Relationship between auditory pathway fractional anisotropy and audibility in patients with cerebellopontine angle tumors on diffusion tensor imaging.

OBJECTIVE: Recent reports demonstrated that acoustic nerve disorders affect the auditory pathway on diffusion tensor imaging (DTI). The aim was to investigate whether auditory pathway fractional anisotropy (FA) values are associated with audibility in patients with cerebellopontine angle tumors. PATIENTS AND METHODS: Patients with cerebellopontine angle tumors were included in this retrospective study. Preoperatively, all patients underwent magnetic resonance imaging (MRI) including DTI. Two regions of interest on the lateral lemniscus (LL) and inferior colliculus (IC) were set bilaterally on DTI. FA values were calculated using software. Correlations between FA values and audibility were evaluated using Spearman's rank correlation coefficient. Statistical significance was defined as p < 0.05. RESULTS: Seventeen patients with cerebellopontine angle tumors were included in this study. FA values in the bilateral LL showed a significant negative correlation with hearing impairment severity (r = -0.758, -0.600, p < 0.05). FA values on the ipsilateral side of the IC showed a significant negative correlation with hearing impairment severity (r = -0.477, p < 0.05). FA values on the contralateral side of the IC did not correlate with hearing impairment severity (r = -0.201, p > 0.05). One patient with a low FA value on the contralateral side of the IC had postoperative hearing impairment despite good preoperative hearing ability. CONCLUSIONS: FA values in the bilateral LL and on the ipsilateral side of the IC reflected hearing impairment severity. Decreased FA values on the contralateral side of the IC might predict hearing impairment postoperatively.

Diagnostic evaluation of neck torsion test in objective examination in patients with vertigo and/or hearing impairment.

INTRODUCTION: The aim of the study was to evaluate the neck torsion test in objective examinations of patients with vertigo and/or hearing loss. MATERIAL AND METHODS: The study was conducted in 100 patients, including 54 women and 46 men aged 17-79 years, who were divided into two groups: I - 50 patients, including 30 women and 20 men aged 17-79 years (mean age 49.92 years) with dizziness and/or hearing impairments, and confirmed asymmetry of intracranial vessels, II - 50 patients - the control group, including 24 women and 26 men aged 20-71 years without dizziness and/or hearing disorders and without disturbance in the construction of intracranial vessels. For each patient, the following tests were carried out: subjective, objective otorhinolaryngological, Doppler ultrasound specifying the diameter of vertebral and carotid arteries and the velocity of blood flow in these vessels, audiological diagnostics, including the examination of latency of waves I, III, V of the auditory evoked potentials of the brain stem, otoneurological diagnostics with used the neck torsion test. RESULTS: It appears from the analysis of the material presented that the application of the neck torsion test in the Doppler ultrasound results in the fact that the difference in the mean systolic velocity of blood flow in vertebral artery is higher on the side opposite to the turning of the neck, and the increase in the average diastolic blood flow velocity in the vertebral artery on the side of the test being performed and its reduction on the opposite side in the study group, when compared to the control group. The value of the wave I, II, V latency in the ABR test during the neck torsion test is extended more in the study group than in the controls, on the side of the performed test. The performed neck torsion test in the VNG test increases the occurrence of both, square waves and nystagmus (much higher in the study group than in the controls). CONCLUSION: The application of the neck torsion test in the Doppler ultrasound, ABR and VNG test in patients with vertigo and/or hearing loss means that these tests become functional, thereby increasing their diagnostic value and may be used to monitor the rehabilitation of inner ear disorders.

Salicylate-induced frequency-map reorganization in four subfields of the mouse auditory cortex.

