摘要:

ObjectiveThis study aimed to demonstrate the temporal bone histopathology of Cornelia de Lange syndrome.Study DesignHistopathologic examination of an infant with this syndrome.SettingThis study was performed at the temporal bone laboratory, Fukushima Medical University, Fukushima.ResultsThe authors describe interesting temporal bone findings of this syndrome: anomalies of the middle and inner ear and the facial nerves.ConclusionsThis is the second case report describing the histologic findings of the temporal bone in Cornelia de Lange syndrome in the English literature.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

HypothesisThe goal of the investigation was to determine if vector analysis of nystagmus in a patient with the Tullio phenomenon could determine the source of the nystagmus.BackgroundThe Tullio phenomenon consists of the combination of vertigo and abnormal eye and/or head movements provoked by sound. Dehiscence of the superior semicircular canal can be found in certain patients with the Tullio phenomenon.MethodsThe patient was tested with pure tones ranging from 250 to 3000 Hz at 95dB HL. The time course of the three-dimensional vector of eye movement, including torsion and vertical and horizontal displacement angles was determined by individual stop-frame analysis of digitized video.ResultsTorsion amplitude varied from 1 to 7 degrees; vertical amplitude varied from 1 to 5 degrees; and horizontal amplitude varied less than 1.5 degrees. The maximal response occurred on stimulation of the right ear with a 1250-Hz 95-dB HL tone. This elicited a reliable counterclockwise torsional and down-beating fast phase nystagmus as seen from the examiner's point of view. Comparison of the nystagmus with known canal vectors identified the right superior semicircular canal as the source of stimulation. High-resolution computed tomography scan of the temporal bone showed a definite right superior canal dehiscence.ConclusionThe origin of nystagmus from the Tullio phenomenon can be identified by calculating the three-dimensional vector of the observed nystagmus. We show that vector analysis of the observed eye movement can be used to infer the source of nystagmus in these patients. The development of real-time, three-dimensional vector analysis of nystagmus is desirable.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveThe purpose of this study was to determine the effectiveness of a new liberatory maneuver in the management of the geotropic variant of horizontal canal paroxysmal positional vertigo (HC-PPV).Study DesignCase review.SettingOutpatient clinic.PatientsThe diagnosis of HC-PPV was based on the history of recurrent sudden crisis of vertigo associated with bursts of horizontal geotropic paroxysmal nystagmus provoked by turning the head from the supine to either lateral position. The patients were 11 men and 21 women ranging in age from 30 to 85 years (average 55.43 years), and the average duration of symptoms was 7.68 days.InterventionsAll patients were treated with a liberatory maneuver based on the hypothesis that the syndrome is caused by the presence of free-floating dense particles inside the endolymph of the posterior arm of the semicircular horizontal canal. The maneuver favors their outmigration into the utricle. Patients were reexamined immediately after the treatment and again 2 days later.Main Outcome MeasureThe treatment outcome was considered as responsive when, after one or more liberatory maneuvers, the clinical signs of PPV disappeared at the end of physical therapy.ResultsThe liberatory maneuver resulted in a complete remission of the positioning vertigo and nystagmus in all patients after the first session.ConclusionsThis approach represents a simple and effective approach to the management of the geotropic form of HC-PPV.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

HypothesisAn objective direct method is proposed to differentiate between drug-induced functional vestibulotoxicity and cochleotoxicity.BackgroundMany substances are ototoxic. Although there are objective methods to directly evaluate functional cochlear toxicity (auditory nerve brainstem responses [ABR]), it is more difficult to assess direct functional ototoxicity to the various vestibular end organs.MethodsShort-latency vestibular evoked potentials (VsEP) from different vestibular end organs and ABR, were used to assess functional impairment of the vestibular and cochlear end organs caused by daily injections of the aminoglycoside amikacin (known to be preferentially cochleotoxic) in guinea pigs.ResultsThere was no significant change in the various VsEPs, whereas ABR thresholds were elevated, confirming the selective functional cochleotoxicity previously reported, as evaluated by other (mainly nondirect) methods.ConclusionThis study demonstrates the feasibility in general of using short-latency evoked potentials to evaluate functional cochleotoxicity and vestibulotoxicity of ototoxic drugs and to differentiate between them.