Jin Kanzaki

New and modified reporting systems from the consensus meeting on systems for reporting results in vestibular schwannoma.

Standardization of reporting results of vestibular schwannoma (acoustic neuroma) surgery has been discussed ever since the first Acoustic Neuroma Conference in 1991 (Copenhagen). However, it has been difficult to reach consensus and to agree on a standardized system because these conferences are held only once every 4 years. In view of this, the Consensus Meeting on Systems for Reporting Results in Acoustic Neuroma (chairman, Dr. Kanzaki) was convened, and 40 neurotologists, neurosurgeons, and neuroradiologists attended on November 7 to 9, 2001, in Tokyo as the 11th Keio University International Symposium for Life Sciences and Medicine. The objectives set for the meeting were as follows: 1. A uniform classification for reporting results should be promoted. 2. The classification should be used by all professionals (general physicians, audiologists, otolaryngologists, neurologists, neurosurgeons, and neuroradiologis ts) involved in the diagnosis and management of vestibular schwannoma. 3. The classification should be easy to learn and practicable for all professionals. 4. The classification should be easily understood by patients. 5. Standard guidelines should be provided that would facilitate interinstitutional comparison of results of surgical studies (1). 6. A new and modified rational version of existing classifications should be provided. The following consensus on reporting systems was achieved:

Steroid-responsive bilateral sensorineural hearing loss and immune complexes.

ZusammenfassungAcht Fälle von beiderseitigem, sensorineuralem Hörverlust wurden mit Kortikosteroiden über einen Zeitraum von mindestens 2 Jahren behandelt. Als Ursache für die Schwerhörigkeit wird neben Lues und Aortitis auch eine Autoimmunreaktion diskutiert.SummaryWe have recognized in recent years that some cases with idiopathic bilateral sensorineural hearing loss which showed acute progression in either ear responded to steroid and that the improved hearing level could be maintained by longterm administeration of steroid only.Forty cases of bilateral sensorineural hearing loss were selected for study. In most of these cases hearing deterioration of either ear was confirmed by audiometry.Eight of fifteen cases with marked improvement have been proved to respond to steroid treatment. These eight cases include three cases with syphilitic deafness and one case with aortitis syndrome. In four other cases the causes are still unknown, but could be due to autoimmune mechanisms. In four of 25 cases the immune complexes (IC) value was higher than normal and three among them responded to steroid.Since a high IC value has been reported in lupus nephritis, immunologic examinations are necessary for patients over 30 years of age with bilateral sensorineural hearing loss.

The Growth Rate of Acoustic Neuromas

Growth rate of acoustic neuromas (AN) was studied in 43 patients. The growth rate was analyzed using tumor increasing size (IS) and tumor volume doubling time (VDT). The growth rate of unilateral AN was lower than that of bilateral AN associated with neurofibromatosis2 (NF2). The growth rate of recurrent tumors was higher than that of non-operative tumors. The relationships between growth rate and age and tumor size were also analyzed. The younger the patient or the greater the tumor size, the higher the growth rate. Several factors, i.e. age and sex of patients, tumor pathology and tumor size, should be considered together for predicting the growth rate on AN.

Evaluation of Hearing Recovery and Efficacy of Steroid Treatment in Sudden Deafness

In cases of sudden deafness (SD), hearing recovery is generally assessed by classifying recovery into four degrees. To evaluate factors that may affect the prognosis for hearing recovery (e.g., hearing level at initial examination, days elapsed from onset of hearing loss to start of treatment) or drug efficacy, however, a quantitative method would be more advantageous. In the present study, therefore, the recovery rate was used as an index in investigating the prognosis and the efficacy of steroid treatments in 183 patients with SD first examined within 14 days after the onset of hearing loss during the period 1980-85. As with the degree of hearing recovery, the recovery rate was found to be favorable in those patients with good hearing at the initial examination, no vertigo, little difference in high-tone and low-tone hearing loss, and only a short lag between onset of symptoms and the initiation of treatment. This indicated that the recovery rate could be used as an index for the evaluation of hearing recovery in cases of sudden deafness. The efficacy of steroid treatment was evaluated in patients in whom the average of the hearing levels at five frequencies from 250 to 4,000 Hz was 40-90 dB. No significant differences were found in either recovery rate or degree of recovery between those patients who received steroids and those who did not. This was believed to be attributable to the variety of pathological conditions involved in SD and to the influence of the other, non-steroidal drugs administered.