Salicylate is the active ingredient in aspirin, and in high-doses it is used as an experimental tool to induce transient hearing loss, tinnitus, and hyperacusis. These salicylate-induced perceptual disturbances are associated with tonotopic-map reorganization and neural activity modulation, and such neural correlates have been examined in the central auditory pathway, including the auditory cortex (AC). Although previous studies have reported that salicylate induces increases in noise-burst-evoked neural responses and reorganization of tonotopic maps in the primary AC, little is known about the effects of salicylate on other frequency-organized AC subfields such as the anterior auditory, secondary auditory, and dorsomedial fields. Therefore, to examine salicylate-induced spatiotemporal effects on AC subfields, we measured sound-evoked neural activity in mice before and after the administration of sodium salicylate (SS, 200 mg/kg), using flavoprotein auto-fluorescence imaging. SS-treatment gradually reduced responses driven by tone-bursts with lower (≤8 kHz) and higher (≥25 kHz) frequencies over 3 h, whereas evoked responses to tone-bursts within middle-range frequencies (e.g., 12 and 16 kHz) were sustained and unchanged in the four subfields. Additionally, in each of the four subfields, SS-treatment induced similar reorganization of tonotopic maps, and the response areas selectively driven by the middle-range frequencies were profoundly expanded. Our results indicate that the SS-induced tonotopic map reorganizations in each of the four AC subfields were similar, and only the extent of the activated areas responsive to tone-bursts with specific frequencies was subfield-dependent. Thus, we expect that examining cortical reorganization induced by SS may open the possibility of new treatments aimed at altering cortical reorganization into the normative functional organization.

Diagnostic evaluation of neck torsion test in objective examination in patients with vertigo and/or hearing-impairment.

INTRODUCTION: The aim of the study was to evaluate the neck torsion test in objective examinations of patients with vertigo and/or hearing loss. MATERIAL AND METHODS: The study was conducted in 100 patients, including 54 women and 46 men aged 17-79 years, who were divided into two groups: I - 50 patients, including 30 women and 20 men aged 17-79 years (mean age 49.92 years) with dizziness and/or hearing impairments, and confirmed asymmetry of intracranial vessels, II - 50 patients - control group, including 24 women and 26 men aged 20-71 years without dizziness and/or hearing disorders and without disturbance in the construction of intracranial vessels. For each patient, the following tests were carried out: subjective, objective otorhinolaryngological, Doppler ultrasound specifying diameter of vertebral and carotid arteries and the velocity of blood flow in these vessels, audiological diagnostics, including the examination of latency of waves I, III, V of the auditory evoked potentials of the brain stem, otoneurological diagnostics with used the neck torsion test. RESULTS: It appears from the analysis of the material presented that the application of the neck torsion test in the Doppler ultrasound results in the fact that the difference in the mean systolic velocity of blood flow in vertebral artery is higher on the side opposite to the turning of the neck, and the increase in the average diastolic blood flow velocity in the vertebral artery on the side of the test being performed and its reduction on the opposite side in the study group, when compared to the control group. The value of the wave I, II, V latency in the ABR test during the neck torsion test is extended more in the study group than in the controls, on the side of the performed test. The performed neck torsion test in the VNG test increases the occurrence of both, square waves and nystagmus (much higher in the study group than in the controls). Conclussion. The application of the neck torsion test in the Doppler ultrasound, ABR and VNG test in patients with vertigo and/or hearing loss means that these tests become functional, thereby increasing their diagnostic value and may be used to monitor the rehabilitation of inner ear disorders.

The evaluation of the neck torsion test in subjective examinations of patients with vertigo and/or hearing loss.

