrovascular disorder. Control of risk factors for cerebrovascular ischemia is combined with treatment of pressure dysfunction in the hydropic ear. Screening for risk factors is performed at presentation. Migraine, dyslipidemia, obesity, diabetes, sleep apnea, hypertension, and atherosclerosis are among the major factors that often require medical management. Migraine prophylactic medications, magnesium supplementation, sodium restriction, migraine trigger elimination, diuretics, anticoagulants, and antihypertensives are among the treatments used initially. Steroids administered orally or intratympanically are used if control is not achieved medically, and ablation remains the definitive treatment in unilateral cases experiencing treatment failure.

Endolymphatic hydrops in Ménière's disease: cause, consequence, or epiphenomenon?

OBJECTIVE: To determine the relationship of endolymphatic hydrops to Ménière's disease. DATA SOURCES: Comprehensive review of articles from 1938 through 2012 via Medline and Index Medicus. STUDY SELECTION: Articles discussing Ménière's disease and/or endolymphatic hydrops that include temporal bone autopsy data. DATA EXTRACTION: Fifty-three case reports and series were studied containing examination of 541 hydropic temporal bones and including 276 patients with Ménière's disease. These were divided into those meeting the 1995 American Academy of Otolaryngology-Head and Neck Surgery criteria for Ménière's disease and those that failed to meet these criteria. CONCLUSION: An individual meeting the 1995 criteria for Ménière's disease has a near certain probability of having endolymphatic hydrops in at least 1 ear. Autopsy data do not support the view that the association of MD and EH is an epiphenomenon or that MD causes EH; this leaves us with the probability that EH causes MD. If it is causative, hydrops alone is insufficient to cause Ménière's disease, indicating that there must be one or more additional cofactors that cause asymptomatic hydrops to become symptomatic Ménière's disease. Vascular risk factors should be studied as possible cofactors.

Canal conversion and reentry: a risk of Dix-Hallpike during canalith repositioning procedures.

OBJECTIVE: To determine the incidence, cause, and prevention of horizontal canal benign paroxysmal positional vertigo (H-BPPV) and reentry into the common crus during canalith repositioning procedures (CRPs). STUDY DESIGN: Prospective case series. SETTING: Academic tertiary referral center. PATIENTS: Forty-four patients with symptomatic posterior canalithiasis (P-BPPV) confirmed on Dix-Hallpike. INTERVENTION: Up to 5 CRP for initial P-BPPV and additional maneuvers for canal conversion or reentry in 1 session. MAIN OUTCOME MEASURES: Incidence and timing of onset of H-BPPV or common crus reentry and the outcome of treatment maneuvers. RESULTS: H-BPPV or reentry occurred during the Dix-Hallpike after a canal-clearing CRP and affected 16% (7/44) of subjects. Approximately 87% (7/8) of reentry or conversion events occurred after the very first CRP. Geotropic H-BPPV occurred in 9% (4/44) overall but in 13% (4/30) whose P-BPPV was resolved by a single CRP. All were cleared with H-BPPV maneuvers, 3 of 4 resolving with a single Gufoni maneuver. Approximately 9% (4/44) of patients developed reentry of particles into the common crus. It was treated by raising the patient to the upright position. By waiting 15 minutes after treatment of reentry, a final normal Dix-Hallpike was obtained without causing reentry into any canal in all patients. CONCLUSION: If the first CRP clears the posterior canal, there is an elevated risk of reentry or canal conversion if another Dix-Hallpike is performed immediately. These complications can be treated by additional maneuvers; if unfamiliar with these techniques, the risk can be reduced by waiting at least 15 minutes between repetitions of CRP.