Correlations of inflammatory biomarkers with the onset and prognosis of idiopathic sudden sensorineural hearing loss

Hypothesis We investigated whether inflammatory biomarkers and stress are involved in the pathophysiology of idiopathic sensorineural hearing loss (ISHL). Study Design Individual cohort study. Setting Two tertiary centers. Patients Forty-three ISHL and 10 non-ISHL patients seen in our ENT departments from 2004 to 2010 within a week from the onset of new symptoms and without steroid administration before visiting our departments. Intervention Multiple audiologic evaluations, blood tests including leukocyte counts, natural killer cell activity (NKCA), interleukin 6 (IL-6), tumor necrosis factor, high-sensitivity CRP (hCRP), and the General Health Questionnaire were used to evaluate the systemic stress and inflammatory response. Main Outcome Measures Correlations between biomarkers and ISHL severity and prognosis were evaluated by statistical analysis. Results In the ISHL patients, a neutrophil count above the reference range was associated with severe hearing loss and poor prognosis, and was accompanied by low NKCA and high IL-6. In the non-ISHL patients, these associations were not present. The abnormal neutrophil count was independent of preexisting vascular diseases. The abnormal counts responded to treatment and decreased into the reference range. Conclusion Neutrophil counts above the reference range of a facility will be a useful indicator of poor prognosis of ISHL. Synchronism of different types of NF-&kgr;B activation pathways could be required to cause severe ISHL. An NKCA decrease, an acute neutrophil count increase, and an IL-6 increase can induce NF-&kgr;B activation in the cochlea and cause severe ISHL. Further epidemiologic surveys should be conducted to evaluate whether stressful life events increase the risk of severe ISHL onset.

Effect of prostaglandin E1 on idiopathic sudden sensorineural hearing loss: a double-blinded clinical study.

Objective and Study Design The authors conducted a prospective, randomized, double-blinded clinical trial for the purpose of elucidating the effects of prostaglandin E1 (PGE1) on idiopathic sudden sensorineural hearing loss. Setting and Patients With the approval of the institute ethics committee, a total of 57 consecutive patients with diagnoses of idiopathic sudden sensorineural hearing loss were included in the study. The patients in the PGE1 group received continuous infusion containing 60 &mgr;g PGE1 and 100 mg hydrocortisone for 7 days, and the patients in the placebo group were treated with continuous infusion containing an inactive placebo and 100 mg hydrocortisone. Results No significant differences were observed in the improvements of pure-tone average and subjective symptoms between the PGE1 and the placebo groups. However, the hearing improvement at high frequencies (4 kHz and 8 kHz) was significantly higher in the PGE1 group than in the placebo group, especially in the patients with severe tinnitus. Conclusions These results failed to prove a beneficial effect of PGE1 in the treatment of idiopathic sudden sensorineural hearing loss. Further studies will be needed to clarify the pharmacologic actions of PGE1 in the cochlea.

Quality of life of vestibular schwannoma patients after surgery

The quality of life of vestibular schwannoma (VS) patients after surgery was investigated. The subjects consisted of 236 unilateral VS patients who underwent tumor removal between 1990 and 1997. A questionnaire was sent to all patients regarding their hearing, tinnitus, dizziness and the changes in their daily life after surgery; 176 out of 204 patients (86%) who received the questionnaire completed and returned it. The answers were compared with recent data reported in other clinical studies. Ninety percent of the patients with postoperative class A hearing were satisfied with their hearing. However, only 30% of patients with postoperative class B hearing were satisfied. Tinnitus worsened after surgery more often in patients who underwent a labyrinthectomy than in those who did not. Dizziness improved after surgery in the majority of VS patients. However, 30% of patients had difficulty driving a car and 50% of patients could not enjoy activities such as playing sport after surgery.The quality of life of vestibular schwannoma (VS) patients after surgery was investigated. The subjects consisted of 236 unilateral VS patients who underwent tumor removal between 1990 and 1997. A questionnaire was sent to all patients regarding their hearing, tinnitus, dizziness and the changes in their daily life after surgery; 176 out of 204 patients (86%) who received the questionnaire completed and returned it. The answers were compared with recent data reported in other clinical studies. Ninety percent of the patients with postoperative class A hearing were satisfied with their hearing. However, only 30% of patients with postoperative class B hearing were satisfied. Tinnitus worsened after surgery more often in patients who underwent a labyrinthectomy than in those who did not. Dizziness improved after surgery in the majority of VS patients. However, 30% of patients had difficulty driving a car and 50% of patients could not enjoy activities such as playing sport after surgery.