INTRODUCTION: The aim of this work was to evaluate the diagnostic value of the neck torsion test in VNG, Doppler ultrasonography and brainstem auditory evoked potentials in patients with vertigo and/or hearing loss due to intracranial vascular malformations. MATERIAL AND METHODS: The study covered 47 patients, 30 female and 17 male (mean age, 55.5 years; range, 19-74 years) with vertigo and/or hearing disorders and the asymmetry of vertebral arteries. Each patient underwent a subjective examination, an otolaryngological examination, otoneurological diagnostics, VNG with gaze tracking in the straight ahead position and in the 600 left and right neck torsion, the neck torsion test, audiological diagnostics including I-, III- and V-wave latency of the brainstem evoked potentials in the straight ahead position and the right ear stimulation in the 600 right neck torsion and the left ear stimulation in the neck torsion to the left, Doppler ultrasonography with measuring the diameter of vertebral arteries and the velocity of the blood flow in these vessels with the use of the neck torsion test. RESULTS: In own study, in VNG, the positive neck torsion test was observed in 76.5% of the study patients, while square waves in both directions were found in 46.5% and in one direction in 10.6%. Cervical nystagmus was noticed in 19.1% of these patients. In the auditory evoked potentials test, the differences in I-, III- and V-wave latency time were not statistically significant, either at rest or in the neck torsion. In the Doppler ultrasound examination, the asymmetry of vertebral arteries were present (below 25%) in 7 women (14.9%) and 4 men (8.5%), whereas large asymmetries (above 25%) were observed in 23 women (48.9%) and 13 (27.7%) men (range, 25% - 215%) and was statistically insignificant. The resting blood flow velocity in vertebral arteries of large asymmetries, both in systole and diastole heart phases, was significantly higher in the artery with larger asymmetry. CONCLUSION: The neck torsion test can be diagnostically useful in monitoring the vertebrobasilar system as well as qualify for microsurgical procedures if the vertebrobasilar insufficiency has been diagnosed.

The relationship between jugular bulb position and jugular bulb related inner ear dehiscence: a retrospective analysis.

OBJECTIVE: High jugular bulb (HJB) can erode inner ear structures creating a jugular bulb related inner ear dehiscence (JBID). The aim of this study was to analyze the relationship between the position of jugular bulb (JB) and JBID using high-resolution computed tomography (HRCT). MATERIAL AND METHODS: In this retrospective study HRCT images of 552 ears of 276 patients with hearing loss, otogenic vertigo, tinnitus or idiopathic peripheral facial nerve paralysis were analyzed. HJB type-1 was defined when JB dome reached above the inferior part of the round window, but was below the inferior edge of the internal acoustic meatus (IAM). HJB type-2 was defined when the dome of JB was higher than the inferior edge of IAM. The frequencies and types of HJB were evaluated. JBID for each HJB type was determined. Frequencies of JBID eroding the vestibular aqueduct (VA), the cochlear aqueduct and the posterior semicircular canal were examined. RESULTS: HJB type-1 and HJB type-2 were found in 19% (105/552) and in 15.8% (87/552) of studied ears. JBID showed to be in 3.8% (21/552) of all ears. 90.5% (19/21) of JBID revealed eroding of VA. Jugular bulb related cochlear aqueduct dehiscence and jugular bulb related posterior semicircular canal dehiscence were found in one ear each. The frequency of jugular bulb related vestibular aqueduct dehiscence (JBVAD) in patients with HJB reaching above IAM was higher than in patients with HJB lower than IAM. CONCLUSIONS: HJB is common, but JBID is rare. JBID prevalently erodes VA. HJB rising above IAM is most at risk to show JBVAD.

Aplasia of the cochlea: radiologic assessment and options for hearing rehabilitation.

OBJECTIVE: Hearing rehabilitation of patients with severe inner ear malformations remains controversial. Our objective was to describe the radiologic findings of aplasia of the cochlea (AC) and evaluate the existing therapeutic options in such patients. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Pediatric and adult patients with AC evaluated at our institution from 1995 to 2010. INTERVENTIONS: The precise radiologic findings were identified using high-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) scans of the inner ear. In cases of auditory implantation on the AC side, the achieved outcome was recorded using categories of auditory performance (CAP). Related surgical aspects were analyzed. RESULTS: Twenty-three patients (28 ears) with AC were found. In 5 patients AC was bilateral. The remaining unilateral cases had contralateral normal ears (2 patients), cochlea hypoplasia (5 patients), common cavity (6 patients), incomplete partition Type I (4 patients), and atresia of the internal auditory canal (1 patient). Four patients (3 bilateral, 1 unilateral ACs) were treated with cochlear implants in ears with AC, and 1 patient underwent auditory brainstem implantation. All implanted patients achieved speech perception with limited vocabulary (CAP scores between 4 and 5). CONCLUSION: AC is defined as the total absence of the cochlea, with a present, although malformed, vestibule. Although a distinct auditory nerve was not seen in these cases of AC, results following cochlear implantation suggest functional cochlear nerve fibers in the remaining dysplastic inner ear structures. In selected cases, cochlear implantation may be a reasonable option for the habilitation of deafness associated with AC.