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveThe authors report a case of mature teratoma of the middle ear in a 3-year-old girl with a 1-year history of otitis media.Methods and ResultsRadiologic investigation revealed a partially cystic lesion of the petrous portion of the right temporal bone. It produced opacification of the middle ear as well as destruction of septal air cells. The patient underwent a subtotal petrosectomy. Histologically, the tumor was composed of an intimate admixture of mature tissues representing all three germ layers, including brain, myelinated nerve trunks, skeletal muscle, bone, immature cartilage, seromucinous glands, and respiratory epithelium. Of note within the brain tissue was choroid plexus within an ependyma-lined rudimentary ventricle. Immunohistochemical studies were also performed. Twenty months after surgery, the patient was well, with complete recovery from symptoms.ConclusionTeratomas of the middle ear are rare neoplasms. Only a few examples have been reported. As a rule, they are cured by resection and do not require adjuvant therapy.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

BackgroundThe indication for surgery of vestibular schwannomas (VS) remains controversial and depends on several factors. The ability to predict their patterns of growth would allow better surgical planning. This growth may depend on tumoral proliferation but also depends on dystrophic changes.ObjectiveThe aim of this study was to evaluate the role of magnetic resonance imaging (MRI) in predicting the evolution of VS. For this purpose, the authors attempted (1) to compare the MRI appearance of VS with its histopathologic features, (2) to correlate the MRI appearance of VS and its histopathologic features with its size, and (3) to evaluate the index of proliferation (IP) of each VS.Patients and MethodsThirty VS were studied with MRI before surgery. The VS were measured and classified as homogeneous, heterogeneous, and cystic. After surgery, IP was evaluated with immunohistochemical study using MIB-1 monoclonal antibody, and compared with tumor size. Pathologic studies evaluated the prevalence of Antoni type A and type B tissue, the amount of fibrosis, and the presence of siderin-loaded macrophages, xanthomatous cells, and cysts.ResultsThe IP was low (0.2%–2.2%) and was not correlated with VS size. On MRI, 13 VS were homogeneous, 12 heterogeneous, and 5 cystic. The 13 homogeneous VS were smaller and were predominantly made of Antoni type A tissue. The 12 heterogeneous and 5 cystic VS were larger and were predominantly made of Antoni type B/mixed tissue. Heterogeneous and cystic VS showed significantly more hemosiderin deposits. There was a significant relation between the amount of hemosiderin deposits and the mean size of VS. Microscopic cysts were observed only in VS with cystic MRI appearance. Fibrosis was present in all tumors regardless of their size and MRI appearance.ConclusionA heterogeneous MRI aspect (correlated with larger mean size) not only is related to the ratio of type A to type B tissue but also is caused by other pathologic changes, mainly hemosiderin deposits and cystic formation. Increasing tumor size probably depends less on IP than on dystrophic changes (hemosiderin, cysts) and/or on the presence of type B tissue.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveThe purpose of this study was to determine whether three magnetic resonance imaging (MRI) findings (tumor size, extension to the fundus, intralabyrinthine signal intensity) have a predictive value to hearing preservation in vestibular schwannoma surgery.Study DesignA retrospective study was conducted of preoperative high-resolution MR images in a series of consecutive hearing preservation attempts for vestibular schwannomas.PatientsTwenty-six consecutive records of patients with an acoustic tumor removed via a retrosigmoid transcanal approach were analyzed, and the MR images were reviewed blindly and compared with postoperative hearing.SettingThe study took place in a tertiary referral center.Imaging TechniquesThe MR sequences used in this study were unenhanced and gadolinium-enhanced T1-weighted spin-echo images and gradient echo images (3DFT-CISS).Main Outcome MeasuresThe predictive value of three MRI signs was analyzed: tumor size, lateral extension of the tumor (with and without obliteration of the fundus), and the maintenance of, or decrease in, the intralabyrinthine signal intensity on the affected side in comparison with the signal intensity on the opposite normal side as seen on 3DFT-CISS images.ResultsThe tumor size in ears in which hearing was preserved averaged 15 mm and was 17 mm in those cases where hearing was not preserved. Hearing was preserved in 50% of ears when the tumor did not extend to the fundus but in only 33% when the fundus was obliterated by tumor. A “normal” intralabyrinthine signal on CISS images (being an isointense signal when compared with the contralateral unaffected ear) was followed by hearing preservation in 82% of ears, whereas in cases where the intralabyrinthine signal was low, hearing was preserved in only 20%. This correlation was statistically significant (p < 0.05).ConclusionThe intralabyrinthine signal intensity on 3DFT-CISS gradient-echo images is a valuable additional tool for determining candidacy for hearing preservation surgery. In two cases with preoperative decrease in signal intensity of the intralabyrinthine fluids, control MRI after surgery showed spontaneous recovery of normal intralabyrinthine signal intensity. The authors hypothesize that vascular compression in the internal auditory canal by the tumor is responsible for the observed intralabyrinthine signal decrease.