Blood-Labyrinth Barrier Permeability in Menière Disease and Idiopathic Sudden Sensorineural Hearing Loss: Findings on Delayed Postcontrast 3D-FLAIR MRI.

BACKGROUND AND PURPOSE: Menière disease and idiopathic sudden sensorineural hearing loss can have overlapping clinical presentation and may have similar pathophysiology. Prior studies using postcontrast 3D-FLAIR MR imaging suggest abnormal blood-labyrinth barrier permeability in both conditions, but the 2 diseases have not been directly compared by using the same imaging techniques. We hypothesized that delayed postcontrast 3D-FLAIR MR imaging would show differences in blood-labyrinth barrier permeability between Menière disease and idiopathic sudden sensorineural hearing loss. MATERIALS AND METHODS: Patients with unilateral Menière disease (n = 32) and unilateral idiopathic sudden sensorineural hearing loss (n = 11) imaged with delayed postcontrast 3D-FLAIR MR imaging were retrospectively studied. Signal intensities of the medulla and perilymph of the cochlear basal turns of both ears in each patient were measured in a blinded fashion. Cochlea/medulla ratios were calculated for each ear as a surrogate for blood-labyrinth barrier permeability. The ears were segregated by clinical diagnosis. RESULTS: Cochlea/medulla ratio was higher in symptomatic ears of patients with Menière disease (12.6 ± 7.4) than in patients with idiopathic sudden sensorineural hearing loss (5.7 ± 2.0) and asymptomatic ears of patients with Menière disease (8.0 ± 3.1), indicating increased blood-labyrinth barrier permeability in Menière disease ears. The differences in cochlea/medulla ratio between symptomatic and asymptomatic ears were significantly higher in Menière disease than in idiopathic sudden sensorineural hearing loss. Asymptomatic ears in patients with Menière disease showed higher cochlea/medulla ratio than symptomatic and asymptomatic ears in patients with idiopathic sudden sensorineural hearing loss. CONCLUSIONS: Increased cochlea/medulla ratio indicates increased blood-labyrinth barrier permeability in Menière disease compared with idiopathic sudden sensorineural hearing loss. Increased cochlea/medulla ratio in asymptomatic ears of patients with Menière disease also suggests an underlying systemic cause of Menière disease and may provide a pathophysiologic biomarker.

Endolymphatic Hydrops Reversal following Acetazolamide Therapy: Demonstration with Delayed Intravenous Contrast-Enhanced 3D-FLAIR MRI.

Endolymphatic hydrops, the primary pathologic alteration in Menière disease, can be visualized by using delayed intravenous contrast-enhanced 3D-FLAIR MR imaging. It is not known whether MR imaging-demonstrable changes of hydrops fluctuate with disease activity or are fixed. We describe the results of baseline and posttreatment MR imaging studies in a group of subjects with Menière disease with hydrops who were treated with acetazolamide. Seven subjects with untreated Menière disease with MR imaging evidence of hydrops had repeat MR imaging during acetazolamide treatment. Symptoms and imaging findings were assessed at each time point. Five subjects showed symptom improvement, of whom 3 had improvement or resolution of hydrops. One subject had recurrent symptoms with recurrent hydrops after discontinuing therapy. Two had unchanged hydrops despite symptom improvement. Subjects with unchanged symptoms had unchanged hydrops. Hydrops reversal may be seen with acetazolamide treatment in Menière disease. MR imaging may provide an additional biomarker of disease.

The relevance of migraine in patients with Menière's disease.

CONCLUSION: Coexistent migraine affects relevant clinical features of patients with Ménière's disease (MD). OBJECTIVE: Epidemiological studies have shown an association between migraine and MD. We sought to determine whether the coexistence of migraine affects any clinical features in patients with MD. PATIENTS AND METHODS: In this retrospective case-control study of University Neurotology Clinic patients, 50 patients meeting 1995 AAO-HNS criteria for definite MD were compared to 18 patients meeting the same criteria in addition to the 2004 IHS criteria for migraine (MMD). All had typical low frequency sensorineural hearing loss and episodes of rotational vertigo. Outcome measures included: sex, age of onset of episodic vertigo or fluctuating hearing loss, laterality of hearing loss, aural symptoms, caloric responses, severity of hearing loss, and family history of migraine, episodic vertigo or hearing loss. RESULTS: Age of onset of episodic vertigo or fluctuating hearing loss was significantly lower in patients with MMD (mean +/- 1.96*SE = 37.2 +/- 6.3 years) than in those with MD (mean +/- 1.96*SE = 49.3 +/- 4.4 years). Concurrent bilateral aural symptoms and hearing loss were seen in 56% of MMD and 4% of MD patients. A family history of episodic vertigo was seen in 39% of MMD and 2% of MD patients.