[Vertigo and ear discomfort caused by a bone defect in os temporalis]. / Svimmelhed og øregener forårsaget af knogledefekt i temporalbenet.

Superior semicircular canal dehiscence is a clinical condition where the bone tissue overlying the superior semicircular canal is absent. The symptoms can be nonspecific and include vertigo, oscillopsia and conductive hearing loss. An audiogram will reveal an air-bone-gap in the lower frequencies in an otherwise healthy ear. Diagnosis is confirmed by performing muscular evoked myogenic potentials examination and temporal bone computed tomography. A case of right side superior semicircular canal dehiscence in a 52-year-old woman and the diagnostic course is described.

Improvement in autophony symptoms after superior canal dehiscence repair.

OBJECTIVE: Autophony, or the unusually loud or disturbing sound of a patient's own voice, can be a prominent and disabling symptom of superior canal dehiscence (SCD) syndrome. The current study measures autophony symptoms before and after SCD plugging to quantify the benefits of surgery. STUDY DESIGN: Patients undergoing SCD plugging between September 2007 and October 2008 completed a questionnaire before and 3 months after surgery. The questionnaire consisted of 26 statements to assess the disability caused by the sound of the patient's own voice. Patients graded each item on a scale from 0 (never) to 4 (almost always) to how often they noted a symptom or experience. Typical statements included "hearing my voice has interfered with my ability to work" and "hearing my voice has caused me to avoid social situations." An autophony index (AI) was generated to grade patient symptoms. SETTING: Tertiary referral center. PATIENTS: Nineteen adults with SCD. INTERVENTION: Superior canal dehiscence plugging via a middle fossa approach. OUTCOME MEASURES: Change in AI. RESULTS: Preoperatively, the mean AI was 42 +/- 27 (mean +/- SD; range, 0-86; 1 patient had no autophony symptoms). Postoperative AI decreased 89% to 9 +/- 22, a significant (p < 0.01) decline. Of the 18 patients with preoperative autophony, 13 had complete postoperative resolution. In 3 remaining patients, the AI decreased but did not resolve. One of these had bilateral SCD with contralateral autophony. One patient's mild autophony remained unchanged, and another patient with coexisting patulous eustachian tube AI increased after SCD plugging. CONCLUSION: In patients with significant autophony symptoms, SCD plugging improved 94% of patients. A simple 5-item AI is provided that will be useful in grading autophony symptoms.

Spectrum of hearing disorders and their management in children with CHARGE syndrome.

OBJECTIVE: The CHARGE syndrome is associated with ear anomalies and deafness in addition to other malformations. Deformations of the ossicles or aplasia of the semicircular canals, cochlear hypoplasia, hypoplasia or aplasia of the VIIIth cranial nerve and abnormal routing of the VIIth cranial nerve, sigmoid sinus, and emissaries are typical findings. The aim of this study is to explore the feasibility and procedure of cochlear implantation in patients with CHARGE syndrome and to assess the outcome. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center; cochlear implant program. PATIENTS: Ten patients with CHARGE syndrome and 3 patients with CHARGE-like syndrome treated in our center due to hearing impairment. Eleven patients were congenitally deaf, 1 patient had progressive hearing loss, and 1 patient had mixed hearing loss. INTERVENTION: Computed tomography of temporal bones and magnetic resonance imaging of the brain; bone-anchored hearing aid surgery, cochlear implantation, rehabilitation results. MAIN OUTCOME MEASURES: Surgical suitability and hearing rehabilitation. RESULTS: We illustrate the management of preoperative diagnostics, surgical planning, and hearing rehabilitation. One patient with mixed hearing loss underwent bilateral bone-anchored hearing aid surgery. Because 2 patients had bilateral aplasia of the auditory nerves, we recommended an auditory brainstem implant. The unilateral cochlear implantation was performed in 9 patients and bilateral in 1 patient. In selected cases, it was helpful to plan the operation using a simulator for temporal bone surgery. Complex malformations, such as in CHARGE syndrome, with an increased intraoperative risk for complications should be facilitated by using intraoperative digital volume tomography-assisted navigation and intraoperative digital volume tomography control of electrode position. The results after CI surgery vary due to the differing extent of additional disabilities such as developmental delay, intellectual delay, and visual impairment. Nine of our patients showed improved responsiveness with the cochlear implant. Open speech comprehension could not be observed in 8 patients, whereas the follow-up period was less than 1 year in 4 patients. The relatively high age of our patients at implantation might be an important factor. CONCLUSION: Careful planning of the treatment of CHARGE syndrome patients with sensorineural hearing loss can, to a limited extent, lead to auditory benefit without increasing surgical complications. Cochlear implantation is therefore indicated after critical assessment.

