Assessment of Cortical Auditory Function Using Electrophysiological and Neuropsychological Measurements in Children with Bone-Anchored Hearing Aids.

Children suffering from conductive or mixed hearing loss may benefit from a bone-anchored hearing aid system (BAHA Attract implantable prosthesis). After audiological rehabilitation, different aspects of development are improving. The objective of this case report is to propose a comprehensive framework for monitoring cortical auditory function after implantation of a bone-anchored hearing aid system by using electrophysiological and neuropsychological measurements. We present the case of a seven-year-old boy with a congenital hearing loss due to a plurimalformative syndrome, including outer and middle ear malformation. After the diagnosis of hearing loss and the audiological rehabilitation with a BAHA Attract implantable prosthesis, the cortical auditory evoked potentials were recorded. We performed a neuropsychological evaluation using the Wechsler Intelligence Scale for Children - Fourth Edition, which was applied according to a standard procedure. The P1 latency was delayed according to the age (an objective biomarker for quantifying cortical auditory function). The neuropsychological evaluation revealed that the child's working memory and verbal reasoning abilities were in the borderline range comparing with his nonverbal reasoning abilities and processing abilities, which were in the average and below-average range, respectively. Cortical auditory evoked potentials, along with neuropsychological evaluation, could be an essential tool for monitoring cortical auditory function in children with hearing loss after a bone-anchored hearing aid implantation.

A painless erythematous swelling of the external ear as a manifestation of Lyme disease: a case report.

BACKGROUND: Lyme disease is the most common tick-borne illness in the USA, Canada, and Europe. Clinical manifestations vary greatly, with localized skin findings functioning as early signs of the disease, followed by disseminated disease. The rarest dermatologic presentation of Lyme is a borrelial lymphocytoma, occurring distinctly in Europe and caused typically by Borrelia afzelii. CASE PRESENTATION: We report a case of a Caucasian 5-year-old European-American boy with slowly progressing, painless edema and erythema of his right pinna. Travel history revealed significant exposure to ticks during a recent trip to Eastern Europe. Laboratory testing for Borrelia burgdorferi demonstrated mixed positivity. He was treated with a 21-day course of amoxicillin, with complete resolution of symptoms and no sign of secondary Lyme disease. CONCLUSIONS: Borrelial lymphocytoma is a rare manifestation of Lyme disease in North America, although not uncommon in Europe. Diagnosis is made by the presence of a painless erythematous swelling typically found on the ear lobe, nipples, or testes. Laboratory tests are available but with low sensitivity, therefore, a high index of suspicion is necessary for a clinical diagnosis to be made. Treatment for isolated borrelial lymphocytoma is doxycycline 4 mg/kg up to 100 mg twice daily, whereas for children less than 8 years of age amoxicillin 50 mg/kg divided three times daily, for 3-4 weeks, is preferred.

Limitations of present models of blast-induced sound power conduction through the external and middle ear.

The use of models to predict the effect of blast-like impulses on hearing function is an ongoing topic of investigation relevant to hearing protection and hearing-loss prevention in the modern military. The first steps in the hearing process are the collection of sound power from the environment and its conduction through the external and middle ear into the inner ear. Present efforts to quantify the conduction of high-intensity sound power through the auditory periphery depend heavily on modeling. This paper reviews and elaborates on several existing models of the conduction of high-level sound from the environment into the inner ear and discusses the shortcomings of these models. A case is made that any attempt to more accurately define the workings of the middle ear during high-level sound stimulation needs to be based on additional data, some of which has been recently gathered.

Periotic sensory dysfunction via postauricular approach after otitis media surgery.

OBJECTIVES/HYPOTHESIS: To assess the somatosensory dysfunction of the auricle and periotic skin in patients undergoing otitis media surgery. STUDY DESIGN: Retrospective study. METHODS: Symptoms of periotic somatosensory function after surgery were investigated in 100 patients (42 males, 58 females, mean age 41.39 years) who underwent otitis media surgery. Questionnaires on periotic somatosensory disturbance were answered after surgery at least over 1 year postoperatively. RESULTS: Of 100 tympanoplasties, all patients were completed within a postauricular approach. The highest incidence rates of periotic sensory disturbance were found in the postauricular region (75%), followed by the auricular region (20.83%); lower rates were found in the preauricular region (2.08%) and the earlobe (2.08%). Periotic somatosensory dysfunction occurred in 48 patients (48%). The most prevalent somatosensory abnormality was tactile hypoaesthesia/numbness, evident in 28% of the patients; more remarkable, inferior postauricular region. Periotic pain was reported by 21% of the cases, mostly located in the upper auricle. Compared to the recovery time of tactile hypoaesthesia (7.36 months), patients' periotic somatosensory pain improved significantly within 4.07 months, which has obvious statistical significance (P < 0.01). CONCLUSION: The likelihood of periotic cutaneous sensory dysfunction should be emphasized to patients undergoing otitis media surgery via postauricular approaches, which occurred in nearly half of patients; the majority recovered within 1 year. Furthermore, the postauricular region is the most vulnerable location to sensory disturbance, followed by the auricular region. Functionally, periotic somatosensory pain was more easily resilient, relative to tactile hypoaesthesia/numbness. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:454-458, 2019.

