Natural history of hearing and tumor growth in vestibular schwannoma in neurofibromatosis type 2-related schwannomatosis.

OBJECTIVES: To determine the natural history of hearing loss and tumor volume in patients with untreated neurofibromatosis type 2 (NF2)-related schwannomatosis. Moreover, we statistically examined the factors affecting hearing prognosis. METHODS: This retrospective cohort study was conducted on 37 ears of 24 patients with NF2-related vestibular schwannomatosis followed up without treatment for more than 1 year. We obtained detailed chronological changes in the PTA and tumor volume in each case over time, and the rate of change per year was obtained. Multivariate analysis was also conducted to investigate factors associated with changes in hearing. RESULTS: The average follow-up period was approximately 9 years, and hearing deteriorated at an average rate of approximately 4 dB/year. The rate of maintaining effective hearing decreased from 30 ears (81%) at the first visit to 19 ears (51%) at the final follow-up. The average rate of change in tumor growth for volume was approximately 686.0 mm3/year. This study revealed that most patients with NF2 experienced deterioration in hearing acuity and tumor growth during the natural course. A correlation was observed between an increase in tumor volume and hearing loss (r = 0.686; p < 0.001). CONCLUSIONS: Although the hearing preservation rate in NF2 cases is poor with the current treatment methods, many cases exist in which hearing acuity deteriorates, even during the natural course. Patients with an increased tumor volume during the follow-up period were more likely to experience hearing deterioration. Trial registration number 20140242 (date of registration: 27 October 2014).

Intratemporal Facial Nerve Schwannomas: A Review of 45 Cases in A Single Center.

There are no established indications for facial nerve schwannoma treatment, including surgery, radiation and follow-up observation, and it is difficult to determine treatment policy uniformly. The treatment policy was examined from each treatment course. Data of patients with facial nerve schwannomas at our hospital from 1987 to 2018 were retrospectively examined. Their age, sex, clinical symptoms, tumor localization, treatment policies and outcomes were reviewed. In total, 22 patients underwent surgery and 1 patient underwent radiotherapy; 22 patients were followed up without treatment. After total resection, there were no tumor recurrences, and most patients had grade 3 or 4 postoperative facial paralysis. After subtotal resection, tumor regrowth was observed in four patients and reoperation was required in two patients. Facial nerve function was maintained in four patients and was decreased in two patients. During follow-up, six patients showed tumor growth. Only one patient had worsening facial nerve paralysis; four patients underwent facial nerve decompression owing to facial nerve paralysis during follow-up. If the tumor compresses the brain or it is prone to growth, surgery may be indicated, and when the preoperative facial nerve function is grade ≤3, consideration should be given to preserving facial nerve function and subtotal resection should be indicated. If the preoperative facial nerve function is grade ≥3, total resection with nerve grafting is an option to prevent regrowth. If there is no brain compression or tumor growth, the follow-up is a good indication, and decompression should be considered in facial nerve paralysis cases.

Anticipating real-ear insertion response using an external auditory canal model.

OBJECTIVES: This study aimed to determine the acoustic characteristics of the external auditory canal (EAC) and predict the real-ear aided response (REAR) using an EAC model that includes the standing wave effect. METHODS: The EAC transfer function equations were derived by summing the incoming and outgoing waves. First, we investigated the real-ear unaided gain (REUG). Second, seven patients (eight ears) wearing hearing aids (HAs) were enrolled as subjects to examine the REAR. We conducted wideband tympanometry (WBT) to measure the absorbance, the frequency response at 65 dB (65dB-FR) of the HAs, and the measured REAR for an international speech test signal (ISTS) at 65 dB. RESULTS: The EAC model that includes the standing-wave effect is considered to be valid from examination of the REUG. A significant correlation was found between the measured and calculated REARs at 900 Hz, 1000 Hz, 2000 Hz, and 3000 Hz in an uncorrelated test. A two-way analysis of variance (ANOVA) found significant differences in the 65dB-FR and the measured REARs at 800, 900, 1000, and 2000 Hz, but this difference disappeared after correction of the calculated acoustic characteristics of the EAC. CONCLUSIONS: By measuring the WBT characteristics and correcting them with an EAC model, the in-situ REAR can be determined from the HA characteristics in the mid-frequency range. There is a risk of insufficient HA amplification in the mid-frequency range when no real-ear measurements are performed.