The circulatory influence on development of age-related macular degeneration and hearing and equilibrium impairments.

This study attempts to answer the question if any level of head and neck circulation takes a part in development of Age-Related Macular Degeneration (ARMD) and hearing and equilibrium impairments. Condition of large blood vessels was examined by Color-Doppler ultrasound, and carotid and ophthalmic arteries were included. The microcirculatory changes were examined directly by fundus photography and fluorescein angiography and indirectly testing hearing and equilibrium. The study group included 40 patients (21 females, 19 males) aging from 53 to 84 years with different stages of ARMD. The control group included 40 patients (18 females, 22 males) aging from 51 to 82 years without ARMD. Patients were inhabitants of Primorsko-Goranska County. There was no relationship between ARMD and condition of large blood vessels because significant stenosis of carotid arteries was found in 2 patients (5%) in study group and 3 patients (7.5%) in the control group (p > 0.05). On the contrary, we found correlation between ARMD and hearing (p = 0.0127) and equilibrium impairments (p = 0.0242). Fluorescein angiograms shows raised number of ischemic retinal capillaries in patients with ARMD (p = 0.0053). Results lead to conclusion that circulatory disorders on microcirculatory level take a great part in development of ARMD and hearing and equilibrium impairments in the elderly. The key is damage of sensory cells of the retina and inner ear caused by microcirculatory disorders. Interesting data was noticed that 9 patients with more serious ARMD on one side of head had greater hearing loss on the same side. If we find a new treatment for microcirculatory disorders, maybe we can treat both sensory impairments in earlier stage.

Three-dimensional computed tomography imaging of the eustachian tube lumen in patients with patulous eustachian tube.

BACKGROUND: The eustachian tube (ET) has a complicated anatomy, which is related to middle ear pathology. The anatomy of the ET has been well investigated in cadavers, but may not accurately reflect that in living subjects. Three-dimensional (3D) computed tomography (CT) was used to examine the ET in patients with patulous ET. METHOD: The subjects were 35 patients (17 males, aged 59 +/- 16 years, and 18 females, aged 44 +/- 15 years) with patulous ET which could be continuously traced from the pharyngeal orifice to the tympanic orifice (53 ears). CT was performed in the sitting position at rest, followed by 3D reconstruction. The course of the ET from the middle ear to the pharynx was visualized in 3D surface images. Morphological measurements of the curvature angle (angle C) and the inclination angle (angle I) were determined from 3D coordinates on the CT images. RESULTS: The full length of the ET could be visualized in most patients suffering from patulous ET in the sitting position. Angle C was 21.7 +/- 5.8 degrees in the patients aged <40 years (n = 20), which is significantly larger than 16.7 +/- 6.8 degrees in the patients aged >59 years (n = 18; Student's t test, p < 0.05). Angle I was 26.5 +/- 4.2 degrees in the patients aged <40 years (n = 20), also significantly larger than 21.6 +/- 5.8 degrees in the patients aged >59 years (n = 18; Student's t test, p < 0.05). CONCLUSION: CT in the sitting position is a potential diagnostic tool of the ET and middle ear pathology.

X-linked stapes gusher: CT findings in one patient.