Mycophenolate mofetil embryopathy: A newly recognized teratogenic syndrome.

Mycophenolate mofetil (MMF) is probably the most common employed immunosuppressant drug in recipients of solid organ transplant and in many autoimmune diseases. In vitro studies, a significant number of single clinical observations and a recent study from a group of different European teratogen information services, have provided very consistent data supporting the existence of a specific MMF embryopathy. The typical malformative pattern of MMF embryopathy includes external ear anomalies ranging from hypoplastic pinna (microtia) to complete absence of pinna (anotia); cleft lip, with or without cleft palate, and ocular anomalies as iris or chorioretinal coloboma and anophthalmia/microphthalmia. Other less frequent features are congenital heart defects, distal limbs anomalies, esophageal atresia, vertebral malformations, diaphragmatic hernia, and kidney and central nervous system anomalies. Neurodevelopmental outcome seems favorable in the small number of patients where information about this issue is available, but neurological deficits have been documented. Physicians in charge of women under MMF therapy should be aware of the potential risk of this drug to cause a specific embryopathy and the need of interrupting the treatment at least six weeks before becoming pregnant.

Radiología del oído externo: indicaciones, anatomía normal y procesos patológicos / Radiology of external ear: indications, normal anatomy, and pathological processes

El oído externo (OE) es accesible al examen directo. En la mayor parte de las enfermedades, la historia clínica y la otoscopia son suficientes para su diagnóstico y tratamiento. Nuestro objetivo es describir la anatomía normal del OE, especificar las indicaciones de pruebas de imagen y revisar las manifestaciones clínicas y radiológicas de las enfermedades más frecuentes, que clasificaremos según su origen en patología congénita, inflamatoria e infecciosa, tumoral ósea benigna, traumática y tumoral maligna. Las pruebas de imagen no desempeñan un papel importante en la patología del OE, pero en determinados escenarios clínicos pueden ser cruciales para alcanzar el diagnóstico concreto y establecer el tratamiento idóneo. La tomografía computarizada es la técnica de elección para la mayor parte de las enfermedades. La resonancia magnética es complementaria, permite discriminar tejidos de diferente naturaleza y evaluar con precisión la extensión de la enfermedad (AU)

The external ear is accessible to direct examination; the clinical history and otoscopy are sufficient to diagnose and treat most diseases of the external ear. We aim to describe the normal anatomy of the external ear, specify the indications for imaging tests, and review the clinical and radiological manifestations of the most common diseases affecting the external ear. We classify these diseases according to their origin into congenital, inflammatory, infectious, or traumatic disease or benign bone tumors or malignant tumors. Imaging does not play an important role in diseases of the external ear, but in certain clinical scenarios it can be crucial for reaching a concrete diagnosis and establishing the best treatment. Computed tomography is the first-choice technique for most diseases. Magnetic resonance imaging complements computed tomography and makes it possible to differentiate among different tissue types and to evaluate the extension of disease accurately (AU)

Comparative analysis of ear-hole closure identifies epimorphic regeneration as a discrete trait in mammals.

Why mammals have poor regenerative ability has remained a long-standing question in biology. In regenerating vertebrates, injury can induce a process known as epimorphic regeneration to replace damaged structures. Using a 4-mm ear punch assay across multiple mammalian species, here we show that several Acomys spp. (spiny mice) and Oryctolagus cuniculus completely regenerate tissue, whereas other rodents including MRL/MpJ 'healer' mice heal similar injuries by scarring. We demonstrate ear-hole closure is independent of ear size, and closure rate can be modelled with a cubic function. Cellular and genetic analyses reveal that injury induces blastema formation in Acomys cahirinus. Despite cell cycle re-entry in Mus musculus and A. cahirinus, efficient cell cycle progression and proliferation only occurs in spiny mice. Together, our data unite blastema-mediated regeneration in spiny mice with regeneration in other vertebrates such as salamanders, newts and zebrafish, where all healthy adults regenerate in response to injury.

