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Objective: The aeration status of the middle ear is presumed to be one of the factors affecting the outcome in acquired cholesteatoma. The present study investigated the impact of postoperative middle ear aeration on hearing and recurrence after intact canal wall tympanoplasty.Methods: We conducted a retrospective chart review of 127 pars flaccida cholesteatoma patients who underwent primary intact canal wall tympanoplasty at a tertiary academic medical center. We collected data on clinical characteristics, including extent of cholesteatoma, surgical procedure, pre- and postoperative middle ear aeration, hearing level, and recurrence. The aeration was measured before and at one year after operation on two axial computed tomography (CT) planes: at the lateral semicircular canal (mastoid cavity) and at the oval window (tympanic cavity). Based on the postoperative air-bone gap (ABG), patients were categorized into two groups: the successful hearing (ABG ≤15 dB) group and the unsuccessful hearing (ABG >15 dB) group. We used aeration ratio to assess the relationship between postoperative aeration improvement and hearing outcome or recurrence. Multivariate logistic regression analysis was used to identify the factors associated with hearing outcome.Results: At one year after operation, aeration ratio in both mastoid and tympanic cavities was significantly improved than the preoperative status (p < 0.001, p < 0.001, respectively, Wilcoxon signed-rank test). The positive correlation was found between postoperative aeration ratios of mastoid cavity and tympanic cavity (r = 0.348, p < 0.001, Spearman's rank-correlation coefficient). In mastoid and tympanic cavities, the postoperative aeration ratio in successful hearing group (n = 57) was significantly higher than that in the unsuccessful hearing group (n = 45) at one year after operation (p < 0.001, p = 0.028, respectively, Mann-Whitney U test). Multivariate logistic regression analysis demonstrated that postoperative aeration ratio in mastoid cavity and preoperative ABG were significant independent prognostic factors for successful hearing (odds ratio [95% confidence interval]: 2.630 [0.985 - 7.024], p = 0.045; 0.891[0.840 - 0.944], p < 0.001, respectively). However, postoperative aeration ratios did not significantly differ between with (n = 14) and without recurrence (n = 113) groups in both cavities.Conclusion: Our results suggest that better postoperative aeration in mastoid cavity is independently associated with satisfactory hearing outcome after intact canal wall tympanoplasty in pars flaccida cholesteatoma. However, no significant differences were observed between middle ear aeration at one year after operation and recurrence. [ABSTRACT FROM AUTHOR]

Objective: Intellectual disability (ID) and autism spectrum disorder (ASD) are common among children who are candidates for cochlear implants. However, the implications of these comorbidities for cochlear implant placement have been not fully established. This study sought to identify these implications by comparing developmental delays among children with these conditions.Methods: Participants were children who were followed up at least every 6 months for 24 months after cochlear implant surgery. Developmental delays were assessed using the Enjoji Scale of Infant Analytical Development (Enjoji Scale) and compared in three groups with hearing loss: those with ID (ID group, n=4); those with ASD and ID (ASD+ID group, n=4); and those with typical development (control group, n=5). Developmental delay was evaluated longitudinally before and after cochlear implant placement for 18 months.Results: Among the six subscales that make up the Enjoji Scale, language development and intelligence development were significantly delayed in all three groups and were exacerbated over time except for language development in the control group. Emotional development and social behavior were significantly delayed only in the ASD+ID group. Comparison of intergroup differences revealed delays in language development in the ID and ASD+ID groups compared with the control group.Conclusion: The Enjoji Scale successfully demonstrated developmental delays characteristic to the underlying comorbidities of ID with or without ASD in children with cochlear implants. The Enjoji Scale can be a useful diagnostic tool for screening children with cochlear implants for ID with or without ASD. [ABSTRACT FROM AUTHOR]

Although most acute peripheral facial palsies can be attributed to Bell's palsy, other factors, such as infection, trauma, and neoplasm, can cause facial palsy as well. Among these, facial nerve tumors are rare but should be considered in the differential diagnosis of facial palsy. Palsies due to facial nerve tumors usually present with slow onset but occasionally present as acute episodes. In such cases, facial nerve decompression is the treatment of choice to allow the tumor room to grow without compressing the nerve or its blood supply. We describe a case of severe, acute facial palsy presenting with a spindle-shaped bone erosion on the mastoid portion of the facial canal. Although facial neuroma was suspected preoperatively, emergency decompression surgery revealed that an unusual inflammatory pseudotumor was responsible for the finding. Postoperative histological analysis revealed extensive destruction of the nerve fibers, with extensive infiltration of foamy macrophages containing characteristic, diastase-resistant, periodic acid-Schiff (PAS)-positive inclusions, which are hallmark of the uncommon bacterial infections. This was a case of facial palsy with an unusual etiology. The case shows the benefit of decompression surgery not only as treatment for the palsy but also as exploratory surgery in cases of facial nerve tumor. [ABSTRACT FROM AUTHOR]

