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Histologic changes in the ageing spiral tract.

To investigate the influence of ageing on the morphology of the bottom of the internal auditory meatus, we analysed 150 undecalcified pyramids of human temporal bones. In most of the histologic sections examined, we found pointed bony spicules in the region of the spiral tract. These develop from layers of active and resting osteoid, reach their final morphologic shape in the fourth decade of life and then protrude into the internal auditory meatus, compressing the cochlear nerve fibers.

Adolescent↗

Histopathological studies of the temporal bones in Hurler's disease [mucopolysaccharidosis (MPS) IH].

The structural basis of the combined conductive and sensorineural deafness has been described in two patients with Hurler's disease. All parts of the ear contained numerous large vacuolated Hurler cells, the vacuoles being distended lysosomes from which accumulated glycosaminoglycans had been dissolved during fixation of the tissue. The external and middle ears also showed chronic inflammation. There was resorption of the bone in the mastoid process by masses of Hurler cells and abnormal new bone with prominent cement lines. The blood vessels were surrounded by a 'blue mantle' of osteoid tissue similar to that which is usually associated with otosclerosis. The stapes appeared deformed and was covered by thickened mucosa and granulation tissue. The bone structure of the ossicles resembled that of the mastoid process. The organ of Corti was degenerate and the Reissner's and tectorial membranes were adherent to one another and covered by haemorrhagic material near the vascular striae. The blood vessels in the striae were congested and the scalae media and tympani contained some blood. The neurons in the basal coil of the spiral ganglion were replaced by Hurler cells. The vestibulo-cochlear nerves were disrupted by numerous Hurler cells. These pathological findings adequately explain the combined conductive and sensorineural deafness in these cases. They are also discussed in relation to some other clinical and pathological aspects of these two specific patients.

Child, Preschool↗

Enhancing hearing preservation in endoscopic-assisted excision of acoustic neuroma via the retrosigmoid approach.

Surgeons using the operating microscope are able to make use of numerous landmarks described for the lateral limits of dissection to preserve hearing in acoustic neuroma surgery via the retrosigmoid approach. Similar landmarks for hearing preservation described specifically for the endoscopic-assisted technique, are lacking. By analysing computed tomography (CT) scans of temporal bones, it was observed that to reach within 3 mm of the lateral end of the internal auditory meatus (IAM) via a 3 cm retrosigmoid craniotomy, drilling should be up to about 3 mm medial to the opening of the vestibular aqueduct. It was hypothesized that in surgery, by keeping 3 mm medial to the opening of the vestibular aqueduct, the integrity of inner ear structures would be preserved. This hypothesis was tested in 30 temporal bones and was found to be true. In addition, the lateral end of the IAM up to the transverse crest could be viewed by the 30-degree rigid angled endoscope. This landmark could, therefore, be utilized in the endoscopic-assisted technique to predict the optimal amount of bone to be removed at a stage before the internal auditory meatal dura is opened when the intact dura affords added protection to the meatal contents during drilling. Well designed dural flaps on the posterior petrous bone could be created by making a longitudinal incision not more than 7 mm from the superior border of petrous bone and a transverse incision at least 17 mm from sigmoid. These flaps minimize injury to the endolymphatic sac and protect the cochlear nerve and vasculature that when damaged, may result in hearing loss.

Endoscopy↗

Auditory neuropathy in a patient with mitochondrial myopathy and multiple mtDNA deletions.

Auditory neuropathy (AN) is a hearing disorder characterized by the absence or severe distortion of the auditory brainstem responses, in the presence of preserved otoacoustic emissions. This peculiar combination suggests the presence of a defect impinging upon the functional complex formed by inner hair cells, the primary afferents (spiral ganglion neurones) and the first order synapses between hair cells and the cochlear nerve. Typically, AN patients show a severe speech perception impairment, which appears reduced out of proportion to pure tone threshold, but the clinical presentation of AN is quite complex. Hearing loss is a common symptom associated with mitochondrial diseases; however, AN has only rarely been reported in these disorders. Here we report a rare association, the first case observed in Italy, in a patient with autosomal recessive mitochondrial myopathy and mitochondrial DNA multiple deletions, and a hearing deficit with the audiological and electrophysiological features of AN.

DNA, Mitochondrial↗

Stereotactically delivered cranial radiation therapy: a ten-year experience of linac-based radiosurgery in the UK.

In 1989, linear accelerator (linac)-based cranial stereotactic radiation therapy ('radiosurgery') was introduced in the UK at St Bartholomew's Hospital; a new, relocatable stereotactic frame was first used at the same time, allowing fractionated stereotactic radiotherapy. In the first decade of clinical practice using this technology, some 200 patients with blood vessel tumours/malformations have been treated, together with another 200 suffering from other conditions. The usefulness of this technique for cerebral arteriovenous malformations (AVM) has been demonstrated, and also a significant cure rate for AVM of >3 cm diameter (which is larger than for those previously reported after treatment on the gamma unit), albeit attended by a higher complication rate. The epilepsy associated with AVM is much improved by successful radiotherapy. The usefulness of radiosurgery for glomus tumours has been confirmed and new data published on the efficacy of the technique for haemangioblastoma, with new radiation therapy strategies designed for patients with von Hippel-Lindau disease. The acoustic neuroma treatment results have included improvements in hearing (a result not reported in the gamma unit literature), which are ascribed to the lower internal dose gradient within the target volume. Fractionation will, it is argued, also lead to sparing of the special sensory cochlear nerve. The risks of radiosurgery to the brainstem for chordoma of the mid-clivus are reduced by using a 'spacer' technique for the prepontine space. For meningiomas involving the cavernous sinus, conventionally fractionated radiotherapy is recommended when the meningeal base diameter exceeds 3.0 cm and radiosurgery (utilizing fractionation where appropriate) is advised for smaller lesions. Thus far, radiosurgery indications for pituitary adenomas have been restricted to recurrences after conventional radiotherapy, usually those in the cavernous sinus. In therapy for recurrent craniopharyngioma, it is argued that fractionation delivered via the relocatable frame will be important, particularly when the disease envelops the optic chiasma. For semicystic/semisolid craniopharyngiomas, the stereotactic delivery of colloidal yttrium-90 into a cystic element is useful, while stereotactic radiosurgery is delivered to the solid component. Staff at this centre consider that radiosurgery for low-grade gliomas, perhaps as boost therapy after conventional fractionation, is worthy of more research. We have been extremely selective in the use of radiosurgery for brain metastases (2% of patients, compared with about 30% in some Gamma Knife units), but future indications may become broader, probably using it as a booster technique after whole-brain conventionally-fractionated radiotherapy. Positron emission tomography scanning, co-registered with magnetic resonance imaging, allows the 'boost' concept in radiosurgery to become a sophisticated and accurate reality. Post-radiosurgical sequelae have been placed within a standard framework classification. New observations are being made with regard to subacute reactions: late-responding intrinsic and extra-axial tumours may swell in the subacute period, prior to shrinkage, and be attended by symptomatic surrounding brain oedema.

Adenoma↗

Intraoperative monitoring for hearing preservation and restoration in acoustic neuroma surgery.

The present article reports on our experience with hearing preservation during 158 acoustic neuroma (AN) operations via the retrosigmoid-transmeatal (RS-TM) approach with the aid of intraoperative auditory monitoring. Several auditory monitoring methods are described. Of these, the bipolar cochlear nerve action potential (CNAP) was found to be the most helpful in preserving hearing. Of 106 patients with useful hearing preoperatively, more than 50% had useful hearing after surgery. Electrical auditory brainstem responses were useful in the placement of an auditory brain stem implant (ABI) in 4 patients with neurofibromatosis type 2 (NF2). All 4 reported speech perception benefit and use their ABIs regularly in their lives. It is our firm belief that intraoperative auditory monitoring has a pivotal role in the preservation and restoration of hearing in AN surgery.

Journal Article↗

[Contralateral modification of transitory evoked otoacoustic emissions].

BACKGROUND: In recent publications the influence of contralateral white noise on transient evoked otoacoustic emissions (TEOAE) is discussed with regard on contributions of the efferent auditory system. METHODS: In the present study the effects have been investigated with regards to middle-ear muscles, efferents and cross hearing. TEOAE to monaural 40-80 dB SPL clicks were recorded in normal-hearing adults under simultaneous presentation of 20-60 dB SPL broadband noise to the contralateral ear. Control runs were performed before, during a short break of, and after contralateral stimulation. The control run before contralateral stimulation was used as a reference. RESULTS: Decrease in TEOAE, and increase in accompanying noise floor, were found to follow the contralateral stimulation. In particular a 1-3 dB decrease was found for contralateral noise levels of 40 and 60 dB SPL, even though the readings at 60 dB only were statistically significant (paired-samples t test, p = 0.05). For both TEOAE and noise floor no systematic dependence on click intensity was seen. The control runs during temporary break and after contralateral noise revealed an increase in both TEOAE and noise floor. As a rule, the TEOAE adapted to the reference within 2-3 min following the cessation of contralateral stimulation, whereas the increased noise floor level was still noted after 10 min. CONCLUSIONS: Traditionally, suppressing effects of contralateral stimulation on TEOAE have been attributed to cochlear efferents (CEs). Occasionally, the middle-ear muscle and cross hearing involvement have been considered as well. Substantially, the present results and findings of other workers are inconsistent with the basic knowledge of CE functioning: (I) The decrease in TEOAE under contralateral stimulation is in conflict with an increase in cochlear microphonics and summating potentials observed during activation of CEs: (II) contralateral suppression of TEOAE exhibited no significant dependence on the test-stimulus level while the CEs are known to be efficient in the range of the low signal intensities only, and (III) acoustic activation of the CEs can hardly be expected to reach levels sufficient to influence the TEOAE mechanism. The present findings, i.e. decrease in TEOAE and increase in noise floor level, can more reasonably be explained as being mainly attributable to activation of the middle-ear muscles.

Adult↗

Expression of vascular cell adhesion molecule-1, alpha4-integrin and L-selectin during inner ear immunity reaction.

The expression of vascular cell adhesion molecule-1 (VCAM-1), alpha4-integrin and L-selectin was dynamically observed using an immunohistochemical approach during labyrinthitis induced by inoculation of keyhole limpet hemocyanin into the scala tympani of animals that had been systemically sensitized to it. At the same time, we used ELISA to monitor interleukin-1(IL-1) in the peripheral blood of these animals. The expression of alpha4-integrin and L-selectin on the surface of leukocytes in peripheral blood was examined by flow cytometry. VCAM-1 was found on the endothelial surface of the spiral modiolar vein (SMV) and its collecting venules (CV) at 24 h post-challenge; the VCAM-1 level reached a maximum on day 2, which was maintained until 1 week post-challenge, and then declined gradually. Alpha4-integrin was found on the surface of leukocytes, mainly monocytes and lymphocytes, that had infiltrated the SMV, CV, cochlear nerve and perilymph by 24 h post-challenge; the alpha4-integrin level reached a maximum on day 2 and then decreased rapidly. At 1 week post-challenge, no significant expression of alpha4-integrin was seen. Expression of alpha4-integrin and L-selectin was also observed on leukocytes in peripheral blood. No L-selectin was observed on the surface of leukocytes during inner ear inflammation. The expression of L-selectin on the surface of leukocytes in the peripheral blood appeared to be downregulated during inflammation. The concentrations of IL-1 in the peripheral blood increased commensurately during inner ear inflammation. These results further elucidate the role of adhesion molecules during inner ear immune responses. The increased expression of VCAM-1 on SMV and CV was correlated with the concentration of IL-1 in peripheral blood during inner ear inflammation.

Animals↗

Effects of lidocaine on basilar membrane vibration in the guinea pig.

The effects of lidocaine on basilar membrane (BM) vibration and compound action potential (CAP) were studied in guinea pigs in order to elucidate the site of lidocaine action in the cochlea. BM vibration was measured with a laser Doppler vibrometer through an opening made in the lateral bony wall of the scala tympani at the basal turn. Ten min after local administration of lidocaine (250 microg) into the scala tympani, the velocity of BM vibration and the CAP amplitude decreased significantly at around the characteristic frequency of the stimulus sound (p < 0.05). The maximum decreases were 4 dB in the velocity of the BM vibration and 40 dB in the CAP amplitude. In contrast, such changes were not observed after i.v. injection of lidocaine (1.5 mg/kg). These results suggest that when lidocaine is administered locally in the cochlea it acts not only on the cochlear nerve but also on the outer hair cells.

Administration, Topical↗

Relationship between three inner ear antigens with different molecular weights and autoimmune inner ear disease.

Crude inner ear antigen (CIEAg) can induce autoimmune inner ear disease (AIED) although it is not known which subcomponent of CIEAg is involved. In this study, we investigated the relationship between 3 purified inner ear antigens (31, 42-45 and 60 kD proteins) and AIED, and determined their distribution in normal guinea pig cochlea. Three groups of guinea pigs were immunized with the three inner ear antigens and one group served as a control. The hearing thresholds, serum IgG level and morphological changes in the inner ear were observed. The expression of the three antigens in the cochlea was detected using immunohistochemical techniques. No obvious changes in hearing thresholds or inner ear morphology were observed between the control and 42-45 kD groups. Animals immunized with the 31 or 60 kD proteins showed a significant increase in hearing thresholds (p < 0.05 vs control), accompanied by morphological changes in the inner ear. The serum IgG level was increased significantly (p < 0.05) in all immunized animals. The 31 kD protein was distributed in the cochlear nerve and spiral ganglion, while the 42-45 and 60 kD proteins were distributed widely, being found in the spiral ganglion, organ of Corti, stria vascularis and spiral ligament. These results suggest that two subcomponents of CIEAg (the 31 and 60 kD proteins) may induce AIED independently, that several inner ear antigens may contribute to the pathogenesis of AIED and that the 31 kD protein is of high tissue specificity and may be used as a marker protein for the clinical diagnosis of AIED.

Animals↗

A new mouse insertional mutation that causes sensorineural deafness and vestibular defects.

This article describes a new recessive insertional mutation in the transgenic line TgN2742Rpw that causes deafness and circling behavior in mice. Histologic analysis revealed virtually complete loss of the cochlear neuroepithelium (the organ of Corti) in adult mutant mice. In association with the neuroepithelial changes, there is a dramatic reduction of the cochlear nerve supply. Adult mutants also show morphological defects of the vestibular apparatus, including degeneration of the saccular neuroepithelium and occasional malformation of utricular otoconia. Audiometric evaluations demonstrated that the mice displaying the circling phenotype are completely deaf. Molecular analysis of this mutant line revealed that the transgenic insertion occurred without creating a large deletion of the host DNA sequences. The mutant locus was mapped to a region on mouse chromosome 10, where other spontaneous, recessive mutations causing deafness in mice have been mapped.

Animals↗

Decreased cochlear DNA receptor staining in MRL.MpJ-Fas(lpr) autoimmune mice with hearing loss.

OBJECTIVES: Previous studies of decreased cochlear DNA binding in autoimmune mice suggested that antibodies against a cochlear cell surface DNA receptor cause autoimmune hearing loss. However, the presence of a cochlear DNA receptor has not been determined. Therefore, immunohistochemistry with an anti-DNA receptor antibody was performed on MRL.MpJ-Fas(lpr) (MRL/lpr) autoimmune mice to determine 1) which inner ear structures contain DNA receptors and 2) whether the receptor staining pattern changes as autoimmune disease progresses and hearing thresholds increase. STUDY DESIGN: A prospective study of the progression of hearing loss in autoimmune mice and correlated alterations in immunostaining for the inner ear DNA receptor. METHODS: One group of MRL/lpr mice (n = 10) was allowed to develop autoimmune disease, and auditory brainstem response (ABR) audiometry was performed at 4, 6, and 9 months of age to measure the progression of hearing loss. A second group (n = 5) was tested for ABR thresholds at 2 months of age and immediately killed to assess receptor staining before the onset of autoimmune disease and hearing loss. The inner ears from all mice were immunohistochemically stained with an anti-DNA receptor antibody, and a qualitative analysis of the staining of cochlear structures was performed. RESULTS: Auditory brainstem response audiometry revealed a significant 20- to 30-dB elevation of thresholds as systemic disease progressed. Anti-DNA receptor staining was heaviest in the spiral ligament and less intense in the spiral ganglion and cochlear nerve. Both groups showed a similar pattern of staining in these structures. The stria vascularis and hair cells also stained in both groups. However, the stria cells of normal-hearing mice showed diffuse intracellular immunoreactivity, whereas older mice displayed less staining that was confined to the cell membranes. CONCLUSIONS: The inner ears of MRL/lpr mice contain DNA receptors. Autoimmune hearing loss was correlated with weaker overall intracellular staining in the stria vascularis and hair cells but increased staining of the cell membranes. This suggested DNA receptors have impaired endocytosis and more receptors remain on the cell membrane, possibly as a result of binding by circulating autoantibodies.

Age Factors↗

Ultrastructural and electrophysiologic changes of rat cochlea after irradiation.

OBJECTIVE/HYPOTHESIS: To determine the acute and subacute cochlear effects of sublethal total body irradiation. STUDY DESIGN: The study was designed prospectively as a Sprague-Dawley (SD) rat model with otoacoustic emission measurement. METHODS AND MATERIALS: Ten rats and 20 ears were included in the study. Distortion product otoacoustic emission (DPOAE) measurements were performed under ketamine anesthesia before and 1 to 8 weeks after sublethal dose total body irradiation. Eight and six rats survived at the first and eighth week measurements, respectively. The frequency bandwidth was set to 1,067 Hz (referenced to f1) - 9,512 Hz (referenced to f2) (2f1 - f2 = 832 - 6,076 Hz) and eight points were sampled per octave, but low-frequency (<2,549 Hz [referenced to 2f1 - f2]) results were not included in the analysis. Averages were obtained at each frequency, and statistical analysis was performed to compare the pre-and postradiation values. Then, three of the rats were killed for transient electron microscopy (TEM) of the temporal bones. RESULTS: The posttreatment DPOAEs at almost all frequencies showed a trend toward decline at both the first and eighth weeks, but significant reduction was only detected at 3,600 and 3,961 Hz (referenced to 2f1 - f2) in the first week. TEM of the cochlea revealed ultrastructural changes in the outer hair cells (OHCs) and stria vascularis of the basal turn. The cochlear ganglion nuclei and cochlear nerve axons were unaffected. CONCLUSION: Radiation can affect the cochlea in the acute phase at the mid to high frequencies; further prediction for long-term effects requires longer follow-ups.

Animals↗

Progressive deterioration of auditory evoked potentials after excision of an acoustic neurinoma: case report.

The authors report the case of a 17-year-old girl who underwent excision of bilateral neurinomas of the cerebellopontine angle. Although her auditory evoked potentials were well maintained during the operation, they deteriorated gradually and progressively for the following 2 months. This seemed to reflect degeneration of the cochlear nerve fibers initiated by operative manipulation in the cerebellopontine angle in a patient who had no hearing when she awoke from surgery.

Adolescent↗

Cavernous malformation presenting as an extra-axial cerebellopontine angle mass: case report.

OBJECTIVE AND IMPORTANCE: We describe a rare case of an extra-axial cavernoma of the cerebellopontine angle without internal auditory canal involvement and with an unusual appearance on preoperative magnetic resonance imaging (MRI). Only three cases of cerebellopontine angle cavernomas have been previously reported, and none of these reports included preoperative MRI. CLINICAL PRESENTATION: A young man presented with subacute left facial hypesthesia and chronic asymmetrical sensorineural hearing loss. MRI revealed a mass, which was isointense on T1- and T2-weighted images, with evidence of hemorrhage (methemoglobin and hemosiderin). The lesion did not enhance after the injection of gadolinium. INTERVENTION: The lesion was removed without difficulty by a suboccipital approach, with intraoperative electrophysiological monitoring of facial and cochlear nerves. Facial paresis cleared postoperatively. CONCLUSION: The present case suggests that the appearance of cerebelopontine angle cavernomas as revealed by MRI may be similar to that of intraparenchymal cavernomas. This is in contrast to previously reported cases of extra-axial cavernomas at other sites. Evidence of hemorrhage in an extra-axial lesion revealed by MRI suggests a possible diagnosis of cavernoma.

Adult↗

Management of vestibular schwannomas (acoustic neuromas): radiological features in 202 cases--their value for diagnosis and their predictive importance.

OBJECTIVE: Tumor-induced bony and soft tissue findings might reflect, in part, the biological characteristics of vestibular schwannomas and could predict clinical findings. In addition, the role of the individual anatomic conditions of the posterior fossa is of interest to the surgeon. METHODS: Of 1000 vestibular schwannomas treated surgically at the neurosurgical department at Nordstadt Hospital, 202 cases, which were consecutively investigated at the same computed tomography (CT) unit and using the same technique (high-resolution CT at bone windows before and after surgery, contrast-enhanced high-resolution CT before surgery, and native high-resolution CT after surgery), were evaluated for special radiological features. Evaluation included, in 103 cases, anatomic parameters of the petrous bone and posterior fossa cavity and, in 202 cases, tumor-induced changes of bony structures, tumor relations with bony structures and with neural structures, and postoperative findings of bony structures and of neural tissues. These radiological parameters and the related clinical pre- and postoperative findings were transferred to a database for statistical evaluation of their positive or negative correlations, i.e., for their reliability in diagnosis and their importance in predicting postsurgical outcome. RESULTS: As important parameters, the following could be identified. The length of the posterior auditory canal wall and the interear difference of the maximum porus width both correlate with the degree of preoperative hearing deterioration (P < 0.05). The extent of the widening of the internal auditory canal is of predictive importance for the chances of postoperative hearing preservation or hearing loss (P < 0.01). The extent of tumor growth anterior and caudal to the internal auditory canal in large tumors is of significant importance for prediction of postoperative hearing function (P < 0.05). The tumor extension in all directions and the extent of cystic tumor components correlate with the pre- and postoperative function of the facial and cochlear nerves. The positions of the labyrinthine structures and their geographical relations to the fundus and the sigmoid sinus and, thereby, to the suboccipital route, enable reliable calculations of the danger of labyrinthine destruction and help improve the planning of the surgical strategy. DISCUSSION AND CONCLUSIONS: In addition to the importance for surgical planning, preoperative bone window CT and contrast-enhanced images offer the opportunity to identify traits of tumor biology, such as bony destruction of the internal auditory canal, tumor shape and cyst formation, and aspects with predictive importance for postoperative outcome. The average size for hearing preservation in tumors was 14.5 x 16.5 x 11.8 mm (coronal x sagittal x axial). The recent finding (8) of a higher presentation age in female patients has an apparently anatomic basis, which is a relatively larger internal auditory meatus.

Brain↗

Retrosigmoid approach to acoustic neuroma (vestibular schwannoma).

The retrosigmoid approach for the microsurgical removal of an acoustic neuroma (vestibular schwannoma) is described, and perioperative medical management of the patient is discussed. The techniques for monitoring facial and cochlear nerve function are presented. The supine-oblique position, skin incision, bone removal, dural opening, and initial exposure are outlined. Important points in the technique for removing acoustic neuromas and preserving hearing, when possible, are described and illustrated.

Hearing↗