[Detection of the Presence of Middle-ear Effusion with Wideband Absorbance Tympanometry].

This study evaluated the ability of wideband absorbance (AB) to predict the presence of middle-ear effusion (MEE) in ears with a flat (type B) 226 Hz tympanogram (TG). AB measures were obtained in a no MEE group of 8 ears with a flat TG and a MEE group of 19 ears also with a flat TG. AB was significantly small at frequencies 2 and 4 kHz in the MEE group than in the no MEE group. Based on our results, it appeared that AB could detect MEE with higher specificity than conventional tympanometry. The presence or absence of MEE could be distinguished based on the average AB using 1, 2, and 4 kHz, with 40% as the cutoff point.

[Chondroblastoma of the Temporal Bone Removed Using a Middle Cranial Fossa Approach].

We report a case of chondroblastoma of the middle cranial fossa, probably arising from the (infra) mandibular fossa, and expanding to the attic and external auditory canal that was successfully removed using a middle cranial fossa approach. No recurrences occurred during an 8-year postoperative follow-up period. Initial biopsy findings suggested a pathological diagnosis of giant cell tumor that was later confirmed to be a chondroblastoma based on an immunohistochemical study of S-100. This case study suggests a profound understanding of the clinical features, histopathological characteristics, and possible treatment. of chondroblastoma.

Psychiatric disorders in patients with dizziness and Ménière's disease.

CONCLUSIONS: The Self-Rating Depression Score (SDS) is a useful metric for identifying patients with possible psychiatric disorders. OBJECTIVE: A dilemma commonly encountered by otolaryngologists is how to diagnose and treat depression and anxiety disorders in patients who have dizziness or Ménière's disease. METHODS: We administered the SDS and Hospital Anxiety and Depression Scale (HADS) to patients with dizziness (n = 116) or Ménière's disease (n = 22). Overall, 31 and 9 patients, respectively, had SDS ≥ 41. We investigated the correlation between the two questionnaire scores and the relationship between scores and a diagnosis of depression by a psychiatrist. RESULTS: We referred 12 patients with dizziness and 4 with Ménière's disease to a psychiatrist. The most common psychiatric comorbidities were anxiety disorder and major depression. Overall, 7 of the 12 patients who had dizziness and all 4 patients with Ménière's disease were diagnosed with major depression. Patients with an SDS ≥ 41 and ≥ 11 on the D portion of HADS were likely to be diagnosed with major depression (9 of 11). No psychiatric disorders were diagnosed in patients with an SDS < 41. No specific characteristics were identified by HADS in patients with a psychiatric comorbidity.

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-κB 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-κB 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.

[A case of transient auditory agnosia and schizophrenia].

We report a case of transient functional auditory agnosia and schizophrenia and discuss their relationship. A 30-year-old woman with schizophrenia reporting bilateral hearing loss was found in history taking to be able to hear but could neither understand speech nor discriminate among environmental sounds. Audiometry clarified normal but low speech discrimination. Otoacoustic emission and auditory brainstem response were normal. Magnetic resonance imaging (MRI) elsewhere evidenced no abnormal findings. We assumed that taking care of her grandparents who had been discharged from the hospital had unduly stressed her, and her condition improved shortly after she stopped caring for them, returned home and started taking a minor tranquilizer.

Long-term prognosis of low-frequency hearing loss and predictive factors for the 10-year outcome.

