Large vestibular aqueduct syndrome with high CT density and high MR signal intensity

We report a case of large vestibular aqueduct syndrome with a markedly dilated endolymphatic sac bilaterally. The density and signal intensity of the extraosseous portion of the sac were higher than those of cerebrospinal fluid on CT and MR studies. The findings may represent protein-rich and hyperosmolar fluid within the endolymphatic sac.     

Long-term hearing results following vestibular surgery in Meniere's disease

OBJECTIVES: The purpose of this study is to evaluate the long-term hearing changes following vestibular surgery in patients with Meniere's disease. Study DESIGN: This is a retrospective analysis of patients operated on in a tertiary referral center setting. METHODS: Preoperative, postoperative, and 3- to 9-year postoperative audiograms were analyzed in two patient groups. Twenty-one patients underwent posterior fossa vestibular neurectomy (VN) and five, mastoid endolymphatic sac decompression and shunt (ELS). All frequencies, four-frequency pure-tone averages (PTAs), spondee thresholds, and speech recognition scores were compared for operated ear against nonoperated ear of VN subjects. The results were subjected to a covariance analysis. VN and ELS patients whose hearing deteriorated from "serviceable" (PTA < or =70 dB hearing level) and speech recognition > or =30%) to nonserviceable status were compared using nonparametric statistics. RESULTS: Progressive hearing loss beyond the rate of change of the normal contralateral ear was evident in all patients. Serviceable hearing dropped from 81% to 43% of patients an average of 4 years following VN. CONCLUSIONS: VN patients have significant hearing deterioration over time in the operated ear. This finding suggests that continued postoperative medical management is necessary for patients undergoing VN.     

Predicting acute denervation in carpal tunnel syndrome

OBJECTIVE: Defects of the cochlear modiolus have been found to be associated with most cases of large vestibular aqueduct. The clinical significance of these modiolar defects has not been studied previously. The purpose of this article is to correlate clinical (functional) parameters, such as hearing outcomes, with the severity of the radiographic findings in these dysplastic inner ears. STUDY DESIGN: The study design was a retrospective chart review, supplemented with telephone interviews and clinic visits. SETTING: The study was conducted at an academic, tertiary care center. PATIENTS: Thirty consecutive patients with large vestibular aqueducts participated. RESULTS: Scores of modiolar deficiencies yielded inconsistent correlations with hearing loss. Vestibular aqueduct morphology and thickness correlated very strongly with the severity of hearing loss. CONCLUSIONS: These observations support the hypothesis that large vestibular aqueduct-related hearing loss may be caused by transmission of subarachnoid pressure forces into the inner ear. However, the thickness and morphology of the vestibular aqueduct may simply be markers for more subtle cochlear dysplasia manifest by modiolar deficiency.     

Macrolides in the management of streptococcal pharyngitis/tonsillitis

OBJECTIVE: This study aimed to compare the hearing changes in the long term after vestibular neurectomy, endolymphatic mastoid shunt, and medical treatment in classic Meniere's disease. STUDY DESIGN: A retrospective case review was conducted based on audiologic follow-up between 5 and 21 years. SETTING: The study was performed at two centers in Bari University Hospital, one performing vestibular neurectomy as the first surgical procedure for Meinere's disease and the other, endolymphatic mastoid shunt. PATIENTS AND INTERVENTIONS: Of 68 patients with intractable idiopathic Meniere's disease, 29 underwent middle fossa vestibular neurectomy, and 17 had endolymphatic mastoid shunt; 22 were offered surgery but declined. MAIN OUTCOME MEASURES: Outcome measures were puretone average (PTA), speech reception threshold, and speech discrimination score before and after treatment. RESULTS: PTA declined by an average of 9.3 dB in neurectomy patients, 13.3 dB in patients undergoing endolymphatic mastoid shunt, and 18.1 dB in patients who were offered surgery but declined. Patients were subdivided into two cohorts based on their preoperative or initial PTA. In the patients who had PTA scores worse than 50 dB initially, the PTA declined an average of 4.3 dB in the vestibular neurectomy group, 11.5 dB in the endolymphatic sac group, and 4 dB in the nonsurgical group. In the patients with PTA > or = 50 dB initially, the PTA declined an average of of 25.3 dB in the vestibular neurectomy group, 16.1 in the endolymphatic sac group, and 26.2 dB in the nonsurgical group. Although shunt patients with good hearing initially deteriorated less than neurectomy patients and less than patients who declined surgery, the difference was not significant. CONCLUSIONS: These results indicate that patients with poor hearing stabilized, while patients with good hearing continued to deteriorate. The same conditions were observed in the patients who had surgery and those who were offered surgery but declined.     

