The efficacy of radiosurgery for neurofibromatosis type 2 (NF2)-associated vestibular schwannoma (VS) remains debatable. We retrospectively analyzed radiosurgical outcomes for NF2-associated VS compared to sporadic VS using our database of 422 consecutive VS patients. Twenty-five patients with 30 NF2-associated VSs with a mean follow-up of 121 months were identified. NF2-associated VSs exhibited excellent tumor control (10-year cumulative rate, 92% vs. 92% in sporadic VSs; p = 0.945) and worse overall survival (73% vs. 97%; p = 0.005), mainly due to tumor progression other than the treated VSs. The presence of NF2 was not associated with failed tumor control via multivariate Cox proportional hazard analyses. No difference in radiation-induced adverse events (RAEs) was confirmed between cohorts, and prescription dose (hazard ratio 8.30, 95% confidence interval 3.19–21.62, p < 0.001) was confirmed as a risk for cranial nerve injuries via multivariate analysis. Further analysis after propensity score matching using age, volume, and sex as covariates showed that NF2-associated VSs exhibited excellent local control (100% vs. 93%; p = 0.240) and worse overall survival (67% vs. 100%; p = 0.002) with no significant difference in RAEs. Excellent long-term tumor control and minimal invasiveness may make radiosurgery a favorable therapeutic option for NF2 patients with small to medium VS, preferably with non-functional hearing or deafness in combination with postoperative tumor growth or progressive non-operated tumors, or with functional hearing by patients’ wish.

Background/Objectives: Since we started endoscopic stapes surgery, we have frequently noticed a white spot (WS) with a clear boundary on the bone surface around the fissula ante fenestrum (FAF) in otosclerosis cases. We investigated the significance of this surgical finding. Methods: We enrolled 27 cases of otosclerosis and 28 control cases of conductive hearing loss due to pathologies other than otosclerosis, both operated on endoscopically at the University of Tokyo Hospital. We retrospectively reviewed surgical videos to determine whether WS was present or absent. We examined the incidence of WS in otosclerosis cases and the controls and also in cases of otosclerosis, the presence or absence of WS was compared with the preoperative hearing level, preoperative air–bone gap, vasodilatation on the promontory, and a low-density area on high-resolution computed tomography (HRCT). Results: WS was present in 11 (41%) of 27 cases of otosclerosis but none in 28 control cases. There were no significant differences in patients’ age and gender, the incidence of vasodilatation on the promontory, preoperative hearing level, or preoperative air–bone gap between otosclerosis cases with and without WS. Although a hypodense focus anterior to the oval window was more frequently present on HRCT in otosclerosis cases with WS (82%) than those without WS (56%), the difference in the incidence failed to reach significance (p = 0.10). Conclusions: We observed WS around the FAF only in cases of otosclerosis, indicating that WS is unique in otosclerosis. WS did not correlate with vasodilatation on the promontory, preoperative hearing level, or air–bone gap. A hypodense focus anterior to the oval window on HRCT tends to be more common in otosclerosis cases with WS.

Background/Objectives: Cytomegalovirus (CMV)-associated hearing loss is common in non-genetic congenital hearing loss. Despite this high prevalence, a wide range of clinical characteristics exists, and the pattern of hearing loss remains unknown. This study aims to describe the clinical manifestations in children with CMV-associated hearing loss and to clarify the timing of hearing level change and the degree of hearing level fluctuation. Methods: A total of 54 patients with hearing loss due to congenital CMV infection were included. Hearing loss type (congenital or later onset), hearing loss laterality (unilateral or bilateral), severity at first and last visit, hearing progression and timing, and the difference between patients with intellectual disability and without intellectual disability were assessed. Results: The number of patients with congenital hearing loss and later onset hearing loss were 19 patients and 13 patients, respectively. Seventy-four percent (14/19) of the congenital hearing loss patients and 62% (8/13) of the later onset hearing loss patients eventually progressed to severe to profound hearing loss bilaterally. Progression occurred in less than 1 year (9 cases), between 1 and 3 years (7 cases), between 3 and 7 years (4 cases), or more than 8 years (1 case). Multiple progression events occurred in 11 cases. Conclusions: Sixty-one percent of patients had progression of hearing loss. Several cases experienced progression over more than one year and showed multiple progression events. CMV patients without intellectual disability tended to suffer later onset hearing loss. Sixty-nine percent of the patients eventually progressed to bilateral severe to profound hearing loss, which means that continuous long-term follow-up is required.