OBJECTIVES: To determine the long-term prognosis of low-frequency hearing loss and predictive factors for the 10-year outcome of low-frequency hearing loss. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: From 1979 to 1998, 466 consecutive patients with low-frequency hearing loss received initial treatment at the Hearing and Tinnitus Clinic of Keio University Hospital. Of the 49 eligible patients, pure-tone threshold data obtained over a period of 10 years after onset of low-frequency hearing loss were available for analysis. To determine the progression of hearing loss, we analyzed audiometric pattern changes. We also examined how the following factors affected 10-year prognosis: sex, age, side of hearing loss, accompanying dizziness, pre-therapeutic hearing thresholds at low frequencies, initial therapy results, and fluctuation of hearing during the first year after onset. RESULTS: High- and pan-frequency hearing loss increased as time progressed. About half of the cases developed high- or pan-frequency hearing loss within 10 years of onset. Audiometric patterns measured at 10 years significantly correlated with those measured at one (r = 0.57), three (r = 0.73), and five years (r = 0.85). The 10-year prognosis significantly correlated with only two factors: initial therapy results (r = 0.49) and fluctuation of hearing during the first year (r = 0.43). CONCLUSIONS: About half of the cases in our study developed high- or pan-frequency hearing loss within 10 years of onset of low-frequency hearing loss. The initial therapy results and fluctuation of hearing during the first year may indicate the long-term prognosis of patients presenting with low-frequency hearing loss.

Long-term prognosis of steroid-dependent sensorineural hearing loss.

We report on the long-term follow-up of 15 patients with steroid-dependent sensorineural hearing loss (SDSNHL), which was steroid dose dependent. We classified the patients into 2 groups: group A consisted of 6 patients with systemic SDSNHL, and group B consisted of 9 patients with inner-ear-specific SDSNHL without systemic disease. Group B patients were further classified as patients with slowly or rapidly progressive hearing loss. The yearly rate of hearing deterioration was greater in group B than in group A. Thus, the long-term hearing prognosis of patients in group B was worse than those in group A. Although SDSNHL may have immune-related origins, we hypothesize that hearing deterioration may be caused by proinflammatory cytokines because many of our cases exhibited abnormal immunological laboratory values and because many of these cases responded favorably to steroid treatment.

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.

Classic hypoglossal-facial nerve anastomosis after acoustic neuroma resection. A review of 46 cases.

To clarify the factors contributing to patient satisfaction with facial movement after the classic hypoglossal-facial nerve anastomosis, we examined 46 such patients who consented to an interview and video-recording. No correlation was seen between the physician's evaluation of returned function (according to Yanagihara's 40-point scale and the grading system of House-Brackmann) and the scores (full marks: 100 points) the patients assigned to their own facial movements. Instead the scores that the patients assigned were closely related to subjective oral dysfunction, such as difficulty in masticating, articulating, and swallowing. Those scores did not correlate with eye-related functional deficits. These findings indicate that modified techniques to minimize the hypoglossal nerve deficit may enhance patient satisfaction without sacrificing recovery of facial movements.

Compensatory changes in static and dynamic subjective visual vertical in patients following vestibular schwanoma surgery.

OBJECTIVE: Among patients with vestibular schwanoma (VS), vestibular function is nonhomogeneous, both before and after surgical removal of the VS. This paper reports investigations of neural changes, especially changes in the contribution of visual input to vestibular system integration, after VS surgery. METHODS: We examined 33 patients who underwent VS surgery via a middle fossa approach. Static and dynamic subjective visual vertical (SVV) was measured once after surgery and compared to those measured in control subjects. SVVs were assessed using a paradigm requiring the subject to manually adjust an image of a bar to the perceived vertical alignment. SVVs were measured when the background was stationary or rotating. RESULTS: In almost all patients, static SVV deviated toward the operated side. In VS subjects, the mean static SVV was 1.8+/-2.2 degrees; the amount of deviation in the dynamic SVV toward the operated side (11.7+/-8.3 degrees ) was significantly larger than that to the intact side (8.8+/-5.5 degrees ). In VS subjects, static SVV was correlated with dynamic SVV only in cases of bar adjustments toward the operated side (R=0.67, P<0.001), but not in cases of adjustments toward the intact, unoperated side. The axis of rotation was defined as the mean value of dynamic SVV for adjustments toward either side. There was only a weak correlation between the static SVV and the axis of rotation (R=0.31; P<0.05) in the control subjects. On the other hand, a more robust correlation between static SVV and axis of rotation was found (R=0.67, P<0.001) in VS subjects. There was no correlation between the static SVV and the deviation of dynamic SVV from static SVV for CCW and CW in control subjects. In contrast, there were significant correlations between static SVV and deviation of dynamic SVV from static SVV for adjustments made toward both operated (r=0.48, P<0.001) and intact sides (r=038, P<0.05). CONCLUSION: It is assumed that the amount of deviation in static SVV reflects the individual level of compensation. In addition, increased visual dependency evoked a symmetrical bias of the dynamic SVV from the measures at initial SVV assessment (i.e. static SVV or the center of tilt). As a result, we conclude that the contribution of visual inputs had changed after surgery, while at the same time, each patient used their static SVV as their reference point for orientation.

