A Pediatric Case of Cogan's Syndrome With Internal Otitis.

Cogan's syndrome is characterized by ocular symptoms and auditory vestibular dysfunction. Auditory vestibular dysfunction in Cogan's syndrome is believed to be similar to Ménière's disease, but the cause is not known in detail. We present the case of a 10-year-old boy with Cogan's syndrome. The patient had panuveitis, bilateral hearing loss, and bilateral vestibular dysfunction. MRI revealed no evidence of endolymphatic hydrops, which is a cause of Ménière's disease, and enhanced contrast effects on the bilateral cochlear and vestibular apparatus. The caloric test, the video-head impulse test, and the vestibular evoked muscle potential test also showed severe vestibular dysfunction. Based on the above, the auditory vestibular dysfunction in this patient was considered to be caused by internal otitis. The patient's vision recovered after treatment with steroids and immunosuppressive drugs, but his hearing did not recover. He underwent bilateral cochlear implantation and had a good postoperative course but we encountered difficulty in deciding when to perform cochlear implantation. This case demonstrates the importance of determining the timing of surgery in consideration of the ossification and fibrosis of the inner ear and the drug administration status.

New non-contrast MRI of endolymphatic hydrops in Ménière's disease considering inversion time.

Objectives: Three-tesla MRI with gadolinium-based contrast agents is important in diagnosing Ménière's disease. However, contrast agents cannot be used in some patients. By using the compositional difference between the inner ear endolymph and perilymph, we performed basic and clinical research focused on potassium ions and protein to find the optimal parameters for visualizing endolymphatic hydrops on MRI without contrast. We then examined the relationship between severity stage and visualization rate of endolymphatic hydrops. Methods: In phantom experiments simulating the endolymph and perilymph, we explored MRI parameters that could be used to separate endolymph from perilymph by gradually changing the inversion time. We then used these parameters to perform both new non-contrast MRI and contrast MRI on the same day in Ménière's disease patients, and we compared the visualization rates of endolymphatic hydrops under the two modalities. Fifty patients were selected from 478 patients with Ménière's disease of different severity stages; 12 patients had asthma and allergy to contrast agents. Results: The higher the disease stage, the higher the endolymphatic hydrops visualization rate. The new non-contrast MRI gave significantly higher (p < .01) visualization rates of endolymphatic hydrops on the affected side in patients at Stage 3 or above than in Stages 1 and 2 combined. Conclusion: New non-contrast MRI with parameters focusing on the endolymph-perilymph difference in the density of protons surrounding the potassium ions and protein can produce images consistent with endolymphatic hydrops. We believe that this groundbreaking method will be useful for diagnosing Ménière's disease in patients. Evidence Level: Clinical studies are at evidence level 3 in non-randomized controlled trials.

Perilymphatic fistula with characteristic findings of the inner ear by contrast-enhanced magnetic resonance imaging: a case report.

A perilymphatic fistula (PLF) presents with abnormal traffic in the otic capsule, causing cochlear and vestibular symptoms. However, the mechanisms underlying symptom recurrence remain controversial. Herein, we report the case of a 27-year-old female who complained of hearing disturbance in her right ear and recurrent vertigo after sudden onset of hearing loss with vertigo. The caloric test revealed unilateral weakness in the right ear, and the video head impulse test (vHIT) showed decreased vestibulo-ocular reflex (VOR) gain. Contrast-enhanced magnetic resonance imaging (MRI) using hybrid of reversed image of positive endolymph signal and negative image of perilymph signal (HYDROPS) indicated a collapsed endolymphatic space. As the vestibular symptoms did not improve, an exploratory tympanotomy was performed on the right ear. Although perilymph leakage was not noted in the oval or round windows, both windows were sealed with connective tissue. The patient's vestibular symptoms rapidly improved after surgery, and postoperative contrast-enhanced MRI showed improvement in the collapsed endolymphatic space. Although the caloric test revealed unilateral weakness, the VOR gain on the vHIT improved to normal on the right side. Thus, these findings indicated that recurrent symptoms caused by PLF are associated with a collapsed endolymphatic space. We speculate that the collapsed endolymphatic space was due to a ruptured Reissner's membrane. We hypothesized that sealing the fistula would promote normalization of perilymph pressure. The ruptured Reissner's membrane may have been gradually repaired as vestibular symptoms improved. This case adds to the existing literature on the occurrence of the "double-membrane break syndrome". Collapse of the endolymph due to a ruptured Reissner's membrane may be the cause of PLF symptoms.