Temporal bone histopathology in dominantly inherited audiovestibular syndrome.

OBJECTIVE: To describe the clinical and pathologic features of a new dominantly inherited audiovestibular syndrome. METHODS: History, examination, and audiometric testing in the proband, brother, and son; quantitative rotational testing in the proband and son; histopathology of the cochlea and vestibular labyrinth in the proband; sequencing candidate genes COCH and MYO7A in the brother and son. RESULTS: Affected family members developed slowly progressive hearing loss beginning in their late 30s and progressive imbalance in their early 70s. Three of four affected had brief (minutes) episodes of vertigo typically occurring a few times per year. Auditory and vestibular function testing documented a slowly progressive loss of auditory and vestibular function. Postmortem examination showed a loss of hair cells in the cochlea and vestibular receptor organs. There were no cellular infiltrates or acidophilic deposits. No mutations were found in the COCH or MYO7A genes. CONCLUSIONS: This dominantly inherited audiovestibular syndrome results in a selective loss of hair cells in the auditory and vestibular end organs. Finding the causative gene could have important implications for understanding the pathophysiology of presbycusis and dysequilibrium of aging.

Drop attacks in older patients secondary to an otologic cause.

The clinical features and treatment of seven patients with drop attacks attributable to inner ear disease presenting after age 65 are described. A neurologic or cardiovascular cause of drop attacks was initially suspected. Audiovestibular testing documented a unilateral inner ear disorder. The salient clinical features of these cases are discussed. The patients underwent ablative vestibular surgery, and all compensated well and were free of vertigo and falls up to 10 years postoperatively.

Differential subcellular immunolocalization of voltage-gated calcium channel alpha1 subunits in the chinchilla cristae ampullaris.

The immunohistochemical localization of alpha1A, alpha1B, alpha1C, alpha1D and alpha1E voltage-gated calcium channel subunits was investigated in the chinchilla cristae ampullaris and Scarpa's ganglia at the light and electron microscopy level with the use of specific antipeptide antibodies directed against these subunits. The stereocilia membrane of type I and type II hair cells was immunoreactive for alpha1B along its entire length. The basolateral membrane of both types of hair cells was alpha1B, alpha1C and alpha1D immunoreactive. Neurons in the Scarpa's ganglia and afferent nerve terminals in the cristae were immunoreactive for alpha1C and alpha1B. No specific immunoreactivity to alpha1A or alpha1E was seen in the sensory epithelia or ganglia. These findings are consistent with the presence of alpha1B (N-type channel), alpha1C and alpha1D (L-type channels) in the vestibular hair cells, and alpha1B (N-type channel) and alpha1C (L-type channel) in primary vestibular neurons.

Subcellular immunolocalization of NMDA receptor subunit NR-1 in the chinchilla vestibular periphery.

The immunohistochemical localization of N-methyl-D-aspartate (NMDA) glutamate receptor subunit, NR-1 was investigated in the chinchilla cristae ampullaris and utricular maculae at the light and electron microscopy level with the use of specific antipeptide antibodies. The afferent calyces that innervate type I hair cell, and the basolateral type I vestibular hair cell is NR-1 immunoreactive. The afferent boutons innervating type II hair cells and the basal portion of type II hair cell are NR-1 non-immunoreactive. These findings are consistent with NMDA receptor mediation of afferent excitatory neurotransmission from type I, but not type II hair cells to the primary afferent vestibular nerve. The NMDA receptors on the type I hair cell are located in areas of synaptic specialization, and may play a role in autoregulation. The localization of the NMDA receptor subunit in type I but not type II hair cells is intriguing.

Histopathology of idiopathic chronic recurrent vertigo.

Vestibular neuritis is a degenerative neuropathy of the peripheral vestibular system. The etiology of this condition is uncertain, although it is generally believed to be viral. A small percentage of patients with vestibular neuritis have chronic recurrent episodes of vertigo. Detailed cytologic descriptions of acute or chronic vestibular neuritis are lacking, and no previous studies have reported evidence of chronic inflammation in human temporal bone specimens. The authors of this study examined temporal bone specimens from three patients with a history of chronic recurrent vertigo of unknown cause. Varying degrees of inflammation and destruction were seen in the vestibular system, and mild involvement of the cochlear system was noted. These findings are consistent with postinfectious inflammatory changes of the cochlear-vestibular system analogous to a postinfectious syndrome involving the central nervous system.