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo evaluate the auditory brainstem response (ABR) findings in cerebellopontine angle (CPA) tumors and focus on those with normal ABR results.Study DesignThis was a retrospective evaluation of ABR findings. All subjects with diagnosed CPA tumors who were referred for treatment were included.PatientsAll 309 patients with radiographically confirmed (computed tomography or magnetic resonance imaging [MRI]) CPA tumors (153 before 1993 and 156 after 1993).InterventionAll patients underwent complete audiologic examination, electronystagmography, ABR testing, and ipsilateral transtympanic electrocochleography. All tumors were histologically confirmed.Main Outcome MeasuresThe patients were categorized according to ABR results (normal or pathologic findings or no response); according to histology (vestibular schwannomas or meningiomas); and according to tumor size by MRI (small, up to 15 mm; medium, 16–25 mm; large, 26–40 mm; and very large, over 40 mm).ResultsNormal ABR results were found in 18.4%; pathologic ABR results in 31.4%; and no response in 50.2%. Small vestibular schwannomas (under 15 mm) showed a higher incidence of normal ABR results (41.7%). CPA tumors with normal ABR results (n = 57) caused no hearing loss (n = 12), symmetrical hearing loss (n = 11), or ipsilateral hearing loss with Short Increment Sensitivity Index 100% and normal acoustic reflex (cochlear deafness, n = 34). Caloric response was normal in 59.7% of these tumors.ConclusionBecause validation of ABR as screening for acoustic tumors is based on diagnosed tumors, and because MRI allows detection of very small tumors, the incidence of normal ABR increases and its sensitivity would be expected to decrease. ABR is not sufficient for early detection of small CPA tumors.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo analyze the value of electromyography in predicting recovery from acute idiopathic facial nerve paralysis.Study DesignRetrospective case-series review.SettingUniversity-based hospital department of otorhinolaryngology/head neck surgery.PatientsThree hundred fifty-five patients with sudden facial paralysis of unknown cause (Bell's palsy).InterventionTreatment consisted uniformly of high-dose prednisolone, dextran, and pentoxifylline. Prognostication was based on electromyography performed not earlier than 10 to 14 days after the onset of palsy. The findings were classified according to Seddon into neurapraxia and axonotmesis/neurotmesis. There is an inherent statement on prognosis in this classification because neurapraxia is presumed to recover completely within 8 to 12 weeks, whereas axonotmesis is most likely to be followed by sequelae.Main Outcome MeasuresFacial nerve function after 6 months.ResultsComplete recovery was predicted correctly in 92.4% of cases. For the relatively rare and therefore principally more difficult predictable event defective recovery prognosis was still accurate in 80.8%.ConclusionThe detection of spontaneous fibrillation in needle electromyography is a reliable sign predicting unfavorable outcome. An accuracy of 80.8% for predicting unfavorable outcome may be sufficient to advise patients what to expect in the course of their facial nerve disorder. However, it seems dubious to build a decision about surgical intervention on such a test, because in the process, unnecessary surgery would be accepted for as much as one fifth of the patient population.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo give a historical perspective of Antonio Scarpa's contributions to otology, specifically the discovery of the inner ear organs as the foundation for the experimental work that followed.Background/MethodScarpa's original descriptions of the human inner ear were translated from the Latin text, and his illustrations were analyzed and compared with current knowledge.ConclusionsAntonio Scarpa's anatomic and clinical studies place him among the great scientists of the eighteenth century. His discoveries about the inner ear established the limit of what could be learned without advanced histologic techniques and provided the foundation for the work that eventually led to the modern understanding of ear physiology.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID