Adult-onset congenital cholesteatoma in the hypotympanum initially presenting as Bell's palsy: A case report.

INTRODUCTION: Cholesteatoma is a rare disease characterized by the accumulation of keratinized squamous epithelial cells in the middle ear or mastoid cavity. Vertigo and facial palsy, which are rare complications, may indicate erosion into the semicircular canals or the fallopian canal. PATIENT CONCERNS: A 40-year-old woman presented to our clinic with progressive right-sided hearing loss over 5 years (primary concern). Approximately 10 years ago, the patient had developed acute right-sided facial weakness with no additional symptoms. A neurologist at another hospital had diagnosed her condition as Bell's palsy and treated it accordingly. DIAGNOSIS: Adult-onset congenital cholesteatoma in the hypotympanum. INTERVENTION: Combined endoscopic and microscopic removal of the cholesteatoma. OUTCOMES: Physical examination revealed slight improvement in right-sided peripheral facial palsy. LESSON: Routine eardrum examination is recommended for patients presenting with isolated peripheral facial palsy. If necessary, a patient should be referred to an otologist for further evaluation and treatment.

Mycobacterial Infection in Recalcitrant Otomastoiditis: A Case Series and Literature Review.

Otomastoiditis caused by mycobacterial infections is uncommon and recalcitrant. Its clinical presentations, sometimes similar to those of common chronic suppurative otitis media, make diagnosis difficult. This retrospective study analyzed the clinical features, treatment course, and therapeutic outcomes of patients with mycobacterial otomastoiditis. The cases of six patients diagnosed with mycobacterial otomastoiditis or suspected mycobacterial infection between January 2007 and January 2019 in a single tertiary medical center in Taiwan were investigated. Information about predisposing factors, clinical features, culture reports, histopathology, treatment course, and outcomes were collected and analyzed. Relevant literature available in English was also reviewed. One patient was infected with tuberculous mycobacteria, two with suspected tuberculous mycobacteria, and three with nontuberculous mycobacteria. All six patients responded poorly to empiric antibiotic therapy, and diagnosis was not possible at their previous clinics. Five patients underwent tympanomastoidectomies; one was administered antimycobacterial medication without undergoing surgery. Mycobacterial infection was confirmed from a tissue culture or from the histopathology of the specimen, but in two patients, no definitive evidence of tuberculosis was found. Antimycobacterial medication was administered based on clinical suspicion, and improvement was noted. With appropriate therapy, all patients recovered, and no sequelae were observed after treatment. If empiric antibiotic therapy cannot achieve acceptable results, atypical infections, such as mycobacteria, should be considered. Antimycobacterial medication could be administered under clinical suspicion, serving as a diagnosis ex juvantibus. Surgical intervention might help reduce the bacterial load and obtain specimens for accurate diagnosis, but this may be unnecessary if appropriate antimycobacterial medication results in improvement. Early diagnosis and treatment can prevent complications in patients with recalcitrant otomastoiditis.

Primary versus salvage intratympanic steroid treatment for idiopathic sudden sensorineural hearing loss.

Objective: The objective of this research is to compare primary and salvage intratympanic (IT) steroid treatments in terms of hearing outcomes in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). Methods: The patients were randomized into two (primary and salvage) groups. Both groups received systemic steroid treatment for 2 weeks. The primary group also received IT dexamethasone injection three times during the treatment period, whereas the salvage group received IT dexamethasone injection only if no or slight recovery was noted at the 2-week follow-up. If needed, salvage steroid injection was administered three times during the following 2 weeks. Hearing recovery was analyzed according to the modified American Academy of Otolaryngology-Head and Neck Surgery criteria. Results: The degrees of hearing improvement at the 3-month follow-up were similar in the two groups. Compared with baseline, the pure-tone average values and speech discrimination scores improved by 38.45 ± 21.95 dB HL and 34.32% ± 30.55%, respectively, in the primary group and 36.80 ± 22.33 dB HL and 31.87% ± 27.88%, respectively, in the salvage group (p = .762 and .659, respectively). In addition, the complete or partial hearing recovery rates were also similar in the primary and salvage groups (67.7% vs. 73.3%, respectively; p = .780). In the salvage group, 18 patients required no IT steroid injection because they recovered after systemic steroid treatment. Conclusion: Primary and salvage IT steroid treatments for ISSNHL led to similar outcomes. In summary, salvage IT steroid injection is recommended for patients with ISSNHL patients to prevent unnecessary IT injection. Level of evidence: 2.

Long-term follow-up of applying autologous bone grafts for reconstructing tympanomastoid defects in functional cholesteatoma surgery.

OBJECTIVES: This study investigated the long-term surgical outcomes of functional cholesteatoma surgery with canal wall reconstruction using autologous bone grafts as the primary material in patients with acquired cholesteatoma. SUBJECTS AND METHODS: Medical charts were retrospectively reviewed for all patients admitted to one institution for surgical intervention between 2010 and 2018. We analyzed 66 patients (66 ears) who underwent functional tympanomastoidectomy involving the use of autologous bone grafts for canal wall defect reconstruction. Surgical outcomes were evaluated by comparing preoperative audiometric results with follow-up data (at least 36 months after surgery). Logistic regression analyses were performed to determine prognostic factors related to long-term hearing success. These factors included classification and stage of cholesteatoma, stapes condition, ossicular chain damage, active infection of the middle ear, state of the contralateral ear, preoperative hearing thresholds, gender, and age. RESULTS: The mean follow-up period was 49.2 months. The recidivism rate was 6% (four of 66 ears). The pure-tone average significantly improved from 50.78 ± 19.98 to 40.81 ± 21.22 dB hearing level (HL; p < 0.001). Air-bone gaps significantly improved from 26.26 ± 10.53 to 17.58 ± 8.21 dB HL (p < 0.001). In multivariate logistic regression analysis, early-stage disease (p = 0.021) and pars flaccida cholesteatoma (p = 0.036) exhibited statistically significant correlations with successful hearing preservation. CONCLUSION: Functional cholesteatoma surgery with autologous bone grafts reconstruction is an effective approach to significantly improve hearing with low recidivism rates. Localized disease and pars flaccida cholesteatoma were two independent predictors of successful hearing preservation.