Effect of single-drug treatment on idiopathic sudden sensorineural hearing loss

OBJECTIVES In order to evaluate the effect of a medical administration for the sudden deafness patients, single-drug treatment for idiopathic sudden sensorineural hearing loss (ISSHL) was assessed at multi-centers participating in the Acute Severe Hearing Loss Study Group sponsored by the Ministry of Health, Labor and Welfare of Japan. METHODS The subjects consisted of ISSHL patients who were (1) 20 years of age or older, (2) diagnosed within 2 weeks after the onset of hearing loss, (3) showing a mean hearing level of 40-90 dB at five frequencies from 250 to 4000 Hz, (4) previously untreated, and (5) with normal for age in hearing of the opposite ear. The drugs used in this study were ATP, alprostadil, hydrocortisone and amidotrizoate, which were administered intravenously, and beraprost sodium and betamethasone, which were given orally. Two drugs were assigned to each center, one of which was selected according to the code hidden in envelopes and administered for 1 week. The treatment after the single-drug administration was conducted at the discretion of each center. The hearing gain and recovery rate at 1 week after the initiation of single-drug treatment and at 1 month or over when the hearing level was fixed, were evaluated based on the criteria for hearing recovery prepared by the Acute Severe Hearing Loss Study Group. RESULTS There was no statistically significant difference in the recovery rate among drugs either at 1 week after the initiation of single-drug treatment or at the time of fixed hearing level. At the time when the hearing level was fixed, a statistically significant difference in the complete recovery rate was detected only between amidotrizoate and beraprost sodium. CONCLUSION From these results, we could not find any specific drugs recommended for ISSNHL. In evaluating the effect of the drugs, however, several problems in the clinical trial for ISSHL should be considered.

A comparison and conversion table of ‘the House–Brackmann facial nerve grading system’ and ‘the Yanagihara grading system’

A comparison between the House-Brackmann facial nerve grading system (the HB-system) and the Yanagihara grading system (the Y-system) was studied with 199 evaluations of 62 cases of postoperative unilateral acoustic neuroma. In the beginning, an original draft of the conversion table was formulated according to the 199 evaluations, in which, 0-6, 8-14, 16-20, 22-28, 30-38, and 40 points in the Y-system were matched with grade VI, V, IV, III, II, and I in the HB-system respectively. The result of the present study for prediction of sequelae showed that it was not necessary to consider the sequelae in a conversion table. And more, the study of an inter-observer variation showed that the lower and upper limits of the scores in the Y-system may shift within about a 2-point range in the draft table. From these aspects, a newly revised conversion table was proposed as a revised conversion table, in which, 0-6, 8-14, 16-22, 24-30, 32-38, and 40 points in the Y-system were matched with grade VI, V, IV, III, II, and I in the HB-system. This revised conversion table is much easier to remember for clinical use, because the score of the lower limit of each grade is simply in multiples of 8, that is, 0, 8, 16, 24, 32 and 40.

Circulating Immune Complexes in Steroid-Responsive Sensorineural Hearing Loss and the Long-Term Observation

Immune complexes (IC) were determined quantitatively in 53 cases of bilateral sensorineural hearing loss (SNHL) by the Raji cell immuno-fluorescence assay. The 53 cases consisted of 47 cases of unknown etiology, 5 cases with positive syphilitic reaction and 1 case of aortitis syndrome. High IC values were indicated in 5 cases, of which one was of aortitis syndrome and 4 were of unknown etiology. The case of aortitis syndrome and 3 out of the above 4 cases of unknown etiology were responsive to steroid. There were 2 cases which showed normal IC values but reacted to steroid. All patients of the steroid-responsive SNHL were female. If hearing improves by steroid administration, it is necessary to suspect autoimmune SNHL. It has been proved that in SNHL which is responsive to steroid, the hearing level before the start of treatment may be maintained by long-term steroid therapy.