摘要:

ObjectiveTo treat patients with sudden sensorineural hearing loss (SSNL) who failed oral prednisone therapy by using a round window membrane (RWM) microcatheter. This topical delivery strategy sought to improve effectiveness of steroid treatment to the inner ear by targeting drug delivery to the RWM.Study DesignNonrandomized prospective design.SettingTertiary care facility.PatientsSix patients with severe unilateral SSHL, five of whom were refractory to a course of oral steroid therapy treated within 6 weeks of SSHL and three additional patients treated more than 6 weeks after SSHL.InterventionTherapeutic use of RWM catheter.Main Outcome MeasuresPure-tone averages (PTAs) and word identification scores (WIS).ResultsFive of the six patients treated within 6 weeks of SSHL improved their WIS. Of the six, four returned to baseline hearing, one recovered hearing that could benefit by hearing amplification, and one regained moderate improvement in PTA but not WIS.ConclusionTargeted topical steroid administration avoids the significant systemic side effects of oral steroids and may offer more effective dosing than simple transtympanic injection of medicine. Although these findings are preliminary, it is possible that after further study, targeted drug delivery may be a useful technique to consider in patients with severe to profound hearing loss that have failed all other management options.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveIndividuals with monaural hearing experience disadvantages compared with normal hearing counterparts because of the loss of the head shadow effect, the squelch effect, and binaural summation. In this study the Minimum Speech Test Battery (MSTB), a battery designed to document word recognition in bilaterally hearing impaired cochlear implant candidates, was administered to unilaterally hearing-impaired and normal hearing subjects to study its possible use in measuring hearing difficulty in monaural subjects.Study DesignRepeated measures design with the MSTB administered in sound-field in a sound-isolated booth in 1) quiet; 2) speech toward good ear, noise (+10 dB S/N) toward impaired ear; 3) speech toward impaired ear, noise toward good ear; and 4) bilateral speech and noise.SettingAcademic otologic practice.PatientsTen adults with normal hearing and 10 adults with normal or near-normal hearing in one ear and profound hearing loss in the contralateral ear.Main Outcome MeasuresThe MSTB, composed of the Consonant-Nucleus-Consonant (CNC) test and the Hearing In Noise Test (HINT).ResultsAs expected, performance differences between the groups were not found in quiet conditions. Analysis of variance and regression analysis confirmed that the impaired group performed significantly worse than control subjects on HINT testing when noise was directed toward the good ear. Analysis of variance and regression analysis confirmed that the impaired group performed significantly worse than control subjects on CNC testing when noise was directed toward the good ear and in bilateral noise.ConclusionsThe MSTB may be useful in measuring the hearing difficulty of patients with monaural hearing.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo assess the magnetic properties of stapes prostheses and manufacturing materials using a vibratory sample magnetometer (VSM).Data SourcesVSM was performed on 16 samples, including ferromagnetic 420F stainless steel, with an LDJ Model 9600 VSM in accordance with American Society for Testing and Materials standard A894.ResultsA VSM measures the magnetic dipole moment of a sample in a magnetic field. The magnetic field is swept over a range of magnetic fields, and the magnetic dipole moment is plotted as a function of field. The prostheses made of 316L stainless steel previously found to be ferromagnetic had the highest specific magnetic moments. The specific magnetic moments ranged from 0.023 electromagnetic units (emu)/g to 156 emu/g. The samples made with 316L stainless steel, which is used in otologic implants, were significantly less magnetic than was the 420F stainless steel.ConclusionVSM demonstrates that prostheses made with 316L stainless steel are relatively nonferromagnetic compared with 420F stainless steel. However, none of the implants are nonmagnetic. The torque and linear force on the prosthesis is a concern. The safety of performing MRI on patients with these implants needs to be further assessed.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo identify the clinical and radiologic characteristics of aplasia and hypoplasia of the eighth nerve.Study DesignRetrospective case-note review.SettingCochlear implant program.PatientsAll children at the authors' institution in whom the cochlear implant assessment failed because of absence or hypoplasia of the eighth nerve.InterventionComputed tomography of petrous bones and magnetic resonance imaging of the brain.Main Outcome MeasuresPresence or absence of eighth nerve and other radiologic factors contraindicating implantation.ResultsOf 143 cochlear implant candidates, 237 were judged ineligible for cochlear implantation. The preimplant assessment failed in 10 candidates of 143 because of bilateral aplasia or hypoplasia of the eighth nerve (7 cases) or unilateral aplasia or hypoplasia of the eighth nerve and a contraindication to operation on the other side (3 cases). The aplasia or hypoplasia of the eighth nerve was confirmed by magnetic resonance imaging in seven cases (5%): six were syndromic (3 CHARGE, 1 VATER-RAPADILLINO, 1 Möbius, 1 Okihiro), and one was nonsyndromic autosomal-recessive. All seven children had delayed motor milestones and absence of auditory brainstem responses.ConclusionAplasia and hypoplasia of the eighth nerve are not uncommon in pediatric cochlear implant candidates, particularly in the presence of a syndrome such as CHARGE. Magnetic resonance imaging of the brain is mandatory before implantation because it can identify the presence or absence of the eighth nerve. Parents of children with profound hearing loss, delayed motor milestones, absence of auditory brainstem responses, and a syndromic diagnosis, should be made aware of this possible abnormality.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