Hearing loss from hemodialysis

A 47-year-old man with normal hearing had chronic progressive renal failure. He experienced hearing loss in additive increments with peritoneal dialysis and hemodialysis, until he was profoundly deaf in both ears. Postmortem studies showed collapse of the endolymphatic system and edema and atrophy of most of the specialized cell types of the auditory and vestibular sense organs. It is our interpretation that the alterations were caused by osmotic disequilibrium associated with hemodialysis.     

Surgery for intractable vertigo: Chiang Mai experience

If medical therapy fails (6-12 months or more), careful consideration of surgical therapy should be followed with; the patient's hearing, severity of symptoms, age, and occupation. Cochleosacculotomy is reserved for elderly Meniere's patients with poor health, poor hearing and good vestibular function. Endolymphatic sac shunt should be considered as the first procedure for disabled Meniere's patients with aidable hearing and may also be used in those patients with bilateral Meniere's disease. Retrolabyrinthine vestibular nerve section (RLVNS) is indicated for patients with disabling vertigo and normal or aidable hearing. It could be done for all peripheral vertigo and for failed endolymphatic sac procedure. A destructive procedure, such as labyrinthectomy, should be a procedure of choice in a patient who presents with peripheral incapacitating vertigo and nonserviceable hearing loss in the solely affected ear. Again, patients with failed cochleosacculotomy, endolymphatic shunt then go on to receive either labyrinthectomy or RLVNS, based on residual hearing. To obtain a satisfactory surgical result, the surgeon must be precise in selecting the patient with the right disease at the right time.     

Endolymphatic sac tumors: report of three cases

OBJECTIVE AND IMPORTANCE: We present three cases of endolymphatic sac tumors and review the previously published cases. Despite frequent extension to the cerebellopontine angle, these rare tumors have only recently been recognized by neurosurgeons. CLINICAL PRESENTATION: A 26-year-old man developed a progressive hearing loss, revealing an intrapetrous retrolabyrinthine tumor on the right side. A 28-year-old woman experienced a left cerebellopontine angle syndrome, with a lytic intrapetrous mass extending into the cerebellopontine angle. A 38-year-old woman presented with an intracranial hypertension syndrome caused by a tumor of the jugular foramen. INTERVENTION: For the first and second patients, the tumors originated from the operculum of the endolymphatic sac. Total removal was achieved, via a transpetrosal approach, in these two cases. No recurrence was detected after a 20-month follow-up period. For the third patient, the tumor originated from the distal part of the sac. Recurrence was observed 8 years after subtotal removal via a retrosigmoid route. Histological analysis revealed a papillary-cystic adenocarcinomatous pattern in all cases, without features of aggressiveness. CONCLUSION: Endolymphatic sac tumors are locally invasive neoplasms characterized by bipolar intrapetrous and posterior fossa involvement. The anatomic complexity of the endolymphatic sac may explain the distinct patterns of extension of these tumors. Early radical surgery is related to good outcomes.     

Endolymphatic sac enhancement surgery in elderly patients with Ménière's disease

Ménière's disease is a pathologic condition of the inner ear that is characterized by vertigo, tinnitus and a progressive loss of hearing. When Ménière's disease is unresponsive to medical treatment and when destructive surgery is not advisable, patients, particularly the elderly, often benefit from endolymphatic sac enhancement, a conservative, nondestructive surgical procedure. We evaluated the outcomes of 62 such patients, aged 65 years and older, who underwent a total of 78 endolymphatic sac enhancements. We assessed their response to surgery by means of a questionnaire, which classified pre- and post-surgical data according to criteria established by the American Academy of Otolaryngology-Head and Neck Surgery. Of the 27 patients who returned questionnaires, 23 reported significant alleviation of vertigo symptoms and 19 said their hearing ability had either improved or was maintained at presurgical levels. Endolymphatic sac enhancement resulted in no mortality, and morbidity was documented in only one patient. We conclude that endolymphatic sac enhancement is a safe and viable treatment for elderly patients with Ménière's disease that is refractory to medical therapy.     