A 31-year-old male patient presented with progressive mixed hearing loss since birth. A stapedectomy was attempted and was unsuccessful because of perilymph gushing. CT of the temporal bones showed bulbous dilatation of the fundi of the internal auditory canals and absence of the bone plates separating them from the base of the cochleas. This unusual abnormality was found after the attempted stapedectomy and explains the clinical findings. The findings in male patients are fairly typical X-linked congenital deafness.

Linear accelerator-based radiosurgery for vestibular schwannoma.

Despite major advances in skull base surgery and microsurgical techniques, surgery for vestibular schwannoma (VS) carries a risk of complications. Some are inherent to general anesthesia and surgery of any type and include myocardial infarction, pneumonia, pulmonary embolism, and infection. Some are specific to neurosurgery in this area of the brain, and include hydrocephalus, cerebrospinal fluid leak, facial nerve paralysis, facial numbness, hearing loss, ataxia, dysphagia, and major stroke. Even in the hands of very experienced acoustic surgeons, these risks cannot be eliminated. Radiosurgery provides an outpatient, noninvasive alternative for the treatment of small acoustic schwannomas. Initially radiosurgery was undertaken in "high-risk" patients, including the elderly, those with severe medical comorbidities, and those in whom tumors recurred after surgery. Additionally, a high rate of cranial nerve morbidity was reported. With improvements in dosimetry planning and dose selection, however, authors practicing at radiosurgical centers now report very low complication rates, as well as high tumor control rates. In this report the authors specifically review the results of linear accelerator-based radiosurgery for VS and compare these outcomes with the best surgical alternatives.

Preservation of functional hearing after gamma knife surgery for vestibular schwannoma.

OBJECT: The goal of this retrospective study was to define the rates of preservation of functional hearing and growth control of vestibular schwannomas (VS) treated by gamma knife surgery (GKS) involving a consistent 12-Gy prescription dose. METHODS: One hundred thirty-four patients with unilateral VS underwent GKS between 1994 and 2000. The mean magnetic resonance (MR) imaging follow-up period was 31.7 months (maximum 72 months), and the mean audiometry follow-up interval was 26.3 months (maximum 60 months). The mean marginal dose was 12 +/- 0.6 Gy. The mean maximum dose delivered to the tumor center was 25.4 Gy (range 17.4-34.3 Gy). The tumor control rate, defined as no change or a reduction in size at last follow up, was 96.7%. Of the patients studied, 97.7% remained free from the need to undergo tumor resection. Overall functional hearing preservation was 61.7%; the preservation rate for intracanalicular tumors was 63.6%, for those with an intracranial diameter less than 1.5 cm it was 54.5%, for those between 1.5 and 3 cm it was 68.2%, and for those larger than 3 cm it was 33.3%. Early in the series, three patients (2.2%) developed temporary facial weakness (House-Brackmann Grade II-III) in the posttreatment period, but this resolved within a few weeks. No case of facial weakness occurred after 1996. CONCLUSIONS: The authors demonstrated the efficacy, safety, and in many ways, the advantage of GKS over microsurgery for VS. Patients harboring tumors 3 cm or smaller in intracranial diameter, regardless of their age and medical condition, should be given the option of undergoing GKS as primary treatment.

Minute findings of inner ear anomalies by three-dimensional CT scanning.

In actual clinical situations, inner ear anomalies must be diagnosed by image diagnosis such as high resolution CT-scanning of the temporal bone before three dimensional (3D) CT-scanning was introduced. In this paper, the usefulness of 3D-CT was investigated in some anomaly cases. It was found that 3D-CT was useful in observing the minute structure of the inner ear in that it could ascertain spatial relationships and minute constrictions and protrusions that could not be detected by 2D analysis. This CT was also capable of assessing the stage of embryological injuries and evaluating anomalies in cochlear turning that are the hidden factors of hearing impairment.

Cochlear implants in children.

Cochlear implants allow the rehabilitation of children with severe to profound hearing loss. They are beneficial for not only postlingual children with hearing loss but also for children with congenital or prelingual hearing loss. Issues regarding cochlear implant candidacy and surgery are discussed. The results of cochlear implants in children and the complications related to cochlear implant surgery in children are reviewed.