HypothesisThis study aimed to assess the possibility of hearing restoration after acoustic tumor removal.BackgroundHearing restoration surgery may be indicated after surgery of bilateral acoustic tumors or surgery of acoustic tumor in the only hearing ear. The choice is between cochlear implantation and brainstem implantation.MethodsFrom the temporal bone collection at the House Ear Institute, Los Angeles, the author histologically examined eight temporal bones from seven patients who had undergone acoustic tumor removal during their lifetime. Special emphasis was put on examining the patency of the cochlear turns and on survival of the spiral ganglion cells and cochlear nerve.ResultsThis study showed that after translabyrinthine acoustic tumor removal, there is progressive osteoneogenesis of the cochlea associated with almost complete degeneration of the spiral ganglion cells and cochlear nerve. Similar findings were noticed after middle fossa removal of acoustic tumor with unsuccessful hearing preservation.ConclusionsThe histologic changes described in the cochlea and cochlear nerve represent the effects of ischemia resulting from inadvertent cutting of the blood supply during acoustic tumor removal. It is possible to do cochlear implantation after acoustic tumor removal provided that the result of promontory electrical stimulation test is positive (the cochlear nerve is intact) and that implantation is done at the time of acoustic tumor removal or shortly thereafter, before cochlear ossification is complete.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

BackgroundOculoauriculovertebral dysplasia (OAVD) (Goldenhar's syndrome) is a congenital syndrome with ipsilateral deformity of the ear and face, epibulbar lipodermoids, coloboma, and vertebral anomalies. Goldenhar's anomaly has often been associated with a degree of congenital hearing deficits, almost always of a conductive origin, but a sensorineural component is also suspected in some cases, evident through malformations of the inner ear.Patients and MethodsBoth temporal bones of a 10-day-old deceased patient with oculoauriculovertebral dysplasia were examined by light microscopy.ResultsThe ear deformities included deformity of the auricle, atresia of the external auditory canal, and malformation of the tympanic cavity and ossicles. Abnormalities of the stria vascularis and the semicircular canals were also demonstrated. Further inner ear deformities were not identified in this case.ConclusionThese histopathologic findings appear to confirm the conductive component of the congenital hearing deficit, but a sensorineural component could not be omitted. The ear alterations favor early developmental field defects. The causes of this condition are controversial. Recent results in genetic research pertaining to the MSX class genes permit better understanding of the variety, variability, and different degrees of severity of the anomalies described here.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo describe the temporal bone histopathologic and genetic abnormalities in a case of Mohr-Tranebjærg syndrome.BackgroundMohr-Tranebjæzrg syndrome (DFN-1) is an X-linked, recessive, syndromic hearing loss, characterized by postlingual sensorineural hearing loss with onset in childhood, followed in adult life by progressive dystonia, spasticity, dysphagia, and optic atrophy. The syndrome is caused by mutations in the DDP (deafness/dystonia peptide) gene, which are thought to result in mitochondrial dysfunction with subsequent neurodegeneration. The temporal bone pathologic changes in this syndrome have not been reported.MethodsHearing loss developed in the patient at age 4, blindness at age 48, and dystonia at age 57. Genetic studies on peripheral blood showed a l51delT mutation in his DDP gene. He died at age 66. The right temporal bone was subjected to light microscopy and polymerase chain reaction–based analysis of the DDP gene sequence.ResultsThere was near complete loss of spiral ganglion cells with loss of nearly all peripheral and central processes. Only 1,765 spiral ganglion cells remained (8.5% of mean normal for age). The organ of Corti (including hair cells), stria vascularis, and spiral ligament were preserved. There was also a severe loss of Scarpa's ganglion cells with preservation of vestibular hair cells. The population of geniculate and trigeminal ganglion cells appeared normal. Sequence analysis from temporal bone DNA showed the 15ldelT DDP gene mutation.ConclusionSensorineural hearing loss in Mohr-Tranebjærg syndrome is the result of a postnatal, progressive, severe auditory neuropathy.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