Submillimeter-resolution MR of the endolymphatic sac in healthy subjects and patients with Menière disease

PURPOSE: To evaluate the utility of submillimeter resolution MR imaging for direct depiction of functional soft-tissue components of the intraosseous endolymphatic duct and sac in healthy subjects and in patients with Menière disease. METHODS: Axial MR images were acquired of 14 patients with Menière disease and 14 healthy volunteers at 1.5 T with a short-echo-time steady-state 3-D gradient-echo sequence. Seven volunteers and eight patients were also studied with a T1-weighted 3-D spoiled gradient-echo sequence. T1/T2 relaxation times were estimated from studies with multiple flip angles. RESULTS: Independent of the acquisition method, intraosseous endolymphatic ducts and sacs were seen unambiguously in the ears of 20 of 21 healthy subjects but in only four of 12 asymptomatic and two of 10 symptomatic ears of patients with Menière disease. Other labyrinthine structures were well depicted in all subjects. Furthermore, shorter relaxation times were measured for the contents of the vestibular aqueduct than for other labyrinthine structures. CONCLUSION: In our high-resolution study, the intraosseous portions of the endolymphatic ducts and sacs were depicted in most of the healthy subjects. They were frequently not seen in either ear of patients with unilateral Menière disease, presumably because of their small size.     

Three familial cases of hearing loss associated with enlargement of the vestibular aqueduct

The present report describes three familial cases of recessive hearing loss associated with enlargement of the vestibular aqueduct (EVA). Six siblings from three families showed EVA. The common characteristic of these patients was the presence of congenital, high-frequency, fluctuating sensorineural hearing loss. These cases suggest that EVA may be a useful discriminator between different types of recessive hearing loss.     

Dexamethasone perfusion of the labyrinth plus intravenous dexamethasone for Ménière's disease

Recent clinical and laboratory evidence indicates that Meniere's disease is an immune-mediated disease. Dexamethasone perfusion of the inner ear through the round window plus intravenous dexamethasone often will stop the dizzy spells, reduce the fullness and low-frequency tinnitus, and sometimes improve the hearing in patients with Meniere's disease. The dexamethasone must act mostly on the endolymphatic sac and, to a lesser extent, on the stria vascularis and spiral ligament, the known targets of immune response in the inner ear, to reduce the endolymphatic hydrops and restore the fluid dynamics of the endolymph. Despite the good results with streptomycin perfusion, the number of patients with further hearing loss is large, so dexamethasone perfusion with intravenous dexamethasone should be tried first. The initial response to dexamethasone perfusion plus intravenous dexamethasone has been very good, with very little risk of further hearing loss, and it holds great promise for the future.     

Suitable fixation condition for rat liver using microwave irradiation and its application to the immunohistochemistry of glutamate dehydrogenase

In 50 patients auditory threshold and brain stem evoked potential studies were carried out before and after myelography. Due to the analysis of amplitudes and latencies of auditory brain stem measurements, significant functional disorders of the hearing organ and the auditory pathway could be demonstrated. In most of the patients these functional disorders were found to be subclinical, whereas 12 patients showed alterations extending from a subjectively slight hearing loss to an audiometrically objectified acute hearing loss depending on its intensity in each case. The reasons of these functional disorders could not be clarified. An open cochlear aqueduct through which perilymph enters the subarachnoidal space leading to a secondary endolymphatic hydrops can be considered as the cause in cases where manifest symptoms develop. The changes in brain stem audiometry can be additionally explained by changes in osmolality of the inner ear fluids which may lead to the development of an endolymphatic hydrops.     

Middle ear pressure and dysfunction of the labyrinth: is there a relationship?

