Auditory brainstem implantation improves speech recognition in neurofibromatosis type II patients.

This prospective study aimed to determine speech understanding in neurofibromatosis type II (NF2) patients following implantation of a MED-EL COMBI 40+ auditory brainstem implant (ABI). Patients (n = 32) were enrolled postsurgically. Nonauditory side effects were evaluated at fitting and audiological performance was determined using the Sound Effects Recognition Test (SERT), Monosyllable-Trochee-Polysyllable (MTP) test and open-set sentence tests. Subjective benefits were determined by questionnaire. ABI activation was documented in 27 patients, 2 patients were too ill for testing and 3 patients were without any auditory perception. SERT and MTP outcomes under auditory-only conditions improved significantly between first fitting and 12-month follow-up. Open-set sentence recognition improved from 5% at first fitting to 37% after 12 months. The number of active electrodes had no significant effect on performance. All questionnaire respondents were 'satisfied' to 'very satisfied' with their ABI. An ABI is an effective treatment option in NF2 patients with the potential to provide open-set speech recognition and subjective benefits. To our knowledge, the data presented herein is exceptional in terms of the open-set speech perception achieved in NF2 patients.

HPV-induced recurrent laryngeal papillomatosis: rationale for adjuvant fatty acid therapy.

The course of human papilloma virus (HPV)-induced recurrent laryngeal papillomatosis (RLP) is variable and unpredictable. Some patients experience spontaneous remission, while others suffer from aggressive growth with dire consequences. Unfortunately, HPV DNA can persist in mucosa after treatment and can be reactivated under immunosuppressive conditions. For this reason, these benign tumors are notoriously recurrent. Better understanding of lipid-driven signaling pathways during tumorigenesis and immune responses in RLP patients can contribute to improve therapeutic approaches in an attempt to obviate this disease. Based on a mountain of evidence in the literature that concerns the immunomodulatory potential of certain FAs, it is clear that there is a rationale for adjuvant FA therapy (concurrent application) in the management of RLP. Of particular importance for immune surveillance is that the Th1 pathway in RLP is down-regulated and it is advocated that conjugated linoleic acid (CLA) and eicosapentaenoic acid (EPA) have the ability to restore the Th1/Th2 balance. Therefore, it is proposed that adjuvant FA therapy with CLA and EPA must be included in the therapeutical regime of RLP, since they are considered excellent anti-viral and anti-tumor agents to improve immune conditions and disease outcome. Immunocompetence plays a pivotal role in the clinical course of RLP and, hence, a new direction with adjuvant FA therapy may be the key to prevent recurrence of this disease.

HPV-induced recurrent laryngeal papillomatosis: fatty acid role-players.

Human papilloma virus-induced recurrent laryngeal papillomatosis is considered a troublesome and dangerous disease, because it can cause airway obstruction. Better understanding of metabolic pathways followed under pathological conditions can contribute to improved therapies by which growth and recurrence may be obviated. Part I of this study presents a clinically relevant total lipid fatty acid profile for papilloma cells, analyzed by gas liquid chromatography and a phosholipid red blood cell profile for RLP patients, analyzed by thin layer chromatography. In the papilloma cells virus interference with delta-6 and delta 5- desaturase activities is prevalent and the n-9 FA metabolic pathway is followed. It is plausible that up-regulated fatty acid synthase and 9 desaturase activities occur, since enhanced saturated fatty acids and monounsaturated fatty acid levels are also prevalent. High saturated fatty acid levels are known for their propensity to interfere with delta-6 and delta-5 desaturase activities and this is reflected in the blood profile of the RLP patients. It is also known that enhanced saturated fatty acid levels can contribute to enhanced cyclooxygenase-2 activity. Furthermore, cumulative oxidative stress with an oxidative burst is responsible for complete exhaustion of exogenous dietary arachidonic acid intake in these patients. The role of linoleic acid needs to be defined. The dietary intakes of lipids and micronutrients in RLP patients and a rationale for adjuvant FA therapy in the management of these patients are discussed in parts II and III of the study.