論文

The results of 248 cases of acoustic neuroma surgery carried out mainly by the extended middle cranial fossa approach during a 16-year period from 1976 to 1991 are analyzed. Hearing preservation was attempted in 69 cases and successfully achieved in 35 (51%) cases. Hearing was preserved in 24 (57%) of 42 cases in which a tumor was 20 mm or smaller in diameter, pure-tone hearing level was 50 dB or lower, and speech discrimination score was 50% or higher. The hearing preservation rate (76%) in the cases with a tumor extending 3 mm or less from the internal auditory canal was much higher than in cases with a larger tumor. Hearing was preserved in three of seven cases with a tumor of 21 mm or larger and in two of four cases of neurofibromatosis type 2. The evaluation of postoperative hearing is also discussed.

In the cochlear (CSE) and vestibular sensory epithelia (VSE), phosphoinositides are hydrolyzed in response to stimulation of phospholipase C (PLC) by cholinergic muscarinic and purinergic P2y agonists. Such receptor-mediated activation of PLC is expected to be coupled through guanine nucleotide-binding proteins (G-proteins). Although several classes of G-proteins have been identified in the inner ear, nothing is known about the type of G-proteins associated with the phosphoinositide second messenger system in CSE and VSE. Phosphoinositide hydrolysis was determined by the release of radiolabeled inositol phosphates (InsPs). Ten mM NaF plus 10 μM AlCl3 increased basal InsPs accumulation 2-fold in both CSE and VSE of the rat. Release of InsPs was also enhanced by guanosine 5'-O-(3-thiotriphosphate) (GTP-γ-S) in saponin-permeabilized tissues. Furthermore, release of InsPs stimulated by both carbamylcholine (CCh) and adenosine 5'-O-[3-thiotriphosphate](ATP-γ-S) was significantly inhibited by 100 μM guanosine 5'-O-[2-thiodiphosphate](GDP-β-S). These results strongly suggest the involvement of G-proteins in the receptor-PLC coupling in CSE and VSE. ADP-ribosylation in membrane fractions of CSE and VSE in the presence of cholera toxin (CTX) or pertussis toxin (PTX) indicated the existence of Gs- and Gi-type G-proteins. However, neither CTX nor PTX affected basal or agonist-stimulated release of InsPs. These observations suggest that muscarinic and P2y purinergic receptors are coupled to PLC via CTX- and PTX-insensitive G-proteins in CSE and VSE.

Neurotransmission and transmembrane signaling are among the cellular mechanisms affected in the aging nervous system. In the inner ear, the phosphoinositide second messenger cascade is of particular interest as a target of the aging process. In both the cochlear (CSE) and vestibular sensory epithelia (VSE), the hydrolysis of phosphatidylinositol 4,5-bisphosphate (PtdInsP2) to the second messenger inositol 1,4,5-trisphosphate (InsP3) is coupled to muscarinic cholinergic and P2y purinergic receptors and may be linked to calcium homeostasis. The present study compared the turnover of phosphoinositides (PtdlnsPs), receptor-mediated release of inositol phosphates (InsPs), and concentrations of endogenous myo-inositol in the CSE and VSE of young (3 months) and aged (24 months) Fischer-344 rats. In the aged rat, there was a significant increase in [3H]inositol incorporation (per mass of protein) into PtdlnsPs plus InsPs in both sensory epithelia while the protein content remained unchanged. In contrast, no age-dependent differences were found when pre-labeled [3H]PtdInsPs were 'chased' with non-radiolabeled myo-inositol indicating that the turnover of these lipids was unaffected. The cholinergic receptor agonist carbamylcholine and the P2 purinergic receptor agonist adenosine 5′-O-(3-thiotriphosphate) stimulated the release of [3H]InsPs two- to six-fold in both organs. This agonist-stimulated release of [3H]InsPs (per mass of protein) was significantly higher in aged animals. However, when the same stimulation was expressed as per cent of control values, there was no age-dependent difference. Finally, the concentration of endogenous myo-inositol decreased by 44% in the aged CSE and by 24% in the aged VSE. In contrast, levels of added myo-[3H]inositol were higher in aged tissues. These results suggest that the increased labeling of PtdlnsPs and InsPs in the aged CSE and VSE is a consequence of the increased specific radioactivity of the myo-[3H]inositol precursor pool. The activity of the phosphoinositide second messenger pathway thus appears unchanged. However, a decreased myo-inositol content may contribute to age-dependent pathology in these tissues, myo-inositol is an organic osmolyte and volume regulator. Changes in osmotic pressure or turgor of hair cells could alter micromechanical coupling on the basilar membrane and vestibular epithelium causing pathophysiological changes in sensory transduction.