Relationships between middle ear pressure and non-infection-related cochleovestibular dysfunction have been suggested by several authors. According to some data, vertiginous attacks can be prevented by the insertion of a ventilation tube in patients suffering from Meniere's syndrome. The aim of our study was to investigate if the incidence of eustachian tube malfunction and pathologic middle ear pressure is frequent, and if routine implantation of ventilation tubes is reasonable in ears with dysfunctions of the labyrinth, including clinical Meniere's syndrome. So, we determined in our pressure chamber all active and passive parameters of eustachian tube function in 40 patients suffering from Meniere's syndrome, sudden sensory hearing impairment (SSHI), or vestibular neuronitis. Our results disclosed no nonrandom incidence of impaired tubal function among our patients compared to healthy control subjects. Pressure equalization was sufficient in most patients suffering from clinical Meniere's syndrome, and only one patient with vestibular neuronitis presented with a patulous tube. Our results show that impairment of vestibular or cochlear function is not regularly accompanied by eustachian tube dysfunction. Furthermore, no patient reported symptoms while pressure variation was performed. We conclude that variation of middle ear pressure does not usually play a role in the genesis of Meniere's syndrome, vestibular neuronitis, or SSHI. Thus, from our data, we cannot recommend routine implantation of tympanic ventilation tubes in patients suffering from Meniere's syndrome, vestibular neuronitis, or sudden hearing loss.     

Vestibular nerve section: long-term follow-up

Vestibular nerve section is considered an effective modality in the treatment of refractory and incapacitating vertigo. Typically nerve section results are described on the basis of short-term follow-up. We have reviewed 41 cases of vestibular nerve section spanning an 18-year period. Although the majority of cases involved classic Meniere's disease, delayed endolymphatic hydrops, vestibular neuritis, and Meniere's syndrome secondary to head trauma were also included. Surgical approaches included translabyrinthine (20 cases), retrolabyrinthine (14 cases), retrosigmoid (six cases), and middle fossa (one case) procedures. Postoperative follow-up time averaged 102 months, with 46% of patients followed for a minimum of 9 years. Results are reported according to standards set forth by the American Academy of Otolaryngology--Head and Neck Surgery. Vertigo was cured or markedly improved in 88% of cases (90% in patients with Meniere's disease) at 18 to 24 months postoperatively. These vertigo results were sustained at the time of latest follow-up. Functional level was also preserved over time despite the development of bilateral symptoms in several cases. The rate of bilateral disease reached 22% of cases. Although vertigo results remained stable, long-term follow-up of successful hearing preservation cases demonstrated deterioration over time. Postoperative continuation of medical treatment is urged to optimize and sustain the vestibular neurectomy result.     

Sudden deafness and Barlow disease

From January 1993 to December 1994 twelve patients were evaluated for sudden hearing loss. The median age was 49 years with a range of 18 to 71. All had severe or profound initial hearing loss. The incidence of bilateral disease was 25%. Total deafness occurred in five (33%) ears. Nine (75%) patients had vestibular symptoms and eight (67%) admitted experiencing tinnitus. Two-dimensional echocardiography revealed mitral prolapse in eight (67%) patients; another patient showed moderate to severe ischemic left ventricular dysfunction with apical aneurysm.     

Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging

PURPOSE: To introduce aplasia or hypoplasia of the vestibulocochlear nerve (VCN) as a possible cause of hearing loss and to identify the magnetic resonance (MR) imaging characteristics of this entity. MATERIALS AND METHODS: In seven patients with congenital deafness or unexplained sensorineural hearing loss, MR imaging enabled diagnosis of aplasia or hypoplasia of the VCN. Axial (0.7-mm) three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) images and parasagittal reconstruction images perpendicular on the course of the VCN were obtained. Twenty normal inner ears were also studied; their findings were compared with those of the patients. RESULTS: The facial nerve and inferior and superior vestibular and cochlear branches of the VCN were identified on the MR images in the 20 normal inner ears. Aplasia of the VCN was detected in two patients with normal labyrinths but with a severe stenosis of the internal auditory canal. A common VCN with absence of the cochlear branch was found bilaterally in two patients with a congenital malformation of the labyrinth. A common VCN with absence or hypoplasia of the cochlear branch was found in three patients with normal internal auditory canals and labyrinths. CONCLUSION: Submillimetric gradient-echo images (eg, 3DFT-CISS) should always be used to exclude aplasia or hypoplasia of the cochlear branch of the VCN in all cochlear implant candidates and patients with congenital deafness. This entity, which can occur with or without associated labyrinthine malformation, should be confirmed in two planes.     

