Contralateral Delayed Endolymphatic Hydrops Research Articles

A 14-year nationwide epidemiological analysis of delayed endolymphatic hydrops in Japan

Background Delayed endolymphatic hydrops (DEH) is an inner ear disease that causes recurrent vertigo in the ipsilateral ear or fluctuating hearing in the contralateral ear due to endolymphatic hydrops secondary to preceding deafness. There are few reports of large, multicentre studies investigating the clinical-epidemiological characteristics of DEH. Objective This study aimed to clarify the characteristics of DEH in Japan. Methods Clinical data on 662 patients with DEH were analysed by nationwide, multicentre surveys conducted by the Peripheral Vestibular Disorders Research Group of Japan. Results The proportion of ipsilateral DEH (IDEH) was slightly higher than that of contralateral DEH (CDEH) at 55.4%. The time delay between onset of precedent deafness and onset of DEH was significantly longer for CDEH than for IDEH. The most common cause of precedent deafness was a disease of unknown cause with onset in early childhood (33.1%). Epidemiological characteristics were not significantly different between CDEH with and without vertigo. Conclusion DEH appearing to be caused by viral labyrinthitis has a high rate of onset within 40 years of precedent deafness. Clinical and epidemiological characteristics of IDEH, CDEH with vertigo, and CDEH without vertigo were very similar. Significance The clinical-epidemiological characteristics of DEH in Japan were clarified.

Dissociation of Caloric and Video Head Impulse Tests in Patients With Delayed Endolymphatic Hydrops.

Delayed endolymphatic hydrops (DEH) represents a rare clinical entity characterized by intermittent vertigo attacks mimicking those of Ménière's disease (MD) in a patient with a prior sensorineural hearing loss. Some vestibular tests have been employed in patients with DEH. These tests provide useful diagnostic information and facilitate clinical decision-making. Here, we retrospectively studied the features of video head impulse test (vHIT) and examined its relationship with caloric test used in DEH patients. Included in this study were 17 patients with ipsilateral DEH and 2 with contralateral DEH. Among them, 73.7% (14/19) showed abnormal caloric test response (76.5% in ipsilateral DEH and 50% in contralateral DEH). Meanwhile, only 15.8% (3/19) of patients yielded abnormal horizontal vHIT results (11.8% in ipsilateral DEH and 50% in contralateral DEH). Abnormal caloric response in the presence of a preserved vHIT was common in DEH patients, especially those with ipsilateral DEH. This dissociation might be a distinctive pattern of vestibular deficit in DEH.

Bilateral delayed endolymphatic hydrops evaluated by bilateral intratympanic injection of gadodiamide with 3T-MRI.

The purpose of this study was to assess the diagnostic performance of 3T MRI after intratympanic injection of gadodiamide for delayed endolymphatic hydrops (DEH), and assess the relationship between endolymphatic hydrops (ELH) and vestibular function in patients diagnosed with DEH and confirmed by 3T MRI. Nineteen patients clinically diagnosed with DEH (11 ipsilateral DEH, 8 contralateral DEH) participated in this study. Diluted gadodiamide was administered to the bilateral tympanic cavity by injection through the tympanic membrane. At 24 hours post-injection, the ELH was evaluated by MRI. Patient vestibular functions were evaluated by caloric testing and cVEMP. ELH was observed in all patients (19/19: positive rate 100%). The distribution patterns of ELH varied between the cochlear or vestibular region. Vestibular ELH was observed in the affected ear in all ipsilateral DEH patients. In the contralateral DEH patients, however, there were individual differences in the distribution patterns of ELH. Six patients (1 ipsilateral DEH, 5 contralateral DEH) had bilateral ELH. No obvious relationships were observed between ELH and vestibular function. ELH distribution was complicated, particularly in the contralateral DEH cases. It was difficult to identify the existence of ELH by vestibular functional testing alone; therefore, 3T MRI is thought to be useful for identifying the affected ear. A significant number of cases had “bilateral” DEH, particularly among the contralateral DEH cases, indicating that we should pay careful attention to this pathology when treating DEH.