The Case of the Woman Who Did Never Dare to Fly: Headache Attributed to Imbalance Between Intrasinusal and External Air Pressure.

BACKGROUND: A new form of headache, Headache attributed to aeroplane travel (AH), has been recognized within the International Classification of Headache Disorders 3 beta (ICHD-3beta). In 8 out of 85 AH cases we identified the coexistence of a headache with identical features of AH, but appearing during the rapid descent by car from a high mountain. Pain began shortly after the rapid descent from a medium altitude of 1920 m above sea level, the maximum peak of intensity developing within a few minutes. All of the patients reported the disappearance of pain within 20 minutes of the rapid descent. CASE: We recently observed a 36-year-old woman who experienced recurrent headache attacks that were constantly triggered by rapid descent from high altitude by car. Negatively shaped by this experience, the patient never dared to fly. CONCLUSION: Headache attacks sharing the same features and occurring in three distinct conditions of pressure variations (aeroplane travel, rapid altitude mountain descent, snorkelling, or scuba diving) have already been reported, although the last two only anecdotally. If confirmed by further case series, they could be gathered together in a unique heading: Headache attributed to imbalance between intrasinusal and external air pressure within the 10th chapter: Headache attributed to disorders of the homoeostasis.

Results of Auricular Conchal Bowl Reconstructions Following Cancer Resections with Postauricular Island Flap.

The aim of the study was to present our experience with the postauricular island flap (pif) and clinical evaluation of the results following auricular conchal bowl reconstructions with the pif in patients after carcinoma resections. MATERIAL AND METHODS: We analyzed results in 13 patients who underwent auricular conchal bowl reconstructions with pif following malignant tumor resection between 2000-2013. The patients were followed-up. We estimated early and long-term results after surgery including plastic surgeon's and patient's opinion. RESULTS: The malignancies were completely excised in all patients, and there were no recurrences within 2 years of follow-up. Observed complications of conchal bowl reconstructions were venous congestion in two cases (15.3 %), and pinning of the operated ear in two patients (15.3%). Postoperative result was very good in 11 cases (both in the opinion of plastic surgeon and patients), whereas in two patients with pinning of the operated ear was satisfied. CONCLUSIONS: 1. Postauricular island flap reconstructions after auricular conchal bowl resections allowed for complete removal of malignant tumors with no evidence of recurrence, and also preserved proper conchal shape in the reconstructed ear. 2. Reconstructions of auricular conchal bowl with the postauricular island flap resulted in very good postoperative results, which confirms the efficiency of the applied technique. 3. Reconstructive surgery with postauricular island flap of individuals with partial auricular conchal bowl defects contributed to postoperative satisfaction in both patients and doctors' estimations.

Dynamometric Analysis of Normative Auricular Stiffness and Comparison With Operated Prominent Ears.

AIM: Stiffness of the auricular cartilage is the main determining factor for the choice of operative technique of the prominent ear deformity. The aim of this study is to evaluate the stiffness of normal appearing ears objectively and quantitatively, compare the results with the operated prominent ear patients, and present prospective short-term dynamometric evaluation of the operated prominent ear patients. PATIENTS AND METHODS: A total of 190 volunteers without ear deformities were recruited and 9 age groups were formed: group (5-9), group (10-14), group (15-19), group (20-24), group (25-29), group (30-34), group (35-39), group (40-49), and group (50+). Total 28 ears (14 patients) with otoplasty were included in the study as group (operated 5-9) and group (operated 10-14). In addition, 3 patients with prominent ear deformity were prospectively followed for dynamometric changes that occur with otoplasty operation. The auriculocephalic angle (ACA) was measured once and auricle to scalp distance was measured at 4 different standardized levels. Ear stiffness was measured on each ear individually at 4 different points over the antihelix using digital computer-aided dynamometry. Each ear was compared in terms of ACA, distance, and dynamometric values. FINDINGS: Dynamometric values tend to increase with age, which increase and peak around 35 years of age and declines after 40 years of age. Measurements of the first 2 age groups were statistically different compared with the other groups. Postoperative dynamometric measurements (DNM) of group (operated 5-9) were similar with normative values of group (5-9) and postoperative satisfaction visual analogue scale (VAS) score was 92.8%. Postoperative DNM of group (operated 10-14) were higher compared with normative values of group (10-14) for each different measuring level and the postoperative satisfaction VAS score was 75.3. A total of 3 patients with prominent ears had lower dynamometric values preoperatively; these values approached closer to normative values of their age group postoperatively. CONCLUSIONS: Results show that auricular cartilage stiffens and malleability decreases with increased age. This stiffness peaks in the 35-39 age group and declines after 40 years of age. Dynamometric values increase, at all levels, suggesting increased cartilage stiffness is related to age. In the scope of these results, cartilage sparing techniques are more suitable for 5 to 14 years of age and cartilage-cutting techniques are more suitable for older patients.