Dynamic and static subjective visual vertical with aging.

OBJECTIVE: Our vestibular function is gradually deteriorating during aging, although, its behavioral consequences are not easily recognized due to a substitution process by other sensory modalities as visual or proprioceptive inputs. METHODS: To reveal such a hidden substitution process by visual signals, the measurement of the static as well as the dynamic subjective visual vertical (SVV) was performed among 63 healthy subjects of different age. RESULTS: The static SVV was found to be stable among all subjects, whereas the shift of the dynamic SVV during rotation of a background scene gradually increased with age. CONCLUSION: This result indicates that the substitution process identified as a function of age in a perceptual test may have its counterpart in postural stabilizing reflex.

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 microg 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.

[Statistical analysis of maxillary sinus squamous cell carcinoma].

Cases of squamous cell carcinoma of the maxillary sinus initially treated at Keio University Hospital between January 1981 and December 1998 studied retrospectively involved 60 untreated cases--46 men and 14 women aged 36 to 86 years (mean: 59.8 years). Of these, 7 had T2, 41 T3 and 12 T4 tumors based on 1997 AJCC TNM classification. Seven (11.7%) had nodal involvement but none had distant metastasis at diagnosis. Of the 60, 53 (88.3%) were stage III or IV. Prognostic factors and survival were statistically analyzed for 59 cases, excluding the 60th who died of another cause. Follow-up was 4 to 227 months, with a median of 59 months and a mean of 38 months. Of the 59, 48 (81.4%) underwent neoadjuvant chemotherapy (NAC). Survival was estimated using the Kaplan-Meier method as univariate analysis. Cause-specific 5-year survival was 56.8% for all stages, 68.2% for T2, 60.0% for T3, and 41.7% for T4. T stage classification was thus a significant independent prognostic factor in multivariate analysis using Cox's proportional hazards model (p = 0.0240). It also identified T stage classification (p = 0.0486) and NAC (p = 0.0419) as significant independent factors affecting survival with organ preservation. We also statistically analyzed 48 cases treated with NAC, which showed complete response (CR) for 11, partial response (PR) for 25, no change (NC) for 11, and progressive disease (PD) for 1. Responders (CR + PR) showed significantly better survival and organ preservation than nonresponders (NC + PD). The NAC response enables us to predict prognosis. T4 cases without NAC response should be treated intensively.

Haemorrhagic venous infarction following the posterior petrosal approach for acoustic neurinoma surgery: a report of two cases.

The authors report two surgical cases with acoustic neurinoma in which haemorrhagic infarction occurred via a compromise in cerebral deep venous outflow. In both cases, surgery was performed via the posterior petrosal approach, and the neurinomas were completely removed. In the first case, the haemorrhagic infarction was considered to have resulted from transection of the tentorial sinus, the presence of which had not been predictable by preoperative angiography. In the second case, the haemorrhagic infarction was caused by a coagulation of the petrosal vein, which was firmly adherent to a tumour. With the posterior petrosal approach, meticulous care is necessary to preserve the deep anastomotic veins into and around the cerebellar tentorium. Thereby, catastrophic morbidity related to compromised deep cerebral venous outflow can be avoided.

Expression of c-Fos after noise-induced temporary threshold shift in the guinea pig cochlea.

c-Fos is known to be a component of a transcription factor, activator protein-1, which is induced by oxidative stress. Guinea pigs were exposed to 4 kHz band noise of 110 dB SPL for 1 or 5 h and the expression of c-Fos in the organ of Corti was determined using Western blotting analysis and immunocytochemistry. c-Fos was expressed only after the noise exposure. The c-Fos expression was mainly found in the Hensen's cells, Claudius' cells and Deiter's cells of the basal and second turns of the cochlea. Since the threshold shift was temporary, the expression of c-Fos is therefore considered to contribute to the survival or protective function of the organ of Corti.