International guidelines for the management and treatment of Morquio A syndrome.

Morquio A syndrome (mucopolysaccharidosis IVA) is a lysosomal storage disorder associated with skeletal and joint abnormalities and significant non-skeletal manifestations including respiratory disease, spinal cord compression, cardiac disease, impaired vision, hearing loss, and dental problems. The clinical presentation, onset, severity and progression rate of clinical manifestations of Morquio A syndrome vary widely between patients. Because of the heterogeneous and progressive nature of the disease, the management of patients with Morquio A syndrome is challenging and requires a multidisciplinary approach, involving an array of specialists. The current paper presents international guidelines for the evaluation, treatment and symptom-based management of Morquio A syndrome. These guidelines were developed during two expert meetings by an international panel of specialists in pediatrics, genetics, orthopedics, pulmonology, cardiology, and anesthesia with extensive experience in managing Morquio A syndrome

Brainstem Auditory Evoked Potentials' Diagnostic Accuracy for Hearing Loss: Systematic Review and Meta-Analysis

Background: Microvascular decompression (MVD) utilizes brainstem auditory evoked potential (BAEP) intraoperative monitoring to reduce the risk of iatrogenic hearing loss. Studies report varying efficacy and hearing loss rates during MVD with intraoperative monitoring. Objectives: This study aims to perform a comprehensive review and study of diagnostic accuracy of BAEPs during MVD to predict hearing loss in studies published from January 1984 to December 2013. Methods: The PubMed/MEDLINE and World Science databases were searched. Studies performed MVD for trigeminal neuralgia, hemifacial spasm, glossopharyngeal neuralgia or geniculate neuralgia and monitored intraoperative BAEPs to prevent hearing loss. Retrospectively, BAEP parameters were compared with postoperative hearing. The diagnostic accuracy of significant change in BAEPs, which includes loss of response, was tested using summary receiver operative curve and diagnostic odds ratio (DOR). Results: A total of 13 studies were included in the analysis with a total of 2,540 cases. Loss of response pooled sensitivity, specificity, and DOR with 95% confidence interval being 74% (60–84%), 98% (88–100%), and 69.3 (18.2–263%), respectively. The similar significant change results were 88% (77–94%), 63% (40–81%), and 9.1 (3.9–21.6%). Conclusion: Patients with hearing loss after MVD are more likely to have shown loss of BAEP responses intraoperatively. Loss of responses has high specificity in evaluating hearing loss. Patients undergoing MVD should have BAEP monitoring to prevent hearing loss

The Future of Cognitive Dysfunction in Elderly Patients: A Clinical Perspective with Recommendations

Background: The U.S. Census Bureau projects the number of Americans age 65 years and older will more than double between 2010 and 2050. The geriatric patient population will likely require the use of surgical services at a higher rate than ever before. To best care for the elderly undergoing procedures with anesthesia, it is imperative to follow the most up to date evidence to avoid post-operative complications, and more specifically post-operative cognitive dysfunction. Purpose: The purpose of this DNP evidence-based practice project is to identify patients that are at high risk for post-operative cognitive dysfunction prior to undergoing anesthesia. Post-operative cognitive dysfunction can lead to increased lengths of stay in the hospital, discharge of patients to places other than home, and leads to unnecessary spending of the healthcare system. By identifying these patients in their pre-op visit, an “anesthesia bundle for the elderly” can be implemented intra-operatively to decrease risk for cognitive dysfunction post-operatively. EBP Model: The “8 A’s Model’ will be used to guide this Evidence Based Practice project. Evidence Based Interventions: Elderly patients that are over the age of 70 should be administered a Mini-Cog exam in their pre-op visit. If the Mini-Cog is failed, and key patient factors that increase the risk for post-operative cognitive dysfunction are identified, this information should be disseminated to the surgical team so the patients can be treated more appropriately intra-operatively. The evidence demonstrates that Benzodiazepines and Anticholinergic drugs specifically should be avoided in patients who are at high risk for post-operative cognitive dysfunction. Evaluation and Results: The initial stages of this project will gather information through a chart review on current practices at a local university hospital. Charts were reviewed of elderly patients aged 70 and older that underwent anesthesia at this hospital in the past month. Per the evidence, patients with a history of alcohol abuse, depression, renal insufficiency, anemia, coronary artery disease, hypertension, and poor functional capacity should be administered a Mini-Cog exam. We will note if a Mini-Cog was done or not and note which medications the patients were on prior to undergoing anesthesia. Additionally, it will be important to know which medications were administered to patients during anesthesia, as well as how the patient did post-operatively. CAM scores conducted in the PACU will be taken from the chart. All information gathered will have no patient identifiers present. Implications for Practice: The goal of this project is to make a practice change in this local hospital’s anesthesia department to improve cognitive outcomes for the elderly post-operatively. Best practices for our growing elderly population are critical for anesthesia administration. Continuity of care between pre-op, intra-op, and post-op is imperative and can help decrease cognitive dysfunction after anesthesia for some of our most fragile patients