Neurofibromatosis type 2 initially presenting as a preauricular mass: a case report.

Neurofibromatosis type 2 (NF2) is a rare genetic disease involving multiple tumors of the central and peripheral nervous systems. Most patients with NF2 have bilateral vestibular schwannomas; nonvestibular schwannomas may also develop. While the majority of patients may present with hearing impairment, tinnitus, dizziness and balance disorders, some may present with cutaneous manifestations. We describe the case of a 20-year-old man who initially presented with a solitary subcutaneous painless nodule in the left preauricular area without any other symptoms. He received excisional biopsy for the preauricular mass and the pathologic diagnosis was schwannoma. Magnetic resonance imaging of brain and neck revealed multiple mass lesions over the bilateral cerebellopontine angle cisterns, extending to the bilateral internal auditory canals, bilateral cervical neuroforamens, cervical and upper thoracic spinal canals, and left posterior neck. The patient was diagnosed with NF2 according to the clinical criteria. He underwent gamma knife stereotactic radiosurgery for bilateral vestibular schwannomas and is now under regular monitoring. CONCLUSION: NF2 patients may present with an isolated solitary cutaneous schwannoma with no other associated clinical findings. Further assessment is thus warranted in young patients presenting with a peripheral schwannoma despite absence of other clinical findings.

Trans-oral robotic surgery versus coblation tongue base reduction for obstructive sleep apnea syndrome.

OBJECTIVES: To compare the efficacy of trans-oral robotic surgery (TORS) with that of coblation assisted tongue base reduction surgery in patients with obstructive sleep apnea syndrome (OSAS). SUBJECTS AND METHODS: The medical charts were retrospectively reviewed for all OSAS patients admitted to one institution for surgical intervention between 2012 and 2017. We analyzed 33 cases; 16 patients received TORS and 17 received coblation surgery for tongue base reduction. Both groups received concomitant uvulopalatoplasty. Surgical outcomes were evaluated by comparing the initial polysomnography (PSG) parameters with the follow-up PSG data (at least 3 months after the surgery). Epworth sleepiness scale (ESS) and complications were also compared between the 2 groups. RESULTS: The success rate (≥50% reduction of pre-operative AHI and post-operative AHI <20) in the TORS group and coblation group were 50% and 58%, respectively, and there was no significant difference (p = .611). The AHI (mean ± SD) reduction in the TORS and coblation groups were 24.9 ± 26.5 events/h and 19.4 ± 24.8 events/h, respectively; the between-group difference was not significant (p = .631). ESS improvement did not differ significantly between the TORS and coblation groups (3.8 ± 6.6 and 3.1 ± 9.2, respectively, p = .873). The rates of minor complication were higher in the TORS group (50%) than that of the coblation group (35.3%) without statistical significance (p = .393). CONCLUSION: TORS achieved comparable surgical outcomes compared to coblation assisted tongue base reduction surgery in OSAS patients. Multilevel surgery using either TORS or coblation tongue base reduction combined with uvulopalatoplasty is an effective approach for the management of OSAS.

Pentoxifylline versus Steroid Therapy for Idiopathic Sudden Sensorineural Hearing Loss with Diabetes.

OBJECTIVES: To compare the efficacy of pentoxifylline with that of conventional steroid therapy in diabetic patients with idiopathic sudden sensorineural hearing loss (ISSNHL) and to compare blood sugar levels during hospitalization. MATERIALS AND METHODS: Medical charts were retrospectively reviewed for all diabetic patients admitted to one institution for ISSNHL between 2000 and 2015. We analyzed 298 cases; 50 patients received pulse steroid treatment (steroid group) and 248 received intravenous administration of pentoxifylline only (pentoxifylline group). Hearing change was evaluated by comparing the initial hearing tests with follow-up hearing tests for up to 3 months. Blood sugar levels were also compared between the 2 groups. RESULTS: At 3 months post-treatment, the degree of hearing recovery was similar between the 2 groups. The pure-tone average was improved from baseline by 17.9±21.2 dB in the steroid group and 18.9±20.7 dB in the pentoxifylline group (p=0.776); hearing recovery rates were also similar (40% vs 39.1%; p=0.826). During hospitalization, average fasting blood sugar levels were higher (203.9±92.0 vs 174.4±54.8 mg/dL; p=0.033) and acute hyperglycemia was more common (48.0% vs 33.1%; p=0.044) with steroid versus pentoxifylline treatment. CONCLUSION: Hearing recovery rates did not significantly differ between steroid and pentoxifylline treatment in diabetic patients with ISSNHL, but pentoxifylline appeared to be associated with better blood sugar control.