Objective: To compare the prevalence of middle ear malformations between patients with and without congenital external auditory canal stenosis (CEACS) and to investigate the outcomes of tympanoplasty and/or canalplasty in terms of tympanic membrane (TM) size and external auditory canal (EAC) stenosis in patients with middle ear malformation.Methods: Twenty-five patients who underwent primary tympanoplasty and/or canalplasty for middle ear malformation at a tertiary academic medical center were retrospectively reviewed. CEACS was defined as an EAC diameter of 4 mm or less. Intraoperative findings, including irregularity of the ossicles, facial nerve, or chorda tympani; size of the TM and EAC on computed tomography; and pre- and postoperative hearing level, were collected.Results: We included 7 and 18 patients with and without CEACS, respectively. The malleus handle defect, anterior deviation of the chorda tympani, and small TM (≤7 mm) (p-values 0.015, <0.001, and 0.003, respectively; Fisher's exact test) had significantly higher prevalence in patients with CEACS than in those without. The mean postoperative air-bone gap (ABG) in patients with CEACS was not significantly different from that in patients with normal-sized EAC (20.6 dB and 19.5 dB, respectively; p-value, 0.121; Mann-Whitney U test). No difference was observed in mean postoperative ABG between patients with small TM and those with normal-sized TM (19.2 dB and 20.0 dB, respectively; p-value, 0.469; Mann-Whitney U test).Conclusion: Patients with CEACS were more likely to have malleus handle defect, anterior deviation of the chorda tympani, and small TM than those without CEACS. Hearing outcome of patients with CEACS and a malformed malleus and/or incus might be equivalent to that of patients without CEACS following tympanoplasty and/or canalplasty, regardless of the EAC or TM size. [ABSTRACT FROM AUTHOR]

Objectives: A broad mastoid extension limits cholesteatoma resection via a transmeatal approach including endoscopic ear surgery. Therefore, a preoperative diagnosis of mastoid extension is a the most critical factor to determine whether to perform mastoidectomy. The purpose of this study was to assess the efficacy of non-echoplanar diffusion-weighted imaging (non-EPI DWI) and T1-weighted imaging in the evaluation of mastoid extension in cholesteatomas of the middle ear.Methods: Patients who underwent magnetic resonance imaging (MRI) for pretreatment evaluation before primary surgery for pars flaccida or tensa cholesteatoma, which revealed a high-signal intensity in the mastoid on T2-weighed imaging were retrospectively evaluated. Two board-certified radiologists retrospectively evaluated the extent of cholesteatomas on MRI with non-EPI DWI, non-EPI DWI- and T1-weighted axial imaging. The presence of a high signal intensity on non-EPI DWI or low or high signal intensity on T1-weighted imaging in the mastoid was evaluated. All cases were subclassified as M+ (surgically mastoid extension-positive) or M- (surgically mastoid extension-negative).Results: A total of 59 patients with middle ear cholesteatoma were evaluated. There were 37 M+ cases and 22 M- cases. High-signal intensity on non-EPI DWI exhibited a sensitivity of 0.89 and specificity of 0.82, whereas partial low-signal intensity on T1-weighted imaging exhibited a sensitivity of 0.84 and specificity of 0.91 for detecting mastoid involvement. Complete high-signal intensity on T1-weighted imaging exhibited a sensitivity of 0.73 and specificity of 0.89 for detecting non-involvement of the mastoid. The sensitivity (0.92) and specificity (0.96) of combined non-EPI DWI and T1-weighted imaging evaluation were higher than those of with non-EPI DWI or T1-weighted imaging alone. The interobserver agreement for the presence of high-signal intensity in the mastoid cavity on non-EPI DWI was very good at 0.82, that of a partial low-signal intensity area in the mastoid cavity lesions on T1-weighted imaging was good, at 0.76 and that of complete high-signal intensity in the mastoid cavity lesions on T1-weighted imaging was good, at 0.67.Conclusions: The signal intensity on non-EPI DWI and T1-weighted imaging of the mastoid could be used to accurately assess the extent of middle ear cholesteatoma, which could facilitate surgical treatment planning. [ABSTRACT FROM AUTHOR]

Objectives: We aimed to clarify the usefulness of high-resolution computed tomography (HRCT) and establish HRCT criteria for presurgical assessment of the mastoid extension in pars flaccida cholesteatomas of the middle ear.Methods: Retrospective observational study. Patients who underwent primary surgery for pars flaccida cholesteatoma and those who underwent temporal bone HRCT for pretreatment evaluation were reviewed. The distance in the anterior-most portion of the mastoid sinus on HRCT was measured, and the presence of surgically verified mastoid extension of cholesteatoma was evaluated. All cases were subclassified as M+ (surgically mastoid extension-positive) or M- (surgically mastoid extension-negative).Results: A total of 107 patients with pars flaccida cholesteatoma were included. The distance in the M+ cases was significantly longer than that in the M- cases, and the cutoff value was 3.6 mm. The difference between the ipsilateral/diseased-side distance and the contralateral/evaluable side (difference value) in M+ cases was larger than that in M- cases, with a cutoff value of 0.6 mm. The inter-rater reliability of this distance measurement was excellent, regardless of imaging experience.Conclusions: The cutoff values of the distance and the difference value can be used for pretreatment HRCT evaluation of mastoid extension in middle ear cholesteatoma with relatively high accuracy, regardless of the experience and skill levels of the evaluator. [ABSTRACT FROM AUTHOR]