Identifying Elderly Patients at High Risk for Post-Operative Cognitive Dysfunction: A Clinical Perspective

Background: The U.S. Census Bureau projects the number of Americans age 65 years and older will more than double between 2010 and 2050. The geriatric patient population will likely require the use of surgical services at a higher rate than ever before. To best care for the elderly undergoing procedures with anesthesia, it is imperative to follow the most up to date evidence to avoid post-operative complications, and more specifically post-operative cognitive dysfunction. Purpose: The purpose of this DNP evidence-based practice project is to identify patients that are at high risk for post-operative cognitive dysfunction prior to undergoing anesthesia. Post-operative cognitive dysfunction can lead to increased lengths of stay in the hospital, discharge of patients to places other than home, and leads to unnecessary spending of the healthcare system. By identifying these patients in their pre-op visit, an “anesthesia bundle for the elderly” can be implemented intra-operatively to decrease risk for cognitive dysfunction post-operatively. EBP Model: The “8 A’s Model’ will be used to guide this Evidence Based Practice project. Evidence Based Interventions: Elderly patients that are over the age of 70 should be administered a Mini-Cog exam in their pre-op visit. If the Mini-Cog is failed, this information should be disseminated to the surgical team so the patients can be treated more appropriately intra-operatively. The evidence demonstrates that Benzodiazepines and Anticholinergic drugs specifically should be avoided in patients who are at high risk for post-operative cognitive dysfunction. Evaluation and Results: The initial stages of this project will gather information through a chart review on current practices at a local university hospital. Charts were reviewed of elderly patients aged 70 and older that underwent anesthesia at this hospital in the past month. Per the evidence, patients with a history of alcohol abuse, depression, renal insufficiency, anemia, coronary artery disease, hypertension, and poor functional capacity should be administered a Mini-Cog exam. We will note if a Mini-Cog was done or not and note which medications the patients were on prior to undergoing anesthesia. Additionally, it will be important to know which medications were administered to patients during anesthesia, as well as how the patient did post-operatively. CAM scores conducted in the PACU will be taken from the chart. All information gathered will have no patient identifiers present. Implications for Practice: The goal of this project is to make a practice change in this local hospital’s anesthesia department to improve cognitive outcomes for the elderly post-operatively. Best practices for our growing elderly population are critical for anesthesia administration. Continuity of care between pre-op, intra-op, and post-op is imperative and can help decrease cognitive dysfunction after anesthesia for some of our most fragile patients. Keywords: post-op cognitive dysfunction, peri-op, anesthesia, mini-cog, CAM score, benzodiazepines, anticholinergics Abbreviations: Confusion Assessment Method (CAM), Post-Anesthesia Care Unit (PACU), Doctor of Nursing Practice (DNP

Vertigo following cochlear implantation: a review

Cochlear implantation may cause a detrimental effect on vestibular function and residual hearing. A significant number of patients with a cochlear implant present with vertigo. There are several mechanisms for dizziness following cochlear implantations. The causes may be surgical trauma, disruption of normal cochlear physiology, or ensuing endolymphatic hydrops. Vibratory trauma affecting the cochlea during cochleostomy plays a vital role in causing paroxysmal vertigo in patients with a cochlear implant. In addition, the vibrations affecting the cochlea are enough to dislodge otoconia particles. During cochlear implantation, it is necessary to insert an electrode array into the cochlea and thus the chance of damage to cochlear and function may happen. Dizziness or vertigo may develop after cochlear implantation. It usually occurs due to vestibular hypofunction. Vertigo following cochlear implantation has not frequently been documented in the literature previously. However, the increasing number of cochlear implantations in the current scenario is showing different postoperative complications like vestibular symptoms among patients with an implant. The vestibular symptoms following cochlear implantation range from a gradual sense of mild unsteadiness or lightheadedness to brief attacks of whirling vertigo. Vertigo following cochlear implantations affects the quality of life although vestibular therapy is often helpful to manage this condition. The article aims to provide a comprehensive review of vertigo following cochlear implantation