Red ear syndrome - case report and review of literature.

Red ear syndrome is characterized by: paroxysmal, unilateral, recurrent pain, redness and discomfort of the ear lobe accompanied by a burning sensation. The duration and frequency of red ear syndrome attacks is very various and the episodes, usually occur spontaneously. The pathophysiology is still unknown and also there are no medications with approved efficacy. The goal of this brief report is to present a 11-year old girls whose symptoms of red ear syndrome preceded migraine without aura and the signs of redness of the ear occurred in clusters. The occurrence of symptoms of our case may have confirmed the observation that red ear syndrome is associated with primary headaches particularly migraine and cluster headaches. The literature on this case report of pediatric idiopathic red ear syndrome has been reviewed.

Congenital aural atresia and stenosis: surgery strategies and long-term results.

OBJECTIVE: To compare the patients who underwent surgery for congenital aural atresia (CAA) with congenital aural stenosis (CAS) for the stability of hearing results and complications during long-term follow-up. DESIGN: Retrospective review. STUDY SAMPLE: Seventy-five CAA patients and fifty CAS patients who underwent congenital meatoplasty with canalplasty and tympanoplasty between 2007 and 2012. RESULTS: Paired comparison analyses detected no significant difference in preoperative ABG but significant changes in postoperative ABG, ΔABG, the number of ABG < 30 dB and ABG < 10 dB between CAA and CAS. Complications such as postoperative stenosis, bony regrowth, external aural canal (EAC) infection, EAC eczema, total deaf, and lateralization of the tympanic membrane (TM) were observed in 61.3% of patients with CAA and 20% of patients with CAS. Chi square test detected significant differences in complications between patients with CAA and CAS (χ(2) = 20.73, p < 0.01). CONCLUSION: Meatoplasty with canalplasty and tympanoplasty in individuals with CAS can yield reliable and lasting positive hearing results with a low incidence of severe complications. The existence and preoperative condition of patients' TM and EAC skin helped improve hearing results and decrease the incidence of complications. However, the final hearing results and complications required stricter indications for CAA patients.

Augmenting endogenous Wnt signaling improves skin wound healing.

Wnt signaling is required for both the development and homeostasis of the skin, yet its contribution to skin wound repair remains controversial. By employing Axin2(LacZ/+) reporter mice we evaluated the spatial and temporal distribution patterns of Wnt responsive cells, and found that the pattern of Wnt responsiveness varies with the hair cycle, and correlates with wound healing potential. Using Axin2(LacZ/LacZ) mice and an ear wound model, we demonstrate that amplified Wnt signaling leads to improved healing. Utilizing a biochemical approach that mimics the amplified Wnt response of Axin2(LacZ/LacZ) mice, we show that topical application of liposomal Wnt3a to a non-healing wound enhances endogenous Wnt signaling, and results in better skin wound healing. Given the importance of Wnt signaling in the maintenance and repair of skin, liposomal Wnt3a may have widespread application in clinical practice.

Otoscopia: exame da orelha / Otoscopy: examination of ear

Otoscopia é o exame que permite ao médico avaliar o conduto auditivo e a membrana timpânica, além de diagnosticar enfermidades da orelha externa e da orelha média. A otoscopia, uma boa anamnese e um exame físico completo são fundamentais para o estudo das doenças da orelha. O presente trabalho faz uma revisão da otoscopia e de suas principais afecções.

Otoscopy is the examination of the external auditory meatus and the tympa nic membrane. It is used for the purpose of diagnosing diseases of the external and middle ear. The anmanesis and the clinical examination associated with ear examination (otoscopy) are essential in ear diaseases. The purpose of this study is to review the examination of the ear (otoscopy) and principal disease of the ear.