Delayed endolymphatic hydrops. Special emphasis on nystagmus associated with episodes and contribution of chemical labyrinthectomy

ObjectivesThe main objective was to describe spontaneous nystagmus characteristics during an episode of delayed endolymphatic hydrops (DEH), including an initial vertical upbeating nystagmus in one patient. The secondary objective was to highlight the contribution of chemical labyrinthectomy. MethodsEpisodic vertigo after a prolonged period of time of sensorineural hearing loss (profound or total) in one ear characterized ipsilateral DEH and was associated with the development of hearing loss in the opposite ear in contralateral DEH. ResultsTen patients met the criteria for DEH: 7 ipsilateral and 3 contralateral. Three (all ipsilateral DEH) were examined during a vertigo episode. Two patients had a typical horizontal-torsional nystagmus beating contralaterally to the hearing loss. One patient showed atypic initial vertical upbeating nystagmus with a slight torsional component, which secondarily became horizontal-torsional beating contralaterally to the hearing loss. Four patients had disabling vertigo with unilateral total deafness (ipsilateral DEH), successfully treated by 1-3 transtympanic gentamycin (Gentalline®) injections. ConclusionNystagmus direction during vertigo episodes varies, and may initially present as vertical upbeating nystagmus, which, to our knowledge, has not been previously reported in DEH or Menière's disease. This nystagmus might reflect an inhibition of the superior semicircular canal (on the hearing-impaired side), suggesting incipient hydrops in this canal. Chemical labyrinthectomy is a simple and effective procedure in unilateral DEH, especially as the patient often suffers from total deafness.

Contralateral type of delayed endolymphatic hydrops may consist of two phenotypes based on a magnetic resonance imaging preliminary study

Objective: This study was performed to determine the volume distribution of EH in contralateral DEH.Patients and methods: Five contralateral DEH patients (age range = 21–77 years) and one ipsilateral DEH patient. Visualization of in vivo EH using 3T magnetic resonance (MR) imaging was performed by intravenous administration of gadolinium. The EH volume was determined quantitatively by adding the area of EH in consecutive MR images.Results: The total EH volume of the patients with contralateral DEH varied from 24.2 to 56.6 μL in the first ear and 20.2 to 35.7 μL in the contralateral ear. EH was dominantly developed in the first ear for Patient no. 3 and 4, and not dominantly developed in the first ear for Patient no. 1, 2, and 5.Conclusions: The volume distribution of endolymphatic hydrops (EH) in contralateral delayed endolymphatic hydrops (DEH) was classified into two phenotypes. This suggests that contralateral DEH may consist of two etiologies.

Gadolinium-enhanced inner ear magnetic resonance imaging for evaluation of delayed endolymphatic hydrops, including a bilateral case

Conclusion The data suggests that gadolinium-enhanced inner ear MR imaging is useful for diagnosis of delayed endolymphatic hydrops (DEH) because it is independent of inner ear function, and the size of the affected endolymphatic space is clearly enlarged. Objective This study was performed to semi-quantitatively evaluate the endolymphatic space in patients with all types of DEH using gadolinium-enhanced inner ear magnetic resonance (MR) imaging. Patients and methods Seven patients (age range = 21–77 years; five female, two male) with ipsilateral DEH (n = 5), contralateral DEH (n = 1), and bilateral DEH (n = 1). All patients underwent 3T MR imaging 4 h after intravenous injection of gadolinium. Software was used to determine the size of the endolymphatic space. Pure tone audiometry and caloric testing using an electronystagmogram were carried out. Results One side of the endolymphatic space was dominantly extended in patients with ipsilateral DEH, and both sides of the space were extended in patients with contralateral and bilateral DEH. In patients with ipsilateral DEH, the volume ratio of endolymph to vestibule was 2.5–4.3-times that in the unaffected ear. The volume ratio of endolymph to vestibule was nearly equal in patients with contralateral and bilateral DEH.

