Ventilation tube insertion is not effective to the treatment of hearing impairment in pediatric patients with Cornelia de Lange syndrome.

OBJECTIVE: Cornelia de Lange syndrome (CdLS) is a multiple developmental disorder including hearing loss. The hearing impairment in CdLS patients is not only sensorineural but also conductive hearing loss (CHL). The aim of this study was to elucidate hearing loss causes in CdLS patients and evaluate the effect of ventilation tube (v-tube) insertion in the cases of CHL. METHODS: Thirty-two patients clinically diagnosed with CdLS were enrolled and analyzed with retrospective case review. Audiologic evaluations and imaging studies such as a temporal bone computed tomogram or brain magnetic resonance imaging (MRI) were performed for all patients. Hearing rehabilitation such as ventilation tube insertion, hearing aid fitting, or cochlear implantation was chosen depending on the audiological condition. RESULTS: Among 32 CdLS patients who underwent auditory brainstem response test, 81.2% presented hearing loss. Imaging studies showed that only middle ear lesions without inner ear anomalies were identified in 56.3%. Notably, the soft tissue lesion in middle ear was identified even in the neonatal MRI. When 7 patients were thought to have CHL due to otitis media with effusion, v-tube insertion was applied first. However, v-tube insertion rarely improved CHL postoperatively. Moreover, middle ear lesion was not fluid effusion but soft tissue lesion according to the intraoperative finding. These lesions were not eradicated even after revision surgery of v-tube insertion. CONCLUSION: V-tube insertion is not effective to improve hearing or eradicate otitis media with effusion in CdLS patients.

Circulating microRNAs as potentially new diagnostic biomarkers of idiopathic sudden sensorineural hearing loss.

BACKGROUND: Early detection of inner ear cell damage can reduce the chances of permanent damage to hearing ability. However, current inner ear cell damage detection methods can detect damage only after the patient has lost hearing ability. MicroRNA expression levels in circulating systems are affected in diseases or conditions arising from the distant lesions. Therefore, detection of circulating microRNA expression levels could be one of the best ways to obtain information on inaccessible lesion sites. AIMS/OBJECTIVES: This study aims to establish a method for monitoring idiopathic sudden sensorineural hearing loss (ISSNHL) by analyzing circulating microRNA expression levels. 21 ISSNHL patients and 24 healthy controls were enrolled. MATERIAL AND METHODS: Real-time quantitative polymerase chain reaction was performed for detecting expression levels of circulating microRNAs. RESULTS: Among eight circulating microRNAs, expression levels of five circulating microRNAs significantly differed between ISSNHL patients and healthy controls. circulating microRNA expression levels correlates with treatment outcomes and hearing ability. CONCLUSIONS AND SIGNIFICANCE: Using methods combining the evaluation of miR-183, miR-210, miR-18b, and miR-23a cut-off values identified in ISSNHL patients and healthy controls during receiver operating characteristic curve analysis, sensitivity and specificity of 80.95% (17/21) and 87.50% (21/24) were obtained, respectively.

Audiologic limitations of Vibrant Soundbridge device: Is the contralateral hearing aid fitting indispensable?

OBJECTIVES/HYPOTHESIS: The purpose of this study was to evaluate the audiologic limitations of the Vibrant Soundbridge (VSB) implant and the benefits of contralateral hearing aid (HA) fitting in VSB recipients. STUDY DESIGN: Retrospective study. METHODS: Twenty-three patients with symmetrical sensorineural or mixed hearing loss were enrolled in this study. The patients underwent VSB implantation in one ear and HA fitting in the other. Aided pure-tone audiometry was performed to measure the functional gains of each device. The Korean version of the Hearing in Noise Test (K-HINT) was used to determine the sentence speech perception in a quiet environment and the signal-to-noise ratio (SNR) in a noisy environment. RESULTS: VSB implantation resulted in hearing gains comparable to that of conventional HAs at high frequencies, whereas the functional gains at low frequencies were not satisfactory in the mixed hearing loss group. In these patients, the contralateral HA sufficiently amplified the low frequencies. The results of the K-HINT of the SNR in the VSB-aided ear were not significantly improved when compared to HA-aided contralateral ear. However, binaural fitting of a VSB and HA resulted in substantially improved SNR when compared to the unaided condition. This improvement of the SNR strongly correlated with functional gains at low frequencies in the contralateral HA-aided ear. CONCLUSIONS: Although unilateral VSB implantation is limited in terms of low-tone enhancement and speech perception in noisy environments, contralateral HA fitting can overcome these limitations and increase the efficacy of hearing rehabilitation. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:2116-2123, 2016.

A cervical vagal schwannoma mimicking a parathyroid cyst.

Cervical vagal schwannomas with cystic degeneration changes are extremely rare. These tumors are typically benign and slow growing. A 44-year-old woman underwent complete resection of a tumor measuring 4×3.5 cm in the right neck using the endoscopic approach, instead of the conventional transcervical approach. We applied the new scarless neck surgery technique with an endoscopic unilateral axillo-breast approach. The tumor originated from the right vagus nerve, and was confirmed to be a vagal schwannoma pathologically. The patient has been followed up for 18 months postoperatively with no evidence of tumor recurrence or neurological deficit. We report an extremely rare vagal schwannoma with cystic degeneration that was removed by an endoscopic approach, along with a review of the relevant literature.

Infrasellar craniopharyngioma originating from the pterygopalatine fossa with invasion to the maxillary sinus.

Craniopharyngiomas that originate in the nasopharynx and sphenoid bone (known as infrasellar craniopharyngiomas) are rare and comprise only 5% of all craniopharyngiomas. The involvement of the maxillary sinus has been reported only twice. We present a very rare case that involved the maxillary sinus.