Further characterization of the DFNA1 audiovestibular phenotype

BACKGROUND: Autosomal dominant, nonsyndromic, hereditary hearing impairment in a large Costa Rican kindred is caused by a mutation in the human homolog of the Drosophila diaphanous gene. OBJECTIVE: To further characterize the phenotype of DFNA1 with comprehensive audiovestibular evaluation and computed tomography of the temporal bone. PATIENTS: One affected child and 2 affected adults of the Costa Rican kindred who harbor a mutation in the diaphanous gene. SETTING: Medical Center at the University of California, San Francisco. INTERVENTION: Otologic and neuro-otologic examination; pure tone audiometry, speech audiometry, and immitance testing; auditory evoked potentials, electrocochleography, and otoacoustic emissions; electronystagmography and vestibular autorotation tests; and computed tomography of the temporal bone. RESULTS: The youngest subject, an 8-year-old boy, had a mild hearing loss, intact stapedial reflexes, otoacoustic emissions at high frequencies, normal auditory evoked potentials, and electrocochleographic findings consistent with endolymphatic hydrops. The two adults had severe to profound bilateral sensorineural hearing impairment. Electronystagmography disclosed normal vestibular function. Computed tomography demonstrated normal external, middle, and inner ear structures. CONCLUSIONS: These results suggest that the early low-frequency hearing loss in this family is associated with endolymphatic hydrops. Elucidation of the role of the diaphanous gene in hearing will therefore lead to a better understanding of the mechanism of endolymphatic hydrops.     

Sudden sensorineural hearing loss associated with anticardiolipin antibody

We experienced three female patients with sudden sensorineural hearing loss. All the patients showed elevation of anticardiolipin antibody in the serum. Immunoglobulin (Ig) M anticardiolipin antibody was present in case 1 (a 34-year old woman). IgG anticardiolipin antibodies were present in cases 2 (a 50-year-old woman) and 3 (a 9 year old girl). The patient in case 1 showed hearing disturbance of low tone in the left ear and normal vestibular function in both ears. The case 2 patient had an average hearing level of 81.7dB at 0.5. 1, and 2kHz and severe canal paresis in the right ear. In case 3 the patient showed an average hearing level of 53.3dB in the right ear and normal vestibular function in both ears. It was suggested that thrombosis which might have been induced by anticardiolipin antibody could have caused the sudden sensorineural hearing loss in these patients. Steroid therapy cured the hearing disturbance completely in case 1. Steroid and prostaglandin E1 therapy did not improve the hearing disturbance in case 2. Steroid, prostaglandin E1, and ticlopidine hydrochloride therapy improved the hearing disturbance in case 3. It appears that not only steroids but also prostaglandin E1 and ticlopidine hydrochloride therapy may be effective in patients with sensorineural hearing loss associated with anticardiolipin antibody.     

A partial area difference analysis for estimating elimination rate constants and distribution volumes of metabolites

A prospective, randomized study was carried out comparing the effect of two surgical modalities in the treatment of patients with Meniere's disease: insertion of an endolymphatic sac shunt and insertion of a ventilating tube in the tympanic membrane. A total of 29 patients, 12 males and 17 females, age 27-71 years, were operated on in two ear, nose and throat (ENT) departments. Of these patients, 15 had an endolymphatic shunt inserted and 14 had a ventilating tube inserted in the tympanic membrane. Postoperative follow-up was carried out in the department in which the patients had not been operated. The severity of the disease was scored pre- and postoperatively, and the results evaluated under the guidelines of the Committee on Hearing and Equilibrium (1995) for the diagnosis and evaluation of therapy in Meniere's disease. The patients in both groups had a statistically significant reduction in dizzy spells, measured 6 and 12 months postoperatively, and there was no difference between the groups. The pathophysiological explanation for the reduction in dizzy spells in each of the treatment modalities is debatable and the effect is non-specific. The patients' hearing and tinnitus were statistically unaffected by the treatment in both groups, though 2 patients in the shunt group developed severe hearing loss (anacusis/70 dB).