Relationship Between the Onset of Ménière's Disease and Sympathetic Hyperactivity.

Objective: The pathogenesis of Ménière's disease is still largely unknown; however, it is known to be strongly associated with stress. Excessive stress can cause hyperactivity of the sympathetic autonomic nervous system. With the aim of understanding changes in sympathetic hyperactivity before and after Ménière's disease, we compared autonomic nervous function in patients in a stable phase of Ménière's disease and that in healthy adults. We also gathered data over about 10 years on autonomic nervous function immediately before a Ménière's attack. Study Design: Prospective study. Patients: Autonomic nervous function was analyzed in 129 patients in a stable phase of Ménière's disease 31 healthy adult volunteers. In nine patients, autonomic nervous function was also measured immediately before and after treatment of a vertigo attack. Main Outcome Measure: Power spectrum analysis of heart rate variability (HRV) of EEG/ECG and an infrared electronic pupillometer were used. Sympathetic and parasympathetic nervous function was measured. Results: There were no statistically significant differences in autonomic nervous function determined by HRV and electronic pupillometry between patients in a stable phase of Ménière's disease and healthy adults. Sympathetic function as measured by electronic pupillometry parameters VD and T5 showed no difference between the affected and unaffected sides in the baseline data measured in the stable phase (VD: affected side is 31.02 ± 6.16 mm/sec, unaffected side is 29.25 ± 5.73 mm/sec; T5: affected side is 3.37 ± 0.43 msec, unaffected side is 3.25 ± 0.39 msec). In contrast, all nine patients whose HRV data had been obtained just before an attack showed marked suppression of the parasympathetic nervous system and activation of the sympathetic nervous system. Electronic pupillometry also revealed an overactivation of the sympathetic nervous system on the affected side, just before the attacks. Analysis of sequential changes after the onset of an attack revealed that overactivation on the affected side was reduced after treatment, and no difference between affected and unaffected sides was observed 3 days after treatment. Conclusion: Detailed analysis of autonomic nervous function showed that immediately before an attack of Ménière's disease, the sympathetic nervous system on the affected side was strongly overactivated.

Effects of using cane and vestibular rehabilitation on the walking function in elderly patients with dizziness.

OBJECTIVE: Adults over the age of 65 years with balance disorders are at about twice the risk of falls, compared with those without balance disorders. Falls contribute to about 74% of the proximal femoral fractures commonly seen in the elderly. Since balance disorders are more prevalent in older adults than in younger adults, it is important to deal with balance disorders in older adults to prevent falls and the resulting deterioration in their ADL (activity of daily living). In this study, we investigated the effects of vestibular rehabilitation (VR) and cane use on improving gait and balance in patients aged over 65 years with balance disorder. METHODS: Patients aged over 65 years presenting to the Department of Otolaryngology at St. Marianna University School of Medicine between July 1 and November 1, 2018, with symptoms of dizziness for ≥ 3 months and a Japanese translation of the Dizziness Handicap Inventory score of ≥ 26 were included in the study. We quantitatively analyzed their gait before and after VR, and with and without the use of a cane. RESULTS: A total of 21 patients participated in the study (14 women; mean age 73.9 ± 6.9 years). Before VR, using a cane made no difference to step length or walking speed. After VR, using a cane increased step length from 50.5 cm (95% confidence interval [CI], 47.4-53.7 cm) to 52.0 cm (95% CI, 48.9-55.1 cm) (p = 0.039). There was no change in walking speed. A comparison of walking assessment results while using a cane before and after VR showed that step length increased from 49.9 cm (95% CI, 46.6-53.2 cm) to 52.0 cm (95% CI, 48.9-55.1 cm) (p = 0.005), and walking speed increased from 90.5 cm/s (95% CI, 82.7-98.4 cm/s) to 96.1 cm/s (95% CI, 88.3-103.9 cm/s) (p = 0.005). CONCLUSIONS: Walking speed and step length with the use of a cane significantly improved following VR. VR and cane use may act synergistically to improve walking.