Audiometric data from 76 patients with unilateral sudden deafness and hearing loss of 80 dB or more were studied to statistically assess the relationship between vestibular findings (vertigo and/or dizziness, nystagmus, canal paresis) and hearing recovery in various frequency ranges. Hearing recovery in patients with vertigo and/or dizziness (V(+ was much poorer than in patients without such findings (V(-)). Concerning hearing recovery in the high frequency range, a significant statistical difference was evident between the V(+ and V(-) group. There were no statistically significant differences in hearing recovery between patients with spontaneous nystagmus or canal paresis and those without.

The usefulness of endoscopic examination for the diagnosis of idiopathic perilymphatic fistula (IPLF) was investigated. Eight patients presenting with unilateral sensorineural hearing loss and vertigo underwent endoscopic examination by the transtubal or transtympanic approach. In 5 out of the 8 patients, transtubal endoscopy was carried out using a superfine flexible endoscope. With this approach, no abnormal findings were visualized. A perilymphatic leak from the round window was observed in 2 patients by means of transtympanic examination using a needle scope. These findings were confirmed in both patients by microscopic observation during tympanotomy. In one patient who was finally diagnosed with IPLF, the transtympanic endoscopy failed to detect perilymphatic leakage. Although incision of the tympanic membrane is necessary for the examination, transtympanic endoscopy is useful for the diagnosis of IPLF. Further improvement of the superfine flexible fiberscope is necessary before transtubal observation of the tympanic cavity can be effectively conducted.

In the present study, the clinical and audiological features of 3 patients with aplastic anemia who subsequently developed sudden sensorineural hearing loss are reported. These patients, for whom a diagnosis of aplastic anemia had previously been confirmed, suddenly developed profound or complete hearing loss accompanied by tinnitus and severe vertigo. In addition to erythrocytopenia and thrombocytopenia, serial hematological examinations revealed a marked drop in the platelet count prior to or just at the time of the acute episode of hearing loss. The prognosis for hearing recovery was poor in each patient. These findings suggest that intracochlear hemorrhage could be a mechanism underlying the sudden sensorineural hearing loss in these patients.

It is still difficult to evaluate the efficacy of treatment of sudden deafness. Effective methods of therapy have been reported in some studies and other studies have reported spontaneous recovery. In The present study, we assessed the correlation between hearing recovery and various parameters measured during recovery. We used three parameters: hearing gain, relative hearing gain, and recovery rate. We found that the recovery rate during recovery and the parameters measured on the 7th treatment day demonstrated the greatest correlation. The recovery rate on the 7th day of treatment was found to be useful for predicting the final hearing recovery. Comparison of the recovery rate on the 7th day of treatment may be useful for the evaluation of the efficacy of treatment of sudden deafness.