HypothesisThe authors believe that anatomic differences render the superior division of the vestibular nerve more susceptible to injury during vestibular neuritis. The purpose of the study was to investigate anatomic differences between the superior vestibular nerve and singular nerve canals.BackgroundPrevious studies of temporal bones have revealed vestibular nerve degeneration in patients with vestibular neuritis. Although the cause of this degeneration has not been established, it has been noted that the superior division of the vestibular nerve is preferentially affected, with sparing of the inferior division. The superior vestibular nerve and the singular nerve, a branch of the inferior vestibular nerve, both pass through canals interlaced with bony networks before reaching the peripheral receptors.MethodsThe authors performed histologic analysis of 40 normal temporal bones randomly selected from their temporal bone library. With a micrometer, measurements were taken of the individual canals. The ratio of the total bony spicule component to the total canal width was obtained for both the superior vestibular nerve and the singular nerve. The length of the canals was also measured. Arteriole:arteriolar canal ratios of the superior vestibular nerve and singular nerve were obtained.ResultsThe bony channel of the singular nerve had an average length of 0.59 mm, and the average length of the superior vestibular nerve was 2.30 mm (p < 0.001). The ratio of total bony spicule width to total canal width was significantly smaller (p < 0.05) for the singular nerve (0.30 mm) compared with the superior vestibular nerve (0.34 mm). The arteriole: arteriolar canal ratio was significantly smaller (p < 0.05) for the singular nerve (0.45 mm) than for the superior vestibular nerve (0.54 mm).ConclusionThe bony canal of the superior vestibular nerve is longer than the singular nerve canal. Additionally, the superior vestibular nerve and arteriole travel through a relatively narrower passage than the singular nerve and its vascular supply. From an anatomic standpoint, this renders the superior division of the vestibular nerve more susceptible to entrapment and possible ischemic labyrinthine changes.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo describe an anatomic variant of the anterior inferior cerebellar artery in a patient with Méniére's disease.Study DesignRetrospective case review and review of the literature.SettingTertiary referral clinic.InterventionVestibular nerve section and microvascular decompression.Main Outcome MeasuresAudiometric testing and control of vertigo.ResultsThe eighth nerve was identified via a retromastoid approach. The anterior inferior cerebellar artery was observed bisecting the eighth nerve. The vestibular nerve was sectioned, and microvascular decompression was performed on the cochlear division. At last follow-up, the patient had not experienced any vertiginous attacks but was observed to have progressive hearing loss.ConclusionsThe course of the anterior inferior cerebellar artery is highly variable and difficult to predict. Knowing the potential paths is a necessity in performing posterior fossa surgery. Although the patient's vertigo was controlled by the vestibular nerve section, microvascular decompression of the cochlear nerve did not result in hearing improvement or stabilization. This case report does not support a benefit of microvascular decompression in Méniére's disease. Vestibular nerve section remains the authors' treatment of choice for controlling disabling vertigo caused by Méniére's disease.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo evaluate the self-rated quality of life associated with vertigo, hearing loss, and tinnitus in Méniére's patients, and to identify potential relationships between these findings, treatment regimens, and sense of coherence in comparison to the classification of the American Academy of Otolaryngology–Head and Neck Surgery (AAO/HNS).Study DesignCross-sectional.SettingTertiary referral hospital centers.Patients112 patients with Méniére's disease, who had undergone endolymphatic sac surgery or intratympanic gentamicin injections, or were surgically untreated.Main Outcome MeasureQuestionnaires concerning quality of life aspects and symptom-specific instruments: the Vertigo Symptom Scale (VSS), the Hearing Disability Handicap scale (HDHS), the Tinnitus Severity Questionnaire (TSQ), the AAO/HNS criteria for reporting results of treatment of Méniére's disease, and the Sense of Coherence Scale.ResultsA majority of the patients reported their quality of life in general as very good or good. There was no difference in general quality of life, present hearing loss, or tinnitus between the three treatment groups, but the gentamicin-treated patients had less vertigo than did the other groups. Sense of coherence showed a strong correlation to reported quality of life in all measurements.ConclusionsEven though the gentamicin-treated patients had less vertigo, no difference in overall quality of life was found between the surgically treated and untreated patients. The sense of coherence seems to be an important factor in the patient's experience of quality of life. Quality of life instruments can measure both specific symptoms and related aspects on quality of life and may give complementary information to the AAO/HNS classification in evaluating the treatment of patients with Méniére's disease.

ISSN:1531-7129    

出版商:OVID    

年代:2001

数据来源: OVID