Intratympanic injection in delayed endolymphatic hydrops

Conclusions: The present study showed that intratympanic dexamethasone injection (ITD) is a promising approach for the treatment of contralateral and ipsilateral delayed endolymphatic hydrops (DEH). Moreover, intratympanic gentamicin injection (ITG), as a chemical labyrinthectomy, is a simple alternative for controlling vertigo in patients with ipsilateral DEH. Objective: This study examined the effect of ITD or ITG on DEH. Methods: Fourteen patients with DEH completed the clinical and audio-vestibular evaluation. Among them, 10 cases (ipsilateral type: nine cases, contralateral type: one case) were treated with intratympanic injection. Four patients with ipsilateral DEH underwent ITG, five patients with ipsilateral type and one patient with contralateral type received ITD. All 10 cases were followed up for 8–48 months. Results: Complete and substantial vertigo control was achieved in four of nine ipsilateral DEH patients treated with ITG. In the other five ipsilateral cases who received ITD, two accomplished complete vertigo control and two had substantial control. In one case, the vertigo was not effectively controlled. One case of contralateral DEH underwent ITD and this case had complete vertigo control. The vertigo intensity, vertigo frequency, vertigo duration and the functional level scale after intratympanic injection was decreased significantly.

Long-term effects of the Meniett device in Japanese patients with Meniere's disease and delayed endolymphatic hydrops reported by the Middle Ear Pressure Treatment Research Group of Japan

Conclusion: The Meniett device is a minimally invasive and safe treatment that may be used to provide longer-term reduction of vestibular symptoms in patients with delayed endolymphatic hydrops (DEH) as well as those with Meniere's disease (MD). Objective: The effects of the Meniett device were evaluated in patients with a diagnosis of definite MD or DEH according to the 1995 AAO-HNS criteria. Methods: Twenty-nine ears of 28 patients with MD and 5 ears of 5 patients with DEH (ipsilateral type 4, contralateral type 1) were treated with the Meniett device by the Middle Ear Pressure Treatment Research Group of Japan. All of the patients had failed to respond to medical treatment including diuretics before the pressure treatment. Results: Sixteen (57%) patients with MD and all five (100%) patients with DEH remained entirely free from vertigo spells; nine (32%) patients with MD responded with a significant decrease in the frequency of vertigo spells. In regard to hearing, 25 ears (74%: MD, n = 21; ipsilateral DEH, n = 4) had stable hearing levels; only 4 ears (12%: MD, n = 3; contralateral DEH, n = 1) showed a significant hearing improvement. No complications were attributable to the Meniett device.

Endolymphatic space imaging in patients with delayed endolymphatic hydrops

Conclusion: Magnetic resonance imaging (MRI) after intratympanic gadolinium injection can reveal endolymphatic hydrops (ELH) in patients with delayed ELH (DELH). Patients with contralateral DELH may have bilateral ELH. Objective: DELH has previously been diagnosed based on clinical history, hearing and vestibular examinations. DELH is classified into three types: ipsilateral, contralateral and bilateral indicate the side with the longstanding hearing loss. Ipsilateral DELH occurs in the ear with a profound hearing loss, contralateral DELH in the better hearing ear and bilateral DELH in both ears. Imaging diagnosis of the endolymphatic space may add a new dimension to the diagnosis and treatment of DELH. Patients and methods: Gadodiamide hydrate was diluted eightfold with saline. The diluted gadodiamide hydrate was injected intratympanically through the tympanic membrane in two patients with ipsilateral DELH and five patients with contralateral DELH. One day after the injection, 3 Tesla MRI was performed to evaluate the endolymphatic space. Results: ELH was observed in all patients. In three patients who underwent bilateral intratympanic injection of gadolinium and were diagnosed with contralateral DELH, ELH was observed bilaterally. In one of these three patients, ELH was observed in the cochlea on the left and in the vestibule on the right.

Delayed Endolymphatic Hydrops: Study and Review of Clinical Implications and Surgical Treatment

Delayed endolymphatic hydrops (DEH) differs from Ménière's disease in that it occurs in pre-existing ear pathology in patients who have a profound unilateral or total deafness that was caused by infection, trauma, or unknown causes during childhood or adulthood. We performed a retrospective review of 160 patients with ipsilateral, contralateral, or bilateral DEH. Eighty-seven patients who did not respond to medical therapy underwent surgical treatment. Our findings indicate that the more conservative surgical procedures--endolymphatic sac surgery, cochleosacculotomy, and streptomycin perfusion--are all as effective as and less destructive than labyrinthectomy for controlling vertigo. The clinical results of this study would seem to support the observations of others that DEH and Ménière's disease are related disorders caused predominantly by cases of viral labyrinthitis with unknown etiology.

Ménière's Disease and Delayed Endolymphatic Hydrops in Children

In order to study the characteristics of pediatric patients (≤ 15 years at onset) with endolymphatic hydrops disorders, we selected 5 patients with Ménière's disease (MD) and 3 patients with delayed endolymphatic hydrops (DEH) from 7418 patients who had visited the Neurotological Clinic of the Toyama Medical and Pharmaceutical University Hospital between 1979 and 1999. Clinical features were characterized as follows: (i) pediatric MD patients were rare, comprising ≈ 1.5% of all the 337 MD patients in the sample; (ii) a fluctuating hearing loss, strong positive response in glycerol test, high SP/AP amplitude ratio in electrocochleography and moderate positive response in furosemide vestibulo-ocular reflex test were recorded; (iii) in 2 of the 5 pediatric MD patients, vertiginous attacks had persisted for a long period and they had undergone surgical procedures (sac operation and vestibular neurectomy); and (iv) the ipsilateral type of DEH was observed in 2/3 children, and contralateral DEH in 1/3.

Clinical features in patients with delayed endolymphatic hydrops

We report clinical features in patients with delayed endolymphatic hydrops (DEH) with juvenile unilateral deafness. Among 23 patients with DEH, 15 cases were diagnosed as ipsilateral DEH and 8 cases as contralateral DEH. The distribution of onset age showed two peaks at ages of < 30 years and > 40 years. In 80% of the ipsilateral DEH cases, the onset of episodic vertigo was at younger ages. On the other hand, in 75% of the contralateral DEH cases, the onset of fluctuation hearing loss of the contralateral ear was at older ages. Ispilateral DEH and Meniere's disease may show different pathophysiologies. The incidence of dominant negative summating potential in the better-hearing ear was 20% in the ispilateral DEH cases and 60% in the contralateral DEH cases. It is suggested that endolymphatic hydrops is in the better-hearing ear of contralateral DEH.

遅発性内リンパ水腫例の検討

The cause of delayed endolymphatic hydrops (DEH) has not been elucidated. Especially in contralateral DEH, it remains controversial whether the fluctuation of hearing loss and repetition of vertigo is due to hearing disturbance in the contralateral ear.We diagnosed 8 patients as the ipsilateral type of DEH and 5 patients as the contralateral type. We investigated the clinical manifestations of DEH and the manner of occurrence of hearing loss and vertigo. With regard to preexisting severe hearing loss, the contralateral type of DEH differed only slightly from the ipsilateral type. The contralateral type showed repeated vertigo less frequently than the ipsilateral type. When occurrence was investigated in patients with the contralateral type, we supposed that it was possible for some cases diagnosed as the ipsilateral type to shift to the contralateral type. On serologic and immunologic examination, all of patients with the contralateral type showed antinuclear antibodies.

Electrocochleography in the Diagnosis of Delayed Endolymphatic Hydrops

It is known that delayed endolymphatic hydrops (DEH) occurs in patients with unilateral and/or bilateral profound hearing loss. In the ipsilateral and bilateral types of DEH, no audiological tests can be performed on the affected ear because of profound deafness, although these tests can be done in the contralateral type. Electrocochleography (ECochG) was recorded on better hearing ears in both types of DEH. The results showed an increased negative summating potential (SP) in 6 out of 10 contralateral DEH patients whereas it could be measured in only one of 10 ipsilateral DEH patients. The mean SP/AP ratio of the contralateral type was 0.42 which was significantly larger than that of the ipsilateral type. The results suggest that contralateral-type DEH has the same pathology in the cochlea as Meniere's disease, and that ECochG can distinguish between these two types of DEH.

Contralateral Delayed Endolymphatic Hydrops in a Patient with Diabetes Mellitus

A case of fluctuating hearing loss related to contralateral delayed endolymphatic hydrops (DEH) was first described Schuknecht in 1978. Our patient was a 63-year-old male with diabetes mellitus who developed fluctuating hearing loss and was found to have contralateral DEH. He had had severe hearing loss in the left ear following otitis media when he was six years old. Since the age of 53, he had occasionally noticed fluctuating hearing loss in the right ear along with episodic vertigo of Meniere's type.After admission to our hospital, the audiograms of his right ear revealed periodically-fluctuating hearing loss without vertigo. His hearing in the right ear fluctuated diurnally ; his hearing depression was severe in the morning, improved at noon and became severe again in the evening. Because he had diabetes mellitus, his blood glucose levels were serially determined. It was interesting that the fluctuations of his blood glucose levels preceded those of his hearing in the right ear by approximately three hours.

Clinical Findings of Delayed Endolymphatic Hydrops

Schuknecht (1985) classified delayed endolymphatic hydrops (DEH) into three types, ipsilateral, contralateral and bilateral. The concept of DEH has been generally accepted; however, there is a problem that contralateral DEH might be unilateral Meniere's disease, accidentally appearing in on the ear opposite to that with severe deafness. In this paper the characteristics of contralateral DEH are compared with ipsilateral DEH and unilateral Meniere's disease.In the past 9 years 20 patients with DEH, 10 ipsilateral and 10 contralateral, were selected from among all the patients who had visited our clinic in the same period for a retrospective investigation of unilateral and bilateral severe deafness. Patients with unilateral Meniere's disease in the ear opposite to that with early onset of severe deafness, were included in the contralateral DEH. Three cases with contralateral DEH had no vertigo or dizziness with fluctuating hearing loss. Four patients with contralateral DEH had episodic vertigo; sensorineural hearing loss, which was of sudden onset in three, appeared from one to 10 years before the fluctuating hearing loss. Thus, in seven patients with contralateral DEH, cochlear disorders were the only symptom of DEH or preceded the onset of DEH. This tendency is different from the nature of the onset of Meniere's disease. In six of the nine patients with contralateral DEH (66%), endolymphatic hydrops was detected by the glycerol test, the ECochG and/or the furosemide VOR. Because not all patients were tested with all three examinations, the percentage of those with endolymphatic hydrops was lower than in those with Meniere's disease in our clinic (90%). In the four patients, who were tested with all three examinations, however, endolymphatic hydrops were demonstrated, There-fore we concluded that the number of findings pointing to endolymphatic hydrops in contralateral DEH was not very different from that in Meniere's disease.These results suggest that the characteristics of contralateral DEH may be different from those of Meniere's disease although both diseases have the same pathogenesis : endolymphatic hydrops.

対側型遅発性内リンパ水腫の聴力変動について

We treated 42 patients with delayed endolymphatic hydrops (DEH) : 33 ipsilateral, 7 contralateral, and 2 bilateral. We conducted a clinical study of the 7 patients with contralateral DEH.Patients with contralateral DEH have a profound hearing loss in one ear, and eventually develop symptoms of endolymphatic hydrops in the opposite ear (only hearing ear) -fluctuating hearing loss with or without vertigo-, which is a very serious problem not only for the patient but also for otologist.The cause of the profound hearing loss in our 7 cases was head trauma, otitis media and mumps (1 case each), and juvenile unilateral deafness of unknown etiology in 4.The chief complaint was fluctuating and progressive hearing loss in the better hearing ear in 6, and rotatory vertigo caused by the normal hearing ear in 1.In the diagnosis of contralateral DEH, the clinical history is most important and the glycerol and furosemide tests are very useful.In the treatment of contralateral DEH diuretics and steroids have been employed. In those who failed to respond to conservative therapy, epidural shunt operation on the endolymphatic sac was performed in our clinic. The treatment was effective in many cases, but in some cases hearing loss progressed in spite of the intensive therapy. In the patients with advanced hearing loss already in the better hearing ear, it is very difficult to perform an operation because of the relatively high risk of making the hearing worse. We have tried γ-globulin therapy in some patients who showed a poor response to conventional therapy. This therapy sometimes yielded good results. The action of the γ-globulin on the inner ear and the endolymphatic sac is still unknown. But we believe that we should always strive to search for a more effective conservative therapy as well as trying to be more skillful in surgical management.