Various neurotransmitters, hormones and other modulators involved in intercellular communication exert their biological action at receptors coupled to phospholipase C (PLC). This enzyme catalyzes the hydrolysis of phosphatidylinositol 4,5-bisphosphate (PtdInsP2) to inositol 1,4,5-trisphosphate (InsP3) and 1,2-diacylglycerol (DG) which act as second messengers. In the organ of Corti of the guinea pig, the InsP3 second messenger system is linked to muscarinic cholinergic and P2y purinergic receptors. However, nothing is known about the the InsP3 second messenger system in the vestibule. In this study, the receptor-mediated release of inositol phosphates (InsPs) in the vestibular sensory epithelia was compared to that in the cochlear sensory epithelia of Fischer-344 rats. After preincubation of the isolated intact tissues with myo-[3H] in-ositol, stimulation with the cholinergic agonist carbamylcholine or the P2 purinergic agonist ATP-γ-S resulted in a concentration-dependent increase in the formation of [3H]InsPs in both epithelia. Similarly, the muscarinic cholinergic agonist muscarine enhanced InsPs release in both organs, while the nicotinic cholinergic agonist dimethylphenylpiperadinium (DMPP) was ineffective. The muscarinic cholinergic antagonist atropine completely suppressed the InsPs release induced by carbamylcholine, while the nicotinic cholinergic antagonist mecamylamine was ineffective. Potassium depolarization did not alter unstimulated or carbamylcholine-stimulated release of InsPs in either organ. In both tissues, the P2 purinergic agonist α,β-methylene ATP also increased InsPs release, but the P1 purinergic agonist adenosine did not. These results extend our previous observations in the organ of Corti of the guinea pig to the rat and suggest a similar control of the InsP3 second messenger system in the vestibular sensory epithelia. In contrast to the cochlear sensory epithelia, atropine also significantly suppressed unstimulated InsPs release in the vestibular sensory epithelia. This suggests that the physiological mechanisms of the efferent nervous systems involving InsP3 second messenger system might be different in vestibular versus cochlear sensory epithelia.

Vestibular sensory epithelia were studied histologically and ultrastructurally in 17 cases with acoustic neurinoma (AN). The superior vestibular nerve (SVN) near the fundus was also histologically studied in 5 of these 17 cases. Histologically, severe fibrotic change of the vestibular sensory epithelia was found in 1 case, and severe fibrotic change of the SVN was also found in this case. Intra-epithelial cysts were found at the edge of the utricular sensory epithelia in 2 cases. The cysts consisted of the transitional epithelium and were filled with the darkly stained substances. Ultrastructurally, abnormal accumulation of darkly stained masses within the nerve ending and abnormal accumulation of the fibrillar material below the normal basal lamina were frequently observed. These morphological changes described above were regarded as the pathological changes due to AN. In addition, the relationship among the histologic changes, ultrastructural changes and clinical data were fully investigated, and some histologic and ultrastructural changes were regarded as artifacts or age-related changes.

Twenty-nine children with psychogenic deafness were investigated from audiological and psychological aspects. ABR and Békésy audiometry were useful for diagnosing psychogenic deafness. The elapsed time from the start of treatment to audiometric recovery was significantly shorter in patients receiving psychological treatment, indicating that treatment by a team of otologists and counselors was able to hasten recovery in children with psychogenic deafness. The pattern of hearing recovery in the treated group was classified into 3 types and in the control group into 4 types. Counseling seemed to have a positive effect on patients with fluctuating improvement. In the patients in the treated group, the elapsed time to audiometric recovery from the beginning of psychological treatment was 7.5 months; however, in the control group recovery it took 17.1 months. Psychological treatment revealed that the clinical course of psychogenic deafness in children seemed to have some relation to the patient's personality and psychological stresses.

Nine cases of intracranial facial nerve reconstruction are reviewed in this paper. All patients underwent this procedure for severe injury or disruption of the facial nerve during surgery for acoustic neruroma through the modified extended middle cranial fossa approach (1). Satisfactory recovery of facial function was obtained in 4 patients. Three patients underwent hypoglossal-facial nerve anastomosis 1.3-1.5 years later for no or poor recovery of the facial function. One patient refused any further surgical treatment despite unsatisfactory recovery. The remaining 1 patient, during a telephone interview, stated that facial function had not returned at all 1 year and 5 months postoperatively. Although some degree of associated movement or mass movement was unavoidable, facial movement and mimetic facial expression were better in the patients with satisfactory recovery, as compared with those after hypoglossal-facial nerve anastomosis (2). Fibrin glue, which we used in the latest 3 cases instead of suture, seemed to possibly solve the technical difficulty in placing a suture. Facial function after intracranial reconstruction with fibrin glue was as good or better than that after repair by suturing.

Audiological examinations are vital in the diagnosis of acoustic neuroma. In interpreting their results, however, it is necessary to consider the patient's hearing level. The most sensitive audiological examination is auditory brain stem response (ABR) audiometry. Its most useful parameter is the IT5. A U-shaped audiometric configuration suggests AN, since it is seen in 10% of patients with small tumors. Psychological audiometric tests can be excluded from the battery of screening tests since they have low rates of positive diagnosis. The stapedius reflex (SR) test also has a low positive diagnostic rate in cases of small tumors. Even with the parameters of absence of reflex, elevated threshold, and decay combined, the overall SR test has a lower positive diagnostic rate than ABR audiometry. Nevertheless, the SR test can be employed as a screening device in cases in which the hearing level at 2 kHz and lower is 70 dB or lower, even if it is 71 dB or higher at 4 kHz and 8 kHz. At present, ABR audiometry is applicable in only about half of AN cases. Therefore, the need for early diagnosis must be further emphasized.

The complications of acoustic neuroma (AN) surgery by the extended middle cranial fossa approach were studied in 160 cases. Death during surgery occurred in 3 cases (1.9%; 2 of these involved large tumors and the third was a case of recurrence. Transient temporal lobe symptoms were seen in 24 cases (15.3%; the average tumor diameter in these cases was 36.0 mm. Beside symptoms of 7th and 8th nerve disturbance, hemiparesis was seen 7.6%; 3rd, 4th, and 6th cranial nerve symptoms in 7.0%; and cerebellar symptoms in 7.6% of the patients. The incidence of these disturbances increased with tumor size. Cerebrospinal fluid (CSF) leakage occurred in 20.4% of the cases and this necessitated surgical treatment in 5% of the cases.

The results of attempted hearing preservation were investigated in 160 cases of acoustic neuroma surgery carried out by a team of otologists and neurosurgeons at Keio University Hospital during a 14-year period from 1976 to 1989. Surgery was carried out by the middle cranial fossa (MCF) approach in the earlier cases and by the extended middle cranial fossa (EMCF) approach in the more recent cases. Measurable postoperative hearing was preserved in 20 of the 160 cases. Preoperatively, 22 cases had tumors of 20 mm or smaller in diameter, hearing levels (HL) of 50 dB or lower, and speech discrimination scores (SDS) of 50% or higher; 8 (36% met these conditions postoperatively. Among those cases with hearing preserved postoperatively, hearing was unchanged from the preoperative level in 9 cases and changed in 11 cases. Total tumor removal was achieved in 19 cases. In one case, part of the tumor was left in order to preserve hearing, but MRI and CT have revealed no change in hearing or tumor enlargement to date, at 4 12 years after surgery. Hearing was preserved but progressively deteriorated postoperatively in one case in which the tumor was believed to have been totally removed but there was recurrence and in another case of total resection of neurofibromatosis II. Postoperatively, there were increased incidences of absence of the stapedius reflex, Type V by Bekesy audiometry, and prolongation of the IT5, disappearance of Wave V, and no response in measurements of the ABR.

The surgical anatomy of the temporal bone and the cerebellopontine angle was studied in cadavers, by performing simulated surgery via the extended middle cranial fossa approach. This approach is classified into 3 types according to extension of the drilling area of the temporal bone. In this paper, the surgical anatomy visualized by the EMCF type II and type III is described. In the EMCF type II, mastoidectomy and labyrinthectomy are performed completely through the middle cranial fossa, and in the EMCF type III permitting hearing preservation surgery, sections of the middle cranial fossa dura and the cerebellar tentorium are added to House's middle cranial fossa approach. The comprehension of these anatomical structures is essential for surgeons in performing acoustic neuroma surgery. We believe that this study will serve to expand our knowledge of surgical anatomy, and that as a result the EMCF approach for acoustic neuroma surgery will become more popular.