Systematic Review of Current Audiological Treatment Options for Patients with Treacher Collins Syndrome (TCS) and Surgical and Audiological Experiences of an Otorhinolaryngologist with TCS.

Treacher Collins syndrome (TCS) is a rare congenital craniofacial condition that affects approximately one out of fifty thousand births. Different ratios of TCS patients have conductive hearing loss: 88%1 vs. 91.4-100.00%2. For this reason, it was examined which hearing solutions can be used with this condition and how effective they are. A systematic literature review was conducted, which showed that the bone-anchored hearing aid (BAHA, OSIA), the bone conduction implant (Bonebridge) or the active implant of the middle ear (Soundbridge) are reliable methods for the treatment of conductive hearing loss in TCS patients. After the implantation of all available hearing solutions, improved hearing and speech comprehension were observed. Additionally, a statement regarding the treatment of TCS and a personalized point of view of a clinical expert with TCS were provided. However, due to the small amount of data, no general recommendations can be given for the treatment of hearing loss in TCS patients; therefore, it is advised to collect more data on hearing solutions for TCS patients in future research.

Hyperbaric Oxygen Therapy as Salvage Therapy for Sudden Sensorineural Hearing Loss.

OBJECTIVE: To measure the effect of hyperbaric oxygen (HBO) therapy as salvage therapy after the failure of steroid therapy for sudden sensorineural hearing loss (SSNHL). MATERIALS AND METHODS: Ninety-three patients with SSNHL were unsuccessfully treated with systemic steroid therapy. Following steroid therapy, 43 patients received additional HBO therapy while 50 did not. Hearing levels at 0.25, 0.5, 1, 2, 4, and 8 kHz before and after therapy were measured. RESULTS: A significant difference in hearing thresholds after HBO therapy was found at all frequencies in patients with a hearing loss of >61 dB. The group of patients with a hearing threshold of ≤60 dB had a significant improvement only at 250 and 500 Hz, while group of patients without additional therapy control group showed no hearing improvement. CONCLUSION: Hyperbaric oxygen therapy therapy as salvage therapy for SSNHL showed some benefits in hearing improvement. Better results could be expected in patients with severe hearing loss, while in patients with mild-or-moderate hearing loss, recovery should be expected only at low frequencies.

Prognostic value of venoarterial carbon dioxide gradient in patients with severe sepsis and septic shock.

AIM: To investigate the changes in the venoarterial carbon-dioxide gradient (V-a Pco(2)) and its prognostic value for survival of patients with severe sepsis and septic shock. METHODS: The study was conducted in General Hospital Holy Spirit from January 2004 to December 2007 and included 71 conveniently sampled adult patients (25 women and 46 men), who fulfilled the severe sepsis and septic shock criteria and were followed for a median of 8 days (interquartile range, 12 days). The patients were divided in two groups depending on whether or not they had been mechanically ventilated. Both groups of patients underwent interventions with an aim to achieve hemodynamic stability. Mechanical ventilation was applied in respiratory failure. Venoarterial carbon dioxide gradient was calculated from the difference between the partial pressure of arterial CO(2) and the partial pressure of mixed venous CO(2), which was measured with a pulmonary arterial Swan-Ganz catheter. The data were analyzed using Kaplan-Meier survival analysis, along with a calculation of the hazard ratios. RESULTS: There was a significant difference between non-ventilated and ventilated patients, with almost 4-fold greater hazard ratio for lethal outcome in ventilated patients (3.85; 95% confidence interval, 1.64-9.03). Furthermore, the pattern of changes of many other variables was also different in these two groups (carbon dioxide-related variables, variables related to acid-base status, mean arterial pressure, systemic vascular resistance, lactate, body mass index, Acute Physiology and Chronic Health Evaluation II, Simplified Acute Physiology II Score, and Sepsis-related Organ Failure Assessment score). Pco(2) values (with a cut-off of 0.8 kPa) were a significant predictor of lethal outcome in non-ventilated patients (P=0.015) but not in ventilated ones (P=0.270). CONCLUSION: V-a Pco(2) was a significant predictor of fatal outcome only in the non-ventilated group of patients. Ventilated patients are more likely to be admitted with a less favorable clinical status, and other variables seem to have a more important role in their outcome.